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Volume 7, Issue 7, July 2009 1Arizona Cancer Center, University of Arizona, Tucson, Arizona; 2Ironwood Cancer and Research Center, Chandler, Arizona [ Advanced Desmoplastic Small Round Cell Tumor: Near Complete Response with Trastuzumab-based Chemotherapy]

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  • DSRCT Articles
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  • Volume 7, Issue 7, July 2009 1Arizona Cancer Center, University of Arizona, Tucson, Arizona; 2Ironwood Cancer and Research Center, Chandler, Arizona [ Advanced Desmoplastic Small Round Cell Tumor: Near Complete Response with Trastuzumab-based Chemotherapy]
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  • Volume 7, Issue 7, July 2009 1Arizona Cancer Center, University of Arizona, Tucson, Arizona; 2Ironwood Cancer and Research Center, Chandler, Arizona [ Advanced Desmoplastic Small Round Cell Tumor: Near Complete Response with Trastuzumab-based Chemotherapy] Case Report Our patient, a 47-year-old Caucasian male, initially presented with complaints of progressive constipation. Computed tomography (CT) of the abdomen revealed a mass. At surgery, this was observed to be located in the mesentery involving the small intestine near the aorta, making it unresectable. Pathologic examination revealed a malignant neoplasm consisting of small blue cells in solid sheets with patchy necrosis and desmoplastic response. The tumor cells were positive by immunohistochemistry (IHC) for placental alkaline phosphatase (PLAP), vimentin, epithelial membrane antigen (EMA), desmin, and cytokeratin AE1/AE3 (focal), and negative for CD117 (c-kit), CD3, CD20, CD30, myogenin, and all other actins. The constellation of findings was consistent with a diagnosis of desmoplastic small round cell tumor (DSRCT). Conclusion We here report a marked response to trastuzumab-based therapy in a case of DSRCT expressing Her-2/neu. Other treatment strategies directed at Her-2/neu, such as the tyrosine kinase inhibitor lapatinib (Tykerb, GlaxoSmithKline), may be relevant in the primary or secondary therapy of patients with Her-2/neu-positive DSRCT.21 More formal investigation of Her-2/neu-based therapy in DSRCT is warranted.
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