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.
Subject(s)
Gastrointestinal Stromal Tumors/genetics , Gastrointestinal Stromal Tumors/pathology , Pleural Neoplasms/genetics , Pleural Neoplasms/pathology , Proto-Oncogene Proteins c-kit/genetics , Anoctamin-1 , Antineoplastic Agents/therapeutic use , Atrial Fibrillation/complications , Benzamides , Chloride Channels , Depression/complications , Gastrointestinal Stromal Tumors/therapy , Humans , Hypercholesterolemia/complications , Hypertension/complications , Imatinib Mesylate , Immunohistochemistry , Male , Membrane Proteins/biosynthesis , Middle Aged , Mutation , Neoplasm Proteins/biosynthesis , Neoplasm Recurrence, Local/drug therapy , Neoplasm Recurrence, Local/pathology , Piperazines/therapeutic use , Pleural Neoplasms/therapy , Polymerase Chain Reaction , Proto-Oncogene Proteins c-kit/metabolism , Pyrimidines/therapeutic useABSTRACT
BACKGROUND: Neurobehavioral impairment is a common complication of coronary bypass surgery. Cerebral microemboli during cardiopulmonary bypass (CPB) are a principal mechanism of cognitive injury. The aim of this work was to study the occurrence of cerebral embolism during CPB and to evaluate the effectiveness of evidence-based CPB circuit component and process changes on the exposure of the patient to emboli. METHODS AND RESULTS: M-Mode Doppler was used to detect emboli in the inflow and outflow of cardiopulmonary circuit and in the right and left middle cerebral arteries. Doppler signals were merged into a single display to allow real-time associations between discrete clinical techniques and emboli detection. One hundred sixty-nine isolated coronary artery bypass grafting (CABG) patients were studied between 2002 and 2008. There was no statistical difference in median microemboli detected in the inflow of the CPB circuit, (Phase I, 931; Phase II, 1214; Phase III, 1253; Phase IV, 1125; F [3,158]=0.8, P=0.96). Significant changes occurred in median microemboli detected in the outflow of the CPB circuit across phases, (Phase I, 702; Phase II, 572; Phase III, 596; Phase IV, 85; F [3,157]=13.1, P<0.001). Significant changes also occurred in median microemboli detected in the brain across phases, (Phase I, 604; Phase II, 429; Phase III, 407; Phase IV, 138; F [3,153]=14.4, P<0.001). Changes in the cardiopulmonary bypass circuit were associated with an 87.9% (702 versus 85) reduction in median microemboli in the outflow of the CPB circuit (P<0.001), and a 77.2% (604 versus 146) reduction in microemboli in the brain (P<0.001). CONCLUSIONS: Changes in CPB techniques and circuit components, including filter size and type of pump, resulted in a reduction in more than 75% of cerebral microemboli.
