Introduction/Background Adenoid cystic carcinoma (ACC) is an aggressive slow-growing tumor occurring in women with mean age 49 years. The neoplasm is similar to those of salivary gland, evolving from chromosomal translocation (NFIB) and having no HPV-correlation. ACC accounts for 15% of Bartholin´s gland (BG) malignancies and for 2–7% for vulvar carcinomas, with less than 90 cases described. Treatment remains individualistic as there is no level I evidence available, making the new cases crucial to be reported.
Methodology A 46-year-old female, presented after a positive biopsy for ACC-BG. She reported a small swelling in her left posterior labia minor during the last 6 months. She had the BG cyst removed and the pathology showed ACC 3 × 3 cm encapsulated inside the cyst. Another 45-year-old female, presented after an excision of a vulvar mass 3,5 × 2,5 × 2,5 cm of the left labia minor after swelling for the last 2 years which resulted histologically in ACC-BG with positive margins and neural and muscular invasion.
Results Supplementary surgery was decided and the first patient underwent radical left hemivulvectomy with ipsilateral inguinofemoral lymph node excision. There was no residual disease and no adjuvant therapy was needed. In the second case we performed imaging control and radical vulvectomy with bilateral inguinofemoral lymph node dissection. The pathology-report described a middle line tumor 2 × 1,25 × 1,2 cm infiltrating the outer third of vagina plus one left lymph node, having negative margins (FIGO stIIIa); she then underwent adjuvant radiotherapy. Both women are disease-free after 16 and 20 months respectively.
Conclusion Radical excision with negative margins is beneficial, while adjuvant radiotherapy should be considered for patients with risk factors (tumor size, lymph node status, surgical margins). Lung is the most common site of distant metastasis and several chemotherapeutic agents have been used such as cyclophosphamide, adriomycin, 5-FU, cisplatin and tamoxifen. ACC should always be considered in patients older than 40 years with BG lesions.
Disclosure Nothing to disclose
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