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Desmoplastic small round cell tumor: characteristics and treatment options

Katarzyna Waśniewska, Magdalena Miedzińska-Maciejewska, Krzysztof Gawrychowski

Affiliacja i adres do korespondencji
CURR. GYNECOL. ONCOL. 2011, 9 (4), p. 276-282
Streszczenie

Desmoplastic small round cell tumor (DSRCT) is an extremely rare malignancy rated among the sarcomas. It was first described in 1989. As there are only a few hundred cases reported in world literature, to date there are no uniform standards of management of patients presenting with such a diagnosis. This malignancy is characterized by a extremely aggressive clinical course. The disease affects mainly young males in their second and third decade. Most often described primary location of DSRCT is abdominal cavity. Identified cytogenetic marker of DSRCT is translocation t(11;22)(p13q12). Therapy relies in surgery, radiotherapy and chemotherapy. In spite of continuous progress in the treatment of DSRCT, prognosis in this group of patients is still very poor. High propensity to dissemination already at the time of first diagnosis considerably reduces the effectiveness of surgical excision and all patients require systemic treatment. Chemotherapy usually resorts to cyclophosphamide, doxorubicin, vincristine, ifosfamide and etoposide (their combination is known as “protocol P6”). Other cytostatics of confirmed effectiveness in the treatment of DSRCT are cisplatin, carboplatin, topotecan, vinorelbine and irinotecan. In spite of clear clinical activity of chemotherapeutics, eventually all patients develop resistance to implemented treatment and suffer disease progression. Therefore, selection of optimal treatment for this group of patients remains a considerable clinical challenge.

Słowa kluczowe
desmoplastic small round cell tumor, symptoms, diagnosis, treatment, chemotherapy