Open AccessEndolymphatic sac tumor in von Hippel–Lindau disease: A rare case report
Author(s) -
Rituparna Biswas,
Kalpana Kumari,
Subhash Gupta,
K.P. Haresh,
Anirban Halder,
G K Rath
Publication year - 2017
Publication title -
journal of pediatric neurosciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.247
H-Index - 18
eISSN - 1998-3948
pISSN - 1817-1745
DOI - 10.4103/jpn.jpn_28_17
Subject(s) - endolymphatic sac , medicine , von hippel–lindau disease , lesion , pathology , disease , radiation therapy , radiology , inner ear
Endolymphatic sac tumor (ELST) is a rare papillary neoplasm with locally destructive behavior which can occur sporadically or in association with Von Hippel-Lindau (VHL) disease. We herein present a case of ELST associated with VHL disease in a 14-year-old girl and discuss clinico-radiological, immunohistopathologic findings, and management by staged surgery and postoperative radiotherapy to the residual lesion.