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A 6-month-old intact male Dalmatian mix puppy was presented for the evaluation of left maxillary swelling due to a suspected cyst and an unerupted left maxillary canine tooth. Removal of the unerupted left maxillary canine tooth (204) and enucleation of the cyst was performed, followed by histological analysis, which identified the maxillary swelling to be a cystic ameloblastic fibroma. Ameloblastic fibromas are rare in companion animals, and to the best of the authors' knowledge, this is the first cystic variant reported in dogs. The clinical, radiographic, cone beam computed tomography, and histological findings of this case are discussed and compared with the findings of previously documented human and domestic animal cases., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Tumours that metastasize to the ovary can occur in conjunction with other ovarian lesions, including benign sex cord stroma tumours like fibroma or fibrothecoma. This case report presents a unique instance of metastatic signet ring carcinoma involving the ovary in a background of fibroma in a Black African woman., Patient Presentation: A 46-year-old gravida 3, para 0 (2 alive), patient was referred from the general outpatient clinic to the gynecology clinic due to progressive abdominal swelling over the past eight months. Abdominal examination revealed marked distension with massive ascites. Physical examination of the chest demonstrated dullness to percussion over both lung bases, with increased dullness noted on the right. Auscultation revealed decreased air entry in the right middle and lower lung zones, with normal to increased air entry in the remaining lung fields. Abdominopelvic ultrasound revealed a large irregularly marginated homogeneous solid mass in the right adnexa measuring 16.4 × 11.7 × 12.7 cm. An abdominal CT scan revealed bilateral pleural effusion, which was more pronounced on the right, ascites, and evidence of pulmonary and hepatic metastasis. Serum chemistry revealed abnormal levels of several analytes, including elevated CA 125 at 1,108.8 (normal range 0-35) U/L and CA 19-9 at 63.8 (normal range 0-35) U/L. She subsequently underwent staging laparotomy with total abdominal hysterectomy and bilateral salpingo-oophorectomy, without any postoperative complications. The histologic sections of the right and left ovaries revealed a moderately cellular lesion composed of intersecting bundles of spindle cells in a fascicular and storiform pattern. Additionally, pockets of small round to oval-shaped cells with intracytoplasmic clear vacuoles pushing the nucleus to the periphery (signet ring cells) were identified in a few foci. These cells were initially thought to be ovarian stroma or theca cells. Microscopic examination revealed signet ring cells with cytoplasmic positivity for periodic acid-Schiff (PAS) staining. The histopathological diagnosis was metastatic signet ring cell carcinoma involving the ovary, with an underlying ovarian fibroma., Conclusions: Ovarian metastatic signet ring carcinoma in a background of fibroma can pose a significant diagnostic challenge, as signet ring cells can mimic the ovarian stroma or theca cells, especially if they are only observed in a few foci of the sections., (© 2024. The Author(s).)

Family physicians are well-positioned to provide injections for patients who have pain due to hand and finger conditions, especially when initial treatments such as splinting and nonsteroidal anti-inflammatory drugs are ineffective. Corticosteroid injections can offer pain relief; however, potential risks such as infection, cartilage damage, and skin depigmentation should be discussed. Techniques and procedures for injections vary. Corticosteroid injections for ste-nosing flexor tenosynovitis (trigger finger) can be performed with or without ultrasound guidance. To maximize benefits of corticosteroid injection for carpometacarpal joint osteoarthritis, topical nonsteroidal anti-inflammatory drugs and other conservative treatment modalities should be used concurrently. Because of the risks of disease recurrence and adverse effects, corticosteroid injections for palmar fibromatosis should be approached with caution in the context of shared decision-making.

