Glomus tumor D18.01

Mostly solitary, rarely disseminated or in segmental arrangement, benign extra-adrenal paraganglioma derived from atriovenous anastomoses(Glomus cutaneum = Hoyer-Grosser organs).

Clinical Variations:

• Classic type: Solid, usually solitary, painful, localized to the acras of the extremities.
• Angiomatous type: Soft, mostly multiple, not painful, ubiquitous (see below Glomangiomatosis).

Occurs at any age, preferably in the age group between 40 -60 years. Women are significantly more frequently affected (up to 10 times more frequently in larger studies)

Mainly in the nail bed area of fingers and toes, also on the acra of fingers or toes, more rarely on the knee, nose and ears or in the oral mucosa. More rarely in the gastrointestinal tract, liver, genital area or respiratory tract (see below parasympathetic paraganglioma).

Mostly solitary, roundish, often spherical protuberant, red or bluish, solid nodule measuring a few millimetres up to 2.0 cm (note: tumour size greater than 2.0 is regarded as malignancy criteria).

Typical, lancing, shooting cold and touch pain, rarely also spontaneous or heat pain.

Frequent hyperhidrosis in the vicinity of the tumour.

As the tumours are frequently localised under the nail plate, they appear there as circumscribed pain, which is felt to be very unpleasant. Usually a blue to blue-grey colour change is detectable under the nail, which is very painful when pressure is applied.

In addition to the solitary type, there is a multiple variant (see Glomangiomatosis generalized).

Nodular, well-demarcated tumour convolutes in the deep dermis, consisting of very uniform, sinusoidal or elongated vascular spaces arranged in strands, which may also be entirely backgrounded. The vascular spaces are lined by flat endothelia. The tumor nodules are surrounded by a connective tissue capsule. Mast cells are frequently present (solid glomus tumor type).

• Glomangioma (most common type of glomus tumor; about 60%): Here, dilated vascular spaces lined by a single- to multi-rowed layer of uniform cuboid cells are in the foreground.
• Solid glomus tumor without conspicuous lumina
• Glomangiomyoma with spindle differentiated myoid cells.

Immunohistology: The tumor cells are positive for the intermediate filaments vimentin and desmin (see below cytoskeleton), but negative (in contrast to the endothelia) for factor VIII and beta2-microglobulin.

Remember! In case of persistent, circumscribed pressure pain of the nail organ always think of glomus tumor! (see below the acronym ANGLES for other painful skin tumors).

Very rarely malignant glomus tumors(glomangiosarcoma) with metastases are described.

Clinical and histological predictors are: size > 2 cm, deep localization, high mitotic rate, atypical mitotic figures.

The term "glomus" comes from the Latin and means ball.

Glomus tumors are not the same as glomangiomas. They are different entities.

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12. Watanabe K et al (1998) Glomangiosarcoma of the hip: report of a highly aggressive tumour with widespread distant metastases. Br J Dermatol 139: 1097-1101