Primary Extragastrointestinal Stromal Tumor of the Pleura: Report of a Unique Case With Genetic Confirmation
- 1 June 2010
- journal article
- case report
- Published by Ovid Technologies (Wolters Kluwer Health) in The American Journal of Surgical Pathology
- Vol. 34 (6), 907-912
- https://doi.org/10.1097/pas.0b013e3181d9f18f
Abstract
Gastrointestinal stromal tumors (GISTs), the most common mesenchymal neoplasms of the tubular gastrointestinal tract, usually originate in the wall of the stomach or small intestine. Most GISTs harbor oncogenic mutations in either the KIT or platelet-derived growth factor receptor alpha (PDGFRA) tyrosine kinase receptor genes and show differentiation along the lines of the interstitial cells of Cajal. Rarely, GISTs arise primarily in the omentum, mesentery, or retroperitoneum, at which sites they are referred to as "extragastrointestinal stromal tumors" (EGISTs). However, primary intrathoracic GIST arising in the pleura or lung has not been previously reported. We describe herein, a 62-year-old male who presented with a pleural-based mass unrelated to the esophagus that was morphologically typical of a spindle-cell GIST, showing strong immunoreactivity for KIT and DOG1, and harboring an exon 11 mutation in KIT. Ten years after resection, the tumor recurred as multiple masses in the pleura and mediastinum and was marginally reexcised. The patient was then treated with adjuvant imatinib mesylate with no evidence of further recurrences 13 months later. This seems to be the first EGIST arising above the diaphragm. This case shows a potential diagnostic pitfall with therapeutic consequences.This publication has 21 references indexed in Scilit:
- Interstitial cells of Cajal are present in human extrahepatic bile ductsJournal of Gastroenterology and Hepatology, 2010
- A Single-Institution Experience with Eight CD117-Positive Primary Extragastrointestinal Stromal Tumors: Critical Appraisal and a Comparison with Their Gastrointestinal CounterpartsJournal of Gastrointestinal Surgery, 2009
- Monoclonal Antibody DOG1.1 Shows Higher Sensitivity Than KIT in the Diagnosis of Gastrointestinal Stromal Tumors, Including Unusual SubtypesThe American Journal of Surgical Pathology, 2009
- Correlation of Kinase Genotype and Clinical Outcome in the North American Intergroup Phase III Trial of Imatinib Mesylate for Treatment of Advanced Gastrointestinal Stromal Tumor: CALGB 150105 Study by Cancer and Leukemia Group B and Southwest Oncology GroupJournal of Clinical Oncology, 2008
- KIT Gene Mutations and Copy Number in Melanoma SubtypesClinical Cancer Research, 2008
- A Novel Monoclonal Antibody Against DOG1 is a Sensitive and Specific Marker for Gastrointestinal Stromal TumorsThe American Journal of Surgical Pathology, 2008
- Interstitial Cajal-like cells in human gallbladderJournal of Molecular Histology, 2007
- KIT mutations and dose selection for imatinib in patients with advanced gastrointestinal stromal tumoursEuropean Journal of Cancer, 2006
- KIT Mutations Are Common in Incidental Gastrointestinal Stromal Tumors One Centimeter or Less in SizeThe American Journal of Pathology, 2002
- Immunohistochemical Staining for KIT (CD117) in Soft Tissue Sarcomas Is Very Limited in DistributionAmerican Journal of Clinical Pathology, 2002