Granular Cell Tumor of Cecum: A Common Tumor in a Rare Site with Diagnostic Challenge

Abstract

Granular cell tumor (GCT) also known as Abrikossoff’s tumor is a benign neoplasm that is usually seen in the fourth to sixth decades of life with slight female preponderance. It is most frequently seen in the oral cavity, skin, and subcutaneous tissue. Gastrointestinal tract involvement is uncommon, in which esophagus is the most commonly affected site. There are case reports of GCT in stomach, appendix, colon and rectum. In this article, we report a case of GCT involving cecum. The cell of origin in GCT is controversial. There are various pools of thoughts regarding its histogenesis, the details of which are reviewed in this article with emphasis on the diagnostic difficulties encountered in this tumor.

Benign Granular Cell Tumor of the Cecum

“…GCTs are mainly found in patients in their fourth to sixth decades of life and have a female predominance with a 2:1 ratio [2]. These are thought to be derived from the neural tissue (Schwann cells); however, other cell lineages have been proposed as histopathogenesis [3-4].…”

A Rare Case: Granular Cell Tumor of the Tongue

“…50% of GHTs in the head and neck are located in the oral cavity. It is predominantly seen in women in the 4th and 5th decades (1,2,5,8,9). It was first reported by Weber in 1854 and then by Abrikossoff in 1926.…”

“…It is thought to develop from skeletal muscle, histiocytes, fibroblasts, nerve sheath cells, neuroendocrine cells and differentiated mesenchymal cells (2). Recent immunohistochemical and electronmicroscopic studies have shown that the origin of these tumors is "schwann "cells (1,2,9). While GCT is immunohistochemically stained with S100, p75, NSE and CD68, inhibinα, and vimentin, it is not stained with smooth muscle actin (SMA), epithelial membrane antigen, HHF-35, synaptophysin, chromogranin, progesterone, androgen, estrogen, carcinoembryonic antigen (CEA), and PanCK (2,3,9).…”

“…It constitutes less than 1% of head and neck tumors (1). 1-2% of the cases show a malignant course (2)(3)(4). GCT is typically located in the extremities and head-neck skin, oral cavity and gastrointestinal system (5).…”

“…Although it can be seen at any age, it is more common in the 4 th and 5 th decades. It is thought that peripheral nerves originate from "schwann" cells (2). Its benign forms can be treated by local excision (6).…”