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Objective: The wrist is the second most commonly involved location for GCTB, while distal ulna is a relatively rare location and limited evidence exists on which surgical approaches and reconstruction techniques are optimal. We carried out a multicenter retrospective study to evaluate the recurrence rate of distal ulna GCTB and the long-term functional outcomes of different surgery options. Methods: All 28 patients received surgical treatment for distal ulna GCTB in one of three tertiary bone tumor centers between May 2007 and January 2021 with a minimum two-year follow-up. Surgical options included intralesional curettage or en bloc resection (one of 3 types). Functional outcomes were assessed by the MSTS score, the QuickDASH instrument, MWS, and MHQ according to the latest treatment. Results: Overall recurrence rate was 14.2%. The curettage group (N = 7) had a significantly higher recurrence rate compared to en bloc resection (N = 21) (42.9% vs 4.8%) (mean follow-up: 88.8 mo). Seven patients received the Darrach procedure, 5 received the original Sauvé–Kapandji procedure, and 9 received the modified Sauvé–Kapandji procedure with extensor carpi ulnaris (ECU) tenodesis. Of the 4 patients having a recurrence, 1 received the Darrach EBR, 2 received the modified Sauvé–Kapandji procedure, and 1 received resection for soft tissue recurrence. Only MWS and esthetics in the MHQ scores were different (curettage, Darrach, Sauvé–Kapandji, and Sauvé–Kapandji with ECU tenodesis [MWS: 96.5 ± 1.3 vs 91.5 ± 4.7 vs 90.8 ± 2.8 vs 91.5 ± 3.6; esthetics in MHQ: 98.5 ± 3.1 vs 89.9 ± 4.7 vs 93.8 ± 4.4 vs 92.6 ± 3.8], respectively). Conclusions: En bloc resection for distal ulna GCTB had a significantly lower recurrence rate compared with curettage and achieved favorable functional outcome scores. Given the higher recurrence rate after curettage, patients should be well informed of the potential benefits and risks of selecting the distal radioulnar joint-preserving procedure. Moreover, reconstructions after tumor resection of the ulna head do not appear to be necessary. [ABSTRACT FROM AUTHOR]

Background: LIPH, a membrane-associated phosphatidic acid-selective phospholipase A1a, can produce LPA (Lysophosphatidic acid) from PA (Phosphatidic acid) on the outer leaflet of the plasma membrane. It is well known that LIPH dysfunction contributes to lipid metabolism disorder. Previous study shows that LIPH was found to be a potential gene related to poor prognosis with pancreatic ductal adenocarcinoma (PDAC). However, the biological functions of LIPH in PDAC remain unclear. Methods: Cell viability assays were used to evaluate whether LIPH affected cell proliferation. RNA sequencing and immunoprecipitation showed that LIPH participates in tumor glycolysis by stimulating LPA/LPAR axis and maintaining aldolase A (ALDOA) stability in the cytosol. Subcutaneous, orthotopic xenograft models and patient-derived xenograft PDAC model were used to evaluate a newly developed Gemcitabine-based therapy. Results: LIPH was significantly upregulated in PDAC and was related to later pathological stage and poor prognosis. LIPH downregulation in PDAC cells inhibited colony formation and proliferation. Mechanistically, LIPH triggered PI3K/AKT/HIF1A signaling via LPA/LPAR axis. LIPH also promoted glycolysis and de novo synthesis of glycerolipids by maintaining ALDOA stability in the cytosol. Xenograft models show that PDAC with high LIPH expression levels was sensitive to gemcitabine/ki16425/aldometanib therapy without causing discernible side effects. Conclusion: LIPH directly bridges PDAC cells and tumor microenvironment to facilitate aberrant aerobic glycolysis via activating LPA/LPAR axis and maintaining ALDOA stability, which provides an actionable gemcitabine-based combination therapy with limited side effects. [ABSTRACT FROM AUTHOR]

Background: Pituitary apoplexy (PA) is a rare but potentially life-threatening condition. While it is uncommon, it can present with findings that are clinically indistinguishable from acute bacterial meningitis. We report an unusual case of simultaneous coexistence of bacterial meningoencephalitis and pituitary apoplexy following transsphenoidal surgery (TSS) for pituitary macroadenoma, emphasizing the possibility of coexistence of the two entities. Case presentation: A 62-year-old man was admitted with a moderate headache, gait disturbance, and progressively decreasing vision. Sellar magnetic resonance imaging showed a giant pituitary tumor of 4 × 5 cm with invasion of the cavernous sinus bilaterally, and a mass effect on the optic chiasm. The patient underwent a TSS and partial resection of a giant pituitary tumor. On postoperative day 11, he presented with an acute, severe headache and altered sensorium. An urgent computed tomography (CT) scan revealed hemorrhagic swelling of the residual. A complete analysis of the anterior pituitary hormones revealed panhypopituitarism. Administering steroid ameliorated the clinical features, but after decreasing the dose, the patient continued to deteriorate in his neurological status, a high fever, and marked stiffness were noted on postoperative day 14. Given the neck stiffness, leukocytosis, and high C-reactive protein level, acute meningitis was suspected, and the cerebrospinal fluid (CSF) was tested, which was consistent with acute bacterial meningitis. In the results of the bacterial cultures of the CSF, E. coli was identified and switched to ceftriaxone. The patient's neurological status and body temperature improved gradually. CSF test results returned to normal levels, and ceftriaxone was discontinued on day 10. Conclusions: This is a report on a case of a giant pituitary tumor that developed late postoperative PA after having undergone a partial tumor resection. A postoperative CT scan showed hemorrhagic expansion of the residual tumor mass. Further, the patient exhibited typical symptoms of acute meningoencephalitis, the result of the cultures of the CSF was positive, and the patient's general condition deteriorated. In addition, laboratory findings indicated leukocytosis, an elevated C-reactive protein level, and neutrophilic pleocytosis. We highlighted diagnostic challenges and therapeutic delays arising as a result of rare concomitance. [ABSTRACT FROM AUTHOR]

Background: Undifferentiated carcinomas of the pancreas with osteoclast-like giant cells (UCPOGC) are rare pancreatic neoplasms that account for less than 1% of all pancreatic malignancies. This case report of a 54-year-old male with metastatic UCPOGC adds to the existing literature and further ascertains the clinical and imaging features, treatment options, and prognosis of this rare entity. Case presentation: We present the detailed clinical course of a 54-year-old Asian male patient with UCPOGC, with focus on the relevant clinical features and imaging findings that are characteristic of this disease entity. Conclusions: UCPOGC is an extremely rare pancreatic tumor with a unique histopathology and clinical course. It is often difficult to distinguish UCPOGCs from other pancreatic tumors, such as traditional pancreatic ductal adenocarcinomas (PDAC), on imaging, and it therefore remains a pathological diagnosis. Surgery is generally regarded as the first-line treatment option, and the roles of chemotherapy and radiation are unclear. Due to the exceeding rarity of this tumor, large-scale clinical studies are not feasible. Therefore, it is important to share individual insights and experiences to improve our understanding and care for patients with this devastating disease. [ABSTRACT FROM AUTHOR]

Objectives: (1) Describe the clinical spectrum, imaging features, management, and outcome of brown tumors (BT) of the jaws. (2) Determine via a literature review the facts and controversies around the characteristics and management of this lesion. Materials and methods: Our study was approved by the institutional committee of Sahloul University Hospital in accordance with the ethical standards of the Declaration of Helsinki. Sixteen patients with BT in the context of a primary, secondary, and tertiary hyperparathyroidism were treated and followed up during their recovery. Results: This study reports 16 patients with a mean age of 48 years old. Brown tumor lesions were associated with primary hyperparathyroidism in 6 cases (38%), secondary hyperparathyroidism to chronic kidney failure in 5 cases (31%), and tertiary hyperparathyroidism in the context of a long lasting CRI in 5 cases. Their location was maxillary in 7 cases, mandibular in 5 cases, and bimaxillary in 4 cases. The treatment consisted of parathyroidectomy in 13 patients, maxillary resection in 3 cases, and vitamin D treatment in 2 cases. Favorable outcomes, characterized by tumor regression, were reported in 9 cases, whereas unfavorable evolution was observed among 7 patients. Conclusion: Parathyroidectomy is the gold standard treatment for primary hyperparathyroidism. It resulted in a total regression in all our cases. Regarding secondary hyperparathyroidism, blood screening and chronic renal insufficiency follow-up are critically valuable to detect this condition at an early stage, hence increasing the success rate of brown tumor regression. Our secondary and tertiary hyperparathyroidism results were miscellaneous; although it is important to emphasize the importance of the chronic renal insufficiency management to ensure a success. Clinical relevance: Brown tumors should be included in the differential diagnosis of giant cell lesions. Parathyroidectomy is usually sufficient to induce the total regression of the lesion in primary hyperparathyroidism cases. A more delicate approach is needed regarding secondary hyperparathyroidism. Meticulous control of chronic renal insufficiency is mandatory in addition to parathyroidectomy. [ABSTRACT FROM AUTHOR]

