Jornal Brasileiro de Patologia e Medicina Laboratorial ()

Aggressive papillary tumor of endolymphatic sac: case report of a rare neoplasia

  • Eduardo Cambruzzi,
  • Karla Lais Pêgas,
  • Leandro P. Almeida,
  • Gerson Evandro Perondi,
  • Leandro I. Dini

DOI
https://doi.org/10.5935/1676-2444.20160010
Journal volume & issue
Vol. 52, no. 1
pp. 31 – 34

Abstract

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ABSTRACT Aggressive papillary endolymphatic sac tumor (ELST) is a rare neoplasm, occasionally related to von Hippel-Lindau disease, characterized by locally aggressive growth with temporal bone destruction. The authors report a case of ELST in a female patient exhibiting fifth through eighth cranial nerve paralysis. Computed tomography (CT) revealed a large lytic process involving the right temporal bone. The patient underwent surgical resection. At microscopy, a neoplastic process was identified exhibiting monomorphic columnar cells with mild atypias, arranged in a papillary pattern. The lesion exhibited positivity for A31/AE3, epithelial membrane antigen (EMA), and vimentin; and negativity for synaptophysin, glial fibrillary acidic protein (GFAP), neuron-specific enolase (NSE), thyroglobulin, transthyretin, chromogranin, thyroid transcription factor 1 (TTF-1), trans-acting T-cell specific transcription factor GATA-3, and intestinal transcription factor CDX-2. The diagnosis of ELST was then established. Six years after surgical resection, lesion recurrence was observed.

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