A s s o c i a ti on o f C li n ical Path ologist of N e p a l - 2 0 1 0 N e p a l M e d ic al A s sociatio n B uil din g Exhibitio n R o a d , K a t h m a n d u Journal of Pathology of Nepal (2023) Vol. 13, 2022 - www.acpnepal.com Journal of of Nepal PATHOLOGY DOI : 10.3126/jpn.v13i1.51477 Case Report Incidental inguinal tubercular lymphadenitis in a female of androgen insensitivity syndrome- A rare presentation Paridhi 1 , Shalini Bahadur 1 , Madhuvan Gupta 1 , Shalini Shukla 1 , Shivani Kalhan 1 , Deepak Goel 1 , Deeksha Singh 1 , Vivek Gupta 1 1 Department of Pathology, GIMS, Greater Noida, India Keywords: Androgen insensitivity syndrome, Tubercular lymphadenitis, Inguinal tuberculosis, testicular feminization, a disorder of sex development, Ambiguous genitalia ABSTRACT Androgen insensitivity syndrome is a rare X-linked recessive disorder occurring in phenotypic women with a male genotype (46, XY) resulting due to mutation in the X chromosome. On the other hand, tuberculosis is a chronic granulomatous infection caused by Mycobacterium tuberculosis, an acid-fast bacillus. We report a case of a 22-year-old unmarried female presenting with bilateral inguinal pain and primary amenorrhea, which, on a comprehensive evaluation, revealed complete female external genitalia and intra-abdominal testes. The karyotype was 46 XY, hence a diagnosis of complete Androgen insensitivity syndrome was made. One of the ‘probable testes’ removed surgically was an inguinal lymph node with tuberculous lymphadenitis. Isolated inguinal tuberculous lymphadenopathy, by itself, is rare. The co- occurrence of complete Androgen insensitivity syndrome and inguinal tuberculous lymphadenopathy is a highly unusual and rare association that has not been documented in the literature reviewed. Correspondence: Dr. Madhuvan Gupta, MD Department of Pathology Government Institute of Medical Sciences, Greater Noida ORCID ID: 0000-0002-7896-7823 Email: drmadhuvangupta@gmail.com Received : January 14, 2023; Accepted : March 22, 2023 Citation: Paridhi, Bahadur S, Gupta M, et al. Incidental inguinal tubercular lymphadenitis in a female of androgen insensitivity syndrome- A rare presentation. J Pathol Nep. 2023; 13(1):2022-5. DOI: 10.3126/jpn.v13i1.51477 Copyright: This is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. INTRODUCTION Androgen insensitivity syndrome (AIS), alternatively known as testicular feminization (TF) is a rare condition with an incidence of 1 in 20,000-64,000 male births. It is associated with a mutation on Xq11-12 for the human androgen receptor, leading to insensitivity of the receptor to testosterone, due to the failure of androgen to bind to its receptor. Patients are usually phenotypic women or young girls with testes. 1,2 The term TF was earlier coined for this entity, which is a misnomer. The terms TF and AIS are interchangeably used. AIS can be partial (PAIS) or complete (CAIS). Partial AIS (PAIS) patients show a variable degree 2025