Indian Journal of Pathology and Microbiology (Jan 2021)

Desmoplastic small round cell tumor of the ovary: A rare but poor prognostic disease in a young woman!

  • Ravi Hari Phulware,
  • Maitrayee Roy,
  • Neeta Singh,
  • Sunesh Kumar,
  • Sandeep R Mathur

DOI
https://doi.org/10.4103/IJPM.IJPM_725_19
Journal volume & issue
Vol. 64, no. 1
pp. 206 – 209

Abstract

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Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive neoplasm of uncertain histogenesis that preferentially involves the abdominal and pelvic cavities. DSRCT mainly develops in adolescent and young adults with a strong male predominance; the male to female ratio is 4:1. Ovarian location is exceptional. DSRCT generally develops in the abdomen and have a tendency towards peritoneal spread, with subsequent metastasis to distant lymph nodes, liver and lungs. It is a poorly understood malignancy with a very characteristic morphology, immunophenotype, cytogenetic features, and poor prognosis. This tumor can co-express epithelial, neuronal, and mesenchymal markers. Despite intensive therapy, including surgery, radiotherapy and chemotherapy, and immunotherapy; the 5-year survival is less than 15%.

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