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Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 161-163
Peri-ampullary collision tumor - high grade neuroendocrine carcinoma and signet ring cell carcinoma: A case report and review of literature

Rajiv Gandhi Cancer Institute and Research Centre, Sector-5, Rohini, Delhi - 110 085, India

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Date of Web Publication7-Mar-2011


We report a case of 50-year-old male with obstructive jaundice diagnosed as peri-ampullary collision tumor comprising of large cell neuroendocrine carcinoma and signet ring cell carcinoma. The association of neuroendocrine (usually carcinoids) and adenocarcinoma is extremely uncommon with only few case reports available in the reported literature.

Keywords: Collision tumor, neuroendocrine, peri-ampullary

How to cite this article:
Khurana A, Sharma A, Gupta G, Gandhi JS. Peri-ampullary collision tumor - high grade neuroendocrine carcinoma and signet ring cell carcinoma: A case report and review of literature. Indian J Pathol Microbiol 2011;54:161-3

How to cite this URL:
Khurana A, Sharma A, Gupta G, Gandhi JS. Peri-ampullary collision tumor - high grade neuroendocrine carcinoma and signet ring cell carcinoma: A case report and review of literature. Indian J Pathol Microbiol [serial online] 2011 [cited 2022 Jan 18];54:161-3. Available from: https://www.ijpmonline.org/text.asp?2011/54/1/161/77391

   Introduction Top

Pancreatic adenocarcinoma represents 98% of peri-ampullary pancreatic neoplasm. [1] One half of the remaining 2% are carcinoids, who have a better prognosis than the patients with conventional adenocarcinoma. [2] Poorly differentiated endocrine carcinomas, on the other hand, are usually aggressive and prognosis is poor. [2] Due to the confluence of numerous closely connected anatomic structures in this area their precise origin is very difficult to establish.

   Case Report Top

A 50-year-old male, non-diabetic and chronic alcoholic, presented with chief complaints of persistent jaundice for 6 months, along with episodes of intermittent fever and melena of 2 months duration and significant weight loss.

An imaging study of the upper abdomen showed enlarged liver, with normal intra hepatic biliary radicals. The gall bladder was distended, without any evidence of calculus/mass. Common bile duct (CBD) dilatation till lower end and a heterogeneous enhancing soft tissue mass (57 × 43 mm) were seen in relation to the head and uncinate process of pancreas with ill-defined infiltrative margins and loss of the fat planes between the mass and second part of the duodenum. It appeared to invade the peri-ampullary region.

Magnetic resonance imaging cholangiopancreatography (MRCP) revealed altered intensity enhancing irregular wall thickening of peri-ampullary region of D2 segment and enhancing the lower end of CBD at the ampulla with proximal biliary dilatation [Figure 1]a. The Wirsung duct was prominent. In addition a confluent inseparable soft tissue mass, possibly nodal in the pancreatico-duodenal region, abutting and partially effacing D2 and D3, was visualized. It was displacing the pancreas anteriorly, abutting it and had ill-defined planes with the pancreatic head and uncinate process. The CBD was visualized in lateral relation to the nodal mass. Liver parenchyma revealed normal signal intensity. Upper gastrointestinal endoscopy revealed a peri-ampullary growth which was biopsied and was reported as poorly differentiated carcinoma. Routine biochemical studies, showed features of obstructive jaundice with raised serum CEA levels of 33.6 ng/mL (normal range < 3 ng/mL) and elevated serum CA 19.9 level being 242 IU/L (normal range <35.0 IU/L). Subsequently the patient underwent Whipples' procedure.
Figure 1: (a) MRI with contrast showing a peri-ampullary growth at the L2-L3 levels. (b) Gross photograph showing a grey white solid tumor in the peri-ampullary region abutting the second part of duodenum.

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Grossly, the specimen showed an irregular tumor measuring 6×5×4 cm in the head of the pancreas and ampullary region [Figure 1]b. Pancreatic duct was not identified. On sectioning the tumor was grey white and was infiltrating the duodenal wall. Gall bladder and bile ducts were unremarkable, with no calculi.

Microscopic examination of the tumor sections taken from the head of pancreas showed a neoplasm composed of solid nests, ribbons, and cords of malignant cells with high nuclear to cytoplasmic ratio, with salt and pepper chromatin. Mitosis was brisk (>20/10 hpf). Focal areas of necrosis were noted. This tumor was infiltrating the pancreatic parenchyma, duodenal muscularis propria (outer half), and the ampulla. In addition, sections taken from the ampulla of Vater revealed another morphologically distinct lesion composed of a pure population of malignant signet ring cells, diffusely infiltrating ampulla and full thickness of duodenal wall. Mitosis was rare in the latter neoplasm. The two tumors were geographically separate with no areas of transition [Figure 2]. A battery of immunohistochemical markers was applied which proved the lesion to be composed of two distinct tumor components. The solid neoplasm (non-signet ring morphology) showed immunoreactivity with CK7 and neuroendocrine markers (synaptophysin, chromogranin and CD56) [Figure 3]. CK20 and CdX-2 were not expressed. Ki67 index was nearly 60%, suggesting a high proliferation index. The signet ring cell component of the tumor was immunoreactive with CK20 and CdX-2 [Figure 4], whereas CK7 was not expressed; indicating an intestinal origin for signet ring cell carcinoma (SRCC) component. Finally a diagnosis of peri-ampullary collision tumor with a high grade neuroendocrine carcinoma and SRCC as its components was made. Single peri-hepatic node showed metastatic deposit of large cell neuroendocrine carcinoma (LCNEC). Patient received six cycles of etoposide and cisplatin based chemotherapy followed by field radiation for sub-centimeter gastro-hepatic and pre-aortic lymph node. He was symptom free 1 year post completion of therapy.
Figure 2: Photomicrograph showing collision site with left upper half showing signet ring cell carcinoma; right lower half showing neuro-endocrine carcinoma (H and E, ×200).

