ABSTRACT
Introduction Endolymphatic sac tumor (ELST) is a slow-growing, low-grade, locallyinfiltrative tumor arising from the endolymphatic sac/duct, which is located in the posterior part of the petrous temporal bone. It may be sporadic in origin, or may be associated with Von-Hippel Lindau (VHL) syndrome. Case description A 40-year-old female patient with an ELST without VHL syndrome who was treated successfully by microsurgical extirpation of the tumor. Discussion We discuss the radiological features and the histopathology of this rare tumor and review the relevant literature. Conclusion The case herein reported adds to the previously-reported cases of this rare tumor.
Subject(s)
Humans , Female , Adult , Paraganglioma/surgery , Petrous Bone/surgery , Skull Neoplasms/surgery , Endolymphatic Sac/surgery , Paraganglioma/diagnosis , Postoperative Complications , Skull Neoplasms/diagnostic imaging , Endolymphatic Sac/pathology , Endolymphatic Sac/diagnostic imaging , Craniotomy/methods , von Hippel-Lindau Disease/pathologyABSTRACT
Persistent mullerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism in which mullerian duct derivatives are present in an otherwise normally differentiated 46 XY male. We report a case of a 33-year-old male with PMDS operated for postchemotherapy seminoma. A diagnosis of PMDS was made on subsequent histopathological evaluation.