Duodenal carcinoids are rare, and there is no consensus on the optimal extent of surgical treatment.

[1]
In a retrospective review of 24 patients with a pathologic diagnosis of duodenal carcinoid tumor, most
tumors (89%) measured smaller than 2 cm in diameter, and most (85%) were limited to the mucosa or
submucosa. Lymph node metastases were identified in surgical specimens in 7 (54%) of 13 patients in
whom lymph nodes were examined, including 2 patients with tumors smaller than 1 cm, which were
limited to the submucosa. At a mean follow-up of 46 months, the disease-specific survival rate was
100%, and only 2 patients had recurrences in regional lymph nodes. No patient was reported to have
distant metastases or the carcinoid syndrome.[1][Level of evidence: 3iiB] The authors of this study
concluded that, although duodenal carcinoids are indolent, the presence of regional lymph node
metastases cannot be predicted reliably on the basis of tumor size or depth of invasion, and their impact
on survival is unclear.

In general, endoscopic excision of primary duodenal carcinoids appears to be most appropriate for
tumors smaller than 1 cm.[1] Duodenal carcinoids smaller than 2 cm may be excised locally; for tumors
between 1 cm and 2 cm, complete resection is ensured by operative full-thickness excision.[1,2] Follow-
up endoscopy is indicated. Tumors larger than 1 cm may be difficult to remove completely
endoscopically and should be evaluated with endoscopic ultrasonography before endoscopic resection is
attempted because of their potential to invade beyond the submucosa.[3]

Appropriate management of tumors larger than 2 cm can be problematic.[2] However, in general, these
tumors can be treated with operative full-thickness excision and regional lymphadenectomy.
Lymphadenectomy is performed even in the face of negative preoperative imaging because of the high
rate of lymph node metastasis for these tumors.[1]

In addition, some authors recommend that for tumors larger than 2 cm, a regional lymphadenectomy
includes the lymph nodes in the following locations:

Posterior to the duodenum and pancreatic head and anterior to the inferior vena cava.

Posterolateral to the bile duct and portal vein.

Anterior to the common hepatic artery.[1,4]

Regardless of the size of the primary tumor, abnormal lymph nodes detected on pretreatment imaging
studies or at the time of surgery should be resected. Because little is known about the natural history of
unresected, grossly evident lymph node metastases, nonoperative management might otherwise be
supported. Node-positive patients should undergo continued radiographic surveillance regardless of the
size of the primary tumor.[1]

Ampullary and periampullary duodenal carcinoids deserve special consideration because they differ
clinically, histologically, and immunohistochemically from carcinoid tumors that occur elsewhere in the
duodenum.[5] Although their rarity precludes the establishment of any definitive natural history, these
tumors appear to behave unpredictably and might be viewed as a distinct category of carcinoid tumor
when treatment options are being considered.[2] Compared with tumors in other duodenal sites, even
small (<1 cm) ampullary and periampullary carcinoid tumors exhibit distinctly different aggressive
behavior, and they may metastasize early.[5,6]

Current Clinical Trials

Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling
patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and
other criteria. General information about clinical trials is also available.

References

Mullen JT, Wang H, Yao JC, et al.: Carcinoid tumors of the duodenum. Surgery 138 (6): 971-7; discussion
977-8, 2005. [PUBMED Abstract]

Zyromski NJ, Kendrick ML, Nagorney DM, et al.: Duodenal carcinoid tumors: how aggressive should we
be? J Gastrointest Surg 5 (6): 588-93, 2001 Nov-Dec. [PUBMED Abstract]

Yoshikane H, Tsukamoto Y, Niwa Y, et al.: Carcinoid tumors of the gastrointestinal tract: evaluation with
endoscopic ultrasonography. Gastrointest Endosc 39 (3): 375-83, 1993 May-Jun. [PUBMED Abstract]

Modlin IM, Latich I, Kidd M, et al.: Therapeutic options for gastrointestinal carcinoids. Clin Gastroenterol
Hepatol 4 (5): 526-47, 2006. [PUBMED Abstract]

Makhlouf HR, Burke AP, Sobin LH: Carcinoid tumors of the ampulla of Vater: a comparison with duodenal
carcinoid tumors. Cancer 85 (6): 1241-9, 1999. [PUBMED Abstract]

Hatzitheoklitos E, Büchler MW, Friess H, et al.: Carcinoid of the ampulla of Vater. Clinical characteristics
and morphologic features. Cancer 73 (6): 1580-8, 1994. [PUBMED Abstract]

https://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq

12.13 11-04-2019