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Abstract This correspondence addresses the article published by Nie et al. titled “Primary extraskeletal osteosarcoma of sigmoid mesocolon: a case report and a review of the literature”. Their report highlighted an extremely rare case of extraskeletal osteosarcoma (EO) in the sigmoid mesocolon that was diagnosed through imaging and histopathological findings. Diagnosing EO has certain challenges; one of them being the lack of characteristic image findings of EO and the other being the lack of appropriate immunohistochemical (IHC) markers in the histopathological findings. Recently, special AT-rich sequence-binding protein 2 (SATB2) has been proposed as an IHC marker for osteoblastic differentiation; however, it has low specificity. Some cases of EO may show findings such as mouse double minute protein 2 expression and deletion of histone H3 lysine 27 trimethylation (H3K27me3), which are similar to those of other soft tissue sarcomas. Therefore, it is essential to consider other soft tissue sarcomas, especially dedifferentiated liposarcoma, before the accurate diagnosis of EO.

Abstract Although stereotactic body radiotherapy (SBRT) is a curative treatment option for stage I non‐small cell lung cancer (NSCLC), limited data are available regarding chest wall (CW) toxicities during an extended follow‐up of over 10 years. We report an unusual case of a bone tumor‐like CW mass lesion with pathological rib fractures observed 13 years after SBRT for peripheral lung cancer. Despite the initial suspicion of radiation‐induced sarcoma, a subsequent incisional biopsy revealed no evidence of malignancy, and a definitive diagnosis of osteonecrosis was made. Thus, long‐term observation of over 10 years is required to identify late chronic complications following SBRT.

Dedifferentiated liposarcoma is a malignant lipomatous tumor that rarely occurs in the gastrointestinal tract, including the ileocecal region. In this case, computed tomography and magnetic resonance imaging showed no fatty mass located in the mesenteric or submucosal lesion, and positron emission tomography–computed tomography showed a high maximum standardized uptake value, collectively indicating the gastrointestinal stroma tumor and lymphoma. The pathological findings resemble leiomyosarcoma; the immunohistochemistry findings including mouse double minute 2 homolog and cyclin D-dependent kinase-4 and amplification of mouse double minute 2 homolog in fluorescence in situ hybridization just favored the diagnosis of dedifferentiated liposarcoma with leiomyosarcoma phenotype and not leiomyosarcoma. Recently, a new inhibitor for mouse double minute 2 homolog and cyclin D-dependent kinase-4 has been developed, and clinical trials for dedifferentiated liposarcoma are currently ongoing. This could change the treatment strategy drastically compared with other soft tissue sarcomas. Hence, a correct diagnosis of dedifferentiated liposarcoma is required.

Myofibroblastomas are benign mesenchymal tumors that frequently occur in the groin. They show variable morphology, and the differential histopathological diagnoses are broad, including lipomatous to myxoid tumors. In addition, both pathological and imaging findings may be complex, which makes diagnosis challenging. We herein present a case of a mixed-variant myofibroblastoma of the wrist in a 73-year-old woman. Considering the long clinical course of more than 20 years and the imaging findings, a benign myxoid tumor including a schwannoma was suspected; however, the histopathological findings from resected specimens suggested a diagnosis of myxofibrosarcoma. Additional histopathological findings led to a diagnosis of mixed-variant myofibroblastoma. The differential diagnosis of myofibroblastoma extends beyond imaging to pathological findings because of the number of possible variants. This case reinforces the notion that the gold standard treatment for soft tissue tumors is to perform surgery only after determining the correct diagnosis by biopsy.

This report describes a rare case of a pediatric tenosynovial giant cell tumor (TSGCT) with a delayed diagnosis. A 9-year-old boy presented with a 3-month history of knee pain and swelling, initially attributed to a femoral non-ossifying fibroma and arthritis based on computed tomography findings and slightly elevated C-reactive protein levels. The symptoms persisted despite medical treatment. Magnetic resonance imaging (MRI) revealed a tumor in the posterior compartment. He underwent surgery, and the pathology confirmed the diagnosis of localized TSGCT. Six months postoperatively, the patient remained asymptomatic. Pediatric knee pain is a complex symptom associated with inflammatory conditions and benign and malignant tumors. Benign tumors, as in this case, can be misdiagnosed as arthritis, delaying diagnosis and treatment. MRI is recommended in cases involving symptom persistence. However, histopathological, immunohistochemical, and morphological examinations are crucial for definitive diagnosis, particularly when the imaging findings are inconclusive.

