Large-cell neuroendocrine carcinoma of the ampulla of Vater

Abstract

Large-cell neuroendocrine carcinoma is a high-grade neuroendocrine carcinoma, originally described in the lung. The tumor rarely occurs in extrapulmonary sites like the gastrointestinal tract, and only few examples have been described in the ampulla of Vater. A new case of large-cell neuroendocrine carcinoma of the ampulla of Vater in a 60-year-old man is reported. After pancreatoduodenectomy, macroscopic examination revealed ulcerated tumor in the region of the ampulla of Vater. Microscopically, the tumor exhibited organoid, predominantly nested growth pattern, consisting of large, polygonal cells with pleomorphic nuclei. Average number of mitoses was 36 per 10 high-power fields. Small and large areas of necrosis were identified. Immunohistochemically, the tumor cells were positive for synaptophysin, chromogranin A, PGP 9.5, neuron-specific enolase, pancytokeratin, CK8 and somatostatin and negative for CK7, CK20, S-100, TTF-1, HMB-45, CD117, E-cadherin and regulatory peptides. Ki-67 proliferative index was 41%. Histone deacetylase (HDAC) analysis showed almost identical results for HDAC1, HDAC2 and HDAC3--60, 60.3 and 61%, respectively. Two months after surgery, liver metastases occurred, confirming highly aggressive behavior of large-cell neuroendocrine carcinoma.

Histopathology of gastrointestinal neuroendocrine neoplasms

“…Large-cell carcinomas are composed of medium- or large-sized tumor cells possessing large atypical nuclei with evident nucleoli. The nuclear to cytoplasmic ratio of large-cell carcinomas is lower than that of small-cell carcinomas (Kajiwara et al, 2009; Stojsic et al, 2010). MANECs are malignant tumors composed of both adenocarcinoma and neuroendocrine carcinoma components, with each component accounting for more than 30% of the tumor (Bosman et al, 2010).…”

Neuroendocrine tumours of the ampulla of Vater: clinico-pathological features, surgical approach and assessment of prognosis

“…WHO classification, ENETS and UICC staging systems, tumour diameter, T stage, lymph node involvement, survival status, overall and disease-free survival). A total of 37 patients who underwent pancreaticoduodenectomy for NET of the ampulla of Vater were introduced in a univariate analysis, including our cases (Table 2) [4, 5, 8, 17–21]. …”

Report of two cases of large cell neuroendocrine carcinoma of duodenal ampulla with contrasting outcomes following pancreaticoduodenectomy according to the use of adjuvant chemotherapy

“…Further, LCNEC is extremely rare, comprising only 4% (6/139) of the ampullary NET reported in that study. Some individual case reports and small series of LCNEC have been documented [1] , [2] , [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] . Beggs et al studied a pooled analysis of previously published reports of LCNEC cases in 2012 and collected only 20 patients [8] .…”

“…The prevalence of NET in the ampulla is less than 5% of all duodenal malignancies and only 0.3% of the NET of the entire digestive tract [2] , [3] , [4] , [5] , [6] . Among NET in the ampulla, LCNEC is extremely rare; only a few examples and small case series are documented in the English literature [1] , [2] , [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] . Because LCNEC of the ampulla is very rare, its clinical features and treatment strategy are not well established.…”