Endolymphatic sac tumor in von Hippel–Lindau disease: A rare case report | Zendy

K.P. Haresh | Zendy; Rituparna Biswas | Zendy; Kalpana Kumari | Zendy; S. Datta Gupta | Zendy; Anirban Halder | Zendy; GK Rath | Zendy

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Endolymphatic sac tumor in von Hippel–Lindau disease: A rare case report

Author(s) -

K.P. Haresh,

Rituparna Biswas,

Kalpana Kumari,

S. Datta Gupta,

Anirban Halder,

GK 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.

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