Multinucleate Cell Angiohistiocytoma

Background

This rare lesion is characterized by multiple dull red papules, usually located on the had and neck and acral regions. It is most common in elderly women.

OUTLINE

Epidemiology

Disease Associations

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Prognosis and Treatment

Commonly Used Terms

EPIDEMIOLOGY CHARACTERIZATION

SYNONYMS

INCIDENCE

AGE RANGE-MEDIAN Elderly 7-8th decades

SEX (M:F)
Women common

GEOGRAPHY

DISEASE ASSOCIATIONS CHARACTERIZATION

MYCOSIS FUNGOIDES

Multinucleate-cell angiohistiocytoma occurring in a patient with mycosis fungoides.

Bader RS, Telang GH, Vonderheid EC.

Department of Dermatology, Allegheny University Hospitals, Philadelphia, Pennsylvania 19107, USA.
Cutis 1999 Mar;63(3):145-8 Abstract quote
Multinucleate-cell angiohistiocytoma (MCAH) is a benign vascular proliferation of unknown etiology. Clinically, MCAH presents as grouped, erythematous, or violaceous papules on the extremities in older women. These lesions often resemble Kaposi's sarcoma.

Histologic examination reveals characteristic bizarre-shaped, multinucleate giant cells, some of which contain three to six nuclei arranged in a ring-like or overlapping pattern, which stain positively for Factor XIIIa. In addition, there is a proliferation of dermal capillaries and venules with a mild lymphohistiocytic infiltrate.

To our knowledge, this is the first reported case of MCAH occurring in a patient with mycosis fungoides.

GROSS APPEARANCE/
CLINICAL VARIANTS CHARACTERIZATION

GENERAL

Multinucleate cell angiohistiocytoma: a clinicopathological, immunohistochemical and ultrastructural study.

Smolle J, Auboeck L, Gogg-Retzer I, Soyer HP, Kerl H.

Department of Dermatology, University of Graz, Austria.

Br J Dermatol 1989 Jul;121(1):113-21 Abstract quote
The term 'multinucleate cell angiohistiocytoma' was first introduced by Smith and Wilson Jones in 1985.

We report the clinicopathological, immunohistological and ultrastructural findings observed in two patients. Multinucleate cell angiohistiocytoma occurs mainly in middle-aged women and is usually located at acral sites, particularly the distal extremities. Grouped, brown-red, slightly elevated, asymptomatic papules slowly develop over several months until further growth ceases. There is no evidence of systemic disease.

Histologically, the dermis shows numerous well developed capillaries with prominent endothelia, large bizarre basophilic and often multinucleate cells with a sparse lymphohistiocytic infiltrate. The immunohistological and ultrastructural findings suggest a fibroblastic differentiation of the large multinucleate cells.

Multinucleate cell angiohistiocytoma: a distinct entity diagnosable by clinical and histologic features.

Shapiro PE, Nova MP, Rosmarin LA, Halperin AJ.

Department of Dermatology, Yale University School of Medicine, New Haven, CT 06510.

J Am Acad Dermatol 1994 Mar;30(3):417-22 Abstract quote
BACKGROUND: Multinucleate cell angiohistiocytoma is a newly described entity; examples from the United States have not yet been reported.

OBJECTIVE: Our purpose was to analyze the clinical and histologic features of this entity and confirm or refute its existence.

METHODS: Seven cases were analyzed clinically and by light microscopy. RESULTS: Multinucleate cell angiohistiocytoma typically occurs in middle-aged women and consists of multiple, grouped, red-brown to violaceous papules that are dome-shaped or flat-topped, roundish and smooth in outline, sharply circumscribed, and occasionally coalescent. Typical sites are the legs, thighs, and backs of hands and fingers. Microscopic features are an increased number of blood vessels (usually capillaries and venules) that are small, rounded, and not well grouped, together with multinucleated histiocyte-like cells with scalloped borders.

CONCLUSION: Multinucleate cell angiohistiocytoma is a distinct entity that is diagnosable clinically and histopathologically.

VARIANTS

LIP

Multinucleate cell angiohistiocytoma of the upper lip.

