| Granular cell tumor | |
|---|---|
 | |
| 2-cm tumor presented as an abdominal wall mass in a middle-aged woman | |
| Specialty | Oncology  |
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Granular cell tumor is atumor that can develop on anyskin or mucosal surface, but occurs on thetongue 40% of the time.
It is also known asAbrikossoff's tumor,[1]granular cell myoblastoma,[1]granular cell nerve sheath tumor,[1] andgranular cell schwannoma.[1] Granular cell tumors (GCTs) affect females more often than males.[2]
Granular cell tumors are derived from neural tissue, as can be demonstrated byimmunohistochemistry and ultrastructural evidence usingelectron microscopy. These lesions characteristically consist of polygonal cells with bland nuclei, abundant cytoplasm and fine eosinophilic cytoplasmic granules. The tumor cells stain positively for S-100 as they are of Schwann cell origin. Both malignant and benign versions of the tumor exist, where malignant tumors are characterized histologically by features such as spindling, high nuclear to cytoplasmic ratios, pleomorphism, and necrosis.[3]
Multiple granular cell tumors may seen in the context ofLEOPARD syndrome, due to a mutation in thePTPN11gene.[4]
These tumors, on occasion, may appear similar to neoplasms of renal (relating to the kidneys) origin or other soft tissue neoplasms.
The primary method for treatment is surgical, not medical. Radiation andchemotherapy are not needed for benign lesions and are not effective for malignant lesions.
Benign granular cell tumors have a recurrence rate of 2% to 8% whenresection margins are deemed clear of tumor infiltration. When the resection margins of a benign granular cell tumor are positive for tumor infiltration the recurrence rate is increased to 20%. Malignant lesions are aggressive and difficult to eradicate with surgery and have a recurrence rate of 32%.
Granular cell tumors can affect all parts of the body; however, the head and neck areas are affected 45% to 65% of the time. Of the head and neck cases 70% of lesions are located intraorally (tongue,oral mucosa,hard palate). The next most common location that lesions are found in the head and neck area is thelarynx (10%).[5] Granular cell tumors are also found in the internal organs, particularly in the upper aerodigestive tract. Vaginal granular cell tumors are generally rare.[6]
Breast granular cell tumors arise from intralobular breast stroma and occurs within the distribution of the cutaneous branches of thesupraclavicular nerve. As they do follow the innervation of the skin, they may demonstrate skin changes like contraction or shrinkage. Unlike traditionalbreast cancers, granular cell tumors are mostly found in the upper inner quadrant of the breast.[7] They can be erroneously diagnosed as an invasive ductal carcinoma viaultrasound andmammography, therefore, it is necessary to consider a diagnosis of invasiveductal carcinoma.
The usual presentation is of a slow growing behavior, forming a polygonal accumulation of secondary lysosomes in thecytoplasm. Granular cell tumors are typically solitary and rarely larger than three centimeters. However, proliferative growth and development of an ulcer indicates likely malignancy as this type of tumor can be eitherbenign ormalignant. Malignancy is rare and constitutes only 2% of all granular cell tumors.[8]
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