1097-0142/asset/cover.gif?v=1&s=a7299bc18f075294c232ade468773cd0672bd470 "Cancer")
Fax: (901) 521-9005
1097-0142/asset/olbannerleft.gif?v=1&s=ca681f5719430b26e1bc15e9ea4c9fc0a7110104)
1097-0142/asset/olbannerright.gif?v=1&s=8142566facf7e76aef9be6c51162a2e920b3b9f9)
Review Article
You have full text access to this OnlineOpen article
Pediatric gastrointestinal stromal tumors and leiomyosarcoma
The St. Jude Children's Research Hospital experience and a review of the literature
Article first published online: 28 MAY 2004
DOI: 10.1002/cncr.20352
Copyright © 2004 American Cancer Society
Additional Information
How to Cite
Cypriano, M. S., Jenkins, J. J., Pappo, A. S., Rao, B. N. and Daw, N. C. (2004), Pediatric gastrointestinal stromal tumors and leiomyosarcoma. Cancer, 101: 39–50. doi: 10.1002/cncr.20352
Publication History
- Issue published online: 18 JUN 2004
- Article first published online: 28 MAY 2004
- Manuscript Accepted: 15 APR 2004
- Manuscript Revised: 9 APR 2004
- Manuscript Received: 30 DEC 2003
Funded by
- United States Public Health Service Cancer Center. Grant Number: CA-21765
- Childhood Solid Tumor Program Project. Grant Number: CA-23099
- American Lebanese Syrian Associated Charities (ALSAC)
- Abstract
- Article
- References
- Cited By
Keywords:
- children;
- c-kit;
- gastrointestinal stromal tumors;
- gastrointestinal autonomic nerve tumors;
- imatinib mesylate;
- KIT;
- leiomyosarcoma
Gastrointestinal stromal tumors (GISTs) and leiomyosarcomas (LMSs) rarely affect pediatric patients. Surgery was the treatment of choice for both entities, and tumor resectability was a key prognostic factor. KIT staining helped to distinguish GISTs from LMSs.
Abstract
BACKGROUND
With the introduction of molecularly targeted therapy for gastrointestinal stromal tumors (GISTs), it became important to distinguish GISTs from leiomyosarcomas (LMSs). The authors sought to characterize the clinicopathologic features of these tumors in pediatric patients.
METHODS
The authors reviewed the medical records of 11 patients for whom GIST or LMS was diagnosed between March 1962 and July 2002 at St. Jude Children's Research Hospital and reclassified the tumors according to current histologic and immunophenotypic criteria. The authors also reviewed the literature pertaining to pediatric GISTs and LMSs.
RESULTS
Seven patients had GISTs, and four had LMS. The median age of the patients at diagnosis was 11.5 years. At diagnosis, metastases were present in one patient with GISTs and in another with LMS. Unlike the focal distribution of CD117 (KIT) in LMS, diffuse and strong immunostaining was observed in GISTs. Only GISTs expressed CD34. Six patients underwent complete resection (four with GISTs and two with LMS), four patients underwent incomplete resection (three with GISTs and one with LMS), and one patient (with LMS) underwent a biopsy only. Radiotherapy or chemotherapy was used to treat one patient with GISTs and three patients with LMS. One patient with a high-risk GIST (largest dimension of 32 cm and high mitotic count) was treated with adjuvant imatinib mesylate outside the preferred setting of a clinical trial, due to concerns regarding the high risk of tumor recurrence. Four patients with GISTs and two with LMS survived median disease-free a median of 10.4 years and 4.3 years after diagnosis, respectively. Tumors in all but one survivor were completely resected.
CONCLUSIONS
KIT staining helped to distinguish GISTs from LMSs. Surgery was the treatment of choice for both entities, and tumor resectability was a key prognostic factor. Cancer 2004. © 2004 American Cancer Society.