Glomuvenous malformation (GVM), previously termed glomus tumor or glomangioma, is a rare benign hamartoma of the glomus body,¹ distinct from venous malformations, and comprising less than 2% of all soft-tissue tumors. It occurs most commonly in adults between the fourth and seventh decades of life,² although it has been reported even in children.³ This tumor most frequently subcutaneously occurs in the arms (14%) and legs (13%), with less common occurrence on the trunk (3%).²

Terminology in the literature is rather confusing and tumors can arise from both the glomus body and the glomus cell. A glomus cell is a peripheral chemoreceptor mainly located in the carotid bodies and aortic bodies, and less commonly in other organs. They are located along some blood vessels and nerves and help the body regulate breathing. It is able to detect changes in the blood’s pH, oxygen saturation, and carbon dioxide levels. Clusters of glomus cells, of which the carotid bodies and aortic bodies are the most important, are called nonchromaffin or parasympathetic paraganglia. They are also present along the vagus nerve, in the inner ears, in the lungs, and at other sites. Tumors arising from glomus cells include glomus jugulare, glomus tympanicum, and paragangliomas in other deep sites. Some publications separate tumors from glomus cells and those from the glomus body as distinct, whereas others refer to both as glomus tumors. Deep-seated GVMs arise in locations that include the mouth, trachea, esophagus, stomach, lungs, colon, vagina, penis, and bones.

The glomus body was first described by Wood in 1812.⁴ It is a normal intradermal arteriovenous shunt scattered in the dermis of the skin and is most frequently found in the pulps of the fingers and toes, the tip of the nose, and pinnae of the ears. Their primary function is thought to be thermoregulation.⁵ The glomus body shunts blood away from the skin surface when exposed to cold temperature, thus preventing heat loss, and allowing maximum heat flow to the skin in warm weather to allow heat to dissipate. This structure is composed of an afferent arteriole that gives rise to several preglomic arterioles, and these anastomose with venules that are surrounded by a thick sheath with an S-shaped lumen, known as a Sucquet-Hoyer canal.⁶,⁷,⁸,⁹,¹⁰ The Sucquet-Hoyer canals are lined by endothelial cells, which are surrounded by smooth muscle cells. The glomus bodies are interspersed within the smooth muscle. Hyperplasia of any of the structures composing the glomus body, glomus cells, vasculature, and smooth muscle cells will result in a GVM.⁸ Malignant and atypical GVMs have been classified into several subtypes,¹¹ but none of these have been reported to occur in the eyelids.