Compound clear cell sarcoma misdiagnosed as a Spitz nevus Journal Article


Authors: Kiuru, M.; Hameed, M.; Busam, K. J.
Article Title: Compound clear cell sarcoma misdiagnosed as a Spitz nevus
Abstract: Clear cell sarcoma (CCS) typically presents as a tumor in the deep soft tissue of extremities, but when centered in the dermis it may be confused with a melanocytic nevus, primary nodular or metastatic melanoma. Compound variants of CCS, i.e. tumor cells present in both the epidermis and underlying soft tissue have not yet been described. Herein we report such a case, which initially presented as a nodule on the left wrist of a young woman at 19 years of age. The lesion was then interpreted as 'Spitz nevus, compound type'. Twelve years later the patient noticed an enlarged lymph node in the right axilla. The excised lymph node was nearly completely replaced by malignant tumor cells, which were immunoreactive for S100 protein. They resembled the tumor cells of the wrist lesion. Cytogenetic analysis of the metastatic tumor revealed a t(12;22) translocation. Fluorescence in situ hybridization confirmed Ewing's sarcoma breakpoint region 1 (EWSR1) rearrangement in 70% of the tumor cells, thereby supporting the diagnosis of metastatic CCS. Our case is of interest because it documents that CCS can involve the epidermis. This observation expands the morphological spectrum associated with this tumor. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
Keywords: melanocytic lesions; soft tissue tumors; dermatopathology; cutaneous neoplasm; s100
Journal Title: Journal of Cutaneous Pathology
Volume: 40
Issue: 11
ISSN: 0303-6987
Publisher: John Wiley & Sons  
Date Published: 2013-11-01
Start Page: 950
End Page: 954
Language: English
DOI: 10.1111/cup.12197
PROVIDER: scopus
PUBMED: 23980901
DOI/URL:
Notes: --- - "Export Date: 2 December 2013" - "CODEN: JCUPB" - "Source: Scopus"
Altmetric
Citation Impact
BMJ Impact Analytics
MSK Authors
  1. Meera Hameed
    281 Hameed
  2. Klaus J Busam
    688 Busam
  3. Maija H.T. Kiuru
    9 Kiuru