Abstract: Dermatofibrosarcoma protuberans (DFSP) is a neoplasm of intermediate malignancy with high local recurrence rates. The sclerosing variant is characterized by the presence of sclerotic areas in more than 50% of tumors and is rarely reported. In this report, we describe a case of sclerosing DFSP with areas histopathologically resembling sclerotic fibroma, where the initial biopsy tissue presented a diagnostic challenge. A 77-year-old man presented with a 2-cm firm, erythematous nodule on the chest. A punch biopsy revealed plywood-like sclerosis and spindle cells with a vaguely storiform pattern. The tumor cells were positive for CD34. Sclerotic fibroma and DFSP were considered differential diagnoses. Subsequent excisional biopsy revealed that the tumor comprised 3 different histopathological areas: classic DFSP, sclerotic fibroma-like, and giant cell fibroblastoma-like. This report highlights the importance of reevaluating the clinical context and excision for further characterization., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Ovarian sex cord-stromal tumours (SCSTs) present diagnostic difficulties during frozen section (FS) consultations due to their diverse morphology. This study aimed to evaluate the accuracy of FS evaluation of SCSTs in our institution, as well as to examine the reasons leading to incorrect FS diagnosis. Cases mimicking SCSTs and diagnosed as such during FS were also highlighted. We analysed 121 ovarian SCST cases and their mimics which underwent FS consultations over a 10-year period, to evaluate FS accuracy, reasons for deferrals and discrepancies. FS diagnoses were concordant, deferred and discrepant compared to the final diagnosis in 50 (41.3%), 39 (32.2%) and 32 (26.5%) cases, respectively. Major discrepancies (9/121, 7.4%) were mostly related to the diagnosis of adult granulosa cell tumour (AGCT). A fibromatous AGCT was misinterpreted as fibroma on FS, while a cystic AGCT was called a benign cyst. Conversely, a mesonephric-like adenocarcinoma, a sertoliform endometrioid carcinoma and a thecoma were misinterpreted as AGCT on FS. Another discrepant case was a Krukenberg tumour with prominent fibromatous stroma in which malignant signet ring cells were overlooked and misinterpreted as fibroma. Minor discrepancies were primarily associated with fibroma (21/23, 91.3%), wherein minor but potentially impactful details such as cellular fibroma and mitotically active cellular fibroma were missed due to sampling issues and misinterpretation as leiomyoma. FS evaluation for ovarian SCSTs demonstrated an overall accuracy of 78.5%, 81.0% and 81.8% for benign, uncertain/low malignant potential and malignant categories, respectively. There was no FS-related adverse clinical impact in all cases with available follow-up information (120/121 cases). Intraoperative FS evaluation of ovarian SCSTs is challenging. A small number of cases were misinterpreted, with AGCTs being the primary group where errors occur. Awareness of common diagnostic pitfalls and difficulties, alongside application of a stepwise approach, including (1) obtaining comprehensive clinical information, (2) thorough macroscopic examination and directed sampling, (3) meticulous microscopic examination with consideration of pitfalls and mimics, (4) effective communication with surgeons in difficult cases, and (5) consultation of subspecialty colleagues in challenging cases, will enhance pathologists' reporting accuracy and management of such cases in the future., (Copyright © 2024 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.)

A wide spectrum of tumors may affect the periungual spaces of the digits. Superficial acral fibromyxoma (SAF) is a rare, benign soft tissue tumor with diverse clinical presentations. We present a case of a 55-year-old woman with a 2-year history of a solitary periungual tumor on the left thumb, subjected to multiple episodes of trauma. Initially suspected to be a periungual squamous cell carcinoma (SCC) based on clinical and dermoscopic features, the tumor was confirmed to be a CD34 - SAF through histopathology and immunohistochemistry. Although CD34 immunoreactivity is common in SAF, one-third of these tumors, including this case, do not stain for this marker. Periungual SCC considered a "great mimicker of nail tumors," may resemble other benign nail tumors such as SAF. The patient underwent complete surgical excision with primary closure, resulting in no recurrence after 1 year. This case highlights SAF as an underrecognized benign entity that may manifest with features suspicious of malignancy, potentially leading to unnecessarily aggressive interventions. Recognizing SAF through accurate biopsy techniques and thorough histopathologic evaluation, even in the absence of CD34 reactivity, is crucial for appropriate treatment and preservation of hand function and appearance., (© 2024 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Sclerotic fibroma (SF) is an uncommon yet benign tumor that may occasionally be associated with Cowden's disease that presents as an asymptomatic, well-circumscribed lesion. We present a rare case of a patient with a solitary SF of the palpebral conjunctiva. The patient was an 85-year-old male who presented with a right lower lid nodule that was initially treated as a chalazion. Excision yielded a dense mass that was sent to pathology for evaluation. Histologically, the lesion showed a bland storiform spindle cell proliferation embedded in a sclerotic stroma with prominent clefting.

