Granular cell tumor - Oral Mucosal Lesion
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Synopsis
Granular cell tumors are more common in females and individuals of African descent. The average reported age is between the fourth and fifth decades. Benign granular cell tumors are usually slow growing. Malignant granular cell tumors represent fewer than 2% of cases. They can have rapid growth and possess metastatic potential. The most common sites for metastasis are regional lymph nodes, lungs, and bones. Patients with malignant granular cell tumor are at risk for development of pancreatic and renal cell cancers.
Granular cell tumors are typically solitary, although the presence of more than one tumor has been reported. Nodules occur most frequently intraorally, especially on the tongue. The skin is another common location, and visceral involvement is rare (esophagus, stomach, bronchus, rectal mucosa, anus mucosa, and breast parenchyma have all been reported as primary sites).
Clinically, granular cell tumors appear as firm, flesh-colored or brownish-red nodules, usually on the head and neck or upper body. They vary in size between 0.5-3 cm. Malignant granular cell tumors are more likely to occur on the lower extremity. Histopathologically, granular cells that give the tumor their name contain lysosomes that are periodic acid-Schiff stain positive, diastase resistant, and stain positively with S100 protein and neuron specific enolase.
Two variants have been reported:
- The non-neural granular cell tumor (primitive polypoid granular cell tumor) has similar clinical features, but lacks S100 staining.
- The congenital granular cell lesion (gingival granular cell tumor of newborns) occurs only in newborns at the gums, and does not stain positive for S100.
Codes
ICD10CM:D10.30 – Benign neoplasm of unspecified part of mouth
D23.9 – Other benign neoplasm of skin, unspecified
SNOMEDCT:
404035005 – Granular cell tumor
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Differential Diagnosis & Pitfalls
- Lymphoepithelial cyst – These have a soft doughy consistency and rarely involve the dorsum of the tongue, but rather the ventral surface or floor of mouth.
- Lipoma – These have a soft consistency and are usually exophytic and nodularnodular.
- Intraoral fibroma
- Sebaceous hyperplasia – This rarely involves the dorsum of the tongue and is soft.
- Amyloidoma (AL type, AA type) – This may appear similar with a waxy, firm consistency. There may be a history of plasma cell dyscrasia.
- Scar – A scar from chronic bite trauma tends to be pink rather than yellow.
- Median rhomboid glossitis – A form of candida, this may present as a firm ovoid to rhomboidal nodule in the midline dorsum of the tongue.
- Oral mucosal wart
- Foreign body granuloma
- Squamous cell carcinoma
- Mucocele
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Therapy
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References
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Last Reviewed:03/13/2018
Last Updated:02/04/2021
Last Updated:02/04/2021
Granular cell tumor - Oral Mucosal Lesion
See also in: Overview