Angiomatoid fibrous histiocytoma (AFH) is a rare intermediate tumor that is often difficult to diagnose radiologically and pathologically. Herein, we report a case of AFH in the knee that was initially misdiagnosed as a cystic lesion. The tumor was first identified eight years earlier during the patient’s initial visit, when plain magnetic resonance imaging (MRI) was performed, leading to a diagnosis of a cystic lesion. At the current visit, the tumor had subsequently enlarged, and pain had appeared. Contrast-enhanced MRI was performed at our hospital, revealing enhancement suggestive of a solid tumor. A needle biopsy was performed, raising suspicion of AFH. Wide resection was performed, and AFH was diagnosed using histopathological findings and fluorescence in situ hybridization (FISH). Although there are several characteristic imaging findings of AFH, they are non-specific, and small tumors can be easily overlooked. Furthermore, histopathological findings lack specific immunohistochemical markers, making morphological appearance, combined with FISH findings recently reported as useful, important for preventing misdiagnosis. Since cystic lesions can occur in various locations besides the knee, it is recommended to perform contrast-enhanced MRI for accurate diagnosis when there is an increase in size or the appearance of symptoms, as plain MRI alone may lead to misdiagnosis.

Purpose: This prospective multicenter study investigated the implant fixation of a cementless cup in primary total hip arthroplasty (THA) for osteoarthritis (OA) secondary to developmental dysplasia of the hip (DDH) in Japanese patients. Methods: Ten hospitals in Japan were enrolled in this study. The cohort comprised 267 hips in 228 Japanese patients who underwent primary THA for OA secondary to DDH. An acetabular cup with advanced in-growth, GRIPTION™ coating, was used in all patients. Mean age at surgery was 66.9 (range 45–89) years, and 201 patients (239 hips) were female. Of these patients, 89.1 ​% (238/267) was Crowe group I. Radiolucent lines around the cup, osteolysis, cup migration, grafted bone status, and clinical scores were evaluated preoperatively and at 6 months, 1 year, and 2 years postoperatively. Adverse events were evaluated throughout the study. Implant survivorship was assessed using the Kaplan–Meier method. Results: Nine hips had radiolucent lines ≥2 ​mm that resolved by 6 months. All cups showed bone in-growth and no cups loosened. At 2 years, the bone grafts had remodeled and incorporated in 68 ​% (43/63) of hips. There were four systemic and five surgical site events, and one procedure-related dislocation. The survivorship with cup revision as the endpoint was 99.6 ​% (95 ​% confidence interval, 97.3–99.9). All clinical scores were improved at 2 years compared with preoperatively (p ​

Abstract The prognosis of osteosarcoma (OS) has remained stagnant over the past two decades, requiring the exploration of new therapeutic targets. Cytokines, arising from tumor-associated macrophages (TAMs), a major component of the tumor microenvironment (TME), have garnered attention owing to their impact on tumor growth, invasion, metastasis, and resistance to chemotherapy. Nonetheless, the precise functional role of TAMs in OS progression requires further investigation. In this study, we investigated the interaction between OS and TAMs, as well as the contribution of TAM-produced cytokines to OS advancement. TAMs were observed to be more prevalent in lung metastases compared with that in primary tumors, suggesting their potential support for OS progression. To simulate the TME, OS and TAMs were co-cultured, and the cytokines resulting from this co-culture could stimulate OS proliferation, migration, and invasion. A detailed investigation of cytokines in the co-culture conditioned medium (CM) revealed a substantial increase in IL-8, establishing it as a pivotal cytokine in the process of enhancing OS proliferation, migration, and invasion through the focal adhesion kinase (FAK) pathway. In an in vivo model, co-culture CM promoted OS proliferation and lung metastasis, effects that were mitigated by anti-IL-8 antibodies. Collectively, IL-8, generated within the TME formed by OS and TAMs, accelerates OS proliferation and metastasis via the FAK pathway, thereby positioning IL-8 as a potential novel therapeutic target in OS.

