生殖器系と疾患のジャーナル

Granular Cell Tumors are Very Rare but Important Vulvar Masses that should not be Overlooked

Al Naimi A, Schulze F, Schmidt-Fittschen M and Bahlmann F

Granular cell tumors are uncommon neoplasms of neural origins affecting the submucosal and subcutaneous tissue in the tongue, neck, head, and genitals. Vulvar tumors represent about 15% of the cases and present as solitary painless swellings with or without accompanying local manifestations. It is more common in Afro- American women in their fourth to fifth decade.

They are predominantly benign tumors with malignant variants in 1-3% of cases. Malignant forms are aggressive, therapy-resistant, and can metastasize. Wide excision with clear margins is the recommended curative treatment. More than 33% of excisions have positive margins, increasing the risk for local recurrence from 2% to about 20%. The combined risk of recurrence and malignant transformation is a reason for recommending wide re-excision in cases with positive margins. We recommend a preoperative biopsy for every solid vulvar lump in order not to miss such a rare diagnosis and to correctly plan treatment.