Desmoplastic Small Round Cell Tumor with Ovarian Involvement - A Case Report -
0
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm that preferentially involves the abdominal and pelvic cavities in relatively young males. We present a rare case of DSRCT arising in the ovary of a 16-year-old girl. During surgery, a 15 cm-sized huge mass was noted in the right ovary and wide spreading of the tumor was identified in the left ovary, uterine wall, and omentum and bowel wall. Histological investigation showed nests of small round cells with round nuclei and scanty eosinophilic cytoplasm accompanied with dense desmoplastic stroma. The immunohistochemistry showed that the tumor coexpressed epithelial, mesenchymal, and neuronal markers. The tumor cells ultrastructurally showed poorly developed cell junctions and occasionally showed intracytoplasmic aggregates of intermediate filaments. Molecular analysis of the tumor revealed chromosomal translocation t(11:22)(p13; q12) associated with the EWS-WT1 fusion protein. DSRCT should be included in the differential diagnosis of ovarian neoplasms in young patients.
Abstract
Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm that preferentially involves the abdominal and pelvic cavities in relatively young males. We present a rare case of DSRCT arising in the ovary of a 16-year-old girl. During surgery, a 15 cm-sized huge mass was noted in the right ovary and wide spreading of the tumor was identified in the left ovary, uterine wall, and omentum and bowel wall. Histological investigation showed nests of small round cells with round nuclei and scanty eosinophilic cytoplasm accompanied with dense desmoplastic stroma. The immunohistochemistry showed that the tumor coexpressed epithelial, mesenchymal, and neuronal markers. The tumor cells ultrastructurally showed poorly developed cell junctions and occasionally showed intracytoplasmic aggregates of intermediate filaments. Molecular analysis of the tumor revealed chromosomal translocation t(11:22)(p13; q12) associated with the EWS-WT1 fusion protein. DSRCT should be included in the differential diagnosis of ovarian neoplasms in young patients.
참고문헌 (12)
- Chang F, "Desmoplastic small round cell tumors: cytologic, histologic, and immunohistochemical features", Arch Pathol Lab Med, 130, pp.728~732 (2006)
- Roganovich J, "Paratesticular desmoplastic small round cell tumor: case report and review of the literature", J Surg Oncol, 71, pp.269~272 (1999)
- Murphy A, "Primary desmoplastic small round cell tumor of bone: report of a case with cytogenetic confirmation", Cancer Genet Cytogenet, 156, pp.167~171 (2005)
- Finke NM, "Sinonasal desmoplastic small round cell tumor: a case report", Am J Surg Pathol, 26, pp.799~803 (2002)
- Karavitakis EM, "Desmoplastic small round cell tumor of the pleura", Pediatr Blood Cancer, 49, pp.335~338 (2007)
- Syed S, "Desmoplastic small round cell tumor of the lung", Arch Pathol Lab Med, 126, pp.1226~1228 (2002)
- Slomovitz BM, "Desmoplastic small round cell tumor with primary ovarian involvement: case report and review", Gynecol Oncol, 79, pp.124~128 (2000)
- Su MC, "Desmoplastic small round cell tumor of the kidney", Am J Surg Pathol, 28, pp.1379~1383 (2004)
- Saab R, "Desmoplastic small round cell tumor in childhood: the ST. Jude children’s research hospital experience", Pediatr Blood Cancer, 49, pp.274~279 (2007)
- Ordo@n)ez NG, "CA 125 production in desmoplastic small round cell tumor: report of a case with elevated serum levels and prominent signet ring morphology", Hum Pathol, 29, pp.294~299 (1998)
- Lae ME, "Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors", Am J Surg Pathol, 26, pp.823~835 (2002)
- Gerald WL, "Intra-abdominal desmoplastic small round-cell tumor. Report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals", Am J Surg Pathol, 15, pp.499~513 (1991)