Background: This study aimed to find out the characteristics in relation to tumor recurrence in diffused-tenosynovial giant cell tumor of temporomandibular joint and to develop and validate the prognostic model for personalized prediction. Methods: From April 2009 to January 2021, patients with diffused-tenosynovial giant cell tumor of temporomandibular joint at a single center were included in this study. The clinical features and local recurrence-free survival were assessed through the expression of the Ki-67 index and colony-stimulating factor 1 receptor expression. Both univariate and multivariate analyses were performed on the prognostic factors for local recurrence-free survival. An independent predictor nomogram and pertinent tumor characteristics were included. Results: The retrospective study enrolling seventy eligible patients at the Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine. During the follow-up time, eleven patients suffered tumor recurrence. Age was an independent risk factor for local recurrence-free survival (P = 0.032). The Ki-67 index varied significantly in different sites (P = 0.034) and tumor volume (P = 0.017). Multivariate logistic regression was used to develop the prediction model using both statistical significance and prognostic indicators. The C-index of the nomogram based on age, site, Ki-67, and colony-stimulating factor 1 receptor was 0.833. These variates provided good predicted accuracy for a nomogram on local recurrence-free survival. Diffused-tenosynovial giant cell tumor from the temporomandibular joint is extremely uncommon, and certain clinical traits are linked to the tumor proliferation index. Conclusions: We identified the risk indicators and developed a nomogram in this study to forecast the likelihood of local recurrence-free survival in patients with diffused-tenosynovial giant cell tumor from temporomandibular joint. [ABSTRACT FROM AUTHOR]

Introduction/Purpose: To determine the diagnostic accuracy and complication rates of ultrasound‐guided, percutaneous core needle biopsies of soft tissue masses in the hand and fingers. Methods: Reports from all ultrasound‐guided procedures between 21 May 2014 and 17 March 2022 were queried for keywords including "hand", OR "finger", AND "biopsy". Patient demographics, lesion size and location, biopsy needle gauge and the number of cores obtained were recorded. The final pathology of the mass excision was then compared with the core needle biopsy (CNB) for each patient. Results: Sixty‐six records were reviewed, and 37 patients met inclusion criteria. Maximum lesion diameter averaged 1.45 cm with a range between 0.4 and 4.3 cm. The frequency of needle gauges used was 14G (14%), 16G (24%), 18G (38%), 20G (11%) and 'not reported' (14%). The mean number of tissue cores obtained was 2.9 (SD 1.2; range 1 to 6), excluding nine cases that reported 'multiple'. The frequency of CNB diagnoses included tenosynovial giant cell tumour (TGCT) at 30%, ganglion cyst at 11% and epidermal inclusion cyst at 5%. CNB was 100% sensitive in detecting the three (8%) malignancies. Of the 37 tumours biopsied, 16 were surgically excised. One angiomyoma was originally diagnosed as a haemangioma on CNB, but all other histologic results were concordant for a diagnostic accuracy of 97%. Discussion: Small soft tissue masses in the hands and fingers, even those less than 1 cm, are often amenable to ultrasound‐guided CNB. Performance under image guidance facilitates retrieval of core specimens adquate for histologic diagnosis with relatively few passes using higher gauge needles. Conclusion: Overall, ultrasound‐guided CNB of the hand and fingers is safe and highly accurate in diagnosing soft tissue tumours. The accuracy is unrelated to the needle's gauge, the number of passes and the size of the lesions. [ABSTRACT FROM AUTHOR]

Sacral masses can be removed using anterior, posterior, or combined approaches. Achieving total sacrectomy through a posterior-only approach results in a shorter procedure time, minimal tissue damage, and a reduced risk of complications. In this study, we aimed to share our experience with performing total sacrectomy using a posterior-only approach in 26 patients and to assess their clinical outcomes at our center. This retrospective study examines the clinical progression, surgical response, and outcomes of 26 patients with various sacral mass pathologies. We accessed patient information from our hospital records. The study included 14 men (54%) and 12 women (46%), with an average age of 49.8 years. Most cases had a normal body mass index, while 6 were overweight. Sacrectomy was performed at a high level in 12 patients and at a middle level in 14 patients. In addition to pain, motor deficits were observed in 9 patients, and sphincter dysfunction was found in 5. Preoperative embolization was conducted for 11 patients. The most prevalent lesions were chordoma (8 patients), malignant peripheral nerve sheath tumor (4 patients), giant cell tumor (3 patients), and solitary plasmacytoma (3 patients). Only 1 patient experienced a temporary partial motor deficit after surgery. There were no instances of cerebrospinal fluid leakage. Five patients experienced local recurrence, and 1 had distant metastasis. Performing sacrectomy for large or giant sacral tumors through a posterior approach is both feasible and safe, resulting in reduced morbidity and no significant change in overall survival. [ABSTRACT FROM AUTHOR]

Paget's disease of bone (PDB) is a common, late-onset bone disorder, characterized by focal increases of bone turnover that can result in bone lesions. Heterozygous pathogenic variants in the Sequestosome 1 (SQSTM1) gene are found to be the main genetic cause of PDB. More recently, PFN1 and ZNF687 have been identified as causal genes in patients with a severe, early-onset, polyostotic form of PDB, and an increased likelihood to develop giant cell tumors. In our study, we screened the coding regions of PFN1 and ZNF687 in a Belgian PDB cohort (n = 188). In the PFN1 gene, no variants could be identified, supporting the observation that variants in this gene are extremely rare in PDB. However, we identified 3 non-synonymous coding variants in ZNF687. Interestingly, two of these rare variants (p.Pro937His and p.Arg939Cys) were clustering in the nuclear localization signal of the encoded ZNF687 protein, also harboring the p.Pro937Arg variant, a previously reported disease-causing variant. In conclusion, our findings support the involvement of genetic variation in ZNF687 in the pathogenesis of classical PDB, thereby expanding its mutational spectrum. [ABSTRACT FROM AUTHOR]

Background: Giant cell tumor of soft tissue (GCT‐ST) is a rare soft tissue neoplasm that is morphologically similar to but genetically distinct from giant cell tumor of bone. A novel keratin‐positive GCT‐ST (KPGCT‐ST) harboring HMGA2::NCOR2 fusions was recently discovered. Fewer than 30 cases have been described; herein is reported an additional seven. Methods: Cases diagnosed as GCT‐ST were retrieved from institutional archives and consultation files. The histopathologic characteristics were assessed, and the electronic medical record was reviewed. Results: Seven tumors were identified in six women and one man with a median age of 23 years. All patients underwent excision; no recurrences or metastases were noted during a median follow‐up period of 7 months. Histopathologically, the tumors were characterized by a multinodular proliferation of keratin‐positive mononuclear cells with evenly admixed osteoclast‐like giant cells and absent neoplastic bone. A fibrous capsule with lymphoid cuffing was frequently seen. Foamy macrophages, inflammation, hemorrhage, and hemosiderin were variably present. The HMGA2::NCOR2 fusion was detected in all cases. Conclusions: Our findings support previously reported hypotheses that KPGCT‐ST is a spectrum of the same entity as the recently described xanthogranulomatous epithelial tumor. Although follow‐up data are limited, to date, KPGCT‐ST appears to follow an indolent course. [ABSTRACT FROM AUTHOR]