Jones AC, Mullins D, Jimenez F.

University of Florida College of Dentistry, Department of Oral Diagnostic Sciences, Gainesville.
Oral Surg Oral Med Oral Pathol 1994 Dec;78(6):743-7 Abstract quote
Multinucleate cell angiohistiocytoma is a rare benign fibrohistiocytic vascular tumor with distinctive histologic features.

It appears clinically as small, slow-growing, grouped reddish-brown to purple papules and nodules that may be mistaken for other vascular proliferative or reactive conditions. Multinucleate cell angiohistiocytoma tends to occur more frequently in women, to favor the extremities, and to appear as multiple lesions.

We report a case of solitary multinucleate cell angiohistiocytoma that appeared as a nodule on the upper lip of a man but that otherwise demonstrated characteristic histologic and immunohistochemical features. Greater awareness of this uncommon benign lesion will facilitate diagnosis and appropriate management.

PLAQUE

Plaque-type multinucleate cell angiohistiocytoma.

Issa AA, Lui H, Shapiro J, Trotter MJ.

Division of Dermatology, Vancouver Hospital & Health Sciences Centre, University of British Columbia, Vancouver, British Columbia, Canada.
J Cutan Med Surg 1998 Oct;3(2):112-4 Abstract quote
BACKGROUND: Multinucleate cell angiohistiocytoma (MCAH) is a rare cutaneous disorder that usually presents as papules or nodules.

OBJECTIVE: This article reports a case of MCAH that appeared clinically as a large cutaneous plaque.

METHODS AND RESULTS: A 74-year-old woman presented with a large painless, dusky red, indurated plaque measuring 12 x 6 cm on the trunk that was found on histopathologic examination to be a MCAH. Based on a literature review, this is the first reported case of MCAH presenting as a plaque rather than a papule or nodule.

CONCLUSION: Multinucleate cell angiohistiocytoma may manifest clinically as a solitary cutaneous plaque.

HISTOLOGICAL TYPES CHARACTERIZATION

GENERAL

Multinucleate cell angiohistiocytoma. A review and report of four cases.

Cribier B, Gambini C, Rainero M, Grosshans E.

Clinic of Dermatology, Strasbourg, France
Acta Derm Venereol 1995 Sep;75(5):337-9 Abstract quote
Multinucleate cell angiohistiocytoma (MCA) was first characterized by Smith & Wilson-Jones. Although only a few cases have been published, this very characteristic benign tumor is probably not rare.

The clinical pictures are firm circumscribed papules, mainly of the hands, which progress slowly over the years. The histologic features show an increase of capillaries and venules in the reticular dermis, overlaid by epidermal hyperplasia. Bizarre-shaped multinucleate cells are present between the vessels, and factor XIIIa-positive interstitial cells are increased in number. MCA can be easily recognised if the pathologist is aware of the diagnosis.

We present here the clinical, histologic and immunopathologic features of 4 new cases and review the literature.

Multinucleate cell angiohistiocytoma: a report of two cases.

Aloi F, Solaroli C, Tomasini C, Pippione M.

Department of Dermatology, University of Turin, Italy.
J Eur Acad Dermatol Venereol 1998 Jul;11(1):51-4 Abstract quote
We report the clinical, histological and immunological features of two cases of multinucleate cell angiohistiocytoma (MCAH) in women of 32 and 53 years of age, respectively.

Clinically, MCAH occurs mostly in middle-aged women and consists of crops of reddish-purple, dome-shaped papules especially on the limbs. Histologically, the reticular dermis presents an increased number of small vascular channels with plump endothelial cells embedded in a fibrohistiocitic stroma with numerous bizarre multinucleate cells.

Bizarre multinucleated cells are not specific to MCAH; they can be observed in numerous other cutaneous conditions. However, MCAH presents quite distinctive clinico-pathological findings and may be easily differentiated from other cutaneous disorders.

Multinucleate cell angiohistiocytoma: a fibrohistiocytic proliferation with increased mast cell numbers and vascular hyperplasia.

