Granular cell tumour of the gastrointestinal tract

Clinical features

Granular cell tumours are commonly found in the tongue and skin, but have also been reported in the gastrointestinal tract. The common sites are oesophagus, caecum and rectum, with rare cases in the stomach, small intestine and anus.

Clinical features

Granular cell tumour of the oesophagus occurs more commonly in women and blacks. Most are solitary and in the distal oesophagus. Usually incidental, large oesophageal tumours may cause dysphagia.

Colorectal granular cell tumours are more common in men. They may be multiple. The most common sites are caecum and rectum.

Macroscopic appearance

The tumours are usually small, causing a yellow submucosal polyp

Histopathology

As in the skin, esophageal and anal granular cell tumours may be overlain by squamous epithelium showing pseudoepitheliomatous hyperplasia1. However, synchronous squamous cell carcinoma of the oesophagus has also been reported.

Immunohistochemistry

S-100

11/111

Nestin

7/71

CD34

0/71

CD117

0/81

inhibin

0/71

desmin

0/71

GFAP

0/71

   

The negativity for inhibin contrasts with the reported positivity in granular cell tumours of the biliary tract2.

Differential diagnosis

Granular cell change has been reported in ameloblastoma, dermatofibroma, leiomyoma, leiomyosarcoma, angiosarcoma, MFH and melanoma.

Prognosis

About 10% of oesophageal granular cell tumours recur and about 4% show malignant behaviour: adverse features are a size greater than 40 mm, infiltrative growth, necrosis, a spindle cell morphology, cytological pleomorphism, mitotic activity, a raised Ki-67 index and positivity for p53.

Malignant behaviour has not been reported in the other sites.

References

1 Parfitt JR, McLean CA, Joseph MG, et al. Granular cell tumours of the gastrointestinal tract: expression of nestin and clinicopathological evaluation of 11 patients. Histopathology 2006; 48:424-30

2 Murakata LA,Ishak KG Expression of inhibin-alpha by granular cell tumors of the gallbladder and extrahepatic bile ducts. Am J Surg Pathol 2001; 25:1200-3

 

This page last revised 24.3.2006.