Background: The giant cell tumour of the tendon sheath (GCTTS) is the second most frequent soft tissue tumour affecting the hand. No consensus exists on the etiology, prognostic factors, or recurrence rate of GCTTS. This article presents a series of 18 cases supplemented by a literature review that examines the epidemiology, presentation, gross and microscopic characteristics, and recurrence rate of GCTTS. Methods: A total of 18 patients with a histo-pathological diagnosis of a GCTTS of the hand or finger were reviewed. The location for the tumour was limited to the wrist and hand. All cases were operated under Wide Awake Local anaesthesia (WALANT), and using a magnifying loupe. Results: A total of 18 patients presented at our institution with a diagnosis of GCTTS from 2016 to 2018. Of the 18 patients, 11 were female and 7 were male. The mean age of included patients was 43.6 years (31–59 years). The most common site for the lesion was the middle finger (4/18), followed by the index finger, wrist, and thumb (3/18 each). The little and ring finger were least commonly affected with one case each. The mean size of the tumour was 2.4 cm (0.5–5 cm). None of the patients reported recurrence of the lesion on an average follow-up of 18.8 months. Conclusion: GCTTS is a benign, slowly growing lesion of the hand that typically does not cause any symptoms and is treated with surgical resection. Meticulous excision of the GCTTS using magnification loupes to ensure appropriate wide excision of the tumour is the treatment of choice to prevent a recurrence. In addition, a radiographic and histopathological examination must be performed on the tumour to rule out other diagnoses. Finally, the function of the hand should be reconstructed to minimize the loss of any functional unit. [ABSTRACT FROM AUTHOR]

Giant cell tumors of the bone (GCTB) are considered moderately malignant bone tumors. Denosumab, as a neoadjuvant therapy, provides new possibilities for treating GCTB. However, even after multiple studies and long-term clinical trials, there are limitations in the treatment process. Research data and Medical Subject Headings terms related to denosumab and GCTB were collected from January 2010 to October 2022 using the Web of Science and MeSH (https://meshb.nlm.nih.gov) browsers. These data were imported into CiteSpace and VOSviewer softwares for bibliometric analysis. Overall, 445 publications on denosumab and GCTB were identified. Over the last 12 years, the growth rate of the total number of publications has remained relatively stable. The USA published the highest number of articles (83) and had the highest centrality (0.42). Amgen Inc. and Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) First Ortoped Rizzoli were identified as the most influential institutions. Many authors have made outstanding contributions to this field. Lancet Oncology had the highest journal impact factor (54.433). Local recurrence and drug dosage are current research hotspots, and future development trends will mainly focus on prognostic markers of GCTB and the development of new therapies. Further research is required to analyze denosumab's safety and efficacy and understand its local recurrence of GCTB, to identify the optimal dose. Future progress in this field will likely focus on exploring new diagnostic and recurrence markers to monitor disease progression and examine new therapeutic targets and treatment strategies. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Desmoplastic round cell tumor, though rare, must be taken into consideration as a differential diagnosis, thus aiding in early evaluation and changing the trajectory of the natural history of the disease condition, and improving the prognosis of patients. Desmoplastic small round cell tumor is a rare, aggressive tumor of mesenchymal origin with an incidence of 0.74 cases per million. We present a young adult with a periumbilical mass who was diagnosed as a desmoplastic round cell tumor and later was treated with exploratory laparotomy and resection of the tumor with no recurrence during a 6‐month follow‐up period. [ABSTRACT FROM AUTHOR]

Background A vascularized fibula graft (VFG) is the vascular autograft most frequently used to restore large segmental long bone defects, particularly in the upper limb. Because the use of a vascularized fibula involves an operation in an uninvolved extremity with potential morbidity, it is important to document that this type of reconstruction is successful in restoring function to the humerus. However, the long-term results of VFG after intercalary resection of the humeral diaphysis for bone tumors are still unknown. Questions/purposes (1) What was the complication rate of reconstruction? (2) What was the functional result after surgical treatment, as assessed by the Musculoskeletal Tumor Society (MSTS) score, the American Shoulder and Elbow Society (ASES) score, and Constant score? (3) What was the survivorship of these grafts free from revision and graft removal at 5, 10, and 15 years? Methods Between 1987 and 2021, 127 patients were treated at our institution with en bloc resection for a primary malignant or an aggressive benign bone tumor of the humerus; we excluded patients treated with extra-articular resection or amputation. Of those, 14% (18 of 127) were treated with intercalary resection of the humeral diaphysis for primary bone tumors and reconstruction with VFG, with or without a bulk allograft, and were analyzed in this retrospective study. Generally, our indications for reconstruction with VFG are intercalary resection of the humerus for primary malignant or aggressive benign bone tumors in patients with long life expectancy and high functional demands, in whom adequate bone stock of the proximal and distal epiphysis can be preserved. In 13 patients, VFG was used alone, whereas in five patients, a massive allograft was used. Our policy was to use VFG combined with a massive allograft in patients undergoing juxta-articular joint-sparing resections in which proximal osteotomy was performed close to the anatomic neck of the humerus to obtain more stable fixation and better tendinous reattachment of the rotator cuff and deltoid. All 18 patients who were treated with a VFG were available for follow-up at a minimum of 2 years (median follow-up 176 months, range 26 to 275 months), and although three have not been seen in the past 5 years and are not known to have died, they had 172, 163, and 236 months of follow-up, and were included. The median age at surgery was 25 years (range 2 to 63 years), the median humeral resection length was 15 cm (range 8 to 21 cm), and the median fibular length was 16 cm(range 12 to 23 cm). Complications and functional scores were ascertained by chart review that was performed by an individual not involved in patient care. Functional results were assessed with the MSTS score (range 0 to 30), the ASES score (range 0 to 100), and the Constant score (range 0% to 100%). Survivorship was estimated using a Kaplan- Meier survivorship estimator, which was suitable because there were few deaths in this series. Results Seven patients underwent a revision procedure (one radial nerve transient palsy because of screw impingement, four nonunions in three patients with one humeral head avascular necrosis, treatment for screw-related pain in one patient, and two VFG fractures), and one patient underwent VFG removal. Donor site complications were observed in four patients (one ankle valgus deformity and three claw toes--the first toe in two patients and the other toes in the third). At the final clinical control, at a median follow-up of 176 months (range 26 to 275 months), the median MSTS score was 30 of 30 (range 28 to 30), the median ASES score was 98.3 (range 93 to 100), and the median Constant score was 93.5% (range 79% to 100%). Revision-free survival was 71% (95% CI 53% to 96%) at 5 years and 57% (95% CI 37% to 88%) at 10 and 15 years; VFG removal-free survival was 94% (95% CI 83% to 100%) at 5, 10, and 15 years. Conclusion VFG appears to be an effective reconstructive option after humeral intercalary resection for primary bone tumors. These are complex procedures and should be performed by an experienced team of surgeons who recognize that complications may occur frequently in the first years after the procedure. The frequency of mechanical complications observed in the first 5 years postoperatively may be lessened by using long spanning-plate fixation, and if successful, this reconstruction provides a long-term, durable reconstruction with excellent functional results. Level of Evidence Level IV, therapeutic study. [ABSTRACT FROM AUTHOR]

Pigmented villonodular synovitis (PVNS) is an infrequent benign tumor-like proliferative lesion developing from the synovial membranes of the joint, tendon sheath, and bursa. A 44-year-old woman with numbness on the right side of her face, severe headaches, and swelling in temporomandibular region is presented. On head and neck magnetic resonance imaging, an encapsulated mass approximately 2 cm was detected. The fine-needle aspiration biopsy resulted as suspicion of mesenchymal tumor. A complete resection with the capsule was performed over the temporal branch by monitoring of the facial nerve. The final histopathologic examination resulted as a giant cell tendon sheath tumor. Headache is not the main symptom in PVNS, but in severe pain spreading from the temporomandibular region, physical examination should be done carefully for slight swelling, and the possibility of pigmented villonodular synovitis should be considered. Because of the high recurrence rate, en bloc resection is necessary. [ABSTRACT FROM AUTHOR]

