Woman with diarrhea and abnormal EGD.

Contributed from the case records of the  Indiana University Department of Radiology

Woman with diarrhea and abnormal EGD.

Woman with diarrhea and abnormal EGD.

Woman with diarrhea and abnormal EGD.

• Carcinoid tumor with metastasis
• Sclerosing mesenteritits
• Intestinal metastases and lymphoma
• Desmoid
• Small bowel carcinoma

Woman with diarrhea and abnormal EGD.

• Background:
  • Usually occur in 50s or 60s, Male>Female (2 to 1)
  • Rare )<2% of GI tumors)
  • Neuroendocrine tumor, primary malignant neoplasm of small bowel arises from enterochromaffin cells of Kulchisky
  • 85% of all carcinoid tumors are from GI tract: Appendix > ileum > duodenum > other
  • Next most common origin is the tracheobronchial system
  • Appendiceal carcinoids are usually incidental finding at appendectomy
  • Most common primary small bowel tumor beyond the ligament of Treitz
  • Second most common small bowel tumor (after adenocarcinoma)
  • 90% of small bowel carcinoids arise in distal ileum
  • Can be associated with MEN1
  • 40-80% of small bowel carcinoids are metastatic to mesentery at time of clinical diagnosis
• Presentation:
  • Mostly asymptomatic or may be symptomatic for years before diagnosis made
  • Carcinoid syndrome (episodic flushing, diarrhea, asthma, pain, and right heart failure)
    • Can be misdiagnosed for years
    • Implies liver metastasis, with systemic circulation of secretory factors produced by the tumor (e.g., serotonin, histamine, dopamine, somatostatin, vasoactive intestinal polypeptide, and substance P)
  • Additional signs/symptoms: abdominal pain secondary to intestinal ischemia, right heart failure and murmurs from valve defects
• Laboratory:
  • Increased srotonin or 5-hydroxtryptophan levels or 24 hours urine 5-HIAA levels
• Imaging:
  • More difficult to detect the primary than metastatic disease
  • CT:
    • Primary tumor: solitary or multiple, submucosal, well-defined enhancing lesion
    • Mesenteric extension of tumor: heterogeneous mesenteric mass, calcifications (70%) with mesenteric fibrosis and desmoplastic reaction (spiculations, finger-like projections of mass and tethering, retraction of small bowel loops), may encase and narrow mesenteric vessels
    • Liver metastasis: intense enhancement on arterial phase with iso- or hypo dense appearance on delayed imaging
  • Nuclear Medicine:
    • Somatostatin-receptor scintigraphy is study of choice in management of patients with carcinoid
    • Indium-111 Octreotide/Pentetreotide is most commonly used agent
    • Positive study for GI carcinoids and liver metastasis
• Prognosis:
  • Excellent prognosis with surgical resection of the small bowel carcinoid if there is no nodal or liver metastases
  • 5-year survival rate is about 50% with liver metastasis, but can be prolonged with treatment of the liver metastasis (such as with transcatheter chemoembolization)
• Treatment:
  • Small bowel tumors: Surgical resection of bowel and mesentery
  • Liver metastasis: palliative surgery of primary tumor, and surgical resection of the liver segment/lobe or chemoembolization
  • Symptomatic relief with octreotide (somatostatin analogue)
  • Systemic chemotherapy (for bone metastasis)

1. Federle, Michael P. Statdx.com. Diagnosis: Carcinoid, accessed on August 28, 2011.
2. Levy, Angela D. From the Archives of the AFIP: Gastrointestinal Carcinoids: Imaging Features with Clinicopathologic Comparison." Radiographics. 27:237-257. January – February 2007.

Woman with diarrhea and abnormal EGD.