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.

Keywords: Bartholin's gland; leiomyosarcoma; soft tissue; vulva; vulvar diseases; vulvar neoplasm; vulvar sarcoma.

Figure 1

Spindle cell neoplasm with storiform pattern. Hematoxylin and eosin staining of tumor tissues at a magnification of (A) x40 and (B) x100.

Figure 2

Moderate to severe cytological atypia. (A) Severe cytological atypia; hematoxylin and eosin staining of tumor tissues at a magnification of x100. (B) Moderate cytological atypia; hematoxylin and eosin staining of tumor tissues at a magnification of x100.

Figure 3

Presence of mitotic figures. (A) Hematoxylin and eosin staining of tumor tissues at a magnification of x200. (B) Focally infiltrated margin, hematoxylin and eosin staining of tissue at a magnification of x40.

Figure 4

Immunohistochemical expression of spindled tumor cells for (A) SMA at a magnification of x100; (B) desmin at a magnification of x100; and (C) HHF35 at a magnification of x40.

Figure 5

Immunoreactivity of spindled tumor cells for (A) caldesmon at a magnification of x100; (B) vimentin at a magnification of x10; (C) estrogen receptors at a magnification of x100; and (D) progesterone receptors at a magnification of x100.