A Rare Case of Endolymphatic Sac Tumour: Clinical, Radiological, Pathological and Immunohistochemical Findings with Revi
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CLINICAL REPORT
A Rare Case of Endolymphatic Sac Tumour: Clinical, Radiological, Pathological and Immunohistochemical Findings with Review of Literature Tarang Patel1
•
Poonam Arora1 • Virendra Kumar Meena2
Received: 13 October 2020 / Accepted: 9 November 2020 Ó Association of Otolaryngologists of India 2020
Abstract Endolymphatic sac tumour (ELST) is a rare low grade malignant epithelial tumour of the petrous temporal bone, thought to arise from papillary epithelium of the endolymphatic sac. They may occur sporadically or in association with Von-Hippel Lindau disease. ELST is extremely rare neoplasm with benign histopathological appearance and clinically destructive behaviour. Because of the rarity of this tumour, it can easily be confused with other tumours such as paraganglioma, middle ear adenoma, metastatic carcinomas or choroid plexus papilloma. We report here a rare case of ELST with review of literature and discuss the differentiating features of ELST from its mimickers, showing a papillary configuration. Keywords Endolymphatic sac Metastatic carcinoma Choroid plexus papilloma Immunohistochemistry
Introduction Endolymphatic sac tumour (ELST) is a rare, locally aggressive hypervascular tumour of papillary architecture which arises from the endolymphatic sac in the posterior petrous bone. Hassard et al. in 1984, described the first case of endolymphatic sac tumour as papillary cystic neoplasm arising in the petrous bone [1]. Since that time, only 200 cases are reported in the literature. It is synonymous with Heffner tumour [2] and low grade adenocarcinoma of endolymphatic sac origin (LGAES), according to the recent World Health Organization classification of Head and Neck tumours [3]. Here we report a case of Endolymphatic sac tumour in 50 year old female who presented with leftsided facial paralysis.
Case Report
& Tarang Patel [email protected] Poonam Arora [email protected] Virendra Kumar Meena [email protected] 1
Department of Pathology, Geetanjali Hospital Campus, Geetanjali Medical College & Hospital, B2/G4, Doctors’ quarters, NH-8 bypass, Manvakhera, Udaipur, Rajasthan 313002, India
2
Department of Radiodiagnosis, Geetanjali Medical College & Hospital, Udaipur, Rajasthan, India
A 50 year old woman presented in ENT department with left-sided facial paralysis and left lower eyelid drooping with other complaints of hearing disturbances, tinnitus in left ear, giddiness & headache for one month. Patient had no other significant past or family history. Otoscopic findings were normal. Magnetic resonance imaging (MRI) of Brain with contrast showed a well defined extra-axial, predominantly solid mass, measuring 5.5 9 3.8 9 3.4 cm, in left cerebellopontine(CP) angle region, broad based to petrous temporal bone. It showed hypointense signals in T1W and heterogeneously isointense signals in T2W/ FLAIR sequences with moderate homogenous contrast enhancement. Anteriorly, lesion infiltrated the left petrous apex, mastoid air cells and left clivus and protruded in the left sphenoid
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