Indian Journal of Pathology and Microbiology
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Year : 2021  |  Volume : 64  |  Issue : 1  |  Page : 206-209

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

1 Department of Pathology, All India Institute of Medical Sciences (AIIMS), New Delhi, India
2 Department of Pathology, Maharishi Markandeshwar University, Mullana, Ambala, Haryana, India
3 Department of Obstetrics and Gynecology, All India Institute of Medical Sciences (AIIMS), New Delhi, India

Correspondence Address:
Sandeep R Mathur
Professor, Department of Pathology, All India Institute of Medical Sciences (AIIMS), New Delhi-110029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_725_19

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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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