Aims: Chondromyxoid fibroma (CMF) is a rare, benign bone tumour which arises primarily in young adults and is occasionally diagnostically challenging. Glutamate metabotropic receptor 1 (GRM1) gene encodes a metabotropic glutamate receptor and was recently shown to be up-regulated in chondromyxoid fibroma through gene fusion and promoter swapping. The aim of this study was to interrogate cases of CMF for the presence of GRM1 gene rearrangements, gene fusions and GRM1 protein overexpression., Methods and Results: Selected cases were subjected to testing by fluorescent in-situ hybridisation (FISH) with a GRM1 break-apart probe, a targeted RNA sequencing method and immunohistochemical study with an antibody to GRM1 protein. Two cases were subjected to whole transcriptomic sequencing. In 13 of 13 cases, GRM1 protein overexpression was detected by immunohistochemistry using the GRM1 antibody. Of the 12 cases successfully tested by FISH, nine of 12 showed GRM1 rearrangements by break-apart probe assay. Targeted RNA sequencing analysis did not detect gene fusions in any of the eight cases tested, but there was an increase in GRM1 mRNA expression in all eight cases. Two cases subjected to whole transcriptomic sequencing (WTS) showed elevated GRM1 expression and no gene fusions., Conclusion: GRM1 gene rearrangements can be detected using FISH break-apart probes in approximately 75% of cases, and immunohistochemical detection of GRM1 protein over-expression is a sensitive diagnostic method. The gene fusion was not detected by targeted RNA sequencing, due most probably to the complexity of fusion mechanism, and is not yet a reliable method for confirming a diagnosis of CMF in the clinical setting., (© 2024 John Wiley & Sons Ltd.)

The clinical, radiological, and histopathological features of chondromyxoid fibroma can sometimes resemble those of other benign or malignant tumors. Recently, recurrent GRM1 rearrangements have been identified in chondromyxoid fibroma, and GRM1 positivity by immunohistochemistry has emerged as a dependable surrogate marker for this molecular alteration. Phosphaturic mesenchymal tumor is a rare tumor that often exhibits overexpression of fibroblastic growth factor 23 (FGF23) through various mechanisms. In this report, we present a case of GRM1 -rearranged chondromyxoid fibroma that also exhibited FGF23 expression via in situ hybridization, posing significant diagnostic challenges during workup of the initial core biopsy. We hope that this case can serve as an educational resource, shedding light on a rare diagnostic pitfall., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: The aim of this study was to elucidate the histogenesis and genetic underpinnings of fibromatosis-like undifferentiated gastric carcinoma (FLUGC), a rare pathological entity., Method: Through a detailed analysis of seven cases, including histopathological evaluation, CTNNB1 gene mutation screening, human epidermal growth factor receptor 2 (HER2) protein level quantification, and HER2 gene amplification assessment to identify the pathological and molecular characteristics of FLUGC., Results: Of the seven patients in this study, five were male and two were female (age: 39-73 years). Four patients presented with lesions in the gastric antrum and three had lesions in the lateral curvature of the stomach. Histopathologically, over 90% of the tumor consisted of aggressive fibromatosis-like tissue, including proliferating spindle fibroblasts and myofibroblasts and varying amounts of collagenous fibrous tissues. Undifferentiated cancer cells, accounting for less than 10%, were dispersed among the aggressive fibromatosis-like tissues. These cells were characterized by their small size and were relatively sparse without glandular ducts or nested mass-like structures. Immunophenotyping results showed positive expression of CKpan, CDX2, villin, and p53 in undifferentiated cancer cells; positive expression of vimentin in aggressive fibromatosis-like tissue; positive cytoplasmic expression of β-catenin; and focal cytoplasmic positive expression of smooth muscle actin (SMA). Genetic analysis did not reveal any mutations in the CTNNB1 gene test, nor was there amplification in the HER2 gene fluorescence in situ hybridization (FISH) test. Additionally, the Epstein-Barr encoding region (EBER) of in situ hybridization was negative; and the mismatch repair (MMR) protein was positive. Programmed cell death-1 (PD-1) was < 1-5%; programmed cell death ligand 1 (PD-L1): TPS = 1-4%, CPS = 3-8., Conclusion: The study highlights the significance of CTNNB1, HER2, EBER, and MMR as pivotal genetic markers in FLUGC, underscoring their relevance for diagnosis and clinical management. The rarity and distinct pathological features of FLUGC emphasize the importance of accurate diagnosis to prevent underdiagnosis or misdiagnosis and to raise awareness within the medical community., (© 2024. The Author(s).)

