8711/0 Glomus tumor, NOS


Definitions

Stomach
ICD-O-3 topography code: C16

A rare benign or malignant mesenchymal neoplasm arising from cells that resemble the modified smooth muscle cells of the glomus body. The majority of glomus tumors occur in the distal extremities.

Gastric glomus tumors are also rare and occur in adults, especially women. It usually presents as a 2?5 cm intramural mass with symptoms similar to those of gastric gastrointestinal stromal tumour (GIST). Histological and immunohistochemical features are similar to those of peripheral glomus tumours. Features that are specific to gastric glomus tumours include plexiform growth within the muscular propria and vascular involvement. The latter has no adverse prognostic significance. Immunohistochemically, there is strong expression of SMA and pericellular laminin/collagen IV, but no KIT, DOG1/ANO1, CD34, or desmin. Focal synaptophysin positivity is not uncommon and should not lead to mistaken diagnosis of a neuroendocrine tumour. Most glomus tumours are benign, but metastasis has been reported in a tumour of > 5 cm in size with mitotic activity 1
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Appelman HD, Helwig EB (1969)
Glomus tumors of the stomach.
Cancer 23: 203-13



2
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Miettinen M, Paal E, Lasota J, Sobin LH (2002)
Gastrointestinal glomus tumors: a clinicopathologic, immunohistochemical, and molecular genetic study of 32 cases.
Am J Surg Pathol 26: 301-11



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Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49

/span>lomus tumours are rare mesenchymal neoplasms composed of cells that resemble the modified smooth muscle cells of the glomus body. The majority of glomus tumours occur in the distal extremities, particularly the subungual region, hand, wrist and foot. They arise in skin or superficial soft tissues; rare cases can also be found in deep soft tissue or viscera. Cutaneous lesions are usually small (<1 cm), red-blue nodules. The glomus cells making up the tumour are small, uniform, rounded cells surrounded by basal lamina. Depending on the relative proportion of glomus cells, vascular structures and smooth muscle, three subtypes of typical glomus tumours are distinguished:
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Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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