Granular cell tumors of the vulva

OBJECTIVE: The objective of this study was to review clinical characteristics, histologic findings, and recommendations for surgical treatment of patients with granular cell tumors of the vulva. STUDY DESIGN: A retrospective study of 20 patients diagnosed with a granular cell tumor of the vulva from December 1963 to June 1994 was undertaken to evaluate age, lesion location, size, histopathologic features, presenting symptoms, progression of disease, and treatment. RESULTS: The average age was 50 years, with a range of 26 to 78 years. Fourteen of the 20 patients were black and six were white. Lesions were identified on the labia majora (18), on the clitoris (one), and in the perineal region (one). Only two patients had multiple lesions. Presenting complaints included increasing growth of the lesion (nine), pain (three), and pruritus (one). Six patients were asymptomatic. Lesion size ranged from 0.4 x 0.4 cm to 7 x 8 x 12 cm. Two lesions were found to infiltrate surrounding connective tissue. The 12 lesions that were stained for S-100 protein all had positive results. Nineteen patients were treated with wide local excision only. The twentieth patient required radical surgery because of the large size of the lesion; she died with pulmonary metastasis. CONCLUSION: Granular cell tumors of the vulva are slow-growing, predominantly solitary tumors that are commonly asymptomatic. The tumors originate at the neural crest, as demonstrated by S-100 staining. The majority of lesions are benign and may be treated with conservative wide local excision. An extremely uncommon histologically benign but clinically malignant form of granular cell tumor of the vulva needs special attention.