Granular Cell Tumors

Updated: Sep 08, 2016
  • Author: Vladimir O Osipov, MD; Chief Editor: Jules E Harris, MD, FACP, FRCPC  more...
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Overview

Background

Granular cell tumors (GCTs), also known as Abrikossoff tumors, are rare soft-tissue neoplasms probably derived from Schwann cells. [1, 2] Most granular cell tumors are benign, although some may be locally aggressive. [3] Less than 2% are malignant, but these are aggressive and associated with a poor prognosis. [4]

Granularity of the cells in these tumors is due to the accumulation of secondary lysosomes in the cytoplasm. This change is rather nonspecific and can be observed in many non-neural tumors, including those arising from smooth muscle, connective tissue, neuroglia, endothelial, and epithelial cells.

This article describes neural granular cell tumors. Non-neural granular cell tumors are discussed only as differential diagnoses.

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Pathophysiology

Granular cell tumors are typically solitary and smaller than 3 cm. They are usually located in the dermis or subcutis and less frequently in the submucosa, smooth muscle, or striated muscle. Granular cell tumors are also found in the internal organs, particularly in the upper aerodigestive tract. Benign and malignant counterparts are known; the latter are rare, comprising fewer than 2% of all granular cell tumors. [5]

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Epidemiology

Frequency

The lesion is uncommon. Exact figures are unavailable. Much of the literature on granular cell tumors consists of reports of single cases.

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Race-, Sex-, and Age-related Demographics

Granular cell tumors appear to be more common in black persons. Multiplicity of lesions is definitely more common in black persons. Up to 10% of granular cell tumors are multiple (ie, two to four lesions).

A slight female predominance exists. The female-to-male ratio is estimated at approximately 3:2. [5]

Granular cell tumors affect persons of varying ages, and the range is wide. Most patients are middle-aged, with a peak incidence in the fourth through sixth decades of life.

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