Primary malignant cardiac tumors rarely occur, and cardiac synovial sarcoma (SS) is especially rare among such tumors. Herein, we present the case of a 35-year-old female with primary cardiac SS treated with surgery, chemotherapy, and radiotherapy. She presented with chest symptoms and underwent imaging examinations. A cardiac tumor was suspected, and an open biopsy was performed. The pathological findings suggested cardiac SS. Next, we performed a resection, and the tumors persisted at a macroscopic level. Immunohistochemistry was negative for SS18-SSX and positive for the SSX C-terminus and cytokeratin CAM5.2, a reduction of SMARCB1/INI1 was observed, and fluorescence in situ hybridization showed positive SS18 split staining. Owing to the FNCLCC grade 3 tumor and R2 margins, adjuvant chemotherapy with ifosfamide, doxorubicin, and radiotherapy was initiated, and the patient was diagnosed with cardiac SS. The differences in patients with cardiac SS compared with general SS include male predominance, larger tumor size, and poorer prognosis. Pathological findings of immunohistochemistry and fluorescence in situ hybridization were found to be more reliable than imaging findings for a correct diagnosis. Additionally, because incomplete resection is frequently performed, adjuvant therapy, including chemotherapy and radiation therapy, may be performed. The findings indicate that multiple therapies, including surgery, chemotherapy, and radiotherapy, are essential treatment strategies for improving the prognosis of patients with cardiac SS.

Angiomatoid fibrous histiocytoma (AFH) is a rare tumor of mesenchymal origin occurring in young adults. Based on its clinical course, it is said to have an intermediate potential. We present a case of a 59-year-old woman with AFH in the hand that was difficult to diagnose. A benign soft tissue tumor was suspected on magnetic resonance imaging, and its size and open biopsy suggested nodular fasciitis or inflammatory myofibroblastic tumor. A diagnosis of AFH was eventually made based on the analysis of the resected specimens. The characteristic findings of histopathology and immunohistochemistry in this case were relatively poor, so fluorescence in situ hybridization contributed to making the correct diagnosis. Considering its prognosis, careful follow-up was decided upon without additional surgery. Our case is a challenging one because of its atypical presentation and inconclusive imaging and histopathological findings.

With great interest, we read the article by Manole et al [...]

Abstract Background Herein, for the first time, we present a case with mixed invasive micropapillary and neuroendocrine mammary neoplasm. Case The patient, a 65‐year‐old postmenopausal woman, had become aware of a tumor in her right breast 11 months prior to presentation at our hospital. The cut surface of the mastectomy specimen contained a well‐circumscribed, multinodular, red‐brown tumor, measuring 15x15x15 cm. Histopathologically, this solid cystic lesion consisted of medullary growth of cancer cells accompanied by a well‐developed vascular network as well as conspicuous hemorrhage. Cancer cell nests of various sizes displayed an “inside‐out” structure surrounded by empty spaces. Most cancer cells were polygonal, though a few were short fusiform‐shaped, and possessed finely granular, eosinophilic cytoplasm and ovoid, fine‐granular nuclei. Eighteen mitotic figures were observed in 10 high‐power fields. Macrometastases, up to 13x8 mm in size, with the same morphological features as the original tumor site, were identified in 3 of 15 dissected right axillary nodes. Immunohistochemically, primary and metastatic cancer cells were diffusely positive for chromogranin A and the estrogen receptor (Allred's total score: 8) and focally reactive for synaptophysin and the progesterone receptor (total score: 5). HER2 and cytokeratin 5/6 were negative, and the MIB‐1 labelling index was 36.2%. MUC1 and EMA lined the stroma‐facing surfaces of the cell membranes, indicating reversed polarity. Conclusion Our current patient, who had an invasive breast carcinoma with concomitant neuroendocrine and micropapillary features, developed multiple nodal metastases in association with a large‐diameter tumor showing a luminal B‐like immuno‐profile. Accordingly, meticulous clinical follow‐up remains essential for this uncommon case.

Extraskeletal osteosarcoma (EO) is a soft tissue sarcoma characterized by the production of bone matrix by neoplastic cells. Benign osteoid in EO, leading to a diagnostic dilemma, is rarely encountered. Herein, for the first time, we present a case with cytogenetically confirmed EO combined with or preceding myositis ossificans (MO). A 21-year-old man had a mildly painful swelling in his left knee. Imaging studies demonstrated a 39-mm mass with peripheral mineralization and cystic change on the posterolateral side of the left fibular head. He was clinically suspected of having either MO or a malignancy, such that wide resection was performed. Macroscopically, the mass was grayish to brown. In the cut section, multiple cystic lesions in addition to solid components were noted. Histopathologically, the solid components demonstrated diffuse proliferation of pleomorphic tumor cells with osteoclast-like giant cells. The malignant tumor cells formed osteoid. In the periphery, the mass was benign, showing mature bone tissue and focally non-malignant woven bone with fibroblasts, compatible with zonation. Fluorescence in situ hybridization (FISH) demonstrated split signals of the USP6 gene. These findings suggested EO with preceding MO. Although the pathogenesis remains to be elucidated, the observed USP6 rearrangement might contribute to both the diagnosis of EO with preceding MO and an understanding of the underlying histopathology.

