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Multiple gastrointestinal stromal tumors and synchronous ileal carcinoids

Nature Clinical Practice Oncology volume 2, pages 166–170 (2005)Cite this article

Abstract

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 × 2 × 2cm 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 4.5cm 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.

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Figure 1: Results from the CT scan.
Figure 2: The ulcerated mass as revealed by gastric-wedge resection.
Figure 3
Figure 4: Hematoxylin and eosin staining of the large gastrointestinal stromal tumor.

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Authors and Affiliations

  1. M Buragas is studying for an MD, M Kidd is a Post-doctoral Fellow, I Modlin is a Professor and C Cha is an Assistant Professor at the Department of Surgery, Yale University School of Medicine, New Haven, CT, USA

    Michele Buragas, Mark Kidd, Irvin M Modlin & Charles Cha

Authors
  1. Michele Buragas
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  2. Mark Kidd
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  3. Irvin M Modlin
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  4. Charles Cha
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Corresponding author

Correspondence to Charles Cha.

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Competing interests

The authors declare no competing financial interests.

Glossary

C-KIT

A proto-oncogene that encodes the cell membrane tyrosine kinase growth factor receptor, KIT

CHROMOGRANIN A

A gastric neuroendocrine cell marker usually absent in gastrointestinal stromal tumors

CD117

A cell surface antigen, which is expressed in mast cells and is an epitope of the tyrosine kinase growth factor receptor, KIT

INTERSTITIAL CELLS OF CAJAL

The gastrointestinal pacemaker cells that regulate intestinal motility and peristalsis

SECRETORY GRANULE NEUROENDOCRINE PROTEIN 1

A marker of neuronal and neuroendocrine cells; also known as the 7B2 protein

NEUROFIBROMATOSIS TYPE I (VON RECKLINGHAUSEN DISEASE)

An autosomal dominant disease characterized by neurofibromas and by spots on the skin

PDGFRA

Platelet-derived growth factor receptor A

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Buragas, M., Kidd, M., Modlin, I. et al. Multiple gastrointestinal stromal tumors and synchronous ileal carcinoids. Nat Rev Clin Oncol 2, 166–170 (2005). https://doi.org/10.1038/ncponc0108

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  • DOI: https://doi.org/10.1038/ncponc0108

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