Background: Since giant cell tumors of bone (GCTB) and other giant cell rich tumors of bone (GCRTB) share the histological presence of osteoclastic giant cells and expression of RANK/RANKL, we hypothesized that GCRTB will respond similarly to denosumab as GCTB. The primary objective of this study was to determine the efficacy of denosumab in patients with GCRTB that have recurred or require morbid surgery. Methods: In this open-label, multicenter, phase II trial, patients with GCRTB were included (June 2018-March 2020). Recruitment was stopped because of low accrual. Patients received denosumab (120 mg) subcutaneously (SC) on day 1 of every 4-week cycle with a loading dose of 120 mg SC on days 8 and 15. Results: Three patients were enrolled. One withdrew consent before start of study. The remaining patients had central giant cell granuloma of the jawbone (CGCG). Median treatment duration was 15 cycles (range 12-18). In both subjects, improvement in ossification of lesions was seen. Median follow-up was 28.5 months (range 20-37). One patient developed a recurrence for which surgery was performed. Conclusion: Due to critical emerging real-world data of denosumab in GCRTBs, the study was prematurely stopped and not supportive of use of denosumab for this indication. (ClinicalTrials.gov Identifier: NCT03605199). Giant cell tumors of bone (GCTB) and other giant cell rich tumors of bone (GCRTB) share the histological presence of osteoclastic giant cells and expression of RANK/RANKL, and so it was hypothesized that GCRTB would respond similarly to denosumab as GCTB. This article reports on the efficacy of denosumab in patients with GCRTB that have recurred or require morbid surgery. [ABSTRACT FROM AUTHOR]

Abstract Background Giant cell tumor (GCT) is a relatively common and locally aggressive benign bone tumor that rarely affects the sternum. Case presentation We report a case of giant cell tumor of the sternum in a 28-year-old Saudi with painful swelling at the lower part of the sternum. Subtotal sternectomy and reconstruction with a neosternum using two layers of proline mesh, a methyl methacrylate prosthesis, and bilateral pectoralis muscle advancement flaps were performed. Conclusions Giant cell tumor of the sternum is a rare diagnosis. Surgical resection with negative margins is the ideal management. To avoid defects or instability of the chest wall, reconstruction of the chest wall with neosternum should be considered.

Giant Cell Tumors of the skull are rare and mostly occur in the middle cranial fossa. Radiological investigations serve as adjunct modalities; however, histopathological confirmation is mandatory. Ten to forty% of GCTs may be recurrent. Complete surgical resection is the treatment of choice, however, partial resection with adjuvant radiotherapy can serve as a secondary alternative. Recurrent cases require post-op radiotherapy. Here, we describe a case of recurrent giant cell tumor of sphenoid bone in a young male, who underwent surgical resection twice, after which he was advised adjuvant radiotherapy and denosumab. The patient did not take radiotherapy. [ABSTRACT FROM AUTHOR]

This article discusses a case of plaque-like cutaneous mucinosis (PCM) in a patient with KRAS-mutated giant cell granuloma of the jaw. PCM is a condition characterized by lichenoid papules, nodules, or plaques with glycosaminoglycan deposition in the dermis. The patient in this case had PCM overlying a benign bone-resorbing neoplasia of the jaw called central giant cell granuloma (CGCG). The PCM rapidly resolved following surgical resection of the tumor. The article also mentions other paraneoplastic dermatoses associated with oral neoplasia and provides information on the diagnosis and differential diagnosis of PCM. [Extracted from the article]

This article discusses the molecular drivers and pathogenesis of giant cell granulomas of the jaws (CGCGJ) and their implications for targeted therapy. The study found that CGCGJ shares histopathological similarities with other giant cell-rich lesions, but does not exhibit certain mutations found in those lesions. Recent advancements in molecular studies have identified somatic mutations in genes such as KRAS, TRPV4, and FGFR1 in CGCGJ. The article also explores the association between CGCGJ and certain syndromes, as well as the potential use of TRPV4 inhibitors as a therapeutic approach for more aggressive cases. [Extracted from the article]

Abstract Background The objective of this study was to assess the expression profile of CD44v6, a potential cancer stem cell marker, and its diagnostic and predictive significance in three distinct types of primary bone tumors. Methods In this study, we utilized real-time qRT-PCR and immunohistochemistry to examine the gene and protein levels of CD44v6 in a total of 138 fresh bone tissues. This included 69 tumor tissues comprising osteosarcoma (N = 23), chondrosarcoma (N = 23), and GCT (N = 23), as well as 69 corresponding non-cancerous tumor margins. Furthermore, we investigated the circulating level of CD44v6 by isolating peripheral blood mononuclear cells from 92 blood samples. Among these, 69 samples were obtained from patients diagnosed with primary bone tumors, while the remaining 23 samples were from healthy donors. The primary objectives of our investigation were to assess the correlation between CD44v6 expression levels and clinic-pathological features of the patients, as well as to evaluate the diagnostic and predictive values of CD44v6 in this context. Results In patients with osteosarcoma and chondrosarcoma tumors, both the gene and protein expression of CD44v6 were found to be significantly higher compared to the GCT group. Furthermore, the circulating level of CD44v6 was notably elevated in patients diagnosed with osteosarcoma and chondrosarcoma in comparison to the GCT group and patients with malignant tumor characteristics. Additionally, we observed a strong correlation between the gene and protein levels of CD44v6 and important tumor indicators such as tumor grade, metastasis, recurrence, and size at the tumor site. CD44v6 shows potential in differentiating patients with bone tumors from both control groups and tumor groups with severe and invasive characteristics from those with non-severe features. Importantly, the expression level of CD44v6 also demonstrated predictive value for determining tumor grade and the likelihood of recurrence. Conclusion CD44v6 is likely to play a role in the development of primary bone tumors and has the potential to serve as a diagnostic biomarker for bone cancer. However, to obtain more accurate and conclusive findings, further mechanistic investigations involving larger population samples are necessary.

Abstract Objective This is the first study to establish the utility of extended curettage with or without bone allograft for Grade II giant cell tumors GCTs around the knee joint with the aim of exploring postoperative functional outcomes. Methods We retrospectively reviewed 25 cases of Campanacci grade II GCTs undergoing extended curettage between January 2014 and December 2019. The participants were divided into two groups: one group of 12 patients underwent extended curettage with bone allograft and bone cement, while the other group of 13 patients underwent extended curettage with bone cement only. Quality of life was assessed by the Revised Musculoskeletal Tumor Society Score and by the Knee score of the Knee Society; recurrence and complications were assessed for each cohort at the last follow-up. The Fisher test and two-sample t-tests were used to compare the categorical and continuous outcomes, respectively. Results The mean age was 28.09 (7.44) years old, with 10 (40%) males and 15 females (60%). The distal femur and the proximal tibia were involved in 13 (52%) and in 12 (48%) patients, respectively. There was no significant difference in the musculoskeletal tumor society score (25.75 versus 27.41; p= 0.178), in the knee society score (78.67 versus 81.46; p= 0.33), recurrence (0 versus 0%; p= 1), and complications (25 versus 7.69%; p= 0.21). Conclusions Extended curettage with or without bone allograft have similar functional outcomes for the knee without any major difference in the incidence of recurrence and of complications for Grade II GCTs. However, surgical convenience and cost-effectiveness might favor the bone cement only, while long-term osteoarthritis prevention needs to be investigated to favor bone allograft.

