Multinucleate Cell Angiohistiocytoma 

Updated: Nov 07, 2019
Author: Diane M Scott, MD; Chief Editor: William D James, MD 

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.

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.

 

Presentation

History

Multinucleate cell angiohistiocytoma presents as insidiously developing asymptomatic papules that usually develop on acral surfaces and tend to persist indefinitely.

Physical Examination

Multiple, grouped, well-circumscribed papules are red to violaceous to brownish in color.[13]

Multinucleate cell angiohistiocytoma lesions are smooth and firm, and they may be either dome shaped or flat topped.

A few cases of multinucleate cell angiohistiocytoma have been in an annular distribution, mimicking granuloma annulare.

Lesions of multinucleate cell angiohistiocytoma occur most commonly on the extremities. Acral areas such as the dorsal aspects of the hands, fingers, wrists, and legs are the most frequent sites. Unusual locations of multinucleate cell angiohistiocytoma include the forehead, the upper lip, the chest, oral cavity, and the orbit.[14, 15, 16]

Lesions usually are unilateral, but a few bilateral cases and one generalized case have been reported.

Multinucleate cell angiohistiocytoma lesions are usually asymptomatic to pruritic.

 

DDx

 

Workup

Procedures

Lesions of multinucleate cell angiohistiocytoma often mimic other conditions. A biopsy is the best way to exclude more serious disorders (eg, sarcoidosis, Kaposi sarcoma).[17]

Histologic Findings

Multinucleate cell angiohistiocytoma has a distinctive histologic appearance characterized by a vascular proliferation and bizarre multinucleated cells. In the upper and mid dermis, a proliferation of small blood vessels occurs, some of which have dilated lumina. The vessels have the histologic appearances of capillaries and small vessels. The endothelial cells lining the vessels are plump but lack atypia. A sparse perivascular lymphocytic infiltrate may be present. In addition, an increased number of mast cells has been reported.

The vascular proliferation is embedded in a fibrous stroma rich in fibrohistiocytic cells. The collagen bundles are slightly thickened and arranged haphazardly or parallel to the epidermis. Bizarre multinucleated cells are amongst the interstitial cells. These cells have 3-10 hyperchromatic nuclei that are closely aggregated or arranged in a ringlike fashion. The cytoplasm of these cells is often angulated or scalloped in outline (see the images below).

Biopsy specimen of multinucleate cell angiohistioc Biopsy specimen of multinucleate cell angiohistiocytoma. A vascular proliferation is embedded in a stroma rich in fibroblasts and histiocytes, some of which are multinucleated.
Bizarre multinucleated cells are present in the st Bizarre multinucleated cells are present in the stroma surrounding the vascular proliferation.

Immunohistochemical studies may be useful in confirming the diagnosis of multinucleate cell angiohistiocytoma and in differentiating it from other vascular and fibrous proliferations. The lesional vessels label with factor VIII–related antigen (factor VIII-RA), EN-4, BMA-120, PAL-E, and Ulex europaeus agglutinin I (UEA-I). These findings indicate a capillary and venular nature of the vessels. Polymerase chain reaction analysis reveals no evidence of human herpesvirus-8 (HHV-8) infection, as may be found in Kaposi sarcoma.

The multinucleated cells stain with vimentin, but they do not label with the macrophage markers lysozyme, MAC 387, and alpha-1-antitrypsin. The multinucleated cells are also negative for S-100 protein, factor XIII, and human leukocyte antigen DR (HLA-DR). The mononuclear interstitial cells label with vimentin, factor XIIIa, and CD68.

 

Treatment

Medical Care

Multinucleate cell angiohistiocytoma has not been shown to respond to any medical therapy.

Surgical Care

Surgical excision of multinucleate cell angiohistiocytoma may be recommended for cosmetic reasons.

Laser therapy has resulted in clinical resolution of the lesions with not scarring. Kopera et al reported successful treatment with an argon laser.[18]  Richer and Lui reported successful treatment with a 585-nm pulsed dye laser.[19]

Treatment using intense pulsed light has also been reported.[20]

Perez et al reported a case with a good response to cryosurgery.[21]

Long-Term Monitoring

Further follow-up care is not needed because multinucleate cell angiohistiocytoma is not known to be associated with any systemic diseases and because it has a biologically benign course.

 

Medication

Medication Summary

No medical therapy is available for multinucleate cell angiohistiocytoma.