Lung Metastasis in a Case of Recurrent Poorly Differentiated Leiomyosarcoma of the Bartholin Gland: A Case Report and Review of the Literature

Abstract

Vulvar neoplasms represent four percent of all gynecological cancers. While most cases of vulvar neoplasms are benign, two percent of patients present with malignant disease. We present the case of a 37-year-old premenopausal female who presented to an outside institution with a lump in her left vulva, which had progressively enlarged to the size of an egg. A wide local excision of the left vulva was performed, and the pathology revealed a high-grade sarcoma, not otherwise specified (NOS), with negative margins. Imaging showed enlarged bilateral external iliac lymph nodes, likely metastatic. After discussion at a multidisciplinary gynecology oncology tumor board, she was treated with gemcitabine/docetaxel chemotherapy, followed by a left inguinal lymph node dissection and a left radical vulvectomy after being referred to our centre. The final pathology at that time showed a residual sarcoma of 3.5 mm in the left vulva with no lympho-vascular invasion (LVI) and negative margins, with the closest, laterally, at 2 mm. A total of three lymph nodes were negative. She received additional chemotherapy postoperatively. Approximately one year later, she returned to her gynecologist with a 1 cm mass on the left vulva. She underwent a left hemi-vulvectomy and lymph node dissection, and pathology confirmed the presence of a high-grade sarcoma with close margins. She received adjuvant radiotherapy. Three months later, she presented with persistent cough and pneumonia. Imaging revealed a 10 cm lung mass, which was believed to be metastasis from the vulva. This was confirmed with biopsy and was completely resected.Any mass in the Bartholin gland area should be investigated carefully. Poorly differentiated vulvar leiomyosarcoma in the Bartholin gland can recur locally but may also lead to distant metastasis. Despite surgical and systemic treatment, as well as adjuvant radiation, the tumor recurred. Due to the rarity of this condition, there are no clear recommendations for treatment of this disease. To our knowledge, this is the first report of vulvar leiomyosarcoma of the Bartholin gland with metastasis to the lung.

Vulvar Leiomyosarcomas: A Case Series with Clinical Comparison to Uterine Leiomyosarcomas and Review of the Literature

“…Three patients died of disease [ 40 ], 1 experienced local recurrence [ 45 ], and 1 was suspected to have a local recurrence but died of an unrelated cause [ 50 ] (information available on 12 cases). In patients <50 years, 1 adverse event, in the sense of local recurrence, occurred (information available on 10 cases=10%) [ 36 ]. These results seem to point towards age (<50 years and ≥50 years) being a possible independent prognostic factor.…”

“…Of the 26 cases summarized in Table 3 , 11 patients had negative margins after the definitive operation, 6 had positive margins, 1 had close surgical margins, and in 8 cases no data were available. Five patients with negative margins (45.5%) were reported to be recurrence-free (follow-up time 4–32 months), 2 patients (18.2%) developed a local recurrence after 12 and 15 months, respectively [ 36 , 45 ], 1 patient (9.1%) was diagnosed with pulmonary metastases after 18 months [ 36 ], and 1 patient (9.1%) died of disease [ 40 ].…”

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

“…However, in the presence of negative prognostic factors radiotherapy and chemotherapy should be given. Important favorite prognostic factors are the lower grade of nuclear atypia, the smaller tumoral size, the pathological confirmation of at least 2-cm negative surgical margins and the absence of local recurrences (1,2,12,14,22,38). Favor immunohistochemical factors include the overexpression of estrogen, progesterone and androgen receptors, the moderate immunopositivity for Ki-67 and the absence of p53 expression (4,22).…”

“…Vulvar sarcomas comprise a highly heterogeneous group of histological types, which include leiomyosarcomas, epithelioid sarcomas, fibrosarcomas, liposarcomas, hemangiosarcomas, rhabdomyosarcomas, angiosarcomas, malignant hemagiopericytomas, neurogenous sarcomas, dermatofibrosarcomas protuberans, Ewing sarcomas, synovial sarcomas, clear cells carcinomas, malignant fibrous histiocytomas and sometimes aggressive angiomyxomas (1,3,4,6,(10)(11)(12). The age of the described patients with vulvar leiomyosarcomas at the time of clinical presentation ranged between 14 and 69 years of age, with an average age of approximately 30-40 years (4,6,13,14). Vulvar embryonal rhabdomyosarcoma typically presents in girls less than 8 years of age (15).…”

“…In these cases, the late symptoms include pain, ulceration, bleeding and voiding dysfunction (3,6,11). Vulvar sarcomas are characterized by a high chance of metastasis; it seems that chemotherapy achieves regression of lung metastasis (6,12,14,16).…”