Myxoid neurofibroma is benign tumours of perineural cell origin that arise from elements in the peripheral nervous system. We report a case of a 60-year-old female patient presented with history of right ear mass which was slowly growing. Her primary complaint was cosmetic deformity but hearing loss was also present. The mass was excised and histologically revealed a myxoid neurofibroma. To the authors' knowledge, this is the first time that a myxoid neurofibroma arising from external auditory canal is reported. It should be included in the differential diagnosis of a mass originating from this location.
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Int J Gynecol Pathol
March 2025
Emory University Hospital, Atlanta, GA.
Cellular angiofibromas (CAFs) are benign mesenchymal neoplasms of the vulva and lower genitourinary tract. Although most cases are benign with excellent prognosis, data on CAFs with cytologic atypia (aCAF) and sarcomatous transformation (tCAF) is limited. We identified 13 vulvar CAFs comprising 4 aCAFs and 9 tCAFs.
View Article and Find Full Text PDFInt J Gynecol Pathol
March 2025
Department of Pathology, National University Health System.
Vulval leiomyosarcomas with variant features are rare with limited data available in the literature compared to their uterine counterparts. Gynecologic leiomyosarcoma with nuclear receptor 4A3 (NR4A3) gene fusion is a rare, recently described neoplasm that has been reported mostly in the uterus and rarely in the pelvis. Herein, we report the first case of this entity occurring as a primary vulva tumor in a 46-year-old patient.
View Article and Find Full Text PDFCureus
February 2025
Department of Plastic Surgery, Dermatology and Plastic Surgery of Arizona, Tucson, USA.
Chondroid syringomas are rare adnexal neoplasms composed of epithelial and mesenchymal components, posing diagnostic challenges due to their diverse histological features. We report an atypical presentation of an apocrine mixed tumor in a 51-year-old female patient who presented with a 0.7 cm firm, flesh-colored nodule on the right lateral cutaneous lip.
View Article and Find Full Text PDFMod Pathol
March 2025
Hospices Civils de Lyon, Centre Hospitalier Lyon-Sud, Service de Pathologie Multi-Site, Pierre Bénite, France; Université Claude Bernard Lyon-1, Faculté de Médecine Lyon Sud, Centre pour l'innovation en cancérologie de Lyon (CICLY), UR 3738, Lyon, France; Université Claude Bernard Lyon-1, Faculté de Médecine Lyon Est, Lyon, France. Electronic address:
MEF2D::NCOA2 fusion was recently reported in two vulvovaginal myxoid epithelioid smooth muscle tumors. We aimed to performed an integrated approach combining clinical, morphological, immunohistochemical, and molecular profiling analyses, including targeted-RNA-sequencing, targeted-gene expression analysis profiling with clustering, DNA mutational analysis, and array comparative genomic hybridization (aCGH) in a series of three MEF2D::NCOA2 fusion-associated vulvovaginal tumors, to better described this entity. The median age at diagnosis was 45 years.
View Article and Find Full Text PDFESMO Open
March 2025
Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. Electronic address:
Background: We report the results of the pre-planned secondary analysis of radiologic responses (RRs) of ISG-STS 1001, a randomized trial comparing anthracycline + ifosfamide (AI) versus histology-tailored (HT) neoadjuvant chemotherapy for primary localized high-risk soft-tissue sarcomas of the extremities and trunk wall.
Patients And Methods: Patients with undifferentiated pleomorphic sarcoma (UPS), leiomyosarcoma (LMS), malignant peripheral nerve sheath tumor, synovial sarcoma or myxoid liposarcoma (MLPS) were randomized, whereas patients with myxofibrosarcoma, pleomorphic liposarcoma, pleomorphic rhabdomyosarcoma or unclassified sarcoma were included in the observational arm (O) and treated with AI. Patients with UPS, LMS or MLPS needing concurrent preoperative radiotherapy were included in O.
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