Composite neuroendocrine and adenomatous carcinoma of the papilla of Vater

Musialik, Joanna; Kohut, Maciej; Marek, Tomasz; Wodołażski, Anatol; Hartleb, Marek

doi:10.3748/wjg.15.4199

Cited by 8 publications

(10 citation statements)

References 5 publications

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“…Other names used to diagnose such tumors include mucin-producing carcinoid [47], composite carcinoid-adenocarcinoma [48,49], composite carcinoid tumor [50], mixed adenocarcinoid tumor [51], and composite glandular-neuroendocrine mixed tumor [30]. These neoplasms have been reported in the esophagus [47,52], stomach [18,48,53,54,55], ampulla of Vater [49,56], ileum [51], and large bowel [30,50,57,58,59,60,61]. To date, about 60 cases have been reported in the literature.…”

Section: Mixed Adenoneuroendocrine Carcinomas (Manecs)mentioning

confidence: 99%

Mixed Adenoneuroendocrine Carcinomas (MANECs) of the Gastrointestinal Tract: An Update

Rosa

Marando

Sessa

et al. 2012

Cancers

238

307

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The systematic application of immunohistochemical techniques to the study of tumors has led to the recognition that neuroendocrine cells occur rather frequently in exocrine neoplasms of the gut. It is now well known that there is a wide spectrum of combinations of exocrine and neuroendocrine components, ranging from adenomas or carcinomas with interspersed neuroendocrine cells at one extreme to classical neuroendocrine tumors with a focal exocrine component at the other. In addition, both exocrine and neuroendocrine components can have different morphological features ranging, for the former, from adenomas to adenocarcinomas with different degrees of differentiation and, for the latter, from well differentiated to poorly differentiated neuroendocrine tumors. However, although this range of combinations of neuroendocrine and exocrine components is frequently observed in routine practice, mixed exocrine-neuroendocrine carcinomas, now renamed as mixed adenoneuroendocrine carcinomas (MANECs), are rare; these are, by definition, neoplasms in which each component represents at least 30% of the lesion. Gastrointestinal MANECs can be stratified in different prognostic categories according to the grade of malignancy of each component. The present paper is an overview of the main clinicopathological, morphological, immunohistochemical and molecular features of this specific rare tumor type.

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Section: Mixed Adenoneuroendocrine Carcinomas (Manecs)mentioning

confidence: 99%

Mixed Adenoneuroendocrine Carcinomas (MANECs) of the Gastrointestinal Tract: An Update

Rosa

Marando

Sessa

et al. 2012

Cancers

238

307

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“…We report here the case of two patients with LCNEC in the duodenal ampulla of Vater, which were extremely rare malignancies with aggressive behaviour. NET of the ampulla account for 2–8% of ampullary neoplasms, and almost all NET are classified as NET G1 and G2, with NEC being very rare [2] , [3] , [4] , [5] , [6] , [8] , [11] , [12] , [13] . Among 6081 malignant neoplasms of the ampulla, NET was reported in 139 cases in a US population-based study from the National Cancer Institute’s Surveillance, Epidemiology, and End Results data from 1973 to 2006 [2] .…”

Section: Discussionmentioning

confidence: 99%

“…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] .…”

Section: Discussionmentioning

confidence: 99%

“…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.…”

Section: Introductionmentioning

confidence: 99%

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

Imamura

Nanashima

Hiyoshi

et al. 2017

International Journal of Surgery Case Reports

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HighlightsLarge-cell neuroendocrine carcinoma (LCNEC) of the duodenal ampulla is very rare.LCNEC of the duodenal ampulla behaves aggressively, and the prognosis is poor.Early postoperative tumor relapse occurred in our first case of ampullary LCNEC.Use of adjuvant chemotherapy after surgery resulted long survival in second case.Surgery and adjuvant chemotherapy are recommended for ampullary LCNEC.

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“…Mixed adenoneuroendocrine carcinomas (MANECs) are rare biphasic tumour types, which have only anecdotally been reported in the ampullary region (7,(9)(10)(11)(12). The current report described a patient with ampullary MANEC, presenting with widespread metastatic dissemination.…”

Section: Introductionmentioning

confidence: 97%

Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A case report

Max

Rothe

Langner

2016

Molecular and Clinical Oncology

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Abstract. Mixed adenoneuroendocrine carcinomas (MANECs) are rare biphasic tumour types, which are morphologically recognisable as both gland-forming and neuroendocrine neoplasms. Within the gastrointestinal tract, MANECs occur predominantly in the stomach or colorectum. The present study described a case of a MANEC originating from the ampullary region. The patient presented with widespread metastatic disease. Biopsy samples obtained from the ampullary primary tumour disclosed a complex lesion with adenocarcinoma and neuroendocrine small cell carcinoma components, positive for the intestinal transcription factor caudal type homeobox-2 and for neuroendocrine markers, including chromogranin A, synaptophysin, cluster of differentiation 56/neural cell adhesion molecule. By contrast, biopsy samples obtained from metastatic tissue revealed pure neuroendocrine carcinoma. As exemplified by this true mixed tumour, tumour heterogeneity evolves as the major challenge in oncology today, with potentially severe implications for the choice of chemotherapy. The assessment of metastatic sites may render valuable diagnostic information that is crucial for clinical decision-making and patient management.

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Composite neuroendocrine and adenomatous carcinoma of the papilla of Vater

Cited by 8 publications

References 5 publications

Mixed Adenoneuroendocrine Carcinomas (MANECs) of the Gastrointestinal Tract: An Update

Mixed Adenoneuroendocrine Carcinomas (MANECs) of the Gastrointestinal Tract: An Update

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

Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A case report

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