Fernandez-Figueras MT, Bielsa I, Lloveras B; Lluis Puig.; Agustin Alomar.

Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Departments of Pathology and Dermatology, Hospital Universitari Germans Trias i Pujol, Badalona, Department of Pathology, General Laboratory, Barcelona, Spain.

J Cutan Pathol 2002 Apr;29(4):232-7 Abstract quote
Background: Multinucleate cell angiohistiocytoma (MCAH) is an uncommon lesion clinically characterized by multiple papules usually located on the face and acral regions of elderly women. Histopathologically, MCAH is characterized by dermal vascular hyperplasia associated with increased number of factor XIIIa-positive fibrohistiocytic cells and multinucleate cells with scalloped borders.

Methods: We report the clinical, histopathological and immunohistochemical features of three cases of MCAH, with ulstrastructural study in one of them. The patients were a woman and two men of 56, 40 and 70 years of age, respectively. They all had multiple dull-red papules, which had appeared over several years and were located on the face, the trunk and the dorsa of the hands, respectively.

Results: The reticular dermis presented a fibrohistiocytic proliferation of factor XIIIa-positive cells, with abundant bizarre multinucleate cells and vascular hyperplasia. Increased mast cell numbers were seen in all cases, often in apposition to multinucleate cells.

Conclusion: Histopathological differential diagnosis of MCAH includes mainly angiofibromas and dermatofibromas, even though vascular hyperplasia can be prominent and has led to many authors to classify MCAH among vascular tumors. Bizarre multinucleate cells can be found in reactive, neoplastic and inflammatory lesions in many sites of the body, and mast cells can play a role in their morphogenesis.

VARIANTS

SPECIAL STAINS/IMMUNOPEROXIDASE/
OTHER CHARACTERIZATION

SPECIAL STAINS

IMMUNOPEROXIDASE

Multinucleate cell angiohistiocytoma.

Annessi G, Girolomoni G, Giannetti A.

Department of Dermatology, University of Modena, Italy.
Am J Dermatopathol 1992 Aug;14(4):340-4 Abstract quote
We report a case of multinucleate cell angiohistiocytoma occurring in a 50-year-old woman and presenting with a 4-year history of asymptomatic red papules on the face.

Histologically, there was a dermal vascular proliferation associated with numerous multinucleate cells. Immunohistochemical studies showed vascular spaces surrounded with mature vascular endothelial cells, and the presence of numerous interstitial factor XIIIa+ cells. Multinucleate cells exhibited intermediate filaments of the vimentin type, but were not stained by endothelial cell or macrophage markers, and were factor XIIIa-.

This condition must be addressed in the differential diagnosis of other skin vascular proliferations, such as Kaposi's sarcoma and angiofibromas.

Weedon D. Weedon's Skin Pathology. Churchill Livingstone. 1997.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.