Background: Jaffe-Campanacci syndrome is a rare syndrome, characterized by multiple non-ossifying fibromas (NOF) and cafe-au-lait patches. The name was coined in 1982 by Mirra after Jaffe who first described the case in 1958. Although it's suggested there is a relation with Neurofibromatosis type 1, there is still no consensus on whether Jaffe-Campanacci syndrome is a subtype or variant of neurofibromatosis-1(NF-1)., Case Presentation: In this article, we present a case series of 2 patients. The first case is a 13-year-old male with Jaffe-Campanacci syndrome who presented with a distal femur fracture. His father had positive features of both Jaffe-Campanacci syndrome and NF-1, while his sister only had features of NF-1, so we presented both., Conclusion: Jaffe-Campanacci has a clear relationship with type 1 neurofibromatosis, which still has to be genetically established. Due to the presence of several large non-ossifying fibromas of the long bones, it is linked to a significant risk of pathological fractures. We concur with previous authors, that an osseous screening program should be performed for all patients with newly diagnosed type 1 neurofibromatosis, to identify non-ossifying fibromas and assess the potential for pathological fracture. Moreover, siblings of patients with NF-1 should be screened for multiple NOFs that may carry a high risk of pathological fractures., (© 2024. The Author(s).)

Objective: Pediatric cardiac tumors are rare and, among them, 90% are benign. Cardiac fibroma is the second most frequent tumor, after rhabdomyoma. The objective of this study is to report a case of cardiac fibroma diagnosed incidentally in a patient admitted with acute viral bronchiolitis., Case Description: A 5-month-old male infant was admitted to the pediatric emergency department with acute viral bronchiolitis requiring hospitalization. He presented a detectable respiratory syncytial virus in oropharyngeal swab, blood test with lymphocytosis and a chest radiography revealed cardiomegaly. Further cardiologic testing was performed detecting elevation of cardiac biomarkers, an electrocardiogram with alteration of left ventricular repolarization and echocardiogram with a heterogeneous mass in the left ventricular, with areas of calcification. A chest angiotomography suggested rhabdomyosarcoma or cardiac fibroma and a magnetic resonance showed a mass, with characteristics suggesting fibroma. The final diagnosis was made after two cardiac catheterizations for biopsy of the lesion, confirming cardiac fibroma by anatomopathological examination. Because the patient had moderate to severe systolic dysfunction, he was submitted to heart transplant., Comments: One third of cardiac fibromas are asymptomatic, generally diagnosed late through tests ordered for other reasons. The gold-standard test for definitive diagnosis is biopsy. Cardiac fibroma usually does not present spontaneous regression and, in most cases, partial or total surgical resection is necessary. When tumors are unresectable, heart transplantation should be indicated. It is essential to have detailed characterization of the cardiac mass to establish the most appropriate therapeutic approach for each patient.

Competing Interests: The author declares no conflicts of interest.

A 35-year-old patient presented with a painless, broad-based exophytic lesion in the buccal interdental region between teeth 13 and 14. Despite oral hygiene efforts the lesion persisted for around one year. Radiology excluded bone involvement, and histopathology after excision confirmed a fibromatous epulis, which is characterized by collagen-rich connective tissue. There was no recurrence within one-year follow-up. Surgical removal proved to be efficient., (Copyright 2024 SWISS DENTAL JOURNAL SSO – Science and Clinical Topics. License: This work is licensed under a Creative Commons Attribution 4.0 International License.)

Left ventricular tumour is a rare condition in children. The causes include vegetations, thrombus, and fibroma. 2-year-old asymptomatic female presented with an innocent heart murmur at 6 months of age. Subsequent follow-ups at 18 months of age showed left ventricular mass. Surgical pathology revealed "nodular fasciitis." This type of tumour has never been described in the heart before.