BACKGROUND: Tumor segment resection is one of the standard methods for the treatment of bone tumors. However, the reconstruction of bone defects after tumor resection faces many challenges. A growing number of researchers are focusing on 3D-printed prostheses for bone defect repair and reconstruction following bone tumor surgery. OBJECTIVE: To explore the feasibility of 3D-printed prostheses in the reconstruction of large bone defect following bone tumor surgery and to evaluate the postoperative outcomes. METHODS: Retrospective analysis of clinical data of 24 patients [19 males and 5 females, age 23.8 (6-61) years] who underwent bone tumor resection and 3D-printed prosthesis implantation in the Department of Bone Oncology, the First Affiliated Hospital of Xinjiang Medical University from December 2020 to September 2021 was conducted. There were 7 cases with distal femur tumor, 5 with pelvis tumor, 4 with proximal tibia tumor, 3 with middle femur tumor, 1 with distal tibia tumor, 1 with proximal humerus tumor, 1 with middle humerus tumor, 1 with scapula tumor, 1 with ulna tumor, and 22 cases with primary tumors (13 osteosarcoma, 4 Ewing sarcoma, 2 giant cell tumor of bone, 1 chondroblastoma, 1 chondrosarcoma, and 1 osteoblastoma), 2 metastatic carcinoma. Preoperative and postoperative imaging data were recorded and neoadjuvant chemotherapy was administered in 17 cases before surgery. The Musculoskeletal Tumour Society score was used to assess limb function before surgery and 6 months after surgery, and pain was assessed by the Visual Analog Scale, as well as the complications were recorded. RESULTS AND CONCLUSION: (1) All patients undergoing resection of the tumor segment and 3D-printed prosthesis implantation for the reconstruction of the bone defect were followed for 6-49 months, and the results showed that the length of osteotomy was (18.2 ± 7.3) cm and an average intraoperative bleeding volume was 740 (100-3 000) mL. (2) Two patients died of systemic metastasis, the remaining 22 had no pulmonary metastasis or recurrence during the followup period, and 1 patient developed aseptic loosening of the prosthesis at 25 months postoperatively. (3) The Musculoskeletal Tumour Society scores were significantly increased, while Visual Analog Scale scores were significantly decreased (P < 0.05) at 6 months postoperatively. (4) The Musculoskeletal Tumor Society score was rated excellent in all 22 patients at the final follow-up. (5) These results suggest that 3D-printed prosthesis is suitable for the reconstruction of large bone defects caused by bone tumor resection. Patients have good postoperative function and few complications. However, further investigations are needed to explore long-term follow-up results. [ABSTRACT FROM AUTHOR]

Background: Giant cell tumor (GCT) is a relatively common and locally aggressive benign bone tumor that rarely affects the sternum. Case presentation: We report a case of giant cell tumor of the sternum in a 28-year-old Saudi with painful swelling at the lower part of the sternum. Subtotal sternectomy and reconstruction with a neosternum using two layers of proline mesh, a methyl methacrylate prosthesis, and bilateral pectoralis muscle advancement flaps were performed. Conclusions: Giant cell tumor of the sternum is a rare diagnosis. Surgical resection with negative margins is the ideal management. To avoid defects or instability of the chest wall, reconstruction of the chest wall with neosternum should be considered. [ABSTRACT FROM AUTHOR]

Simple Summary: Prostate cancer remains a leading cause of death in men worldwide. Polyploid giant cancer cells (PGCCs) and chromosomal instability have been proposed to drive the progression of cancer. Given that HCMV infection has been implicated in malignant diseases from different cancer entities, in the present study, we assessed its transformation potential in vitro and evaluated the obtained cellular and molecular phenotypes of prostate epithelial cells (PECs) using HCMV high-risk clinical strains, DB and BL, which were previously isolated in our laboratory. HCMV-induced PGCC formation, Myc and EZH2 upregulation, as well as the stemness and epithelial–mesenchymal transition features verified the transformation process of PECs. Our research work deserves to be distributed among the scientific community, as it paves the way for upcoming studies targeting the potential role of HCMV and PGCCs in prostate cancer development and treatment. Background: Prostate cancer is the most commonly diagnosed malignancy and the sixth leading cause of cancer death in men worldwide. Chromosomal instability (CIN) and polyploid giant cancer cells (PGCCs) have been considered predominant hallmarks of cancer. Recent clinical studies have proven the association of CIN, aneuploidy, and PGCCs with poor prognosis of prostate cancer (PCa). Evidence of HCMV transforming potential might indicate that HCMV may be involved in PCa. Methods: Herein, we underline the role of the high-risk HCMV-DB and -BL clinical strains in transforming prostate epithelial cells and assess the molecular and cellular oncogenic processes associated with PCa. Results: Oncogenesis parallels a sustained growth of "CMV-Transformed Prostate epithelial cells" or CTP cells that highly express Myc and EZH2, forming soft agar colonies and displaying stemness as well as mesenchymal features, hence promoting EMT as well as PGCCs and a spheroid appearance. Conclusions: HCMV-induced Myc and EZH2 upregulation coupled with stemness and EMT traits in IE1-expressing CTP might highlight the potential role of HCMV in PCa development and encourage the use of anti-EZH2 and anti-HCMV in PCa treatment. [ABSTRACT FROM AUTHOR]

Granular cell tumors are extremely uncommon soft tissue neoplasms that mostly occur in the head and neck regions. Granular cell tumors are generally benign, asymptomatic, and rarely involve the median nerve. Due to the lack of awareness about granular cell tumors, they are easily misdiagnosed and mistreated in primary hospitals. Here, we report a giant atypical granular cell tumor located on the median nerve, approximately 12cm in size, with unusual symptoms of median nerve damage. Magnetic resonance imaging revealed a fusiformmass that was hyperintense on T2-weighted images and iso-hypointense on T1-weighted images. The mass was subsequently biopsied and found to be a granular cell tumor. The tumor was resected, and a pathological examination was performed. Pathological examination revealed necrotic foci, abundant eosinophilic granules, pustular ovoid bodies, and multiple mitoses. Immunohistochemical staining revealed that the tumor cells were positive for S-100, CD68, SMA, SOX-10, Calretinin, and TFE3. The integrated diagnosis was an atypical granular cell tumor. To the best of our knowledge, this is the first report of an atypical granular cell tumor involving the median nerve. Furthermore, we comprehensively reviewed the existing literature to provide a concise summary of the diagnostic criteria, imaging findings, and pathological features of granular cell tumors. Given the high recurrence and metastasis rates of this disease, granular cell tumors of the median nerve should be considered when a patient presents with symptoms of median nerve impairment. The diagnosis of atypical granular cell tumors relies on pathological examination. In addition, extensive resection and long-term follow-up are necessary to improve prognosis. [ABSTRACT FROM AUTHOR]

Background: Primary hyperparathyroidism (PHPT) is an uncommon disorder characterised by hypercalcemia with an increased parathyroid hormone level. We reported a PHPT familial case with two subjects, a father and a daughter, and both of them had suffered from the brown tumor. Case presentation: The proband, a 43-year-old patient, developed parathyroid adenomas at the age of 15; a histologically confirmed right parathyroid adenoma was removed by parathyroidectomy; and after six months follow-up, the serum calcium level was normalised. At the age of thirty-three, a CT scan of his head and neck revealed a mass in the right maxilla, as well as PHPT (i.e., left inferior parathyroid adenoma). Then, he underwent a biopsy of an exophytic lesion in the right maxilla and was diagnosed by pathology as a brown tumor, with the serum calcium and PTH levels at 2.78 mmol/L and 221 pg/mL, respectively. Subsequently, the patient took a left inferior parathyroid microwave ablation with ultrasound guidance. After three months of follow-up, the serum calcium and PTH levels returned to normal, and the brown tumor was resolved. After three years, it mineralised as revealed in a CT scan. By the time he was 43 years old, during the 28-year follow-up period, the serum calcium and PTH levels were still within the normal range, and there was no discomfort reported. He has consistently taken calcium supplements throughout the 28 years. Since the initial diagnosis, his blood indicators of kidney function have been normal, and ultrasound showed renal calculus in the right kidney and a normal left kidney. The proband's daughter, a 15-year-old girl, experienced left upper extremity pain for ten months. CT scan revealed a mass in the distal left radius, and a giant cell tumor was suspected. A surgical internal fixation was performed, and the pathology showed a brown tumor. Laboratory tests revealed a serum parathyroid hormone (PTH) level of 1554pg/mL, calcium level of 3.14 mmol/L, phosphorus level of 0.72 mmol/L, and alkaline phosphatase level of 1892 U/L. Given the osteitic changes and elevated levels of calcium and PTH, ultrasonography was performed, after which a mass was detected measuring 19 × 9 × 7 mm mixed with solid components and cystic fluid in the right thyroid gland. The results of 99mTc-MIBI scintigraphy confirmed the abnormal accumulation of 99mTc-MIBI in the right thyroid gland but not seen in the bilateral parathyroid glands. The patient underwent thyroidectomy, and the postoperative pathology report indicated an intra-thyroid ectopic parathyroid adenoma. The serum calcium and PTH levels became normal at 4 h after surgery. One to three months after operation, the serum calcium level was low, while the serum PTH level was high. Then, the patient was advised to take calcium supplements. Until the sixth month after the operation, the serum calcium level and serum PTH level returned to normal, and the bone pain was relieved. The patient's blood tests for kidney function remained normal. There was no evidence of bilateral kidney disease (such as nephrolithiasis or nephrocalcinosis) detected by ultrasound scan. There were several similarities in the state of illness between these two subjects. Both the father and the daughter developed parathyroid adenomas at the age of 15, and there was no lesion in other endocrine glands. And genetic testing revealed mutations in the CDC73 genes in both father and daughter. On the other hand, there were also a few differences. The father's first signs of brown tumor were in the right maxilla, while the daughter's appeared in the distal left radius. The father presented pathological changes in the left and right parathyroid glands, whereas the daughter presented with an ectopic parathyroid adenoma in the right thyroid gland. Conclusion: We report a familial case in which father and daughter were diagnosed to have brown tumors due to parathyroid adenoma and ectopic parathyroid adenoma, and genetic testing revealed CDC73 gene mutations in both. Therefore, in the diagnostic and differential process of young patients having bone disease, clinicians should not only focus on the clinical manifestations of the skeleton, but also implement a comprehensive analysis of systemic symptoms, considering the possibility that the patient has familial PHPT. [ABSTRACT FROM AUTHOR]

