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.
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]
Etiology
Multinucleate cell angiohistiocytoma is an acquired disorder of unknown cause. Puig et al propose the existence of an interaction between mast cells and factor XIIIa–positive fibrohistiocytic cells, which might contribute to vascular proliferation by the release of various proangiogenic cytokines. [3] Shapiro postulates that the condition may be related to trauma because it tends to occur on the dorsal aspects of the hands and around the knees. [4]
Epidemiology
Frequency
Multinucleate cell angiohistiocytoma is a rare disorder. [5] Fewer than 100 cases have been reported as of 2015. [6, 7, 8, 9, 10, 11]
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.
Prognosis
Multinucleate cell angiohistiocytoma is a benign condition. Spontaneous resolution is uncommon. [12] No morbidity or mortality is associated with multinucleate cell angiohistiocytoma.
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Biopsy specimen of multinucleate cell angiohistiocytoma. A vascular proliferation is embedded in a stroma rich in fibroblasts and histiocytes, some of which are multinucleated.
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Bizarre multinucleated cells are present in the stroma surrounding the vascular proliferation.
