A Case of Synchronous Malignant and Benign Granular Cell Tumor of the Vulva

Abstract

Granular cell tumors (GCT) are rare soft tissue neoplasms, which seldom occur in the vulva. They are more commonly benign, but malignant GCT do occur. We report a case of a 50-yr-old postmenopausal woman who presented with a vulvar lesion that was diagnosed as GCT on biopsy. Imaging and clinical examination revealed an enlarged, likely positive lymph node. Pathology of the subsequently resected total deep vulvectomy specimen showed 2 histologically distinct GCTs. The larger lesion met criteria for malignancy and histologically corresponded to metastatic deposits seen in the pelvic lymph nodes. The separate smaller lesion was histologically benign. This case illustrates a malignant GCT with a synchronous, likely benign GCT both occurring in the vulva. Our case demonstrates the application of histologic criteria in the diagnosis of malignant and benign GCT with discussion on the diagnosis and treatment of this rare tumor.

Granular cell tumor of the vulva: Case report and systematic review of the literature

“…Three studies [ 7 , 14 , 26 ] reported 2 cases of malignant as well as 1 synchronous malignant and benign GCT of the vulva were identified. Firstly, Ramos et al [ 7 ] reported a 17 years patient with malignant GCT of the left labia majus, of whom were treated with left hemivulvectomy and left groin lymph node dissection soon after local resection, which involved positive surgical resection margin.…”

“…Unfortunately, the patient reported pulmonary, hepatic and skeletal metastases and died of hepatic failure 4 months after demission. Interestingly, Baranova et al [ 26 ] presented a 50 years old postmenopausal patient with 7 × 4 cm tumor in the left labia and partially the left mons pubis. The patient initially underwent biopsy firstly and the histological results demonstrated an infiltrative lesion with positive for S100 protein immunohistochemically.…”

“…Malignant GCT is a high grade-invasive disease with widespread metastases 3 to 5 years after diagnosis, which means lymphadenectomy and vulvectomy were needed to improve the prognosis. [ 7 , 26 , 33 ] Whether adjuvant chemotherapy or radiotherapy for malignant GCT remains controversial. For example, Leib et al [ 35 ] disapproved radiation therapy was used for GCT of the vulva.…”

“…In addition, Ki-67 immunostain values greater than 10% can help to classify malignant cases histologically. [8,34] Before planning treatment, we should exclude multicentric lesions via taking a detailed history combined with systematic physical examination and exclude metastases by imaging examinations, like chest Ellison [23] 2003 1 Asymptomatic nodule 48 Left labia majus Solitary S100, D-PAS Benign N/No recurrence Laxmisha [24] 2007 1 Asymptomatic lesion 12 Clitoris Solitary N N 6/No recurrence Cui [4] 2018 1 Asymptomatic lesion 12 Right labia majora Solitary S100, CD68 N N/No recurrence Garg [25] 2020 1 Growing swelling 48 Left labia majus Solitary S100, PAS Benign N Baranova [26] 2021 1 Itching N Left labia and partially the left mons pubis Solitary S100, Ki-67 (1%-19%) Synchronous malignant and benign 1/No recurrence Cheewakriangkrai [5] 2005 1 Asymptomatic lesion 24 Left labia majus Solitary S100, NSE, PAS N N/No recurrence Manning-Geist [27] Kavak [28] 2021 1 Enlarged mass 24 Left labia majus Solitary S100 N N Sonmez [29] 2016 1 Asymptomatic lesion N Right labia majus Solitary S100 Benign 7/No recurrence Hong [30] 2013 Patabendige [31] 2019 1 Enlarged lump 24 Mons pubis Solitary S100 Benign 4/No recurrence Rivlin [13] 2013 1 Growth lesion 144 Left labia majus Solitary S100 N 18/No recurrence Kardhashi [16] 2010 2 Asymptomatic nodular × 2 72/24 Left labia majus × 2 Solitary × 2 S100, CEA N 72/No recurrence 10/Recurrence Ramos [7] 2000 1 Enlarged mass 8 Left labia majus Solitary S100, CD68, Ki-67 (5%-10%) Malignant 16/No recurrence Schmidt [14] 2003 1 N N Left labia majus spreading to the mons pubis Solitary S100, p53 (10%), MIB-1 (20%) Malignant 14/ Pulmonary,hepatic and skeletal metastases Mehta [32] X-ray, abdominal sonography or pelvic MRI. Due to the rarity of the disease, there is clearly, no standard surgery regimen for GCT of the vulva.…”