Background: This case reports the synchronous diagnosis of two rare unrelated diseases; leiomyosarcoma and tenosynovial giant cell tumor of the knee. It focuses on the challenges of diagnosing tenosynovial giant cell tumor, including cognitive biases in clinical medicine that delay diagnosis. It also demonstrates the pathogenic etiology of tenosynovial giant cell tumor, evidenced by the transient deterioration of the patients' knee symptoms following the administration of prophylactic granulocyte colony-stimulating factor given as part of the chemotherapeutic regime for leiomyosarcoma. Case presentation: A 37-year-old Caucasian man presented with a left groin lump and left knee pain with swelling and locking. Investigations including positron emission tomography-computed tomography and biopsy revealed leiomyosarcoma in a lymph node likely related to the spermatic cord, with high-grade uptake in the left knee that was presumed to be the primary site. His knee symptoms temporarily worsened each time granulocyte colony-stimulating factor was administered with each cycle of chemotherapy for leiomyosarcoma to help combat myelosuppressive toxicity. Subsequent magnetic resonance imaging and biopsy of the knee confirmed a tenosynovial giant cell tumor. His knee symptoms relating to the tenosynovial giant cell tumor improved following the completion of his leiomyosarcoma treatment. Conclusions: Tenosynovial giant cell tumor remains a diagnostic challenge. We discuss the key clinical features and investigations that aid prompt diagnosis. The National Comprehensive Cancer Network clinical practice guidelines for soft tissue sarcoma have recently been updated to include the pharmacological management of tenosynovial giant cell tumor. Our case discussion provides an up-to-date review of the evidence for optimal management of patients with tenosynovial giant cell tumor, with a particular focus on novel pharmacological options that exploit underlying pathogenesis. [ABSTRACT FROM AUTHOR]

Pexidartinib is a colony-stimulating factor-1 receptor inhibitor approved in the United States for treatment of adult patients with symptomatic tenosynovial giant cell tumor (TGCT) associated with severe morbidity or functional limitations and not amenable to improvement with surgery. Because of the risk of severe and potentially fatal hepatotoxicity, pexidartinib is only available through a Risk Evaluation and Mitigation Strategy (REMS) program. Pexidartinib pharmacokinetics are influenced by the fat content of meals: compared with the fasted state, consuming a high-fat meal with pexidartinib increases pexidartinib absorption by 100%; a low-fat meal increases absorption by approximately 60%. Pexidartinib was initially authorized to be taken at 800 mg/day on an empty stomach; therefore, if this same dose of pexidartinib is taken with food, there is a risk of overexposure and potential toxicity. To reduce the risk of hepatotoxicity and improve patient compliance, pexidartinib has undergone a revised dosing regimen, from 800 mg/day (400 mg twice daily) fasted to 500 mg/day (250 mg twice daily) with a low-fat meal (approximately 11–14 g of total fat). The objective of this report is to educate clinical and allied health professionals on the revised dosing regimen and the importance of patient compliance with a low-fat meal. Healthcare professionals need to understand the rationale for the switch from pexidartinib dosing on an empty stomach to dosing with a low-fat meal and how meal composition and timing influence pharmacokinetics. Finally, we provide guidance for the healthcare team of prescribing providers, nurses, pharmacists, and dietitians who are caring for patients with TGCT on pexidartinib. It is important for healthcare providers to deliver consistent messaging on the low-fat meal requirement and help patients fit pexidartinib into their regular meal schedules. Consulting a dietitian may be helpful for patients, especially those with complex dietary needs. We provide an overview of the roles and responsibilities of each healthcare professional and outline steps to best support patients, including key questions and answers related to the revised dosing regimen. This report provides the information necessary to guide the multidisciplinary team caring for patients with TGCT and to support them through the pexidartinib dosing regimen change. [ABSTRACT FROM AUTHOR]

Introduction. One of the possible manifestations of primary hyperparathyroidism (PHPT) is the appearance of a benign bone tumor. We hereby present a case of a young woman whose first clinical manifestation of PHPT was a brown tumor of the mandible. Case report. A 27-year-old female patient was hospitalized at the Clinic for Endocrinology, Diabetes, and Metabolic Diseases due to problems occurring in the form of nausea, exhaustion, the feeling of suffocation, dysphagia, pain in the right ear and the right half of the cheeks, with suspected PHPT. Initial laboratory findings pointed out the high levels of parathyroid (PT) hormone (PTH) and calcium (Ca2+) ions, low levels of vitamin D, and increased parameters of bone metabolism with signs of osteopenia. Cone beam computed tomography revealed the presence of bilateral radiolucent lesions of the mandible. Scintigraphy verified a retrosternal hot focus consistent with PT adenoma. After parathyroidectomy was performed, there was a normalization of PTH values, bone metabolism parameters, and the Ca2+ values. Four months after parathyroidectomy, a significant regression of the mandibular tumefaction was confirmed, clinically and radiologically. Conclusion. Brown tumors are rare first clinical manifestations of PHPT. Owing to their histological similarities with other giant-cell lesions (GCLs), definitive diagnosis is sometimes difficult and is based on a correlation of pathohistological, radiological, and laboratory findings. Due to the spontaneous regression of bone lesions after treatment of the basic cause of PHPT, brown tumors should be considered in the differential diagnosis of any GCLs in order to avoid unnecessary surgical procedures. [ABSTRACT FROM AUTHOR]

Surgery for spinal giant cell tumors (GCTs) is challenging because these tumors often exhibit a poor clinical course owing to their locally aggressive features. This study aimed to investigate the prognostic factors of GCT recurrence in the spine by focusing on surgical factors. We retrospectively reviewed patients who underwent surgery for spinal GCTs between January 2005 and December 2016. Using the Kaplan-Meier method, surgical variables were evaluated for disease-free survival (DFS). Since tumor violation may occur at the pedicle during en bloc resection of the spine, it was further analyzed as a separate variable. Multivariate Cox proportional hazard regression analysis was performed for other clinical and radiographic variables. A total of 28 patients (male:female = 8:20) were included. The mean follow-up period was 90.5 months (range, 15–184 months). Among the 28 patients, gross total resection (GTR) was the most important factor for DFS (P = 0.001). Any form of tumor violation was also correlated with DFS (P = 0.049); however, use of en bloc resection technique did not show a significant DFS gain compared to piecemeal resection (P = 0.218). In the patient group that achieved GTR, the mode of resection was not a significant factor for DFS (P = 0.959). In the multivariate analysis, the extent of resection was the only significant variable that affected DFS (P = 0.016). Conflicting results on tumor violation from univariate and multivariate analyses suggest that GTR without tumor violation should be the treatment goal for spinal GCTs. However, when tumor violation is unavoidable, it would be important to prioritize GTR over adhering to en bloc resection. [ABSTRACT FROM AUTHOR]

