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.
Highlights
Aggressive papillary tumor of the endolymphatic sac is an uncommon process arising in temporal bone/endolymphatic sac that is characterized by an aggressive local behavior without metastasizing potential[1,2,3]
A) a neoplastic process exhibiting papillary structures lined by a single layer of epithelial cells, 100×; B) monomorphic columnar cells with clear cytoplasm, ovoid central nuclei, and mild atypias covering papillary structures with numerous capillaries, 200×
endolymphatic sac tumor (ELST) is a rare invasive tumor of the temporal bone/ middle ear that is described in patients aged 4 years to 85 years(1, 5, 6, . 8-10) In general, the affected patients complain of audiological symptoms, vertigo, cerebellar disorders, and facial nerve paralysis
Summary
Aggressive papillary tumor of the endolymphatic sac is an uncommon process arising in temporal bone/endolymphatic sac that is characterized by an aggressive local behavior without metastasizing potential[1,2,3]. It has been reported as an aggressive papillary middle-ear tumor, endolymphatic sac tumor (ELST), aggressive papillary tumor of temporal bone, low-grade adenocarcinoma of endolymphatic sac origin, among others[1,2,3,4,5]. The authors report an example of large recurrent ELST arising in the temporal bone, and discuss clinical and pathologic aspects of this rare tumor
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