Clinical Advances in Hematology & Oncology 2009

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.

Sarcoma Journal 2008

Volume 2008, Article ID 261589, 9 pages doi:10.1155/2008/261589


Outpatient and Home Chemotherapy with Novel Local Control Strategies in Desmoplastic Small Round Cell Tumor

DollyAguilera, 1AndreaHayes-Jordan, 2PeterAnderson,1 ShiaoWoo,3 MargaretPearson,1 andHollyGreen2

1 Department of Pediatrics, University of Texas MDAnderson Cancer Center,Houston,TX77030, USA 2 Department of Surgical Oncology, University of Texas MDAnderson Cancer Center, Houston,TX 77030, USA 3 Department of Radiation Oncology, University of Texas MDAnderson Cancer Center,Houston,TX 77030, USA

Correspondence should be addressed to Peter Anderson,

Received 14 December 2007; Accepted 29 April 2008

Desmoplastic Small Round Cell Tumor (DSRCT) has a very poor prognosis. This report illustrates novel chemotherapy and local control interventions in a 5-year old patient. The patient was treated in the outpatient setting, achieved remission, with excellent quality of life. The patient presented with massive ascites and >1000 abdominal tumors. Neoadjuvant chemotherapy included vincristine (1.5mg/m2), ifosfamide (3 g/m2 /day ×3), dexrazoxane/doxorubicin (750/75 mg/m2), and etoposide (150 mg/m2). Continuous hyperthermic peritoneal perfusion (CHPP) with cisplatin (100 mg/m2) was given after extensive cytoreductive surgery. This was followed by irinotecan (10 mg/m2/day ×5 ×2 weeks) + temozolomide monthly ×2, then abdominal radiation 30 Gy with simultaneous temozolomide (100 mg/m2/day ×5). A total of 12 cycles of irinotecan and temozolamide were given. Except for initial chemotherapy, subsequent courses were in the outpatient setting. Focal retroperitoneal relapse at 18 months was treated with IMRT with bevacizumab (5 mg/kg) and 2 perihepatic metastases with radio frequency ablation/cryoablation followed by chronic outpatient maintenance chemotherapy (valproic acid, cyclophosphamide, and rapamycin). Almost 2 years from diagnosis, the patient maintained an excellent quality of life. This is a novel approach to the treatment of children with massive abdomino-pelvic DSRCT.

Copyright © 2008 Dolly Aguilera et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.