Giant cell tumors of bone are a rare entity, usually occurring in young patients and characteristically arising in the long bones. The spinal location is rare and usually presents with pain and/or neurological symptoms. The treatment of choice is surgery. Treatment with Denosumab, a bisphosphonate inhibitor of RANK-L, which is highly expressed in these tumors, has shown extensive activity in unresectable patients or those undergoing incomplete surgery. Preoperative treatment with this drug is gaining increasing interest, as its high potency in tumor reduction in this subtype of neoplasm has allowed resectability in selected patients. We present the case of a young patient with a large spinal tumor who, after neoadjuvant Denosumab, underwent complete en bloc surgery with clean margins and a great pathological response. [ABSTRACT FROM AUTHOR]

Background: The management of the GCT continues to be controversial. The most accepted treatment reported is extended curettage with various adjuvants. However, the rate of recurrence has been very high (10–66%). For better understanding of the lesion and its management, it has become necessary to revise the existing grading system. Materials and Methods: The recommendation of the proposed grading is based on 480 cases of GCT along with the analysis of other major reports published. Out of these, 279 were grade-II and 109 grade-III which were subjected to en bloc excision and reconstruction with non-vascularized fibular graft. Depending on the site, the grafts were stabilized with a suitable plate or extra long Kirschner wires. Results: The observations made in 480 cases over a period of 5 decades revealed that for Campanacci grade-II and grade-III lesions, en bloc excision and suitable reconstruction resulted in minimum recurrence rate of 1.2%. The fibular reconstruction after excision has proved advantageous to our patients as it provides a stable joint in the form of an arthrodesis of the neighboring joint. Conclusion: GCT of bone has been a challenging lesion. Extended curettage has been recommended as the choice of treatment but the reported rate of recurrence has been high. En bloc excision and suitable reconstruction with fibular strut graft have resulted in 1.2% recurrence. It is, therefore, suggested the existing grading system should be revised mainly for grade-II and grade-III lesions which can bring down the high rate of recurrence in this unpredictable tumor. Level of Evidence: Level-IV. [ABSTRACT FROM AUTHOR]

Although seroma is a common complication following breast augmentation surgery, it is very rare in the late period (> 1 year) after implant removal. Here, we present bilateral seromas mimicking breast implants that developed 2 years after explantation. A 55-year-old female patient presented with bilateral round implant-like formations in the breasts. It was learned that the patient had undergone breast surgery three times before. First, she had undergone breast augmentation with textured round silicone breast implants 20 years before. Second, she had undergone implant removal 4 years prior because of painful capsule contracture. Finally, she had undergone seroma evacuation 2 years before. We performed surgical exploration and total capsulectomy to rule out malignancy. While restoring the shape of the breast with mastopexy, malignancy was ruled out. No recurrence was observed at the 1-year follow-up visit. We recommend complete removal of the regenerative layer via total capsulectomy is the most appropriate surgical option to prevent the recurrence of seroma. Level of Evidence: V [ABSTRACT FROM AUTHOR]

Purpose: Bone diseases account for an enormous cost burden on health systems. Bone disorders are considered as age-dependent diseases. The aging of world population has encouraged scientists to further explore the most effective preventive modalities and therapeutic strategies to overcome and reduce the high cost of bone disorders. Herein, we review the current evidence of melatonin's therapeutic effects on bone-related diseases. Methods: This review summarized evidences from in vitro, in vivo, and clinical studies regarding the effects of melatonin on bone-related diseases, with a focus on the molecular mechanisms. Electronically, Scopus and MEDLINE®/PubMed databases were searched for articles published on melatonin and bone-related diseases from inception to June 2023. Results: The findings demonstrated that melatonin has beneficial effect in bone- and cartilage-related disorders such as osteoporosis, bone fracture healing, osteoarthritis, and rheumatoid arthritis, in addition to the control of sleep and circadian rhythms. Conclusion: A number of animal and clinical studies have indicated that various biological effects of melatonin may suggest this molecule as an effective therapeutic agent for controlling, diminishing, or suppressing bone-related disorders. Therefore, further clinical studies are required to clarify whether melatonin can be effective in patients with bone-related diseases. [ABSTRACT FROM AUTHOR]

Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disease of unknown etiology. Typically presenting with cervical adenopathy and constitutional symptoms, RDD involves bone in less than 10% of cases—and rarely presents as a primary intraosseous lesion. In this report, we describe the presentation of primary, bilateral intraosseous RDD, the first known case in the literature. Asymmetrically involving the lateral femoral condyles of a 59-year-old male, the lesion was discovered incidentally during evaluation and workup for giant cell tumor of bone involving the left tibia. Confirmation of the diagnosis required multiple biopsies and extensive evaluation—reflecting the diagnostic challenge associated with this case. We discuss the clinical, radiological, and pathological findings that allowed us to establish the diagnosis—as well as key differential diagnostic considerations and clinical outcome to date. [ABSTRACT FROM AUTHOR]

(C) CD34 positivity of tumor cells in immunohistochemistry (×200). gl DISCUSSION SAF is 1 of the rare benign myxoid neoplasms recently identified. Keywords: acral fibromyxoma; benign tumor; dermoscopy; histology EN acral fibromyxoma benign tumor dermoscopy histology 2110 2113 4 10/11/23 20231001 NES 231001 INTRODUCTION Superficial acral fibromyxoma (SAF) is a benign and rare tumor of the soft tissues. Biopsy showed a regular epidermis, well-limited tumor proliferation made up of regular spindle or stellate cells dissociated by collagen fibers with an abundant myxomatous stromal background (Figure 3A,B). [Extracted from the article]

The modification of the used and development of new treatment regimens significantly improved the overall, recurrence-free survival and quality of life of patients with malignant oncological diseases. Recently, drugs used in non-oncological pathology have been introduced into cancer treatment regimens. This phenomenon is associated with a better understanding of the biology of tumor cells and the mutations that can change this biology. Metformin is actively researched in terms of the treatment of various oncological diseases. For the most part, the modification of the neoadjuvant treatment regimen for early or locally advanced stages of breast cancer results in a less traumatic variant of surgical procedure, thereby increasing recurrence-free survival. The aim is to systematize the data on the possibilities of the antitumor metformin usage for neoadjuvant treatment of breast cancer and to study the results of clinical and morphological effectiveness of the treatment by reviewing the literature. A search of PubMed from February 2023 showed 258 results on the antitumor effects of metformin, of which only 159 were published in the last 5 years. On this subject only four clinical studies were carried out, and only one of them pertained to the implementation of metformin in the systemic treatment of breast cancer. For this review, 55 sources of general and specialized information on anticancer effects of metformin were analyzed. It should be noted that approximately 60% of the study results were published more than 5 years ago and primarily focused on the biological, not clinical aspects of metformin usage. Only one study regarding the implementation of metformin for systemic treatment of breast cancer was carried out by Ukrainian scientists. Currently, there are 2 main hypotheses regarding the antitumor effect of metformin. First one is driven by its impact on the metabolic function of cells and energy deficit. Second – the method of regulating the proliferation of tumor cells involves the PIK3 branch of the regulatory cascade of biological reactions in cancer cells. In addition, metformin reduces the development of resistance in breast cancer cells, thus allowing active chemotherapy agents to act in synergism. However, further studies on the effect of metformin used alone or in combination with standard chemotherapy regimens require a more adequate definition of proto-oncogenic mutations and somatic mutation load. However, it should be considered that more aggressive therapy of oncological diseases can be a nosocomial selector of more aggressive clones of the pool of tumor cells. The main questions are whether metformin can be a targeted drug for the treatment of tumor, whether it is appropriate to use it at the time when the manifestation of evolution disturbances of tumor cell is minimal and homogeneity is maximal. [ABSTRACT FROM AUTHOR]

