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Granular Cell Variant of Dermatofibrosarcoma Protuberans (DFSP)
Author(s) -
Saadat P.,
Vadmal M.
Publication year - 2006
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.0303-6987.2006.0511h.x
Subject(s) - pathology , cd34 , dermatofibrosarcoma protuberans , vimentin , biology , desmin , dermis , cytoplasm , anatomy , intermediate filament , immunohistochemistry , cell , microbiology and biotechnology , medicine , cytoskeleton , genetics , stem cell
To date, there are three reported cases of granular cell variant of DFSP. We describe the fourth case in a 21‐year‐old female who presented with an eight months history of a growing, irregular, firm, 2.5 × 2.0 × 1.5 cm nodule on the right cheek. Biopsy revealed a spindle cell neoplasm involving the deep dermis and subcutis exhibiting the characteristic storiform arrangement. The spindle cells had thin, elongated, hyperchromatic nuclei and a moderate amount of cytoplasm. The superficial dermis revealed larger polygonal cells with central oval nuclei and abundant cytoplasm intermingled with the spindle cells. The distinct feature in both cell types was the presence of a characteristic pink granular cytoplasm. The cells exhibited minimal cytological atypia with rare mitotic figures. Immunohistochemically, both cell types demonstrated a positive immunoreaction for vimentin and CD68, the spindle cells stained positively with muscle actins, and dermal dendrocytes stained with Factor XIIIa. The tumor was negative for cytokeratins, S‐100, HMB‐45, desmin, NSE, and CD34. PAS failed to stain the cytoplasmic granules. Ultrastructurally, abundant cytoplasmic organelles composed of pleomorphic secondary lysosomal structures and lipid droplets were noted. The histological, immunohistochemical and ultrastructural features of this tumor are compatible with previously reported cases of granular variant of DFSP.