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Skin and soft tissue
Published in Tor Wo Chiu, Stone’s Plastic Surgery Facts, 2018
Histologically, lesions show atypical fibroblasts with generous blood supply; there may be large amounts of mucin (low-grade fibromyxoid sarcoma). Less well-differentiated tumours may be best described as anaplastic sarcomas. Better techniques have shown that many lesions previously labelled as fibrosarcoma were actually MFH, synovial sarcoma, etc.; this means that earlier literature should be interpreted cautiously.
Perineurioma and Malignant Peripheral Nerve Sheath Tumor
Published in Dongyou Liu, Tumors and Cancers, 2017
Intraneural perineurioma often causes segmental, uniform expansion of the nerve. Microscopically, intraneural perineurioma shows pseudo-onion bulbs surrounding nerve fibers with bundles of spindle-shaped perineurial cells (containing ovoid to elongated nuclei and pale cytoplasm) in longitudinal sections, fine collagenous stroma, irregular borders with the adjacent lamina propria, and entrapped colonic crypts. Perineurioma stains positive for epithelial membrane antigen, Claudin-1, and CD34 (33%) and negative for S-100, glial fibrillary acidic protein (GFAP), neurofilament protein, smooth muscle actin (SMA), desmin, caldesmon, KIT, and pan-keratin. Cytogenetically, perineurioma harbors monosomy 22, and deletion of 22q11-13.1 [7]. Differential diagnosis for intraneural perineurioma includes localized reactive Schwann cell proliferations, while that for soft tissue perineurioma includes low-grade fibromyxoid sarcoma. Notably, low-grade fibromyxoid sarcoma displays prominent stromal collagen deposition and an abrupt transition into myxoid nodules in a curvilinear vascular pattern. However, low-grade fibromyxoid sarcoma is positive for MUC4 expression and a FUS rearrangement, while perineurioma is negative for both.
Fibrous tumors
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Low-grade fibromyxoid sarcoma is a rare deep soft tissue tumor with spindle-shaped cells with no or few mitoses and characteristic, though not specific, areas of myxoid stroma. Local recurrences are frequent and late metastases are observed. It is due to a translocation of the FUS/CREB3L2 gene.147
Orbitofacial dermatofibrosarcoma protuberans with intranasal extension
Published in Orbit, 2018
Siti Nurhuda Sharudin, Siow Wei Tan, Nor Fadhilah Mohamad, Suresh Kumar Vasudevan, Hanizah Khairan, YC Mun, Jothi Shanmuganathan
Excision biopsy of the left-nasal mass and nasolabial tissue was performed by the otorhinolaryngology team. The specimen obtained was a grey whitish tissue measuring 45 mm x 25 mm x 10 mm and an exophytic tumor mass measuring 40 mm x 17 mm x 6 mm. Microscopically, the tumor cells were spindled with no definite pattern of arrangement embedded within slightly fibromyxoid stroma. The cells were mildly pleomorphic with ill-defined cytoplasmic borders and inconspicuous to small nucleoli. Mitosis is occasionally seen (2/10 hpf). There was no necrosis or carcinomatous differentiation seen. The tumor cells were positive for CD34 and vimentin stains, and negative for desmin and S100. These findings were consistent with low-grade fibromyxoid sarcoma.
Recurrent Giant Cell Fibroblastoma in an Infant: A Diagnostic Challenge
Published in Fetal and Pediatric Pathology, 2022
Priyanka Maity, Uttara Chatterjee, Mou Das, Sabita Patra
The malignant entities included in the differential diagnoses of GCF on histopathology are liposarcoma, myxofibrosarcoma, malignant fibrous histiocytoma, rhabdomyosarcoma and low grade fibromyxoid sarcoma. Considering the young age of the patient, liposarcoma, malignant fibrous histiocytoma and myxofibrosarcoma were excluded. Low grade fibromyxoid sarcoma is positive for MUC4 and may show atypia, necrosis and mitotic figures. CD34 positivity in GCF helps to exclude this long list of differentials.