The aim of our study was to review current concepts in targeted therapies for benign tumors of the jaw. Benign odontogenic and maxillofacial bone tumors often require radical surgery, with consequent morbidity that impacts patients' postsurgical quality of life. Currently, targeted therapies and novel nonsurgical therapeutics are being explored for management of non-resectable tumors, with the aim of avoiding surgery or minimizing surgical scope. However, data on clinical applications of targeted therapies for benign tumors of the jaw remain sparse. Therefore, a literature review was conducted, based on the PubMed database, which included in vivo human clinical studies describing clinical application of targeted therapy for benign tumor of the jaw. The review assessed the outcomes of BRAF and MEK inhibitors for treatment of ameloblastoma, RANKL monoclonal antibody for treatment of giant cell tumor, cherubism, aneurysmal bone cyst, and fibrous dysplasia, and tyrosine kinase inhibitor for treatment of odontogenic myxoma and cherubism. Targeted therapies decreased tumor size, slowed down tumor progression, and reduced bone pain. Surgery remains the gold standard, but targeted therapies are promising adjuvant or alternative treatment options for reducing tumor progression and morbidity of tumor surgery. [ABSTRACT FROM AUTHOR]

Giant Cell Tumor of Bone is a benign tumor with high local aggressive expansion, which, in rare cases, spreads metastasis. Surgical treatment, which often consists of wide curettage to reduce recurrence risk, can lower the quality of life for those affected. Along with aggressive surgery, adjuvant intraoperative techniques have been implemented such as PMMA and cryotherapy. One of the most widely used cryotherapy techniques involves the use of probes to generate ice balls, which have been scientifically shown to have various impacts on the tumor. Although this has been acknowledged, no one has yet tested a way to accurately plan the positioning of cryotherapy probes before surgery, according to the research conducted by the authors. CRIO2AR is a randomized clinical prospective ongoing study by which it will be experimented via preoperative planning of ice probes placement using AR and 3D printing technologies. By studying a single clinical case with these technologies, the surgeon gains better awareness of patient's anatomy and tumor localization. Preliminary results are shown in the article. The first results are confirming that these technologies are applicable in clinical practice. Secondly, preoperative planning is proving to be reliable, easily replicable, and useful for the surgeon. [ABSTRACT FROM AUTHOR]

Symptomatic colon lipoma is a rare occurrence in clinical practice, and its association with sigmoid volvulus is even rarer. We present a case of a man in his 70s who presented to our emergency department with suspected intestinal obstruction. Upon examination, sigmoid volvulus was diagnosed and successfully treated endoscopically through decompression and detorsion. However, the patient experienced a recurrence, leading to the decision to perform sigmoid resection as a Hartmann's procedure. Subsequently, a prolapsed tumor was observed through the stoma, which was endoscopically resected, revealing a pedunculated submucous colonic lipoma. This case report highlights the potential association between sigmoid volvulus and the presence of a large colon lipoma. Thus, giant colonic lipoma should be considered as a differential diagnosis among the causes of colonic volvulus. [ABSTRACT FROM AUTHOR]

CX3CL1 secreted in the tumor microenvironment serves as a chemoattractant playing a critical role in metastasis of CX3CR1 expressing cancer cells. CX3CR1 can be expressed in both cancer and immune-inhibitory myeloid cells to facilitate their migration. We generated a novel monoclonal antibody against mouse CX3CR1 that binds to CX3CR1 and blocks the CX3CL1-CX3CR1 interaction. We next explored the immune evasion strategies implemented by the CX3CL1-CX3CR1 axis and find that it initiates a resistance program in cancer cells that results in 1) facilitation of tumor cell migration, 2) secretion of soluble mediators to generate a pro-metastatic niche, 3) secretion of soluble mediators to attract myeloid populations, and 4) generation of tumor-inflammasome. The CX3CR1 monoclonal antibody reduces migration of tumor cells and decreases secretion of immune suppressive soluble mediators by tumor cells. In combination with anti-PD-1 immunotherapy, this CX3CR1 monoclonal antibody enhances survival in an immunocompetent mouse colon carcinoma model through a decrease in tumor-promoting myeloid populations. Thus, this axis is involved in the mechanisms of resistance to anti-PD-1 immunotherapy and the combination therapy can overcome a portion of the resistance mechanisms to anti-PD-1. [ABSTRACT FROM AUTHOR]

Simple Summary: The purpose of our study was to examine remaining controversies in key issues about the treatment of GCTB. To achieve that we analysed a cohort of 102 patients treated in our institution between 2006 and 2020. We identified the main risk factors for local recurrence, evaluated the recurrence-free survival in dependence on neoadjuvant denosumab use and the type of procedure, and compared the functional outcomes after curettage and en bloc resection. Giant cell tumour of bone (GCTB) is one of the most common local aggressive tumourous lesions with a wide variety of biological behaviour. However, there are no clear indicative criteria when choosing the type of procedure and the complication rates remain high, especially in terms of local recurrence. The purpose of the study was to (1) identify the main risk factors for local recurrence, (2) evaluate the recurrence-free survival in dependence on neoadjuvant denosumab use and the type of procedure, and (3) compare the functional outcomes after curettage and en bloc resection. The group included 102 patients with GCTB treated between 2006 and 2020. The mean age of patients was 34.4 years (15–79). The follow-up period was 8.32 years (2–16) on average. Local recurrence occurred in 14 patients (29.8%) who underwent curettage and in 5 patients (10.6%) after en bloc resection. Curettage was shown to be a factor in increasing recurrence rates (OR = 3.64 [95% CI: 1.19–11.15]; p = 0.023). Tibial location was an independent risk factor for local recurrence regardless of the type of surgery (OR = 3.22 [95% CI: 1.09–9.48]; p = 0.026). The recurrence-free survival rate of patients treated with resection and denosumab was higher compared to other treatments at five years postoperatively (p = 0.0307). Functional ability and pain as reported by patients at the latest follow-up were superior after curettage compared to resection for upper and lower extremity (mean difference: −4.00 [95% CI: –6.81 to −1.18]; p < 0.001 and mean difference: −5.36 [95% CI: −3.74 to −6.97]; p < 0.001, respectively). Proximal tibia tumour location and curettage were shown to be major risk factors for local recurrence in GCTB regardless of neoadjuvant denosumab treatment. The recurrence-free survival rate of patients treated with resection and denosumab was higher compared to other treatments. The functional outcome of patients after curettage was better compared to en bloc resection. [ABSTRACT FROM AUTHOR]

Propose: This study aimed to describe the design and surgical techniques of a three-dimensional (3D) printed strut-type prosthesis with a porous titanium surface for distal femur giant cell tumors of bone (GCTB) and evaluate the short-term clinical outcomes. Methods: From June 2018 to January 2021, 9 consecutive patients with grade I or II GCTB in the distal femur underwent extended intralesional curettage followed by 3D-printed strut-type prosthesis combined with autograft reconstruction were retrospectively reviewed to assess their clinical and radiographic outcomes. Results: All patients were followed up for 30.8 ± 7.5 months (18-42 months) after surgery. The mean affected subchondral bone percentage and the mean subchondral bone thickness before surgery was 31.8% ± 9.6% (range, 18.2% ~50.2%) and 2.2 ± 0.8 mm (range, 1.2-4.0 mm), respectively. At the final followup, all the patients were alive without local recurrence; no postoperative complications were observed. Patients had significant improvements in postoperative MSTS-93 score [(26.7 ± 2.4) vs. (18.8 ± 3.7), P < 0.05], and ROM [(122.8° ± 9.1°) vs. (108.3° ± 6.1°), P < 0.05] compared with their preoperative statuses. Furthermore, the mean subchondral bone thickness has increased to 10.9 ± 1.3 mm (range, 9.1-12.1 mm). Conclusion: 3D-printed strut-type prosthesis combined with autograft reconstruction provides acceptable early functional and radiographic outcomes in patients with grade I or II GCTB in distal femur due to the advantages of the prosthesis such as good biocompatibility, osseointegration capacity, and subchondral bone protection. If our early outcomes can be further validated in studies with more patients and sufficient follow-up, this method may be evaluated as an alternative for the treatment of grade I or II GCTB in the distal femur. [ABSTRACT FROM AUTHOR]