A Rare Collision Tumor Composed of Follicular Lymphoma and Adenocarcinoma in the Ampulla of Vater: A Case Report

Abstract

The duodenum is infrequently affected by malignant lymphoma, and follicular lymphomas of the duodenum are rare histological subtypes. There are no reported cases of collision of follicular lymphoma and other tumors in the ampulla of Vater. A 57-year-old Japanese man presented with jaundice, and abdominal computed tomography revealed a tumor of the ampulla of Vater invading the pancreatic head with biliary dilatation and a thickened duodenal wall. The patient underwent subtotal stomach-preserving pancreaticoduodenectomy. Histopathology of the resected specimen revealed lymphoid follicular formations with small-to-medium-sized centrocyte-like cells and some centroblast-like cells. The atypical lymphoid cells were immunohistochemically positive for CD10, CD20, and CD79a but negative for CD5 and cyclin D1. BCL2 protein was highly expressed in the follicle centers. The diagnosis was duodenal follicular lymphoma, Grade 1. The follicular lymphoma, 40 mm in diameter, involved duodenal submucosa and regional lymph nodes without distant metastasis. This duodenal follicular lymphoma was partially overlapped by adenocarcinoma of the ampulla of Vater, measuring 25 × 20 mm, which involved the lower common bile duct, pancreas, and duodenum. We report the first case of a surgically treated collision tumor composed of a rare mass-forming follicular lymphoma and adenocarcinoma of the ampulla of Vater.

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“…Diagnosis is confirmed with microscopic examination and immunostaining (5). Microscopy reveals neoplastic cells invading the lamina propria, being confined to the submucosal layer.…”

“…The duodenum is the most commonly affected location (1)(2)(3)(4). It is mainly an asymptomatic condition, which usually represents an incidental diagnosis during GI endoscopy (5). The most common reported finding consists of multiple small mucosal whitish nodules or polyps.…”

“…Within the GI tract, immunostaining of follicular lymphoma shows expression of pan B cell markers, such as CD19 and CD20, and, the anti-apoptotic protein, BCL-2. They are also positive for BCL-6 and CD10 (2,5). Due to its low prevalence and absence of guidelines, treatment of ampullary lymphomas is very heterogenous: watch and wait, monotherapy with rituximab, chemotherapy with R-CHOP and R-CHOP-like regimens, surgery, radiotherapy and Helicobacter pylori eradication.…”

Synchronous or collision solid neoplasms and lymphomas: A systematic review of 308 case reports

“…1). [12–262] The data were extracted from the abstract in 34 articles, [17,37–39,64,75,76,78,81,94,97,103,129,134,151,162,166,170,174,176,192,195,212,221,222,229,232,234,251,252,256,260–262] of which 21 were in English, 5 in Japanese, 3 in Spanish, 2 in French, 2 in Chinese, and 1 in Czech.…”

Simultaneous occurrence of pancreatic mixed acinar-ductal adenocarcinoma and primary follicular lymphoma of the duodenum, accompanied by increased number of IgG4 plasma cells in tumor-free parenchyma as concomitant IgG4-related disease or reaction to tumor? A case report

“…According to some authors, several factors may lead to the tumorigenesis of collisional tumors: (i) a carcinogenic agent can affect multiple targets at the same time; (ii) the decrease of the systemic and local immune defense system after the development of the tumor facilitates the occurrence of another tumor; and (iii) dysfunction of the tumor suppressor mutated gene leads to inadequate repair of a gene mutation, which could result in formation of multiple tumors [10]. Constant irritation of the duodenal mucosa by bile and pancreatic juice with a superimposed bacterial infection may also lead to mucosal damage and induce cell proliferation activity and multiple gene mutations, which could explain duodenal predilection for neoplasms [10,11]. Hypothetically these factors could also be stimuli for FL and mixed pancreatic carcinoma in our case.…”