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

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

doi:10.5935/1676-2444.20160010

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Jornal Brasileiro de Patologia e Medicina Laboratorial

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Sumário

Pathology, Case report • J. Bras. Patol. Med. Lab. 52 (1) • Jan-Feb 2016 • https://doi.org/10.5935/1676-2444.20160010 copy

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

Tumor papilar agressivo do saco endolinfático: relato de caso de uma rara neoplasia

Authorship SCIMAGO INSTITUTIONS RANKINGS

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.

Key words:
endolymphatic sac; temporal bone; pathology; immunohistochemistry; prognosis

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[1] CORRESPONDING AUTHOR: Eduardo Cambruzzi, Universidade Luterana do Brasil (Ulbra); Av. Farroupilha, 8001; São José; CEP: 92425-900; Canoas-RS, Brasil; Phone: +55 (51) 9979-6026; e-mail:dudacambruzzi@yahoo.com.br.

Authors	Age/gender	Clinical findings	Topography	Treatment modality	Linked to von Hippel-Lindau syndrome	Outcome
Patel et al .⁽⁸8 Patel PC, Pellitteri DO, Reams CL. Aggressive papillary tumor of the temporal bone: delayed extensive recurrence following radiation therapy. Skull Base Surg. 1997; 7(1): 45-8.⁾	63/F	Facial right paralysis	Mastoid and petrous portion of the temporal bone	Total surgical resection	No	No signs of recurrence after 24 months
Horiguchi et al .⁽⁷7 Horiguchi H, Sano T, Toi H, et al. Endolymphatic sac tumor associated with a von Hippel-Lindau disease patient: an immunohistochemical study. Mod Pathol. 2001; 14(7): 727-32.⁾	20/F	Asymptomatic	Left temporal bone	Partial surgical resection	Yes	Large mass after 19 months
Kupferman et al .⁽¹⁰10 Kupferman ME, Bigelow DC, Carpentieri DF, et al. Endolymphatic sac tumor in a 4-year-old boy. Otol Neurotol. 2004; 25(5): 782-6.⁾	4/M	Otitis media and facial paralysis	Right temporal bone	Surgical resection	No	No signs of recurrence after a six-month follow-up
Reijneveld et al .⁽⁹9 Reijneveld J, Hanlo P, Groenewoud G, et al. Endolymphatic sac tumor: a case report and review of the literature. Surg Pathol. 1997; 48(4): 368-73.⁾	63/F	Fifth through eleventh cranial nerve paralysis, ataxia, pyramidal/ pseudobulbar syndrome	Pars squamous and petrosa of temporal bone	Partial surgical resection	No	Unavailable
Schick et al .⁽¹1 Schick B, Kronsbein H, Kahle G, et al. Papillary tumor of the temporal bone. Skull Base. 2001; 11(1): 25-33.⁾	32/M	Hearing loss and vertigo	Temporal bone	Surgical resection	No	No signs of recurrence after 22 months
Sun et al .⁽⁵5 Sun YH, Wen W, Wu JH, et al. Endolymphatic sac tumor: case report and review of the literature. Diagnostic Pathology. 2012; 7: 36.⁾	31/M	Hearing loss	Temporal bone	Partial surgical resection and radiotherapy	No	Unavailable
Ferri et al .⁽¹⁸18 Ferri E, Amadori M, Armato E, Pavon I. A rare case of endolymphatic sac tumour: clinicopathologic study and surgical management. Case Rep Otolaryngol. 2014; 2014: 376761. doi: 10.1155/2014/376761. https://doi.org/10.1155/2014/376761... ⁾	37/F	Hearing loss	Mastoid process of temporal bone and middle ear	Surgical resection	No	No signs of recurrence after 36 months
Present case	38/F	Fifth through eight cranial nerve paralysis	Right temporal bone	Surgical resection	No	Large mass after six years