Endolymphatic sac tumour
| Endolymphatic sac tumour | |
|---|---|
| Diagnosis in short | |
 Endolymphatic sac tumor H&E stain. | |
|
| |
| LM | Papillary epithelial structures. |
| LM DDx | Plexus papilloma |
| IHC | CK7 +ve |
| Site | usually inner ear |
|
| |
| Symptoms | Tinnitus, dizziness |
| Prevalence | very rare |
| Prognosis | locally agressive |
Endolymphatic sac tumour, abbreviated ELST, is a very rare tumour associated with von Hippel-Lindau disease.
General
- Rare.
- Locally aggressive.[1]
- Associated with von Hippel-Lindau disease.
- May be sporadic.[1]
Clinical - common:[2]
- Hearing loss (sensorineural).
- Tinnitus.
- Dizziness.
Treatment:
- Surgical excision.[2]
Microscopic
Features:[1]
- Cystic spaces.
- Papillary structures.
- Low grade cytomorphology (lack nuclear pleomorphism).
DDx:
- Choroid plexus papilloma - when infiltrating cerebellopontine angle via petrous bone
- Metastatic adenocarcinoma
Images
www:
ELST low power, HE (WC/Soca1zim)
ELST high magnification, HE (WC/Soca1zim)
ELST very high magnification, HE (WC/jensflorian)
Intraoperative frozen section, ELST, HE (WC/jensflorian)
IHC
- CK7 +ve
- S-100 +ve (focally)
- CD31 +ve - marks the outline of the papillary structures.[1]
- Kir 7.1-ve - discriminates tumor from plexus papilloma.[4]
See also
References
- ↑ 1.0 1.1 1.2 1.3 1.4 Künzel, J.; Agaimy, A.; Hornung, J.; Lell, M.; Ganslandt, O.; Semrau, S.; Zenk, J. (2014). "Sporadic endolymphatic sac tumor--a diagnostic and therapeutic challenge.". Int J Clin Exp Pathol 7 (5): 2641-6. PMID 24966979. Cite error: Invalid
<ref>tag; name "pmid24966979" defined multiple times with different content - ↑ 2.0 2.1 Friedman, RA.; Hoa, M.; Brackmann, DE. (Feb 2013). "Surgical management of endolymphatic sac tumors.". J Neurol Surg B Skull Base 74 (1): 12-9. doi:10.1055/s-0032-1329622. PMID 24436884.
- ↑ Yang, X.; Liu, XS.; Fang, Y.; Zhang, XH.; Zhang, YK. (2014). "Endolymphatic sac tumor with von Hippel-Lindau disease: report of a case with atypical pathology of endolymphatic sac tumor.". Int J Clin Exp Pathol 7 (5): 2609-14. PMID 24966975.
- ↑ Schittenhelm, J.; Roser, F.; Tatagiba, M.; Beschorner, R. (Jul 2012). "Diagnostic value of EAAT-1 and Kir7.1 for distinguishing endolymphatic sac tumors from choroid plexus tumors.". Am J Clin Pathol 138 (1): 85-9. doi:10.1309/AJCPPRKNNL09JTLP. PMID 22706862.