The plexiform fibromyxoma is a rare mesenchymal tumor in adults that generally originates in the antrum of stomach, being its occurrence in pediatric patients exceptional. It was classified as a distinct entity by World Health Organization in 2010. No recurrences and metastases have been documented in many of the reported patients to date, being the surgical treatment curative. We report the case of a 3-month-old infant who presented to the emergency department with an episode of intestinal subocclusion requiring an emergent surgery. During the surgical intervention, a mass was identified in the jejunum, causing partial occlusion of its lumen. The surgical pathology report revealed an infiltrative tumor composed of spindle-shaped cells disposed in a stroma with a plexiform pattern alternating myxoid areas. These findings and the immunohistochemical characteristics of the neoplastic cells led to classify the tumor as a plexiform fibromyxoma. A description of the immunophenotype of this tumor is made and differential diagnosis with other gastrointestinal tumors is also discussed., Competing Interests: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Cardiac fibromas are benign tumours of the heart and are composed of fibroblasts and collagen. They are one of the most common cardiac tumours encountered in children and adolescents but are rare in adults., Case Report: The patient was a 57-year-old man who presented, complaining of a two-year history of chest tightness at rest. Transthoracic echocardiography revealed a severely calcified mass protruding from the anterior wall of the right ventricle near the apex. The patient was referred for tumour resection. Postoperative histopathological examination identified the calcified mass to be a cardiac fibroma. The patient's postoperative recovery was unremarkable and he was discharged eight days after surgery. During follow up, he has been free from any troublesome symptoms., Conclusions: Pre-operative diagnosis using various imaging modalities and early surgery are key to optimising the prognosis of patients with a cardiac fibroma.

Objective: Nasal glioma, also known as nasal glial heterotopia, is a rare tumor-like lesion that often affects newborns or infants with no hereditary predisposition., Case Report: A 4-year-old child with a growth on the nasal dorsum since birth was diagnosed with nasal glial heterotopia/nasal glioma. The lesion showed a sclerotic fibroma/collagenoma-like storiform pattern with entrapped glial tissue that was S100 and GFAP positive., Conclusion: When a biopsy of the nasal dorsum demonstrates sclerotic microscopic findings with a storiform pattern, nasal glioma should be considered before making a diagnosis in the collagen-rich tissue spectrum (collagenoma or Gardner's fibroma), and an immunohistochemical panel should be requested to demonstrate the presence of an unrecognized light microscopically visible glial component.

Surgical treatment of chondromyxoid fibroma of ribs is described. The diagnosis was verified after histological analysis. The patient underwent resection of multinodular tumor of anterolateral thoracic wall invading abdominal cavity via thoracoabdominal access. Postoperative period was uneventful. This case demonstrates the need for total en-bloc resection of tumor with surrounding tissues. Surgery is the only effective method for these patients.

Objective: Differential diagnosis between the non-calcifying variant of calcifying epithelial odontogenic tumor (NCLC-CEOT) and amyloid-rich central odontogenic fibroma (AR-COdF) has become a debate, particularly regarding the frequency of CD1a positivity in both entities. This has led to the growing consensus that CD1a-positive staining in AR-NC lesions confirms the diagnosis of AR-COdF. Here, we assess the validity of this consensus., Study Design: We collected the data of a case series of histopathologically distinct CEOTs, NCLC-CEOTs, and COdFs and stained them for CD1a and amyloid. Of the 9 CEOTs and NCLC-CEOTs, we diagnosed 4 as classic, 3 as associated with a dentigerous cyst, and 2 as combined CEOT/adenomatoid odontogenic tumors. Of the 9 COdFs, we diagnosed 3 as epithelial poor, 3 as epithelial rich (lacking amyloid), 2 as hyalinized with amyloid, and 1 as hyalinized without amyloid and assessed the staining results., Results: Of the 9 CEOTs and NCLC-CEOTs, 7 stained positively for CD1a, 5 diffusely and 2 focally. Notably, 2 classic NCLC-CEOTs stained strongly CD1a positive. All 3 of the epithelial-poor COdFs were predictably CD1a negative. Of the 6 remaining COdFs, 2 were CD1a positive, 1 hyalinized-with-amyloid COdF diffusely and 1 epithelial-rich-without amyloid focally., Conclusions: CD1a positivity, which occurs in classic CEOT and NCLC-CEOT, does not help distinguish between NCLC-CEOT and AR-COdF and is inconsistent in all AR-COdFs. The diagnosis of CEOT and AR-COdF should be guided by appropriate histopathologic criteria irrespective of CD1a staining or the presence of amyloid or calcifications., Competing Interests: Declarations of interest None., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Medical records of dogs with an initial histopathological diagnosis of odontogenic sarcoma were reviewed for information on signalment, body weight, medical history, clinical signs, physical examination and diagnostic imaging findings, surgical procedure performed, and histologic characteristics. Twenty dogs were deemed to fit the criteria following the rigorous histology review process. These tumors were characterized by hypercellularity of the spindle cell component with less intervening stroma, mitotic activity, and variable presence of odontogenic epithelium and hard substance (cementum/dentin/bone). Non-invasive or locally invasive hypercellular peripheral odontogenic fibroma is suggested by the authors to describe these tumors. There were no signs of recurrence of any of the masses following surgical excisions with varying margins., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Phosphaturic mesenchymal tumor (PMT) is a rare neoplasm that causes tumor-induced osteomalasia (TIO) in most affected patients, usually through the production of fibroblast growth factor 23 (FGF23). This tumor is often misdiagnosed due to its relative rarity and its widely varied histomorphologic spectrum. Here we describe a case of a 78-year-old woman who presented with a left middle tumor without symptoms of TIO. The histological features resembled chondromyxoid fibroma with smudgy calcification in the tumor matrix. In addition, we evaluated FGF23 expression through immunohistochemical study and reverse transcription polymerase chain reaction. PMT with chondromyxoid fibroma features are extremely rare. Examining the expression of FGF23 is useful in the diagnosis of PMT., (© 2023 Japanese Dermatological Association.)