Atypical spindle cell/pleomorphic lipomatous tumors (ASPLTs) were recently categorized as benign lipomatous tumors. However, accurate and complete preoperative diagnosis of ASPLTs may be difficult. Furthermore, diagnosis based on magnetic resonance imaging (MRI) findings is uncertain because of the varying ratios of the fat component within the tumor. Here, we report a case of ASPLT masquerading as a myxoid tumor. Although MRI findings were consistent with a myxoid liposarcoma, needle biopsy findings suggested a myxoma, and we performed marginal resection. Histopathological findings revealed infiltrating spindle cells with atypia. In addition, immunohistochemistry (IHC) showed positive staining for CD34 and heterogeneous retinoblastoma deficiency, and fluorescence in situ hybridization (FISH) showed no amplification of mouse double minute 2 homolog and no rearrangement of FUS or EWSR1. When MRI and histopathological findings suggest a myxoid tumor, IHC and FISH should be considered and performed for a precise and accurate diagnosis.

Abstract ETS homologous factor (EHF) belongs to the epithelium-specific subfamily of the E26 transformation-specific (ETS) transcription factor family. Currently, little is known about EHF’s function in cancer. We previously reported that ETS1 induces expression of the ZEB family proteins ZEB1/δEF1 and ZEB2/SIP1, which are key regulators of the epithelial–mesenchymal transition (EMT), by activating the ZEB1 promoters. We have found that EHF gene produces two transcript variants, namely a long form variant that includes exon 1 (EHF-LF) and a short form variant that excludes exon 1 (EHF-SF). Only EHF-SF abrogates ETS1-mediated activation of the ZEB1 promoter by promoting degradation of ETS1 proteins, thereby inhibiting the EMT phenotypes of cancer cells. Most importantly, we identified a novel point mutation within the conserved ETS domain of EHF, and found that EHF mutations abolish its original function while causing the EHF protein to act as a potential dominant negative, thereby enhancing metastasis in vivo. Therefore, we suggest that EHF acts as an anti-EMT factor by inhibiting the expression of ZEBs, and that EHF mutations exacerbate cancer progression.

Non-thermal atmospheric pressure plasma (NTAPP)-activated liquids have emerged as new promising anticancer agents because they preferentially injure malignant cells. Here, we report plasma-activated infusion (PAI) as a novel NTAPP-based anti-neoplastic agent. PAI was prepared by irradiating helium NTAP to form a clinically approved infusion fluid. PAI dose-dependently killed malignant melanoma and osteosarcoma cell lines while showing much lower cytotoxic effects on dermal and lung fibroblasts. We found that PAI and salinomycin (Sal), an emerging anticancer stem cell agent, mutually operated as adjuvants. The combined administration of PAI and Sal was much more effective than single-agent application in reducing the growth and lung metastasis of osteosarcoma allografts with minimal adverse effects. Mechanistically, PAI explicitly induced necroptosis and increased the phosphorylation of receptor-interacting protein 1/3 rapidly and transiently. PAI also suppressed the ambient autophagic flux by activating the mammalian target of the rapamycin pathway. PAI increased the phosphorylation of Raptor, Rictor, and p70-S6 kinase, along with decreased LC3-I/II expression. In contrast, Sal promoted autophagy. Moreover, Sal exacerbated the mitochondrial network collapse caused by PAI, resulting in aberrant clustering of fragmented mitochondrial in a tumor-specific manner. Our findings suggest that combined administration of PAI and Sal is a promising approach for treating these apoptosis-resistant cancers.

Calcific tendinitis of the supraspinatus tendon in adults is common, but it is extremely rare in children. This report presents an unusual case of a 2-year-old boy with calcific tendinitis of the supraspinatus tendon. A mother brought her 2-year-old son to our hospital with a fever and severe left shoulder pain. Examination revealed a temperature of 38.6°C accompanied by a swollen shoulder with extreme pain and restricted movement. The radiographs of his left shoulder showed a large radio-opacity in the subacrominal region, and magnetic resonance imaging showed an elongated T1 and T2 hypointense signal above the supraspinatus tendon. Although these images were suggestive of calcific tendinitis of the supraspinatus tendon, we performed an open biopsy and resection in order to differentiate between a suspected diagnosis of calcific tendinitis, which is incredibly rare within pediatric patients, and infection or a soft tissue tumor. Finally, calcific tendinitis of the supraspinatus tendon was diagnosed by pathologic experiment and successfully treated, with complete resolution of pain and movement. Because only four other pediatric cases of calcific tendinitis of the supraspinatus tendon have ever been reported, there is a lack of information on the diagnostic process, management, and treatment of such a condition in young patients. Calcific tendinitis of the supraspinatus tendon still should be considered when encountering cases with typical findings even if the patient is a child.

