Glomangiomatosis: a case report

Abstract

Glomangiomatosis is a benign vascular variant of a glomus tumor. The lesion represents only 5% of glomus tumors with unusual or atypical features and even fewer glomus tumors with typical features. The lesions are most commonly located in the distal extremities and are multiple, deep, extensive, and often pain producing. They develop from small arteriovenous anastamoses and are most often identified in young adults. The lesions may recur. We present a case of a 33-year-old male who presented clinically with multiple slowly enlarging masses of the leg over a 5-year period, of which one caused significant pain. One of the lesions was reported to be present at birth. Imaging studies were performed and were suggestive of neurofibromatosis. Biopsies from multiple lesions led to an eventual diagnosis of multiple glomangiomatosis.

Glomangiomatosis - immunohistochemical study: A case report

“…Skeletal muscle infiltration was observed in 5 cases, and extensive nerve and peripheral invasion was seen in 1 case. However, some authors suggest that the glomus cells and angiomatosis components in glomus hemangioma, often accompanied by adipose tissue, are a sign of mesenchymal proliferation and not simple glomus cell proliferation[ 3 , 10 ]. Further study is necessary to determine whether adipose is one of its components or whether it infiltrates into the adipose tissue.…”

“…Immunohistochemistry was positive for SMA and negative for CD34. Fitzhugh et al [ 10 ] reported a 33-year-old man with a mass on the distal right calf. Immunohistochemistry showed that the tumor cells were diffusely and strongly positive for SMA, and the vascular components were strongly positive for CD31 and CD34.…”

“…For example, epithelioid angiomyolipoma is composed of blood vessels, smooth muscle, and fat. The tumor cells immunohistochemically show the presence of HMB45 and Melan A[ 10 ]. In paraganglioma, the tumor cells are arranged in an organ-like structure, and the stroma is rich in capillaries.…”

Glomangiomatosis of the lower leg

“…They are a subtype of atypical glomus tumor as it falls outside the normal clinical and/or histologic spectrum of typical glomus tumors. It has an estimated incidence if 2 in 1,000,000 and accounts for 2%-3% of glomus tumors in adults [ [3] , [4] , [5] ]. Both typical glomus tumors and glomangiomas are painful and typically occur in the extremities [ 5 , 6 ].…”

“…To the best of our knowledge, this is the first reported case of a glomangioma involving the distal tibiofibular syndesmosis. Glomangiomas are typically seen as soft tissue masses that are homogenously T1 hypo-to–isointense, heterogeneously T2 hyperintense and demonstrate post-contrast enhancement [ 3 , 4 , 8 ]. This case demonstrates non-fat suppressing T1 hyperintensity ( Fig.…”