Giant Cell Angioblastoma

Background

This is a rare tumor of the skin and soft tissue. It is important to correctly diagnose this tumor of infancy as these lesions have a slow growing but locally infiltrative growth pattern which may recur following inadequate excision or treatment.

INCIDENCE Less than 10 cases reported

AGE RANGE-MEDIAN Infancy

PATHOGENESIS CHARACTERIZATION

Chromosomal rearrangements None reported to date

GROSS APPEARANCE/
CLINICAL VARIANTS CHARACTERIZATION

General Extremities and hard and soft palate

VARIANTS

HISTOLOGICAL TYPES CHARACTERIZATION

General
Concentric arrays of oval-to-spindle cells and multinucleate giant cells around small endothelium-lined channels.

Nodular, linear, and plexiform aggregates of oval-to-spindle cells with pink flocculent cytoplasm and round, reniform, or spindle nuclei with dispersed chromatin, small nucleoli, and occasional mitoses

Large mononucleate cells and multinucleate giant cells with one to several nuclei, often with prominent nucleoli, and abundant granular eosinophilic cytoplasm

Round conglomerates of oval-to-spindle cells and giant cells mimicked non-necrotizing granulomas

Perineural and intraneural involvement by small vessels common

Brisk mitotic rate, anaplasia, and necrosis are absent

VARIANTS

Giant Cell Angioblastoma Three Additional Occurrences of a Distinct Pathologic Entity

Sara O. Vargas, M.D.; Antonio R. Perez-Atayde, M.D.; Frank González-Crussi, M.D.; Harry P. Kozakewich, M.D.

From the Department of Pathology (S.O.V., A.R.P.-A., H.P.K.), Children's Hospital, Boston, Massachusetts, U.S.A., and Department of Pathology (F.G.-C.), Children's Memorial Hospital, Chicago, Illinois, U.S.A.

Am J Surg Pathol 2001;25:185-196 Abstract quote

Giant cell angioblastoma was described previously in a single case report as a congenital soft-tissue tumor with a unique morphology.

In the current report, we describe three cases of giant cell angioblastoma found in three infants; one case was congenital and located in the hand, one appeared neonatally in the palate, and one on the scalp of an infant.

Clinical findings and results of light microscopy, immunohistochemistry, and electron microscopy were evaluated. All tumors were ulcerated; the hand and palate tumors also infiltrated soft tissue and bone. They exhibited a solid, nodular, and plexiform proliferation of oval-to-spindle cells with a frequent striking, concentric aggregation around small vascular channels. These cells had characteristics of undifferentiated mesenchymal cells, fibroblasts, myofibroblasts, and pericytes. Co-mingled with these cells were large mononuclear and multinucleate giant cells with histiocytic features.

The palatal giant cell angioblastoma, excised with positive margins, was managed with interferon- and showed no progression after nearly 5 years. The hand tumor diminished in size after management with interferon-, was subtotally excised, and did not progress after 27 months. Follow-up data are unavailable for the patient with the scalp lesion.

Our findings validate the classification of giant cell angioblastoma as a distinct and rare entity that is locally infiltrative but slow growing. The morphology and diverse cellular differentiation are consistent with an unusual form of neoplastic angiogenesis.

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES

Giant cell fibroblastoma

Angiectoid or sinusoidal areas
Solid, often mucoid, nodular mass with pseudovascular spaces
CD34 positive
Rearrangements of chromosomes 17 and 22

Epithelioid hemangioendothelioma with osteoclast-like giant cells Young adults
Attached to large vascular structures
Blister cells
Lack the concentric orientation of cells around small vessels

Plexiform fibrohistiocytic tumor
Children and young adults
Plexiform and nodular growth of rounded histiocytoid cells with interspersed osteoclast-like giant cells

Characteristic onion-skin layering of tumor cells around vessels and the hemangioma-like areas are absent

PROGNOSIS AND TREATMENT CHARACTERIZATION

PROGNOSIS

Recurrence Initial report had a large tumor which extensively involved the arm of an infant requiring amputation

Treatment Complete excision

González-Crussi F, Chou P, Crawford SE. Congenital, infiltrating giant-cell angioblastoma: a new entity? Am J Surg Pathol 1991;15:175–83.