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Figure 3: Synaptophysin immunostaining showing strong positivity in the solid component and negative in signet ring cell areas (DAB, ×200).

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Figure 4: CdX-2 immunostaining showing nuclear positivity in the signet ring cell areas; solid neuro-endocrine areas being negative (DAB, ×200).

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   Discussion Top

A collision tumor is defined as the simultaneous co-existence of two independent tumors located or not in the same area. The other subtype is the composite variant which originates in a common precursor cell, differentiates in two directions, and usually has a transition zone. The most common malignancy of the peri-ampullary area is adenocarcinoma, which accounts for more than 90% of the tumors. These tumors are more common between 60 and 65 years of age, and predominate in women. [1]

Neuro-endocrine tumors of this region are exceedingly rare (0.4%) and the majority being well-differentiated endocrine tumors (carcinoid). [1] LCNEC occurs in patients in fifth decade and are predominate in males. According to WHO classification (2000), neuroendocrine tumors as compared to their pulmonary counterparts are classified into well, moderate and poorly differentiated NEC (NEC Grade-I to III). [2] Common symptoms and signs are related to obstructive jaundice and upper abdominal discomfort. Lymph node and liver metastasis are common. [5],[6],[7] Signet ring cell adenocarcinomas are rare in the peri-ampullary region with less than 15 cases reported so far in the literature. [4] It mainly occurs in elderly people (median age 57 years).

After review of the literature, seven cases of co-existing neuroendocrine tumor and adenocarcinoma in peri-ampullary region were found. [2],[3] To the best of our knowledge, only one case of large cell endocrine carcinoma colliding with adenocarcinoma in the ampulla of Vater has been reported. [2]

Pancreatico-duodenectomy forms the mainstay of the treatment, followed by adjuvant chemotherapy dictated by the prognostic factors used in cases of composite and collision tumor which include tumor size of individual component, grade of the neuro-endocrine component, the presence of lymph node metastasis and distant metastasis.

LCNEC are highly aggressive and prognosis is dismal even after complete resection. [5],[6],[7] According to review of literature, small size of the tumor, absence of invasion into pancreas, and node negative status translate into long disease free survival in cases of SRCC. [4] The prognosis in the indexed case is proposed to depend upon LCNEC, as it produced nodal involvement. These tumors have 100% mortality according to the previous reports, while in this case, the patient is disease free after 1 year of follow up inspite of nodal metastasis at the time of presentation.

Collision tumors of the peri-ampullary region are rare, and combination of signet ring carcinoma and high grade NEC rarer. In the present case, disease-free survival, in spite of nodal metastasis, for 1 year highlights the importance of aggressive adjuvant chemotherapy followed by radiation in improving survival.

   References Top

1.Ferrando MJ, Pallas RA, Moro Valdezate D, Fernández MC. Collision tumor of the ampulla of Vater: Carcinoid and adenocarcinoma. Rev Esp Enferm Dig 2007;99:235-8.  Back to cited text no. 1
2.Liu SH, Tsay SH. Coexistence of large cell neuroendocrine carcinoma of the ampulla of vater: Case report. J Chin Med Assoc 2008;71:536-40.  Back to cited text no. 2
3.Williams IM, Williams NW, Stock D, Foster ME. Collision tumor of the ampulla of vater: Carcinoid and adenocarcinoma. HPB Surg 1997;10:241-4.  Back to cited text no. 3
4.Akatsu T, Aiura K, Takahashi S, Kameyama K, Kitajima M, Kitagawa Y. Signet-ring cell carcinoma of the ampulla of Vater: Report of a case. Surg Today 2007;37:1110-4.   Back to cited text no. 4
5.Cheng SP, Yang TL, Chang KM, Liu CL. Large cell neuroendocrine carcinoma of ampulla of vater with glandular differentiation. J Clin Pathol 2004;57:1098-100.  Back to cited text no. 5
6.Huang SS, Jan YJ, Cheng SB, Yeh DC, Wu CC, Liu TJ, et al. Large cell neuroendocrine carcinoma of the ampulla of Vater: Report of a case. Surg Today 2006;36:1032-5.  Back to cited text no. 6
7.Nassar H, Albores SJ, Klimstra D. High-grade neuroendocrine carcinoma of the ampulla of Vater: A clinicopathologic and immunohistochemical analysis of 14 cases. Am J Surg Pathol 2005;29:588-94.  Back to cited text no. 7

Correspondence Address:
Anuj Khurana
98 SFS Flats, Phase-4 Ashok Vihar, Delhi - 110 052
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.77391

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

This article has been cited by
1 Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A case report
Molecular and Clinical Oncology. 2016; 5(1): 95
[Pubmed] | [DOI]


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