Minute ampullary carcinoid tumor with lymph node metastases: a case report and review of literature

Abstract

Background: Carcinoid tumors are usually considered to have a low degree of malignancy and show slow progression. One of the factors indicating the malignancy of these tumors is their size, and small ampullary carcinoid tumors have been sometimes treated by endoscopic resection.

Case presentation: We report a case of a 63-year-old woman with a minute ampullary carcinoid tumor that was 7 mm in diameter, but was associated with 2 peripancreatic lymph node metastases. Mild elevation of liver enzymes was found at her regular medical check-up. Computed tomography (CT) revealed a markedly dilated common bile duct (CBD) and two enlarged peripancreatic lymph nodes. Endoscopy showed that the ampulla was slightly enlarged by a submucosal tumor. The biopsy specimen revealed tumor cells that showed monotonous proliferation suggestive of a carcinoid tumor. She underwent a pylorus-preserving whipple resection with lymph node dissection. The resected lesion was a small submucosal tumor (7 mm in diameter) at the ampulla, with metastasis to 2 peripancreatic lymph nodes, and it was diagnosed as a malignant carcinoid tumor.

Conclusion: Recently there have been some reports of endoscopic ampullectomy for small carcinoid tumors. However, this case suggests that attention should be paid to the possibility of lymph node metastases as well as that of regional infiltration of the tumor even for minute ampullary carcinoid tumors to provide the best chance for cure.

Figure 1

Contrast-enhanced CT shows the markedly dilated CBD and 2 enlarged lymph nodes in the peripancreatic region. (a) The marked dilated CBD (arrow) and one of 2 enlarged lymph nodes near the upper border of the pancreas (arrow head) are detected. (b) Another enlarged lymph node near the lower border of the pancreas (arrow head) is found.

Figure 2

Endoscopy shows a slightly enlarged ampullary region, suggesting the existence of a submucosal tumor because the epithelium has a normal appearance.

Figure 3

ERCP shows severe stenosis of the distal portion of the CBD and marked proximal dilation. The main pancreatic duct is not dilated.

Figure 4

(a) The resected specimen contains a small yellowish submucosal tumor (approximately 7 mm in diameter) located at the ampulla of Vater (arrow). (b) Monotonous tumor cells with small round nuclei are seen (hematoxylin and eosin staining, × 400). (c) Carcinoid tumor cells within a peripancreatic lymph node (× 200). (d) The tumor cells are positive for synaptophysin, a neuroendocrine marker (× 40). (e) Endolymphatic tumor emboli are shown by staining with D2-40 antibody (× 400). (f) Positive staining for MIB-1 antibody is seen in approximately 3.2% of the tumor cell nuclei (× 400).