Pure seminoma in the setting of androgen insensitivity syndrome treated with surgical resection and para-aortic radiation: a case report and review of literature.
Complete androgen insensitivity is a rare X-linked disorder characterized by a female phenotype in a chromosomally male individual. Malignant transformation of the un-descended testis is a rare phenomena compared to other inter-sex syndromes. This is a case of a 32-year-old female who was diagnosed with androgen insensitivity and presented to the emergency room with pelvic pain. Later the pelvic pain was found to be due to testicular masses, one of which was pure seminoma. We reviewed the literature emphasizing the biochemical and endocrinologic abnormalities leading to the syndrome, as well as the potential for malignant changes of the un-descended testes, diagnosis, and therapeutic management. We discuss the importance of early diagnosis and the consequence associated with misdiagnosis.
Stich Radiation Center, Weill Cornell Medical Center, 525E 68th street, New York, NY 10021, USA.
Michael
M
Herman
Gabriella A
GA
Wernicke
Weisi
W
Yan
Dattatreyudu
D
Nori
Bhupesh
B
Parashar
D000328:Adult; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D018239:Seminoma / Q000503:physiopathology* / Q000532:radiotherapy / Q000601:surgery