Vulvar carcinoma in Fanconi Anaemia: A case report with review of literature

• Main Authors: Vysakh Visweswaran (Author), Hridya Jayamohanan (Author), Anupama Rajanbabu (Author), Keechilat Pavithran (Author)
• Format: Book
• Published: Elsevier, 2021-08-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 2352-5789; 10.1016/j.gore.2021.100841
• Keywords: Fanconi anaemia; Vulvar cancer; Squamous cell carcinoma; Genome instability; DNA repair; Vaginal cancer; Gynecology and obstetrics; RG1-991; Neoplasms. Tumors. Oncology. Including cancer and carcinogens; RC254-282
• Source: Gynecologic Oncology Reports, Vol 37, Iss , Pp 100841- (2021)

Fanconi anaemia is a rare autosomal recessive disorder associated with bone marrow failure and congenital malformations. The impaired DNA repair pathways in Fanconi anaemia predispose patients to a high risk of cancers of squamous cell origin, particularly in the head and neck region. Cancers of the vagina and vulva are rare in Fanconi anaemia. Here, we report a case of a 44-year-old female with Fanconi anaemia who developed an ulcerated lesion on the clitoris that extended into the labia majora. A biopsy of the lesion showed well-differentiated squamous cell carcinoma. The patient was treated with wide local excision of the vulval lesion. The patient developed neutropenia post-procedure but recovered in one week time. We have followed up the patient regularly since the procedure. No further issues have been detected to date.