Multinucleate Cell Angiohistiocytoma

Updated: Jun 27, 2016
  • Author: Diane M Scott, MD; Chief Editor: William D James, MD  more...
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Overview

Background

Multinucleate cell angiohistiocytoma is a benign fibrohistiocytic and vascular proliferation that was first reported by Smith and Wilson Jones in 1985. [1] Multinucleate cell angiohistiocytoma occurs predominantly on the extremities of middle-aged and elderly women.

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Pathophysiology

The pathogenesis of multinucleate cell angiohistiocytoma is unknown. Some authors consider it a connective-tissue disorder, while others categorize it as a vascular tumor. Wilson Jones concluded that multinucleate cell angiohistiocytoma is a chronic inflammatory disorder of unknown cause manifested by vascular hyperplasia and connective-tissue cells that have lost their function after prolonged stimulation. Cesinaro et al argue for a role played by estrogen in the development of this lesion. [2]

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Epidemiology

Frequency

Multinucleate cell angiohistiocytoma is a rare disorder. Fewer than 100 cases have been reported as of 2015. [3, 4, 5, 6, 7, 8]

Race

The frequency of multinucleate cell angiohistiocytoma in various races has not been reported.

Sex

The female-to-male ratio of persons affected with multinucleate cell angiohistiocytoma is approximately 5:1.

Age

Reported cases of multinucleate cell angiohistiocytoma have occurred in patients aged 24-74 years. Most cases of multinucleate cell angiohistiocytoma occur in middle-aged and elderly women.

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Prognosis

Multinucleate cell angiohistiocytoma is a benign condition. Spontaneous resolution is uncommon. No morbidity or mortality is associated with multinucleate cell angiohistiocytoma.

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