Benign Granular Cell Tumor of the Vulva: First Report of Multiple Cases in a Family

Abstract

Granular cell tumors (GCTs) are uncommon soft tissue tumors of neural derivation, as supported by immunohistochemical and ultrastructural evidence. Vulvar involvement has been reported in 7–16%. This paper presents the cases of a 60-year-old woman and her 32-year-old niece with a strong family history of cancer, both presenting with an enlarging mass on their left labia majora. The lesions were treated by simple surgical excision. Histopathological examination revealed a benign vulvar GCT in both lesions. This is the first reported case of GCT of the vulva in the same family. The possible familial component of GCT needs further investigation. A systematic review of the literature on vulvar GCTs is carried out, the most complete one to date. This review unexpectedly reveals that there have been more than 130 cases of GCT of the vulva reported to date, only 7 of which were malignant. Since 5–25% of patients have multiple lesions, before planning treatment, clinicians should exclude multicentric lesions. After surgical treatment, if there is any evidence of tumor in the surgical margin, wider local excision should be performed. Regular follow-up is important for diagnosing a possible recurrence or a new lesion.

A rare granular cell tumor of the vulva: a case report

“…Yapılan bir çalışmada, aynı ailede iki GHT saptanmış ve ilk kez genetik geçiş olabileceğinden bahsedilmiştir. [10] Tümör dermis ve subkutan dokudan kaynaklanır. Ülsere ya da depigmente bir lezyon olarak karşımıza çıkabilir.…”

“…[18] Vulvadaki GHT'nin %98'inin benign ve %2'sinin de malign olduğu saptanmıştır. [10] Malign olan GHT oldukça agresif olup, genellikle lokal ve uzak metastaz yaptıktan sonra tanı alır. Malign olguların görülme yaşı ortalama 50'dir.…”

Granular cell tumor of the clitoris in the pediatric age. A case report and review of the literature

“…Granular cell tumors (GCTs), initially described by Wirchow and Weber in 1854 [1], are uncommon skin and soft tissue neoplastic lesions, occurring in less than 0.017e0.025% of all surgical specimens [1,2].…”

“…GCTs are two or three times more common in females than in males, and black patients are more prevalent than whites [4]; although described in patients of any age, they are more frequently seen in the fourth to sixth decades of life, being rare in children [1]. Occasionally, familial and congenital occurrences have been reported [1,3,5].…”

“…Female genital locations are rarely described (7%e16%) and the vulvar tumors are benign in 98% of cases, with only 2% reported as malignant [1]. A single case of vulvar malignant GCT in a 17-yearold patient is described in the Literature [7].…”