Asymptomatic Ileal Carcinoid Tumors Are Common, But Intussusception Strongly Suggests Malignancy
In adults presenting with ileal intussusception on CT, a carcinoid tumor is highly likely to be the underlying cause, and while many ileal carcinoids can be asymptomatic for years, the presence of intussusception itself represents a symptomatic manifestation requiring urgent surgical intervention.
Prevalence of Asymptomatic Ileal Carcinoids
The true burden of neuroendocrine tumors far exceeds what is diagnosed clinically:
- Autopsy studies reveal that gastrointestinal NETs are substantially more common than detected during life, indicating many patients harbor these tumors asymptomatically 1
- In surgical series, 60% of all gastrointestinal carcinoid tumors were discovered incidentally as asymptomatic lesions 2
- However, among patients specifically with ileal carcinoid tumors, 48% were symptomatic at presentation 2, making ileal primaries more likely to cause symptoms compared to other GI sites
- There is typically a 7-year delay between first symptoms and diagnosis, suggesting prolonged periods of minimal or non-specific symptoms 1
Clinical Context of Intussusception
Your patient's presentation changes the clinical picture significantly:
- Adult intussusception is pathologic in 90% of cases, with an identifiable structural lesion serving as the lead point 3
- Carcinoid tumors are a recognized cause of adult ileocecal intussusception 4, 3, though less common than adenocarcinoma in colonic intussusception
- The intussusception itself represents a symptomatic complication of the tumor, even if the patient had no prior carcinoid-specific symptoms (flushing, diarrhea) 4
Metastatic Potential at Presentation
Ileal carcinoids have aggressive metastatic behavior that is often clinically silent:
- Ileal carcinoids demonstrate 60% nodal metastases and 30% liver metastases at diagnosis 5, the highest rates among GI carcinoids
- Critically, 18% of ileal carcinoids smaller than 1 cm already have metastases 2, emphasizing that tumor size does not predict benign behavior
- Carcinoid syndrome occurs in only 20% of ileal NET cases 1, meaning 80% lack the classic hormonal symptoms even with advanced disease
- The syndrome typically requires liver metastases to manifest, as hepatic first-pass metabolism normally clears vasoactive substances 1
Diagnostic and Management Algorithm
For your patient with ileal intussusception on CT:
Proceed directly to surgical exploration - adult intussusception requires resection without attempted reduction due to high malignancy risk 3
Expect to find a carcinoid tumor - this is a well-documented cause of ileocecal intussusception in adults 4, 3
Perform extended resection with wide mesenteric lymph node sampling - given the 60% rate of nodal involvement in ileal primaries 5
Obtain intraoperative assessment for mesenteric disease and liver metastases 4
Send tissue for immunohistochemistry (chromogranin A, synaptophysin) and Ki-67 proliferation index for WHO grading 6
Postoperative biochemical evaluation with 5-HIAA and chromogranin A to assess for residual disease 4
Critical Pitfalls to Avoid
- Do not assume the tumor is benign based on absence of carcinoid syndrome - 80% of ileal NETs lack hormonal symptoms 1
- Do not rely on tumor size alone - even sub-centimeter ileal carcinoids metastasize in 18% of cases 2
- Do not attempt radiologic or endoscopic reduction - formal surgical resection is required for adult intussusception due to high malignancy rates 3
- Do not overlook synchronous malignancies - 20% of NET patients develop other cancers, one-third in the GI tract 1