Glomangiomatosis generalized D18.0

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 29.10.2020

Synonym(s)

Familial glomangiomatosis; familial glomuvenous malformation; Glomangiomas; glomuvenous malformations; Multiple familial glomangiomas

Definition
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Rare (10-20% of all glomus tumors), clinical and histological glomus tumor variant with multiple, disseminated or segmentally arranged, angiomatous tumors of the skin

Etiopathogenesis
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Autosomal-dominantly inherited genetic defect of the VMGLOM gene (glomin gene), which maps on the gene locus 1p21-p22 Sporadic glomangiomas can probably be classified as somatic mutations of this gene (punctual mosaic).

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Vascular medicine

Hemangioma, cavernous

Benign vascular tumor. Variant of the capillary-cavernous hemangioma. S.u. vascular tumor, cutaneous.

Manifestation
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With children and young adults.

Localization
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Extremities mainly at the acra (fingers, toes, subungual); rarely is an infestation of the mucous membrane.

Clinical features
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0.5 - 3.0 cm in size, blue, to blue-black, isolated or aggregated to beet-like tumor volumes, soft papules and/or nodules, typically without pain symptoms (see below glomus tumor). Rarely found on mucous membranes and internal organs.

Histology
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In the dermis and subcutis, detectable convolutes of large, dilated, thin-walled vessels, which are reminiscent of venous malformations but are distinguished from these by a surrounding single- or multi-row cell row of cuboid cells with very uniform chromatin-tight nuclei. Thrombi or phlebolites can be found in the vascular spaces. No mitoses detectable.

Differential diagnosis
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Multiple cavernous hemangiomas; neurofibromatosis type I; leiomyoma; blue-rubberleb nevus syndrome.

Therapy
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Excision for functionally or cosmetically disturbing localization. S.a.u. Glomus tumor. Successes have also been described with argon or CO2 laser therapies.

Literature
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Ahmed A et al (2003) Intranasal glomangioma. Rhinology 41: 58-60
Brouillard P et al (2002) Mutations in a novel factor, glomulin, are responsible for glomuvenous malformations ("glomangiomas"). At J Hum Genet 70: 866-874
Brauer JA et al (2011) Glomuvenous malformations (Familial generalized multiple glomangiomas).
Dermatol Online J 17(10):9.
Irrthum, H et al (2001) Linkage disequilibrium narrows locus for venous malformation with glomus cells (VMGLOM) to a single 1.48 Mbp YAC. Hum Genet 9: 34-38
Jacobi H et al. (1996) Congenital familial plaque-type glomus tumors. Dermatologist 47: 387-390
Kapur N et al (2002) Local and systemic expression of basic fibroblast growth factor in a patient with familial glomangioma. Br J Dermatol 146: 518-522
Schmiedseder M et al (2014) Multiple blue knots. Familial glomangiomatosis. Dermatologist 65: 556-558
Wolff HW et al (1981) Multiple glomus tumors. dermatologist 32: 354-358