Commonly Used Terms

Soft Tissue Tumors

Last Updated 6/14/2002

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
GENERAL
Multinucleate cell angiohistiocytoma: a clinicopathological, immunohistochemical and ultrastructural study. Smolle J, Auboeck L, Gogg-Retzer I, Soyer HP, Kerl H. Department of Dermatology, University of Graz, Austria.	Br J Dermatol 1989 Jul;121(1):113-21 Abstract quote The term 'multinucleate cell angiohistiocytoma' was first introduced by Smith and Wilson Jones in 1985. We report the clinicopathological, immunohistological and ultrastructural findings observed in two patients. Multinucleate cell angiohistiocytoma occurs mainly in middle-aged women and is usually located at acral sites, particularly the distal extremities. Grouped, brown-red, slightly elevated, asymptomatic papules slowly develop over several months until further growth ceases. There is no evidence of systemic disease. Histologically, the dermis shows numerous well developed capillaries with prominent endothelia, large bizarre basophilic and often multinucleate cells with a sparse lymphohistiocytic infiltrate. The immunohistological and ultrastructural findings suggest a fibroblastic differentiation of the large multinucleate cells.
Multinucleate cell angiohistiocytoma: a distinct entity diagnosable by clinical and histologic features. Shapiro PE, Nova MP, Rosmarin LA, Halperin AJ. Department of Dermatology, Yale University School of Medicine, New Haven, CT 06510.	J Am Acad Dermatol 1994 Mar;30(3):417-22 Abstract quote BACKGROUND: Multinucleate cell angiohistiocytoma is a newly described entity; examples from the United States have not yet been reported. OBJECTIVE: Our purpose was to analyze the clinical and histologic features of this entity and confirm or refute its existence. METHODS: Seven cases were analyzed clinically and by light microscopy. RESULTS: Multinucleate cell angiohistiocytoma typically occurs in middle-aged women and consists of multiple, grouped, red-brown to violaceous papules that are dome-shaped or flat-topped, roundish and smooth in outline, sharply circumscribed, and occasionally coalescent. Typical sites are the legs, thighs, and backs of hands and fingers. Microscopic features are an increased number of blood vessels (usually capillaries and venules) that are small, rounded, and not well grouped, together with multinucleated histiocyte-like cells with scalloped borders. CONCLUSION: Multinucleate cell angiohistiocytoma is a distinct entity that is diagnosable clinically and histopathologically.
VARIANTS
LIP
Multinucleate cell angiohistiocytoma of the upper lip. Jones AC, Mullins D, Jimenez F. University of Florida College of Dentistry, Department of Oral Diagnostic Sciences, Gainesville.	Oral Surg Oral Med Oral Pathol 1994 Dec;78(6):743-7 Abstract quote Multinucleate cell angiohistiocytoma is a rare benign fibrohistiocytic vascular tumor with distinctive histologic features. It appears clinically as small, slow-growing, grouped reddish-brown to purple papules and nodules that may be mistaken for other vascular proliferative or reactive conditions. Multinucleate cell angiohistiocytoma tends to occur more frequently in women, to favor the extremities, and to appear as multiple lesions. We report a case of solitary multinucleate cell angiohistiocytoma that appeared as a nodule on the upper lip of a man but that otherwise demonstrated characteristic histologic and immunohistochemical features. Greater awareness of this uncommon benign lesion will facilitate diagnosis and appropriate management.
PLAQUE
Plaque-type multinucleate cell angiohistiocytoma. Issa AA, Lui H, Shapiro J, Trotter MJ. Division of Dermatology, Vancouver Hospital & Health Sciences Centre, University of British Columbia, Vancouver, British Columbia, Canada.	J Cutan Med Surg 1998 Oct;3(2):112-4 Abstract quote BACKGROUND: Multinucleate cell angiohistiocytoma (MCAH) is a rare cutaneous disorder that usually presents as papules or nodules. OBJECTIVE: This article reports a case of MCAH that appeared clinically as a large cutaneous plaque. METHODS AND RESULTS: A 74-year-old woman presented with a large painless, dusky red, indurated plaque measuring 12 x 6 cm on the trunk that was found on histopathologic examination to be a MCAH. Based on a literature review, this is the first reported case of MCAH presenting as a plaque rather than a papule or nodule. CONCLUSION: Multinucleate cell angiohistiocytoma may manifest clinically as a solitary cutaneous plaque.

