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Composite Extrarenal Rhabdoid Tumor of the Skin: Cytology, Morphology, Electron Microscopy and Special Stain Findings
Author(s) -
Petitt M.,
Doeden K.,
Harris A.,
Bocklage T.
Publication year - 2005
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.0303-6987.2005.320fl.x
Subject(s) - pathology , desmin , cytokeratin , cd34 , nodule (geology) , stain , differential diagnosis , biopsy , vimentin , rhabdomyosarcoma , medicine , immunohistochemistry , biology , staining , sarcoma , stem cell , paleontology , genetics
We present the findings of a rare and very aggressive primary skin tumor, composite extrarenal rhabdoid tumor (CERT) with features of rhabdomyosarcoma. Clinical history: A 53‐year‐old woman presented with a 2.5 cm ulcerated nodule on her right medial calf. On exam, she also exhibited inguinal lymphadenopathy. Pathology: Fine needle aspiration of a lymph node and punch biopsy of the skin nodule disclosed an epithelioid cell morphology with rhabdoid cells, solid growth, and a high mitotic rate (>70 figures/10 HPF). Upon wide excision of the tumor, electron microscopy (EM) and special stains were performed. Since the differential diagnosis was broad, a large number of stains were performed: tumor was positive for vimentin, desmin, muscle specific actin, WT‐1, CD56, and CD10, and negative for S‐100, melan A, cytokeratins, EMA, CD34, fast myosin, myoglobin, myogenin, myoD1, smooth muscle actin, and NSE. EM revealed rhabdoid cells with perinuclear globules of intermediate filaments corresponding to the positive desmin stain. Summary: The patient was treated with chemotherapy without regression of presumed lung metastases. The CERT is a rare, aggressive tumor that extraordinarily presents as a primary skin tumor. The differential is broad and nomenclature confusing. Extensive ancillary pathology studies are required for appropriate diagnosis.