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Malignant peripheral nerve sheath tumor with perineurial cell differentiation (malignant perineurioma)
Author(s) -
Zámečník Michal,
Michal Michal
Publication year - 1999
Publication title -
pathology international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
eISSN - 1440-1827
pISSN - 1320-5463
DOI - 10.1046/j.1440-1827.1999.00815.x
Subject(s) - pathology , cd34 , vimentin , cytokeratin , s100 protein , pleomorphism (cytology) , dermatofibrosarcoma protuberans , nerve sheath neoplasm , perineurium , sarcoma , endoneurium , malignant peripheral nerve sheath tumor , biology , histiocyte , immunohistochemistry , medicine , anatomy , stem cell , genetics , peripheral nerve , sciatic nerve
A unique case of malignant peripheral nerve sheath tumor (MPNST) with perineurial cell differentiation occurring in a 63‐year‐old woman in a subcutis of the forearm is described. The tumor contained cellular and myxoid areas. The neoplastic cells were fusiform with distinct cell borders. They were arranged in storiform pattern and in wavy parallel cell cords in the cellular areas. Focally, a pleomorphism and mitotic activity (including atypical mitoses) similar to those of malignant fibrous histiocytoma were seen. The myxoid parts contained haphazardly oriented cells and scarce lipoblast‐like multivacuolated cells mimicking a liposarcoma. In the differential diagnosis, myxoid liposarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma and low‐grade fibromyxoid sarcoma were considered. Immunohistochemically, perineurial differentiation was indicated by the diffuse expression of epithelial membrane antigen and focal reactivity for CD34. The tumor was negative with antibodies to S‐100 protein, Leu‐7, CD68 (KP1), vimentin and cytokeratin AE1/AE3. Ultrastructure of tumor cells revealed features of MPNST. No recurrence occurred in the patient during 2 years follow up.