Ampullary Mixed Adenoneuroendocrine Carcinoma: Surprise Histology, Familiar Management

Introduction. Mixed adenoneuroendocrine carcinoma (MANEC) has recently been defined by the World Health Organization in 2010. These are rare tumors and MANECs of ampullary region are even rarer. Only 19 cases have been reported in literature. We present 3 cases; the largest series, second case of am...

Full description

Saved in:
Bibliographic Details
Published in:International journal of surgical pathology 2017-10, Vol.25 (7), p.585-591
Main Authors: Mahansaria, Shyam Sunder, Agrawal, Nikhil, Arora, Asit, Bihari, Chhagan, Appukuttan, Murali, Chattopadhyay, Tushar Kanti
Format: Article
Language:English
Subjects:
Adenocarcinoma - diagnostic imaging
Adenocarcinoma - mortality
Adenocarcinoma - pathology
Adenocarcinoma - therapy
Adult
Ampulla of Vater - diagnostic imaging
Ampulla of Vater - pathology
Ampulla of Vater - surgery
Biomarkers, Tumor - blood
Carcinoma, Neuroendocrine - diagnostic imaging
Carcinoma, Neuroendocrine - mortality
Carcinoma, Neuroendocrine - pathology
Carcinoma, Neuroendocrine - therapy
Chemotherapy, Adjuvant
Common Bile Duct Neoplasms - diagnostic imaging
Common Bile Duct Neoplasms - mortality
Common Bile Duct Neoplasms - pathology
Female
Humans
Male
Medical prognosis
Middle Aged
Mixed Tumor, Malignant - diagnostic imaging
Mixed Tumor, Malignant - mortality
Mixed Tumor, Malignant - pathology
Mixed Tumor, Malignant - therapy
Neoplasm Grading
Neoplasm Recurrence, Local - diagnostic imaging
Neoplasm Recurrence, Local - mortality
Neoplasm Recurrence, Local - pathology
Neoplasm Recurrence, Local - therapy
Pancreaticoduodenectomy
Prognosis
Rare Diseases - diagnostic imaging
Rare Diseases - mortality
Rare Diseases - pathology
Rare Diseases - therapy
Retrospective Studies
Survival Analysis
Tomography, X-Ray Computed
Tumors
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Introduction. Mixed adenoneuroendocrine carcinoma (MANEC) has recently been defined by the World Health Organization in 2010. These are rare tumors and MANECs of ampullary region are even rarer. Only 19 cases have been reported in literature. We present 3 cases; the largest series, second case of amphicrine tumor and first case associated with chronic pancreatitis. Methods. Retrospective review of 3 patients who were diagnosed to have ampullary MANEC. Results. All 3 patients were diagnosed preoperatively as neuroendocrine carcinoma and underwent margin negative pancreaticoduodenectomy. The histopathology revealed MANECs of small cell, mixed type in 2 patients and large cell, amphicrine type in 1 patient. The neuroendocrine component was grade 3 in all, the tumor was T3 in 2 and T2 in 1 and all had nodal metastases. Two patients received adjuvant chemotherapy and 2 of them had recurrence at 13 and 16 months. The median survival was 15 months. Conclusion. Ampullary MANECs are rare tumors. They are diagnosed on histopathologic examination of the resected specimen. Clinical presentation, management, and prognosis is similar to ampullary adenocarcinoma in literature.
ISSN:1066-8969
1940-2465
DOI:10.1177/1066896917712454