Nonossifying fibroma (NOF) is a common benign bone neoplasm and is usually observed in the first 2 decades of life. Most NOFs occur in the metaphysis of long bones of the lower extremities and migrate toward the diaphysis during skeletal maturation. Epiphyseal involvement by NOF has been rarely reported, with only one case found in the English literature. The authors report the second case of NOF involving the epiphysis of a long bone, the proximal tibia of a 21-year-old woman. Clinicians and pathologists should be aware of the rare possibility of epiphyseal involvement of long bones by this condition. Pathologists should select appropriate immunohistochemistry markers to rule out alternative diagnoses., Competing Interests: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Nevoid basal cell carcinoma syndrome (NBCCS, Gorlin syndrome) is a rare autosomal dominantly inherited disorder that is characterized by multisystem disorder such as basal cell carcinomas, keratocystic odontogenic tumors and skeletal abnormalities. Bilateral and/or unilateral ovarian fibromas have been reported in individuals diagnosed with NBCCS., Case Presentation: A 22-year-old female, presented with low back pain, and was found to have bilateral giant adnexal masses on pelvic ultrasonography, which had been suspected to be malignant ovarian tumors. Positron emission tomography/computed tomography showed multiple intracranial calcification and skeletal abnormalities. The left adnexa and right ovarian tumor were resected with laparotomy, and pathology revealed bilateral ovarian fibromas with marked calcification. We recommended the patient to receive genetic testing and dermatological examination. No skin lesion was detected. Germline testing identified pathogenic heterozygous mutation in PTCH1 (Patched1)., Conclusions: The possibility of NBCCS needs to be considered in patients with ovarian fibromas diagnosed in an early age. Skin lesions are not necessary for the diagnosis of NBCCS. Ovarian fibromas are managed with surgical excision with an attempt at preserving ovarian function. Follow-up regime and counseling on options for future fertility should be offered to patients., (© 2023. The Author(s).)

Plexiform fibromyxoma (PF) is a rare mesenchymal neoplasm of the stomach usually arising in the gastric antrum, and its main differential diagnosis is gastrointestinal stromal tumor. Most common symptoms are hematemesis, anemia. Immunohistochemically, positivity for smooth muscle actin (SMA) and vimentin suggests the diagnosis of PF. We report the case of a 56-year-old female patient with a 30-day history of nausea at presentation 4 years ago. Gastroscopy at that time revealed a subepithelial lesion (SEL) in the gastric antrum, measuring approximately 20 mm in diameter, with leakage of serous fluid after biopsy. Histopathology showed only an inflammatory process. Follow-up gastroscopies were performed 24, 36, and 48 months later, with surveillance biopsy at each follow-up. The last gastroscopies showed changes in lesion appearance, reduction in size, and absence of fluid leakage. Histopathology showed bland spindle cell proliferation, with a vaguely plexiform/multinodular pattern, in a fibromyxoid stroma with an arborizing capillary network without mitoses. The tumor cells were positive for SMA and negative for DOG1, CD117, CD34, S100, desmin, EMA, CD10, calponin, and beta-catenin. The choice of treatment and follow-up depends on the SEL features, but because no cases of malignancy or metastatic disease have previously been reported, the patient chose a conservative approach.

