Pontoserebellar Köşeye Uzanan Endolenfatik Kese Tümörünün Metastatik Tümörler ile Ayrımındaki Güçlükler

Öz

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.

Anahtar Kelimeler

• Endolenfatik kese
• metastaz
• PAX8

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

Öz

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.

Anahtar Kelimeler

• Endolymphatic sac
• metastasis
• PAX8

Kaynakça

1. 1. Thompson LDR, Maglioca KR, Andreasen S, Kiss K, Rooper L, Stelow L, Wenig BM, Bishop JA. CAIX and pax-8 Commonly Immunoreactive in Endolymphatic Sac Tumors: A Clinicopathologic Study of 26 Cases with Differential Considerations for Metastatic Renal Cell Carcinoma in von Hippel-Lindau Patients. Head Neck Pathol. 2019;13: 355-363.
2. 2. Schnack DT, Kiss K, Hansen S, Miyazaki H, Bech B, Thomasen PC. Sporadic Endolymphatic Sac tumor-A Very Rare Cause of Hearing Loss, Tinnitus, and Dizziness. J Int adv Otol. 2017;13: 289-91.
3. 3. Asano K, Sekiya T, Hatayama T, Tanaka M, Takemura A, Suzuki S, Kubo O, Ishihara Y. A case of endolymphatic sac tumor with long-term survival. Brain Tumor Pathol. 1999;16: 69-76.
4. 4. Maher ER. Von Hippel-Lindau disease. Eur J Can.1994; 30:1987–90
5. 5. Varshney N, Kebede AA, Owusu-dapaah H, Lather J, Kaushik M, Bhullar JS. A Review of Von Hippel-Lindau Syndrome. Journal of Kidney Cancer and VHL.2017; 4: 20-29.
6. 6. Maher ER, Neumann HP, Richard S. Von Hippel–Lindau disease: A clinical and scientific review. Eur J Hum Genet. 2011;19: 617–23.
7. 7. Hassard AD, Boudreau SF, Cron CC. Adenoma of the endolymphatic sac. J Otolaryngol. 1984;13: 213-6.
8. 8. Sun YH, Wen W, Wu JH, Song JM, Guan H, Wang KX, Xu MQ. Endolymphatic sac tumor:case report and review of the literatüre. Diagn Pathol. 2012;7: 36.

9. 9. Önder S, Güler G, İnci S, Ruacan Ş, Söylemezoğlu F. Pontoserebellar açı tümörlerinin histopatolojik ayırıcı tanısında düşünülmesi gereken bir antite: Endolenfatik kese tümörü. Türk Nöroşirürji Dergisi. 2001;11: 226-230
10. 10. Du J, Wang J, Cui Y, Zhang C, Li G, Fang J, Yue S, Xu L. Clinicopathologic study of endolymphatic sac tumor (ELST) and differential diagnosis of papillary tumors located at the cerebellopontine angle. Neuropathology. 2015;35: 410-20.
11. 11. Husseini ST, Piccirillo E, Taibah A, Paties CT, Almutai T, Sanna M. The Gruppo Otologic experience of endolymphatic sac tumor. Auris Nasus Larynx. 2013;40:25-31.
12. 12. Poletti AM, Dubey SP, Colombo G, Cugini G, Mazzonia A. Treatment of endolymphatic sac tumour (Papillary adenocarcinoma) of the temporal bone. Reports of Practical oncology and radiotherapy. 2016;21:391-394.