Familial complete androgen insensitivity syndrome (Morris syndrome or testicular feminization syndrome) in 2 sisters

In 2005, at the age of 15 and 13 years, respectively, 2 phenotypically similar female siblings with an established clinical diagnosis of Morris syndrome underwent bilateral orchiectomy.On sectioning, the cut surface of all testes showed multiple, well-delimited yellowish to tan intraparenchymal nodules and a solid, whitish area, rubbery in consistency, peripherally located at the medial pole of each testis. The epididymis was not identified in any of the 4 testes. The spermatic cord of each testis seemed grossly normal on external inspection.Histologically, most of the inner parenchymal nodules were composed of hamartomatous proliferations of immature (nearly completely solid) seminiferous tubules, which were filled with immature Sertoli cells, being completely devoid of germ cells, and in places surrounded by numerous Leydig cells. The outer rubbery area, which was notable in each of all 4 testes, was represented by a smooth muscle pseudoleiomyomatous body. Epididymal tissue was not seen microscopically in any specimen. The vas deferens was not found in any of the spermatic cords.