Glomangiosarcoma of the Hand: A Case Report and Review of the Literature

Abstract

Glomangiosarcomas, or malignant glomus tumors, are very rare, cutaneous, soft tissue tumors. Despite having histologic features of malignancy, these tumors usually do not metastasize. We describe a 74-year-old woman with a glomangiosarcoma on her hand and review the literature. The woman presented with a five month history of a painful mass on the right palm. An excisional biopsy of the mass was undertaken. Histologically, the tumor was composed of uniform, round cells and numerous vascular components. The tumor cells were pleomorphic and had large nucleoli. Frequent mitotic figures were identified. Immunohistochemical stains showed strong positivity for vimentin and weak, focal positivity for smooth muscle actin. Ultrastructurally, the tumor cells were characterized by pinocytic vesicles, well formed basal lamina, and scattered junctional complex of the plasma membrane.

Painful Forearm Mass in a 75-year-old Man

“…Although there have been isolated reports of glomangiomas with aggressive features, including a report of a locally aggressive glomangioma, this is rare [3]. Glomangiosarcoma, the term for a malignant glomus tumor, is extremely rare [3,13].…”

Glomus tumors of the fingers: Expression of vascular endothelial growth factor

“…Whereas glomangiomas exhibit prominent vascular structures with dilated veins surrounded by clusters of glomus cells, and glomangiomyomas consisted of prominent vascular and elongated, spindled smooth muscle cells[7]. In malignant forms, glomangiosarcoma, pleomorphic tumor cells with marked nuclear atypia and frequent mitotic figures are found in variable numbers[8]. Angiogenesis has been implicated in the progression from benign to malignant tumors.…”

Malignant Glomus Tumors of the Hand

“…Park et al reported a 74-year-old woman with a 1.5-cm ulcerating mass on her palm that was determined to be a glomangiosarcoma with no evidence of metastasis. 6 Finally, Perez de la Fuente et al described a 0.5-cm glomangiosarcoma arising in a glomus tumor in the hypothenar eminence in a 36-year-old woman. 7 Wide local excision has traditionally been recommended as adequate treatment, as most malignant glomus tumors are considered to be unlikely to metastasize.…”

“…There are only 6 reports of malignant glomus tumors in the hand-ranging from 5 mm to 4.5 cm in size. 3,[5][6][7][8][9] Re-classification of glomus tumors has suggested that a diagnosis of malignant should be reserved for tumors with a risk of metastasis, which seems to correlate with a deeper location, size greater than 2 cm, atypical mitotic figures equal or greater than 5 mitotic figures per 50 high-power field, and moderate to high nuclear grade. 1 We describe the largest malignant glomus tumor of the hand ever reported.…”