Commonly Used Terms

Soft Tissue Tumors

Last Updated 8/28/2001

INCIDENCE	Less than 10 cases reported
AGE RANGE-MEDIAN	Infancy

PATHOGENESIS	CHARACTERIZATION
Chromosomal rearrangements	None reported to date

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
General	Extremities and hard and soft palate
VARIANTS

HISTOLOGICAL TYPES	CHARACTERIZATION
General	Concentric arrays of oval-to-spindle cells and multinucleate giant cells around small endothelium-lined channels. Nodular, linear, and plexiform aggregates of oval-to-spindle cells with pink flocculent cytoplasm and round, reniform, or spindle nuclei with dispersed chromatin, small nucleoli, and occasional mitoses Large mononucleate cells and multinucleate giant cells with one to several nuclei, often with prominent nucleoli, and abundant granular eosinophilic cytoplasm Round conglomerates of oval-to-spindle cells and giant cells mimicked non-necrotizing granulomas Perineural and intraneural involvement by small vessels common Brisk mitotic rate, anaplasia, and necrosis are absent
VARIANTS
Giant Cell Angioblastoma Three Additional Occurrences of a Distinct Pathologic Entity Sara O. Vargas, M.D.; Antonio R. Perez-Atayde, M.D.; Frank González-Crussi, M.D.; Harry P. Kozakewich, M.D. From the Department of Pathology (S.O.V., A.R.P.-A., H.P.K.), Children's Hospital, Boston, Massachusetts, U.S.A., and Department of Pathology (F.G.-C.), Children's Memorial Hospital, Chicago, Illinois, U.S.A.	Am J Surg Pathol 2001;25:185-196 Abstract quote Giant cell angioblastoma was described previously in a single case report as a congenital soft-tissue tumor with a unique morphology. In the current report, we describe three cases of giant cell angioblastoma found in three infants; one case was congenital and located in the hand, one appeared neonatally in the palate, and one on the scalp of an infant. Clinical findings and results of light microscopy, immunohistochemistry, and electron microscopy were evaluated. All tumors were ulcerated; the hand and palate tumors also infiltrated soft tissue and bone. They exhibited a solid, nodular, and plexiform proliferation of oval-to-spindle cells with a frequent striking, concentric aggregation around small vascular channels. These cells had characteristics of undifferentiated mesenchymal cells, fibroblasts, myofibroblasts, and pericytes. Co-mingled with these cells were large mononuclear and multinucleate giant cells with histiocytic features. The palatal giant cell angioblastoma, excised with positive margins, was managed with interferon- and showed no progression after nearly 5 years. The hand tumor diminished in size after management with interferon-, was subtotally excised, and did not progress after 27 months. Follow-up data are unavailable for the patient with the scalp lesion. Our findings validate the classification of giant cell angioblastoma as a distinct and rare entity that is locally infiltrative but slow growing. The morphology and diverse cellular differentiation are consistent with an unusual form of neoplastic angiogenesis.

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
Giant cell fibroblastoma	Angiectoid or sinusoidal areas Solid, often mucoid, nodular mass with pseudovascular spaces CD34 positive Rearrangements of chromosomes 17 and 22
Epithelioid hemangioendothelioma with osteoclast-like giant cells	Young adults Attached to large vascular structures Blister cells Lack the concentric orientation of cells around small vessels
Plexiform fibrohistiocytic tumor	Children and young adults Plexiform and nodular growth of rounded histiocytoid cells with interspersed osteoclast-like giant cells Characteristic onion-skin layering of tumor cells around vessels and the hemangioma-like areas are absent

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSIS
Recurrence	Initial report had a large tumor which extensively involved the arm of an infant requiring amputation
Treatment	Complete excision