Isolated and Synchronous Vulvar Granular Cell Tumors: A Clinicopathologic Study of 17 Cases in 13 Patients

Abstract

Granular cell tumors (GCTs) are benign Schwann cell-derived neoplasms occurring throughout the body. Vulvar GCTs are usually isolated, but occasionally multifocal. On account of their anatomic location, surgical interventions aiming for negative resection margins can result in significant morbidity. We describe the clinicopathologic features of 17 vulvar GCTs in 13 patients followed for an average of 7 years. The average age at presentation was 46 years, and 84% of the patients were black. The tumors were multifocal in 3 (23%) patients, and all, either at presentation or subsequently also developed extravulvar foci. Patients with multifocal vulvar GCTs were nearly 10 years younger at presentation than patients in whom the disease was isolated. The most common complaint was a slow-enlarging mass occasionally associated with pruritus or overlying hyperpigmentation. Clinically, the tumors were subcutaneous, mobile, and nodular (2.1 cm on average), without overlying ulceration, and most often were found in the labia majora (6/17). The neoplasms were histologically heterogeneous, but exhibited either a predominantly nodular (3/17) or infiltrative (13/17) pattern of invasion. Cytologically, the tumors displayed round to polygonal cells with a granular cytoplasm, small hyperchromatic nuclei with minimal pleomorphism, and less than 2 mitoses per 10 high power fields. One tumor (1/17) consisted of cells with predominantly vesicular nuclei and prominent nucleoli and was classified as an atypical vulvar GCT. All tumors so examined were reactive for S-100 protein. Eight of 17 tumor excision specimens had positive margins. Of these, 5 tumors remained stable whereas the other 2 with follow-up progressed to require reexcisions after periods of 14 and 8.0 years, respectively. All patients with excisions with negative margins remained stable. Patients with multifocal tumors did not have a higher risk of recurrence per tumor, compared with patients with isolated disease, regardless of the margin status. No patient died from her disease. As granular cell neoplasms have such a low risk or recurrence and behave generally in an indolent manner, aggressive therapy is usually unwarranted.

Giant myoma associated with uterine prolapse during the second trimester of twin pregnancy: case report

“…3 Vulvar GCTs often present as a slowly enlarging subcutaneous, mobile mass that may present with pruritis and hyperpigmentation. 10 Microscopically, GCTs are distinguished by the presence of granulosa cells -small, pale, round cells with coarse, eosinophilic cytoplasmic granules which stain positive for neural markers such as S100. 3,7,9 To date, few cases of clitoral granulosa cell tumors have been reported.…”

“…3,5,11 There is ongoing debate regarding treatment for GCTs as most studies indicate that radiation and chemotherapy have not been successful. 10,12 A study by Golchai J et al however, found that neoadjuvant chemotherapy successfully treated GCT of the uterine cervix. 11 This discrepancy illustrates the need for more research and development of treatment guidelines for GCTs.…”

A Case of Granular Cell Tumor of the Clitoris in a Postmenopausal Woman

“…There have been attempts to treat radiation and chemotherapy, but they have not been successful. 14 The recurrence rate is 2% to 8% when negative resection margin is negative, and increases to 20% when resection margin is positive. 15 One study reported that surgery after isophosphamide, etoposide, and cisplatin neoadjuvant chemotherapy was successful in a GCT of the uterine cervix and there was no recurrence.…”

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

“…Bu yedi vakanın ikisi 8 yıl ve 14 yıl sonra progresyon göstermiştir. [25] Malign GHT'ler radyoterapi ve kemoterapiye oldukça dirençlidir. [19] Uzak metastazı olan vulvar GHT olan bir hastaya 5-FU+Cisplatin denenmiş, tedavi başarısız olunca paklitaksel ve karboplatin verilmiş yine de tümör cevabı sağlana-mamıştır.…”