Results of multimodal treatment for desmoplastic small round cell tumors

Presented at the 35th Annual Meeting of the American Pediatric Surgical Association, Ponte Vedra, Florida, May 27-30, 2004.
https://doi.org/10.1016/j.jpedsurg.2004.09.046Get rights and content

Abstract

Purpose:

Desmoplastic small round cell tumors (DSRCTs) are rare aggressive neoplasms that frequently present with large symptomatic intraabdominal masses. We examined the effects of multimodal therapy including induction chemotherapy, aggressive surgical debulking, and external beam radiotherapy on patients with DSRCT.

Methods:

Institutional Review Board permission was obtained. Sixty-six patients were diagnosed by histology, immunohistochemistry, and or cytogenetics as having DSRCT at our institution from July 1, 1972, to July 1, 2003. Data were collected on patient demographics, presenting symptoms, tumor location and extent, treatment regimen, and overall survival.

Results:

A majority of patients were male (91%), Caucasian (85%), and with a median age of 19 (7-58) years old at diagnosis. The most common presenting complaint was an intraabdominal mass (64%). In 63 patients (96%), the primary tumor was located in the abdomen or pelvis. Thirty-three (50%) had positive lymph nodes and 27 (41%) had distant parenchymal metastases at diagnosis. Overall, 3- and 5-year survivals were 44% and 15%, respectively. Twenty-nine of these patients (44%) underwent induction chemotherapy (P6), surgical debulking, and radiotherapy. Three-year survival was 55% in those receiving chemotherapy, surgery, and radiotherapy vs 27% when all 3 modalities were not used (P < .02). Gross tumor resection was highly significant in prolonging overall survival; 3-year survival was 58% in patients treated with gross tumor resection compared to no survivors past 3 years in the nonresection cohort (P < .000 01). Ten patients (15%) have no evidence of disease with a median follow-up of 2.4 years (range, 0.4-11.2 years).

Conclusions:

Multimodal therapy results in improved survival in patients with DSRCT. Aggressive surgical resection of these extensive intraabdominal neoplasms correlates with improved patient outcome.

Section snippets

Materials and methods

After approval was obtained from the Institutional Review Board in compliance with The Health Insurance Portability and Accountability Act, a retrospective review of all patients with DSRCT treated at Memorial Sloan-Kettering Cancer Center from July 1, 1972, to July 1, 2003 was compiled. Sixty-six patients were identified, 40 of whom had been previously reported. Diagnosis of DSRCT was established by histology, immunochemistry, and/or cytogenetics. The median length of follow-up was 2.0 years

Patient characteristics

The study included 60 male (91%) and 6 female (9%) patients. A majority of patients were Caucasian (85%); the remainder were Hispanic (9%) and African American (6%). The median age at diagnosis was 19 years (range, 7-58 years). Sixty-three patients (96%) had their primary tumor located in the abdomen or pelvis; other sites included the thoracic cavity (1) and testicle (2). Local or distant lymph node involvement was seen in 33 (50%) patients at presentation. Twenty-seven (41%) patients

Discussion

Desmoplastic small round cell tumor is an aggressive malignancy with few long-term survivors. Due to the diffuse serosal spread of DSRCT, systemic chemotherapy utilizing the P6 regimen has been the cornerstone of initial treatment. Debulking surgery is then attempted with a goal of at least 90% reduction of tumor bulk. Aggressive surgical resection continues to be a major determinant in patient survival. With continued follow-up of these patients, surgical resection greater than 90%, P6

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