Difficulties in Differentiation of Endolymphatic Sac Tumor That Extends Into Pontocerebellar Angle from Metastatic Tumors

Year 2024, Volume: 46 Issue: 5, 801 - 806, 12.09.2024

Ganime Çoban , Nurhan Şahin , Mustafa Hatiboğlu , Ayşe Aralaşmak

https://doi.org/10.20515/otd.1403334

Abstract

Endolymphatic Sac Tumor is a local aggressive tumor that grows slowly, associated with Von-Hippel-Lindau disease and originated from endolymphatic canal. A 64-year-old male patient with VHL disease admitted to the neurosurgery department of our hospital and presented pain behind the ear and loss of sensation on the cheek. Cranial magnetic resonance imaging revealed an aggressive tumor located in the middle ear and left acoustic canal and extending to the pontocerebellar angle that eroded the mastoid and temporal bones. Histopathological examination of the lesion revealed a simple cuboidal epithelium forming glandular and papillary structures. The patient diagnosed with endolymphatic sac tumor as the presence of the morphological findings, positive staining with immunohistochemical markers such as Vimentin, NSE, EMA, PAX8, Cytokeratin 7. Our case showed neuroectodermal origin, which is even rare for von Hippel-Lindau disease, and has been discussed with histopathological findings and differential diagnosis.

Keywords

Endolymphatic sac, metastasis, PAX8

Pontoserebellar Köşeye Uzanan Endolenfatik Kese Tümörünün Metastatik Tümörler ile Ayrımındaki Güçlükler

Abstract

Endolenfatik kese tümörü (ELKT), özellikle von Hippel-Lindau hastalığı ile ilişkili, endolenfatik kanaldan orjin alan, yavaş büyüyen lokal agresif bir tümördür. 64 yaşında erkek hasta kulak arkasında ağrı ve yanakta his kaybı ile başvurdu. Kranial manyetik rezonans (MR) incelemesinde sol akustik kanal, orta kulakta yerleşen mastoid, temporal kemikleri erode eden pontoserebellar köşeye uzanan agresif seyirli tümör mevcuttu. Lezyonun histopatolojik incelemesinde glandüler ve papiler yapılar oluşturan tek sıra kuboidal hücreler ile döşeli epitel izlendi. Morfolojik bulgular, Vimentin, NSE, EMA, PAX8, Sitokeratin 7 gibi immünhistokimyasal belirteçlerin pozitif boyanması ile endolenfatik kese tümörü tanısı verildi. Nöroektodermal orjinli, Von Hippel Lindau hastalığında bile nadir görülen endolenfatik kese tümörü olgumuz histopatolojik bulguları ve ayırıcı tanıları ile tartışılmıştır.

Keywords

Endolenfatik kese, metastaz, PAX8

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