Case. Generally, well-differentiated liposarcoma (WDL) has recurrence potential but lacks metastatic potential. We present a rare case of spinal metastasis of WDL component in retroperitoneal dedifferentiated liposarcoma (DDL) treated by tumor curettage and L1 laminectomy followed by percutaneous pedicle screw fixation. Histological examination showed metastasis of the WDL component of DDL. The patient was ambulatory until death. Conclusion. To our knowledge, no case of spinal metastasis of WDL component in retroperitoneal DDL has been reported. We should carefully consider characteristics of DDLs during treatment. Minimally invasive surgery may be a powerful tool in patients with spinal metastasis.

Radiographic imaging plays a crucial role in the diagnosis of osteosarcoma. Currently, computed-tomography (CT) is used to measure tumor-induced osteolysis as a marker for tumor growth by monitoring the bone fractional volume. As most tumors primarily induce osteolysis, lower bone fractional volume has been found to correlate with tumor aggressiveness. However, osteosarcoma is an exception as it induces osteolysis and produces mineralized osteoid simultaneously. Given that competent bone is highly anisotropic (systematic variance in its architectural order renders its physical properties dependent on direction of load) and that tumor induced osteolysis and osteogenesis are structurally disorganized relative to competent bone, we hypothesized that μCT-derived measures of anisotropy could be used to qualitatively and quantitatively detect osteosarcoma provoked deviations in bone, both osteolysis and osteogenesis, in vivo. We tested this hypothesis in a murine model of osteosarcoma cells orthotopically injected into the tibia. We demonstrate that, in addition to bone fractional volume, μCT-derived measure of anisotropy is a complete and accurate method to monitor osteosarcoma-induced osteolysis. Additionally, we found that unlike bone fractional volume, anisotropy could also detect tumor-induced osteogenesis. These findings suggest that monitoring tumor-induced changes in the structural property isotropy of the invaded bone may represent a novel means of diagnosing primary and metastatic bone tumors.

Case: Traumatic pediatric Achilles ruptures are rare, and few cases have been reported among patients with a history of Achilles tenotomy. A 17-year-old boy with a history of Achilles tenotomies for congenital clubfoot as an infant presented with acute midsubstance Achilles tendon rupture of the right leg. The gastrocnemius fascia was dissected to allow for approximation of the ruptured Achilles tendon segments. Furthermore, the plantaris tendon was harvested and sutured around the Achilles tendon-rupture site to improve strength. Conclusions: Patients with a history of clubfoot and chronic Achilles tendon pain should be alerted to the risk of Achilles tendon rupture. [ABSTRACT FROM AUTHOR]

Objectives: To clarify the longitudinal changes in patients with preoperative Stage-3 locomotive syndrome LS) according to different types of surgeries, we investigated the changes in the LS stage in patients who underwent surgery for degenerative musculoskeletal diseases. Methods: A prospective cohort study was conducted on 168 patients with degenerative diseases [46 spinal deformities treated with thoracolumbar interbody fusion (T/LIF), 86 hips with osteoarthritis treated with total hip arthroplasty (THA), and 36 knees with osteoarthritis treated with total knee arthroplasty (TKA)]. The results for the LS stage, stand-up test, two- step test, and 25-question Geriatric Locomotive Function Scale (GLFS-25) were evaluated preoperatively and at 6 months, 1 year, and 2 years postoperatively. Results: Preoperatively, most patients had Stage-3 LS (89.1, 90.8, and 80.6% in the T/LIF, THA, and TKA groups, respectively). At 2 years postoperatively, the Stage-3 LS improved in 41.5, 75.6, and 55.2% of patients in the T/LIF, THA, and TKA groups, respectively. All groups showed similar improvements in the two-step test. The THA group showed the best result in the GLFS-25. Conclusions: LS stage improved in different patterns over 2 years postoperatively and the LS risk test revealed differences in postoperative movement ability according to the type of surgery. [ABSTRACT FROM AUTHOR]