Anaplastic Large Cell Lymphoma In Undescended Testis Of A Patient With 46, Xy Disorder Of Sex Development (Dsd): A Case Report

A rare congenital abnormality, 46, XY DSD affects development of chromosomal, gonadal, or anatomical sex characteristics. Disorders of sex development (DSD) patients frequently present with an undescended testis or cryptorchidism, which may result in low levels of testosterone and high levels of follicle-stimulating hormone (FSH) and luteinizing hormone (LH). The LH receptor is involved in development of T-cell lymphoma. Cryptorchidism is associated with increased risk of development of malignancies, although lymphoma is rare in DSD patients. This present study presents a case of a 54-year-old woman with low levels of testosterone and high levels of FSH and LH due to 46, XY DSD. She was diagnosed with cryptorchidism-related anaplastic large cell lymphoma. Based on this case, it was hypothesized that testicular dysfunction and secondary increases in FSH and LH levels increase the risk of lymphomagenesis in individuals with congenital 46, XY DSD.