A Case of Primary Cutaneous Extraskeletal Ewing Sarcoma on the Abdomen
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초록
Primary cutaneous extraskeletal Ewing sarcoma (EWS) is a primitive neuroectodermal tumor that usually occurs as a small, localized tumor on the trunk or extremities of young adults. The prognosis is typically reported to be quite favorable. It is extremely rare; only three cases of primary cutaneous EWS have been reported in Korea. In the first report, molecular genetic testing was not performed to make a definitive diagnosis. In the second report, reverse transcription polymerase chain reaction (RT-PCR) for EWS-FLI1 gene ar- rangement was done, but the result was negative. Although RT-PCR and fluorescence hybridization (FISH) were performed in the third report, none of the results were shown in the article. Considering that genetic testing is an essential diagnostic tool for certain dis- eases, such as some brain tumors, we report a case of primary cutaneous extraskeletal EWS, including the result of RT-PCR. A 36-year-old Korean female presented with a cutaneous mass on the abdomen. Histological evaluation revealed solid sheets of primitive, small, uni- form cells with hyperchromatic nuclei and scant cytoplasm. Immunohistochemistry stains were positive for CD99 and FLI1. RT-PCR showed a t(11;22) (Ewing sarcoma region 1)- (Friend leukemia virus integration 1) translocation.
Abstract
Primary cutaneous extraskeletal Ewing sarcoma (EWS) is a primitive neuroectodermal tumor that usually occurs as a small, localized tumor on the trunk or extremities of young adults. The prognosis is typically reported to be quite favorable. It is extremely rare; only three cases of primary cutaneous EWS have been reported in Korea. In the first report, molecular genetic testing was not performed to make a definitive diagnosis. In the second report, reverse transcription polymerase chain reaction (RT-PCR) for EWS-FLI1 gene ar- rangement was done, but the result was negative. Although RT-PCR and fluorescence hybridization (FISH) were performed in the third report, none of the results were shown in the article. Considering that genetic testing is an essential diagnostic tool for certain dis- eases, such as some brain tumors, we report a case of primary cutaneous extraskeletal EWS, including the result of RT-PCR. A 36-year-old Korean female presented with a cutaneous mass on the abdomen. Histological evaluation revealed solid sheets of primitive, small, uni- form cells with hyperchromatic nuclei and scant cytoplasm. Immunohistochemistry stains were positive for CD99 and FLI1. RT-PCR showed a t(11;22) (Ewing sarcoma region 1)- (Friend leukemia virus integration 1) translocation.
참고문헌 (12)
- Machado I, "Superficial small round-cell tumors with special reference to the Ewing’s sarcoma family of tumors and the spectrum of differential diagnosis", Semin Diagn Pathol, 30, pp.85~94 (2013)
- Delaplace M, "Primary cutaneous Ewing sarcoma : a systematic review focused on treatment and outcome", Br J Dermatol, 166, pp.721~726 (2012)
- Di Giannatale A, "Primary cutaneous and subcutaneous Ewing sarcoma", Pediatr Blood Cancer, 62, pp.1555~1561 (2015)
- 이우진, "피부를 침범한 골외성 Ewing 육종 1예", 대한피부과학회지, 46(10), pp.1427~1431 (2008), 대한피부과학회
- Bahk WJ, "Primary cutaneous Ewing’s sarcoma", primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses : its complete remission by chemotherapy and magnetic resonance imaging findings, 2010, pp.39, 595~600
- 최재우, "피부에서 발생한 골외성 Ewing 육종", 대한피부과학회지, 50(2), pp.147~151 (2012), 대한피부과학회
- Tefft M, "Paravertebral"round cell"tumors in children", Radiology, 92, pp.1501~1509 (1969)
- Collier AB 3rd, "Cutaneous Ewing sarcoma : report of 2 cases and literature review of presentation, treatment, and outcome of 76 other reported cases", J Pediatr Hematol Oncol, 33, pp.631~634 (2011)
- Scotlandi K, "Immunostaining of the p30", 32MIC2 antigen and molecular detection of EWS rearrangements for the diagnosis of Ewing’s sarcoma and peripheral neuroectodermal tumor, 1996, pp.27, 408~416
- Galyfos G, "Extraosseous Ewing sarcoma : diagnosis, prognosis and optimal management", Indian J Surg, 78, pp.49~53 (2016)
- Wang L, "Undifferentiated small round cell sarcomas with rare EWS gene fusions : identification of a novel EWS-SP3 fusion and of additional cases with the EWS-ETV1 and EWS-FEV fusions", J Mol Diagn, 9, pp.498~509 (2007)
- Machado I, "Superficial Ewing’s sarcoma family of tumors : a clinicopathological study with differential diagnoses", J Cutan Pathol, 38, pp.636~643 (2011)
