Primary Cutaneous Ewing's Sarcoma/Peripheral Primitive Neuroectodermal Tumors in ChildhoodA Molecular Cytogenetic, and Immunohistochemical Study
作者:
C. Lee,
Melissa Southey,
Howard Slater,
Alexander Auldist,
C. Chow,
Deon Venter,
期刊:
Diagnostic Molecular Pathology
(OVID Available online 1995)
卷期:
Volume 4,
issue 3
页码: 174-181
ISSN:1052-9551
年代: 1995
出版商: OVID
关键词: Molecular diagnosis;Malignant skin tumor;Sweat gland tumor;Ewing's sarcoma;Peripheral neuroectodermal tumor;Translocation;t(11;22)-(q24;q12);EWSgene;MIC2 antigen;Rearrangement.
数据来源: OVID
摘要:
Childhood cutaneous and subcutaneous malignancies are rare and include metastatic tumors of diverse histogenesis as well as primary lesions, such as sweat gland carcinomas. Some cutaneous malignancies exhibit a small round cell tumor morphology with few definitive differentiating features; they can thus pose a significant diagnostic problem. We describe two primary malignancies of the skin and superficial subcutis, which were originally diagnosed as sweat gland carcinomas on the basis of their morphological features. A cytogenetic analysis performed on one of these lesions showed the t(11;22)(q24;q12) rearrangement, believed to be unique to the Ewing's sarcoma/ peripheral primitive neuroectodermal tumor (ES/pPNET) group of neoplasms. In view of this unexpected result, reverse transcriptase-polymerase chain reaction analysis was performed on both lesions and showed that they expressedEWS/FLI-1fusion gene mRNA transcripts, the molecular equivalent of t(11;22)(q24;ql2). The two tumors also had an immunohistochemical profile suggesting ES/pPNET, including strong expression of the MIC2 antigen. Both patients were treated with wide local excision, and one was given a course of chemotherapy. Neither patient showed evidence of tumor elsewhere after follow-up periods of 2 years and 16 years. These findings suggest that these tumors are indeed a form of primary ES/pPNET arising in the skin or superficial subcutis, which may be of low-grade malignancy and curable by local surgery.
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