Primary cutaneous Ewing sarcoma - Case report

Abstract

Ewing sarcoma is a primitive neuroectodermal tumor rarely occurs in the skin and sobcutaneous tissues. Generally Ewing's sarcoma is a primary bone tumor, but when present in soft tissues it characterizes an extremely uncommon clinical picture. It usually involves the deep subcutaneous tissue or muscles, and more rarely occurs like a primary skin cancer. Most patients are white, women, and in the second decade of life. The clinical features are a superficial mass, in average measuring 2-3 cm, of soft consistency, freely mobile and sometimes painful. The more affected locations are upper and lower extremities, trunk, head, neck or multiple lesions. The presence of metastases is very rare.

Primary Cutaneous Ewing Sarcoma: Case Report and Review of Literature

“…superficial subcutis They were first described by Angerwall and Enzinger in 1975. The diagnosis is made by aspiration cytology, histochemical stains, immunohistochemistry, electron microscopy, cytogenetics and molecular genetics of translocations [1,3,4]. The differencial diagnosis of ES include primary cutaneous small round cell tumors like Merckel cell carcinoma, eccrine spiradenoma, lymphoma, melanoma, clear cell sarcoma, rhabdomyosarcoma, malignant rhabdoïd tumor, malignant primitive neuroectodermal tumor and poorly differenciated adnexal tumors [5,6].…”

“…The differencial diagnosis of ES include primary cutaneous small round cell tumors like Merckel cell carcinoma, eccrine spiradenoma, lymphoma, melanoma, clear cell sarcoma, rhabdomyosarcoma, malignant rhabdoïd tumor, malignant primitive neuroectodermal tumor and poorly differenciated adnexal tumors [5,6]. It includes also cutaneous metastases from osseous ES, large cell neuroendocrine carcinoma, small cell lung carcinoma and neuroblastoma [1,7]. The ES is composed of small round cells which express the CD99 and molecular study shows a specific chromosomal translocation t(11;22) involving gene EWSR1 in chromosome 22q12 or a fusion or combination between EWSR1 gene and gene of ETS family [1,3,8].…”

“…It includes also cutaneous metastases from osseous ES, large cell neuroendocrine carcinoma, small cell lung carcinoma and neuroblastoma [1,7]. The ES is composed of small round cells which express the CD99 and molecular study shows a specific chromosomal translocation t(11;22) involving gene EWSR1 in chromosome 22q12 or a fusion or combination between EWSR1 gene and gene of ETS family [1,3,8]. This study is made by FISH or real time polymerase chain reaction (RT-PCR) [8].…”

“…This study is made by FISH or real time polymerase chain reaction (RT-PCR) [8]. Cutaneous ES has better prognosis than primary bone or soft tissue ES with a survival rate of 91% in 10 years [1].…”

“…It's usually a primary bone tumor but rarely occurs in the skin and subcutaneous tissues [1], and generally appears as a single small lesion, circumscribed mid to deep dermis or involving subcutis. Due to their rarity and morphological similarity to other cutaneous tumors, cutaneous Ewing sarcoma (CES) are subject to being clinically and pathologically sub-diagnosed [1]. We report a new case of a 20-yearold female with a lesion in the left elbow.…”