Vulvar granular cell tumor

Abstract

Granular cell tumors are rare, usually benign, soft tissue neoplasms of neural origin. They occur more often in females than males, the peak age incidence is in the fourth through fifth decades. They can occur anywhere in the body with up to 15% situated in the vulva. The commonest presentation is as an asymptomatic mass. Microscopic findings are usually sufficient, but immunohistochemistry can also be helpful in confirming the diagnosis. The vulvar tumors are benign in 98% of cases with 2% reported as malignant. In this case report we describe a woman with a granular cell tumor confirmed by biopsy who underwent excision of the mass but with focal extension to the resection margin on microscopy. Our recommendation of re-excision was declined. Since it is not uncommon with these tumors to find groups of tumor cells extending beyond the macroscopic limits of growth, we conclude that it is advisable to have margins assessed intraoperatively by frozen section such that further excision can be performed for positive margins. Our patient has been followed for 18 mo without recurrence, should the tumor recur, re-excision, with frozen section control, is indicated. Recurrence rates are reported as 2%-8% with clear margins and 20% with positive margins.

Incidental Diagnosis of Granular Cell Tumor of Vulva: Importance of Discussing Vulvar Health at the Annual Well-Woman Examination

“…Granular cell tumors are rare tumors of neurogenic or Schwann cell origin and hence can present in virtually any tissue with nerve cells. Accordingly, they have been reported in virtually all organs of the body including the head and neck region, with up to 15% occurring in the vulvar area [5]. They occur most commonly in the third to sixth decades of life.…”

“…In addition to being poorly understood, they present as nodules that are often palpable on the surface of the skin. Majority of these tumors are benign; however, about 1-2% are malignant [5].…”

“…Most lesions are well circumscribed, but up to 50% may show poorly defined margins [5]. Granular cell tumors can mimic most known vulva cystic (Bartholin's gland and sebaceous cysts tumors) and solid benign painless lesions (fibromas, lipomas, papillomas and hidradenomas).…”

“…However, because of the potential for recurrence and indeed malignancy, the current recommendation is wide local excision with clear margins [6]. Reports of the rate of recurrence of granular cell tumors vary anywhere from 2% to 10% [5,6]. Local recurrence usually occurs within 2 years and is likely a result of inadequate excision of the primary lesion.…”

Tumor de células granulares de la vulva

“…Las tasas de recurrencia son reportadas como 2-8% con márgenes libres y un 20% con márgenes positivos 4 .…”