Key result
Palliative chemotherapy in a 26-year-old woman with desmoplastic small round cell tumor achieved a maximal response of stable disease, with the patient expiring 9 months after diagnosis.
Case Report (n=1)
No
Molecular genetic methods such as FISH or RT-PCR are helpful in diagnosing DSRCT, and chemotherapy can provide temporary disease stabilization in advanced cases.
Palliative chemotherapy may stabilize advanced DSRCT temporarily; leaves open optimal regimens and molecular diagnostics in this rare tumor.
Desmoplastic small round cell tumor is a very rare malignancy. We report the case of a 26-year-old woman who suffered from dyspepsia and abdominal pain for 2 months. We performed an endoscopic biopsy of the duodenal mass and diagnosed her disease as desmoplastic small round cell tumor using immunohistochemical staining, fluorescence in situ hybridization, and reverse transcriptase polymerase chain reaction. Because the mass invaded the pancreas and superior mesenteric vein as well as duodenum and the disease was disseminated to liver and peritoneum, she received palliative chemotherapy using vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide. The maximal response to chemotherapy was stable disease. The patient expired 9 months after diagnosis.
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Kim et al. (2009) conducted a case report in Desmoplastic small round cell tumor (n=1). Palliative chemotherapy (VAC/IE and VIP) was evaluated on Tumor response and overall survival. Palliative chemotherapy in a 26-year-old woman with desmoplastic small round cell tumor achieved a maximal response of stable disease, with the patient expiring 9 months after diagnosis.
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