Carcinoid heart disease

Stephen A Geller,Fernando Peixoto Ferraz De Campos

doi:10.4322/acr.2013.010

Abstract

The images are of the tricuspid valve and the pulmonic valve from the autopsy of a patient with mid-gut neuroendocrine carcinoma, carcinoid pattern, extensively metastatic to the liver. The patient had typical “carcinoid syndrome,” including clinical evidence of tricuspid and pulmonic stenosis and insufficiency. The tricuspid valve (left) shows slight retraction and distortion by the overlying endothelial deposition of plaque composed of acid mucopolysaccharide-rich matrix with varying amounts of smooth muscle cells and collagen fibers. The plaque material causes partial coalescence of chordae tendinae with effacement of the usual delicate strands. The pulmonic valve (right) shows more marked distortion with shrinkage and obliteration of cusps and coalescence at the commissures. Beneath the plaque the valves are intact.

Highlights

Neuroendocrine neoplasms (NEN) derive from scattered cells throughout the body, classically identifiable by their histochemical reactivity for chromium and silver salts, named as enterochromaffin cells, argentaffin cells, clear cells, enteroendocrine cells or Kultschitsky cells
Carcinoid syndrome is a symptom complex manifested by episodic flushing, secretory diarrhea, bronchospasm and hypotension
Tricuspid valve involvement predominantly results in regurgitation

Summary

Introduction

Neuroendocrine neoplasms (NEN) derive from scattered cells throughout the body, classically identifiable by their histochemical reactivity for chromium and silver salts, named as enterochromaffin cells, argentaffin cells, clear cells, enteroendocrine cells or Kultschitsky cells. Shunting, as with a patent foramen ovale. Carcinoid syndrome is a symptom complex manifested by episodic flushing, secretory diarrhea, bronchospasm and hypotension. This syndrome occurs in 20% to 30% of patients at the time of diagnosis. Carcinoid heart disease (CHD) occurs in as many as 60% of patients with the carcinoid syndrome.

Results

Conclusion