Primary vulvar leiomyosarcoma localized in the Bartholin's gland area: A case report and review

Abstract

Vulvar sarcomas located in the Bartholin's gland area are extremely uncommon mesenchymal vulvar tumors. These neoplasms can be mistaken as Bartholin' gland benign lesions such as cysts or abscesses, leading to a delay in the diagnosis of underlying malignancy. Currently, only a few cases of these aggressive cancers have been reported in the literature. A 42-year-old female patient without any previous complaint presented to Obstetrics and Gynecology Department of 'G. Chaztikosta' General Hospital due to a vulvar lump in the area of the left Bartholin's gland with a 6-month history of progressive swelling. Pelvic examination showed a solid mass of 6.5-cm in maximum diameter, localized in the left Bartholin's gland. The patient underwent wide local excision and histopathological examination of hematoxylin and eosin-stained sections indicated intersecting fascicles of spindle cells, with moderate to severe atypia. The number of mitoses was up to 8 per 10 high power fields. The neoplasm to its greatest extent was circumscribed and in places had an invasive growth pattern. Tumoral necrosis was not seen. Involved Bartholin' gland by the tumor was identified. The tumor extended focally to the surgical margin. The neoplastic cells showed positive staining for smooth muscle actin, desmin, HHF35, caldesmon, vimentin and estrogen and progesterone receptors. Immunohistochemistry was negative for S100, myoglobulin, keratin 116, CD117, CD34 and CD31. The patient denied further surgery or/and local radiotherapy, although the mass was >5-cm and a focally infiltrative surgical margin was found. During the close follow-up, no local recurrences or metastases were observed 53 months after surgery. In conclusion, wide local tumor excision with free surgical margins is a good option of surgery for vulvar leiomyosarcomas. In recurrences, a new extensive surgical resection of the lesion and radiotherapy are suggested. Ipsilateral lympadenectomy is indicated when there is a pathologic lymph node. Chemotherapy is provided in cases of distal metastases.

Vulvar Leiomyosarcoma in Pregnancy

“…This case is the first case of vulvar leiomyosarcoma showing a rapid increase in size during pregnancy reported in the Gulf region. The literature review revealed few documented cases of leiomyosarcoma located in the female genital tract [1][2][3][4][5][6][7].…”

“…Women often have difficulty articulating vulvar symptoms to medical practitioners, therefore, all women with vulvar symptoms should be examined. It arises most frequently from the labia majora, followed by, in decreasing order, the Bartholin's gland area, clitoris, and labia minora with clinical similarities between them leading to difficulties in clinical diagnosis [2,4,5,7,9,10]. The mean age of incidence has been reported to be between 33 and 40 years, with a range between 15 and 84 years [3].…”

“…Many vulvar cancers are initially misdiagnosed as inflammatory conditions, Bartholin's gland cyst, lipoma, fibroma, or leiomyoma, which could delay the diagnosis and worsening prognosis [2,4,5,7]. Diagnostic and staging workup should be carefully performed for appropriate maternal and fetal management.…”

“…Vulvar sarcomas are rare representing 1-3% of all malignancies arising in the vulva. They are of a mesenchymal origin and the most common types of vulvar sarcomas are leiomyosarcoma, rhabdomyosarcoma, liposarcoma, angiosarcoma, malignant peripheral nerve sheath tumor, malignant fibrous histiocytoma, and epithelioid sarcoma [1][2][3][4].…”

“…Especially given the increasing incidence over the past several decades, however, this increase may be attributed to genetic predisposition, lifestyle, and sexual habits [2,3] In an attempt to add to the scarce literature data on this rare tumor, we are presenting this case report. A review of the course, incidence, diagnosis, and therapeutic approach of leiomyosarcoma of the vulva in pregnancy will be provided in this case report.…”