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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...

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Autores principales: Visweswaran, Vysakh, Jayamohanan, Hridya, Rajanbabu, Anupama, Pavithran, Keechilat
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2021
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
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author Visweswaran, Vysakh
Jayamohanan, Hridya
Rajanbabu, Anupama
Pavithran, Keechilat
author_facet Visweswaran, Vysakh
Jayamohanan, Hridya
Rajanbabu, Anupama
Pavithran, Keechilat
author_sort Visweswaran, Vysakh
collection PubMed
description 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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spelling pubmed-83478192021-08-15 Vulvar carcinoma in Fanconi Anaemia: A case report with review of literature Visweswaran, Vysakh Jayamohanan, Hridya Rajanbabu, Anupama Pavithran, Keechilat Gynecol Oncol Rep Case Reports and Case Series 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. Elsevier 2021-07-27 /pmc/articles/PMC8347819/ /pubmed/34401436 http://dx.doi.org/10.1016/j.gore.2021.100841 Text en © 2021 The Authors. Published by Elsevier Inc. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Reports and Case Series
Visweswaran, Vysakh
Jayamohanan, Hridya
Rajanbabu, Anupama
Pavithran, Keechilat
Vulvar carcinoma in Fanconi Anaemia: A case report with review of literature
title Vulvar carcinoma in Fanconi Anaemia: A case report with review of literature
title_full Vulvar carcinoma in Fanconi Anaemia: A case report with review of literature
title_fullStr Vulvar carcinoma in Fanconi Anaemia: A case report with review of literature
title_full_unstemmed Vulvar carcinoma in Fanconi Anaemia: A case report with review of literature
title_short Vulvar carcinoma in Fanconi Anaemia: A case report with review of literature
title_sort vulvar carcinoma in fanconi anaemia: a case report with review of literature
topic Case Reports and Case Series
url 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
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AT rajanbabuanupama vulvarcarcinomainfanconianaemiaacasereportwithreviewofliterature
AT pavithrankeechilat vulvarcarcinomainfanconianaemiaacasereportwithreviewofliterature