Stephanie Verta

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

BACKGROUND Leiomyosarcomas of the vulva (VLMS) are very rare among gynecological malignancies, with a lack of knowledge on clinical presentation, prognosis, and therapeutic management. CASE REPORT The database of the German Clinical Center of Competence for Genital Sarcomas and Mixed Tumors in Greifswald (DKSM) was reviewed between the years 2010 and 2020. A total of 8 cases of VLMS were retrieved and analyzed retrospectively. One exemplary case of VLMS was outlined in detail: A 45-year-old premenopausal woman presented with increasing vulvar swelling and discomfort. Given the suspicion of a Bartholin's gland abscess, the mass was excised. Final pathology revealed a solid tumor consistent with a moderately differentiated leiomyosarcoma of the vulva. A wide local excision was subsequently performed followed by adjuvant external beam radiation. The clinical features of these 8 cases of VLMS were compared to 26 cases of VLMS found in a review of the literature and to a total of 276 cases of uterine leiomyosarcoma (ULMS) from the same database (DKSM). CONCLUSIONS In addition to rapid growth, observed in both tumor entities, VLMS most commonly presented as Bartholin's gland abscess or cyst and ULMS as leiomyoma. In this cohort, the prognosis of VLMS was much better than that of ULMS, most probably due to the significantly smaller tumor size of VLMS at diagnosis. Further data and larger studies on VLMS are needed to calculate recurrence and survival rates more accurately and define the role of adjuvant radiotherapy.

Adenoid Cystic Carcinoma of Bartholin’s Gland: A Case Report with Emphasis on Surgical Management

BACKGROUND Adenoid cystic carcinomas of Bartholin's gland are rare among gynecological malignancies, accounting for 0.1% to 7% of vulvar carcinomas and 0.001% of all female genital tract malignancies. There are no specific guidelines regarding treatment recommendations; therefore, they are commonly treated like vulvar cancer. CASE REPORT We present the case of a 42-year-old premenopausal woman with an adenoid cystic carcinoma of Bartholin's gland diagnosed upon biopsy of a palpable, predominantly vaginally located mass causing foreign-body sensation, vaginal pain, and extreme dyspareunia. The adenoid cystic carcinoma of Bartholin's gland was treated by radical resection in an extensive interdisciplinary surgical approach including bilateral inguinal lymph node dissection, partial posterior colpectomy, amputation of the rectum, and creation of a descendostomy, as well as reconstruction of the vagina and defect coverage using flap plastic. CONCLUSIONS With the presentation of this case, we propose a possible therapeutic approach to adenoid cystic carcinomas of Bartholin's gland with emphasis on surgical management. Especially in young patients, we recommend primary radical surgery with the objective to obtain negative resection margins. However, additional data on the adenoid cystic carcinoma of Bartholin's gland is needed to better understand its biological behavior and thus optimize and standardize treatment. The role of systematic inguinal-femoral lymphadenectomy and adjuvant and neoadjuvant treatment modalities need further evaluation.