Competing Interests: Competing interests: None declared.

Objective: To present a series of patients with elastofibroma dorsi (ED) in order to recall the pathology and justify its diagnostic and therapeutic management in the primary care setting., Materials and Methods: Retrospective observational and longitudinal study of 12 patients with 18ED. Epidemiological, clinical and imaging characteristics were analysed. Treatment outcomes were assessed in terms of pain (VAS scale), shoulder function (Constant and Murley scale) and quality of life (EuroQol-5 dimension scale, EQ-5D). The mean follow-up of the cases was 60.5months (5years, range 1-161months)., Results: Six patients were male and six were female, with a mean age at diagnosis of 59years. The diagnosis of suspicion in all cases was based in clinical and imaging findings. Only three required surgery. The results of all were satisfactory., Conclusions: Both the diagnosis and the indication for treatment of an ED can be made in the primary care setting. In typical cases, which are the majority, the clinical history and an ultrasound study allow a diagnosis of certainty. The indication for conservative or surgical treatment depends on the informed patient. When there are diagnostic doubts or the patient wishes surgical resection of the tumour, the patient should be referred to a hospital specialist., (Copyright © 2023 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España, S.L.U. All rights reserved.)

Background: Hyaline fibromatosis syndrome is a rare progressive autosomal recessive connective tissue disorder caused by a mutation in the ANTXR2/CMG2 gene. According to its severity, patients may present with skin nodules or visceral infiltration, which carries a poor prognosis. Hypercalcemia has not been reported as a presenting feature of this syndrome. Stimulation of osteoclasts by inflammatory factors and immobilization--induced hypercalcemia have played role in the pathophysiology. To our knowledge, this is the first report of hypercalcemia-associated hyaline fibromatosis syndrome., Case Presentation: Here, we describe cases of two Sudanese patients, a boy aged 9 months and a girl aged 3.5 years with hypercalcemia as an associated presenting feature of hyaline fibromatosis syndrome. Other features include gingival hypertrophy, painful joint swellings, and restriction of movement, which was misdiagnosed as juvenile rheumatoid arthritis. Workup showed normal phosphate, normal to mildly elevated parathyroid hormone, low vitamin D 25. Genetic testing confirmed the mutation of the ANTXR2/CMG2 gene. Both patients responded well to medical therapy for hypercalcemia, but one of them with the severe form of juvenile hyaline fibromatosis died due to sepsis, while the other one has maintained normocalcemic status., Conclusions: These cases highlight the rare presentation of this syndrome and reflect the importance of biopsy and genetic testing in reaching the diagnosis, especially when the clinical presentation can mimic other inflammatory bone disorders. Calcium levels should be checked in such cases., (© 2023. The Author(s).)

An otherwise healthy 32-year-old woman suffered from finger ischemia. An echocardiogram and computed tomography scan revealed a mobile mass in the left ventricle that was attached to the anterior papillary muscle and did not involve the valve leaflets. The tumor was resected, and histopathology confirmed it to be a papillary fibroelastoma. Our case emphasizes the significance of a comprehensive diagnostic work-up for a peripheral ischemic lesion. This resulted in the discovery of an unusual intra-ventricular origin for a commonly benign tumor.

BACKGROUND Ossifying fibromyxoid tumor (OFMT) is a rare mesenchymal tumor predominantly involving the subcutaneous tissues or skeletal muscles in the proximal extremities, typically in middle-aged men. OFMT in the spine is extremely rare, with only 3 previously reported cases in the literature. CASE REPORT Here, we present a rare case of an 82-year-old man presenting with paresthesia of both arms and weakness of both legs, who underwent magnetic resonance imaging (MRI) of the spine, which showed an aggressive extradural tumor. Following surgical debulking, histology examination revealed a tumor of stromal origin with myxoid and ossifying components and pleomorphic features. Overall findings were suggestive of a malignant OFMT. The patient underwent postoperative adjuvant radiotherapy. However, the first follow-up MRI study at 8 months showed residual tumor, which also demonstrated avid tracer uptake on technetium-99m scintigraphy and PET-CT scans. The second MRI follow-up about 9 months later showed several metastatic foci along the craniospinal axis. Despite subsequent resection of the spinal metastasis, the patient eventually died of sepsis about 21 months after the initial tumor diagnosis. CONCLUSIONS We presented a case of extradural spinal malignant OFMT and highlighted the difficulty distinguishing this rare primary tumor from spinal metastases. In this case, MRI signal intensities and identifying intratumoral bone formation, combined with histopathology following surgical resection, confirmed the diagnosis. This case has also shown the importance of follow-up by a multidisciplinary team to monitor for the recurrence of primary OFMT.

