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Intraabdominal desmoplastic small round cell tumor presenting as a gastric mural mass with hepatic metastases.

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OBJECTIVE An atypical case of childhood intraabdominal desmoplastic small round cell tumor (DSRCT) is presented. METHODS An 11-year-old boy presented with progressive nausea and vomiting, abdominal pain, hepatomegaly, and an epigastric mass. Computed tomographic scanning as well as findings at

Requirement for novel surgical and clinical protocols for the treatment of desmoplastic small round cell tumor: A report of two cases and a review of the literature.

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Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive neoplasm that was initially described in 1989. DSRCT predominantly affects young men and typically occurs in the intra-abdominal area. The present study describes the cases of two patients with DSRCT. The first patient was a

The use of interval-compressed chemotherapy with the addition of vincristine, irinotecan, and temozolomide for pediatric patients with newly diagnosed desmoplastic small round cell tumor

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Background: Desmoplastic small round cell tumor (DSRCT) is a rare aggressive sarcoma that affects children and young adults, and portends poor outcomes despite intensive multimodal treatment approaches. We report toxicity, response, and

Diffuse intraabdominal desmoplastic small round cell tumor: a ten-year experience.

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BACKGROUND Intraabdominal desmoplastic small round cell tumors (IDSRCT) are rare in children and predominantly affect male adolescents and young adults. We present our experience in the management of five children with diffuse IDSRCT, managed with aggressive chemotherapy, surgery, radiotherapy and

Whole abdominopelvic intensity-modulated radiation therapy for desmoplastic small round cell tumor after surgery.

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OBJECTIVE Desmoplastic small round cell tumor (DSCRT) is an uncommon pediatric tumor with a poor prognosis. Aggressive multimodality therapy is the current treatment approach; however. treatment toxicity is of concern. We report our results with whole abdominopelvic intensity-modulated radiation

Desmoplastic small round cell tumor with primary ovarian involvement: case report and review.

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BACKGROUND Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm that has recently been characterized. It has not been associated with a primary visceral organ. In women, cases are even more rare and often have some ovarian involvement. METHODS An 11-year-old girl

A phase I trial and pharmacokinetic study of a 24-hour infusion of trabectedin (Yondelis®, ET-743) in children and adolescents with relapsed or refractory solid tumors.

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BACKGROUND The objectives of this phase I study were to determine the maximum tolerated dose (MTD), toxicity profile, and pharmacokinetics of a 24-hour continuous intravenous infusion of trabectedin administered to children and adolescents with refractory or relapsed solid tumors. METHODS Patients

Sclerosing Encapsulating Peritonitis in a Pediatric Patient Treated With Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy

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Background: Sclerosing encapsulating peritonitis (SEP) is a rare chronic inflammatory condition characterized by small bowel encapsulation by a thick fibrocollagenous membrane. Patients with SEP often present with nonspecific symptoms,

Pediatric phase I trial and pharmacokinetic study of the platelet-derived growth factor (PDGF) receptor pathway inhibitor SU101.

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OBJECTIVE To determine the maximum tolerated dose and the toxicity profile of the PDGF receptor pathway inhibitor SU101 in pediatric patients with refractory solid tumors, and to define the plasma pharmacokinetics of SU101 and its active metabolite SU0020 in children. METHODS Patients between 3 and

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