Multiple gastrointestinal stromal tumors and synchronous ileal carcinoids.

BACKGROUND: A 74-year-old African-American male presented with a 3-day history of hematemesis and melena. The patient reported no abdominal pain, constitutional symptoms, bright red blood per rectum, constipation, or diarrhea. His physical examination and medical history were unremarkable except for benign prostatic hypertrophy. An esophagogastroduodenoscopy showed a 3 x 2 x 2 cm smooth round mass in the cardia, 2 cm distal to the gastroesophageal junction. Biopsy of the mass revealed an ulcerated tumor composed of spindle cells. Immunohistochemistry showed positive staining for a number of biochemical markers, including KIT, Ki-67 and smooth muscle actin, but was negative for the markers S100 and desmin. A gastric-wedge resection revealed an ulcerated 4.5 cm mass in the stomach, and exploration of the abdomen revealed two ileal carcinoid tumors, jejunal diverticula and reactive mesenteric lymphadenopathy.

INVESTIGATIONS: Esophagogastroduodenoscopy, biopsy, CT scan, immunohistochemistry, DNA microarray analysis and quantitative reverse transcriptase-PCR.

DIAGNOSIS: Multiple gastrointestinal stromal tumors occurring concomitantly with ileal carcinoids.

MANAGEMENT: Gastric-wedge resection and segmental resection.