ABSTRACT
Endolymphatic sac tumor (ELST) is a rare neoplasm which is seldom evaluated by cytopathology. We report the clinicopathologic course and cytologic cerebrospinal fluid (CSF) findings in a 58-year-old patient with brainstem lesions who originally presented with vertigo but progressed to having left 7th, 8th, 9th, and 10th cranial nerve palsies, right-sided weakness, and occipital headaches. Cytospin of the CSF revealed large epithelioid cells similar to cells seen in a surgical resection of a brain mass three months previously. Review of the surgical specimen revealed a well-differentiated glandular and papillary neoplasm, most consistent with an endolymphatic sac tumor.
Subject(s)
Ear Neoplasms/cerebrospinal fluid , Ear Neoplasms/pathology , Endolymphatic Sac/pathology , Neoplasms, Glandular and Epithelial/cerebrospinal fluid , Neoplasms, Glandular and Epithelial/pathology , Brain Stem/pathology , Brain Stem/radiation effects , Diagnosis, Differential , Ear Neoplasms/radiotherapy , Endolymphatic Sac/radiation effects , Humans , Male , Middle Aged , Neoplasms, Glandular and Epithelial/radiotherapyABSTRACT
Endolymphatic sac tumor (ELST) is a rare low-grade locally aggressive neoplasm of the inner ear that may occur sporadically or in the setting of von Hippel-Lindau syndrome. We herein present a case of sporadic ELST in a 39-year-old man, treated using an interdisciplinary approach (surgery+radiotherapy), with a 10-year follow-up. The patient presented with hearing loss of sudden onset. The treatment of choice for ELST is radical tumor resection, which is associated with a good long-term prognosis. Remission may last for years, but there may be local recurrences, probably as a result of incomplete resection. Adjuvant radiotherapy is an option in case of recurrence and could be discussed after incomplete resection. The purpose of this report is to call attention to ELSTs, which are difficult to diagnose due to their rarity and variety of presentations.
