Familial Generalized Multiple Glomangiomyoma: Report of a New Family, with Immunohistochemical and Ultrastructural Studies and Review of the Literature

Abstract

Glomus tumors are benign lesions which often appear as solitary bluish nodules. They can also be multiple, and can be either acquired or congenital. Histopathologically glomus tumors are classified into three different variants: solid glomus tumor, glomangioma, and glomangiomyoma, which is the least frequent type. We report three instances of familial generalized multiple glomangiomyoma in a woman and her two children. Seven members of the family in two consecutive generations were affected, suggesting autosomal dominant inheritance. We performed a thorough clinical study, complete blood and platelet counts, stool for occult blood, karyotype, abdominal echography (which showed an asymptomatic solitary hepatic vascular lesion in one of the patients), gastrointestinal endoscopy, and skin biopsy with immunohistologic and ultrastructural studies. We believe that the terms glomangioma and glomangiomyoma actually designate the same lesion, with transitional areas from typical glomus cells to well-defined conventional smooth muscle cells. Although complete evaluation should be performed in all patients with multiple glomus tumors in order to detect possible occult systemic lesions, we only recommend treatment for symptomatic lesions. To our knowledge, this is the first report of an instance of familial multiple glomangiomyoma.

Clinical and Histopathological Diagnosis of Glomus Tumor

“…4 Glomangioma and glomangiomyoma are classic variants of the common form of glomus tumors. 5,6 Glomus tumors are typically composed of 3 components: glomus cells, smooth muscle cells, and vasculature. The classical histological features of the glomus tumor include angiocentric uniform sheets of cells with oval nuclei, forming a perivascular “collar” around vessels.…”

Malignant glomus tumour of larynx: first case report and literature review

“…4,6,10,13,[17][18][19][23][24][25] The various reported cases of benign and malignant glomus tumours have differed in their immunostaining pattern, but most, like ours, have shown positivity for smooth muscle actin, type IV collagen, cluster of differentiation 34 glycoprotein and vimentin.…”

Multiple Glomangiomas Involving the Glans Penis in Pediatric Patient

“…The hypothesis is supported by ultrastructural and immunohistochemical findings, as well as the typical occurrence of tumors in regions rich in glomus bodies, namely, nail beds and fingertip pads, palms, wrists, followed by the foot and forearms [4,11]. However, their sporadic location in ectopic sites devoid of glomus bodies favors the concept of neural crest cells or pericytes origin [12].…”

“…The latter are thought to be char-acterized by some different clinicopathologic features. Multiple tumors are more often hereditary and transmitted in an autosomal dominant fashion with incomplete penetrance and variable expression [4]. They have been recently linked with the glomulin gene located in chromosome 1p21-22 [5].…”