Background: Lipofibromatosis is a relatively new entity, considered in the differential diagnosis of soft tissue tumors in children, involving mainly the extremities. Most cases can be completely resected without recurrence. Atypical forms have been described and their highly infiltrative capability can lead to severe clinical impairment. Case report: We report an infant with rapidly growing posterior cervicothoracic tumor, extending from the bulbo-medullary junction to T6 vertebra, leading to loss of spontaneous breathing, quadriparesis and devastating long-term consequences. Spinal tomography scan at 35 days suggested myelomeningocele. Prenatal and early postnatal ultrasounds were reviewed and no central nervous system involvement or fusion defects were present. Magnetic resonance at 40 days showed infiltrative mass, later confirmed by pathology as lipofibromatosis. Two resections failed to completely remove the tumor. Discussion: Lipofibromatosis can involve critical structures in the central nervous system and is difficult to treat surgically in those circumstances.

In cases of growth of FA on imaging, core needle biopsies (CNB) are often performed to rule out phyllodes tumor (PT). We aim to focus on "growing FAs" and to identify clinical and histopathologic features that are likely to predict a PT on excision. Thirty-four FAs with radiologic documentation of growth were included. Various clinical and pathological features such as age, body mass index (BMI), lesion size, and growth rate were recorded. On excision, 17 cases (50 %) were FAs, whereas 16 (47 %) were re-classified as benign PT despite only 19 % being suspicious for PT on CNB. PT patients were older (mean age 42.6) than those with FAs (mean age 28.2), p = 0.0002. All false negative cases demonstrated intracanalicular growth. Mitotic rate was the most significant histologic feature in PT on excision compared to others, such as lesion circumscription and stromal cellularity. Recognition and careful counting of mitotic rate, especially with intracanalicular patterns in growing FAs, can potentially prevent missing a PT on CNB. In patients with "growing FAs" who are ≥40 years of age, excision may be recommended due to the high likelihood of PT diagnosis on excision and high false negative rate on CNB., Competing Interests: Declaration of competing interest All authors declare no conflict of interest. Author SH is cofounder of CloudPath Diagnostics LLC, New York. Part of this study has been accepted for abstract presentation at the USCAP 2020 Annual Meeting., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Context.—: Spindle cell lesions of the breast represent a broad spectrum of entities, ranging from nonneoplastic reactive conditions to high-grade malignant tumors. The wide range makes breast spindle cell lesions a diagnostic pitfall., Objective.—: To review the classification of spindle cell lesions of the breast, including clinical features, morphologic characteristics, and the role of immunohistochemistry as well as molecular tools in assisting the differential diagnosis. A diagnostic algorithm will be proposed., Data Sources.—: Literature and personal experience are the sources for this study., Conclusions.—: Spindle cell lesions of the breast can be classified as biphasic or monophasic, with the former including both spindle cell and epithelial components, and the latter including only spindle cell elements. Each category is further subclassified as low or high grade. In the biphasic low-grade group, fibroadenoma and benign phyllodes tumor are the most common lesions. Other uncommon lesions include hamartoma, adenomyoepithelioma, and pseudoangiomatous stromal hyperplasia. In the biphasic high-grade group, borderline/malignant phyllodes tumor and biphasic metaplastic carcinoma are the main lesions to consider. In the monophasic low-grade group, reactive spindle cell nodule, nodular fasciitis, myofibroblastoma, fibromatosis, and fibromatosis-like metaplastic carcinoma have to be considered. In the monophasic high-grade group, the possible lesions are monophasic spindle cell metaplastic carcinoma, primary breast sarcoma, and metastases. Awareness of the clinical history and careful evaluation of any epithelial differentiation (with a large immunohistochemical panel) are crucial in the distinction., (© 2023 College of American Pathologists.)