High-grade neuroendocrine carcinoma of the Ampulla of Vater

Definition

High grade carcinomas resembling those of the bronchial tree, including both small cell carcinoma and large cell neuroendocrine carcinoma. The corresponding low grade tumours are carcinoids of the ampulla of Vater.

Epidemiology

These are predominantly tumours of the elderly, more common in males.

Histopathology

Small cell carcinomas show cells that grow in sheets, sometimes forming rosettes. Necrosis is extensive. The mitotic rate exceeds 50/10HPF. Small cell carcinoma may occur in combination with adenoma and/or adenocarcinoma.

Large cell carcinoma shows a variable nested, trabecular, rosetting or organoid pattern of growth. There may be a hyalinised or myxoid stroma. The cells have prominent nucleoli. Necrosis is extensive. The mitotic rate is high.

Immunohistochemistry

High grade neuroendocrine carcinomas are positive for one or more neuroendocrine marker, usually for two or more. (Poorly differentiated ampullary adenocarcinomas show at most staining of a few scattered cells.)

 

 

High-grade neuroendocrine carcinoma

Poorly differentiated ampullary adenocarcinoma

 

Chromogranin

13/131

0/121

Synaptophysin

12/131

4/121

CD56

5/81

0/111

Cam5.2

6/91

12/121

AE1/AE3

11/111

12/121

CK7

7/81

8/111

CK20

3/81

6/111

p53

2/71

6/121

p27

8/91

0/111

Rb protein

4/101

12/121

Smad4

8/91

8/111

TTF-1

0/91

0/111

MIB1 (mean % of cells)

63%1

50%1

     

Management

There is a favorable but short-lived responsiveness, particularly of small cell carcinoma, to platinum-based chemotherapy.

Prognosis

These are aggressive tumours, with a high frequency of distant metastases. There may be a propensity for cerebral metastases.

References

1 Nassar H, Albores-Saavedra J,Klimstra DS 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

This page last revised 28.6.2005.

©SMUHT/PW Bishop