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL
Multinucleate cell angiohistiocytoma. A review and report of four cases. Cribier B, Gambini C, Rainero M, Grosshans E. Clinic of Dermatology, Strasbourg, France	Acta Derm Venereol 1995 Sep;75(5):337-9 Abstract quote Multinucleate cell angiohistiocytoma (MCA) was first characterized by Smith & Wilson-Jones. Although only a few cases have been published, this very characteristic benign tumor is probably not rare. The clinical pictures are firm circumscribed papules, mainly of the hands, which progress slowly over the years. The histologic features show an increase of capillaries and venules in the reticular dermis, overlaid by epidermal hyperplasia. Bizarre-shaped multinucleate cells are present between the vessels, and factor XIIIa-positive interstitial cells are increased in number. MCA can be easily recognised if the pathologist is aware of the diagnosis. We present here the clinical, histologic and immunopathologic features of 4 new cases and review the literature.
Multinucleate cell angiohistiocytoma: a report of two cases. Aloi F, Solaroli C, Tomasini C, Pippione M. Department of Dermatology, University of Turin, Italy.	J Eur Acad Dermatol Venereol 1998 Jul;11(1):51-4 Abstract quote We report the clinical, histological and immunological features of two cases of multinucleate cell angiohistiocytoma (MCAH) in women of 32 and 53 years of age, respectively. Clinically, MCAH occurs mostly in middle-aged women and consists of crops of reddish-purple, dome-shaped papules especially on the limbs. Histologically, the reticular dermis presents an increased number of small vascular channels with plump endothelial cells embedded in a fibrohistiocitic stroma with numerous bizarre multinucleate cells. Bizarre multinucleated cells are not specific to MCAH; they can be observed in numerous other cutaneous conditions. However, MCAH presents quite distinctive clinico-pathological findings and may be easily differentiated from other cutaneous disorders.
Multinucleate cell angiohistiocytoma: a fibrohistiocytic proliferation with increased mast cell numbers and vascular hyperplasia. Fernandez-Figueras MT, Bielsa I, Lloveras B; Lluis Puig.; Agustin Alomar. Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Departments of Pathology and Dermatology, Hospital Universitari Germans Trias i Pujol, Badalona, Department of Pathology, General Laboratory, Barcelona, Spain.	J Cutan Pathol 2002 Apr;29(4):232-7 Abstract quote Background: Multinucleate cell angiohistiocytoma (MCAH) is an uncommon lesion clinically characterized by multiple papules usually located on the face and acral regions of elderly women. Histopathologically, MCAH is characterized by dermal vascular hyperplasia associated with increased number of factor XIIIa-positive fibrohistiocytic cells and multinucleate cells with scalloped borders. Methods: We report the clinical, histopathological and immunohistochemical features of three cases of MCAH, with ulstrastructural study in one of them. The patients were a woman and two men of 56, 40 and 70 years of age, respectively. They all had multiple dull-red papules, which had appeared over several years and were located on the face, the trunk and the dorsa of the hands, respectively. Results: The reticular dermis presented a fibrohistiocytic proliferation of factor XIIIa-positive cells, with abundant bizarre multinucleate cells and vascular hyperplasia. Increased mast cell numbers were seen in all cases, often in apposition to multinucleate cells. Conclusion: Histopathological differential diagnosis of MCAH includes mainly angiofibromas and dermatofibromas, even though vascular hyperplasia can be prominent and has led to many authors to classify MCAH among vascular tumors. Bizarre multinucleate cells can be found in reactive, neoplastic and inflammatory lesions in many sites of the body, and mast cells can play a role in their morphogenesis.
VARIANTS

Epidemiology
Disease Associations
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Prognosis and Treatment
Commonly Used Terms

EPIDEMIOLOGY	CHARACTERIZATION
SYNONYMS
INCIDENCE
AGE RANGE-MEDIAN	Elderly 7-8th decades
SEX (M:F)	Women common
GEOGRAPHY

SPECIAL STAINS/IMMUNOPEROXIDASE/ OTHER	CHARACTERIZATION
SPECIAL STAINS
IMMUNOPEROXIDASE
Multinucleate cell angiohistiocytoma. Annessi G, Girolomoni G, Giannetti A. Department of Dermatology, University of Modena, Italy.	Am J Dermatopathol 1992 Aug;14(4):340-4 Abstract quote We report a case of multinucleate cell angiohistiocytoma occurring in a 50-year-old woman and presenting with a 4-year history of asymptomatic red papules on the face. Histologically, there was a dermal vascular proliferation associated with numerous multinucleate cells. Immunohistochemical studies showed vascular spaces surrounded with mature vascular endothelial cells, and the presence of numerous interstitial factor XIIIa+ cells. Multinucleate cells exhibited intermediate filaments of the vimentin type, but were not stained by endothelial cell or macrophage markers, and were factor XIIIa-. This condition must be addressed in the differential diagnosis of other skin vascular proliferations, such as Kaposi's sarcoma and angiofibromas.