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Vulvar carcinoma in Fanconi Anaemia: A case report with review of literature
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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8347819/ https://www.ncbi.nlm.nih.gov/pubmed/34401436 http://dx.doi.org/10.1016/j.gore.2021.100841 |
Sumario: | 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. |
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