Objective And Importance: We present three cases of endolymphatic sac tumors and review the previously published cases. Despite frequent extension to the cerebellopontine angle, these rare tumors have only recently been recognized by neurosurgeons.
Clinical Presentation: A 26-year-old man developed a progressive hearing loss, revealing an intrapetrous retrolabyrinthine tumor on the right side. A 28-year-old woman experienced a left cerebellopontine angle syndrome, with a lytic intrapetrous mass extending into the cerebellopontine angle. A 38-year-old woman presented with an intracranial hypertension syndrome caused by a tumor of the jugular foramen.
Intervention: For the first and second patients, the tumors originated from the operculum of the endolymphatic sac. Total removal was achieved, via a transpetrosal approach, in these two cases. No recurrence was detected after a 20-month follow-up period. For the third patient, the tumor originated from the distal part of the sac. Recurrence was observed 8 years after subtotal removal via a retrosigmoid route. Histological analysis revealed a papillary-cystic adenocarcinomatous pattern in all cases, without features of aggressiveness.
Conclusion: Endolymphatic sac tumors are locally invasive neoplasms characterized by bipolar intrapetrous and posterior fossa involvement. The anatomic complexity of the endolymphatic sac may explain the distinct patterns of extension of these tumors. Early radical surgery is related to good outcomes.
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http://dx.doi.org/10.1097/00006123-199804000-00139 | DOI Listing |
Clin Nucl Med
January 2025
From the Department of Nuclear Medicine and PET-CT, AIG Hospitals, Hyderabad, India.
Endolymphatic sac tumors (ELSTs) are rare, slow-growing, and locally aggressive neoplasms that originate from the epithelial lining of the endolymphatic duct and sac. These are characterized by their infiltrative growth pattern and the potential for local destruction of surrounding structures, including the inner ear and temporal bone. We report a case of an incidentally diagnosed sporadic ELST.
View Article and Find Full Text PDFLaryngoscope
January 2025
Department of Otology and Skull Base Surgery, Eye, Ear, Nose, and Throat Hospital, Fudan University, Shanghai, China.
Objective: Endolymphatic sac tumors (ELSTs), as rare low-grade neoplasms, are primarily treated with surgery. This study analyzes the characteristics of tumor-infiltrating leukocytes (TILs) in ELSTs and their relationships with clinical features to explore the potential for immunotherapy in ELSTs.
Methods: Clinical data and tumor specimens of 10 ELSTs patients who underwent surgery were retrieved.
Eur Arch Otorhinolaryngol
December 2024
ENT Department, Sydney Adventist Hospital, Sydney, NSW, Australia.
Background: Meniere's disease (MD) is a disabling disease of the inner ear, having a substantial effect on a patient's quality of life. While various postulations regarding its aetiology exists, due to the difficulty with accessing inner ear tissue, there have been limited histological studies in patients with active MD.
Methods: Tissue was collected during labyrinthectomy from 8 patients with intractable MD who had failed medical therapy (22 samples), and 9 patients undergoing translabyrinthine resection of vestibular schwannoma (19 samples).
Cureus
November 2024
Otolaryngology - Head and Neck Surgery, Sri Devaraj Urs Academy of Higher Education and Research, Kolar, IND.
Objectives: Surgical treatments for Ménière's disease differ in efficacy. Endolymphatic duct blockage (EDB) is favored for its minimal risk and ability to preserve hearing. One of the main challenges in the technique is the difficulty in accurately identifying the endolymphatic duct (ED).
View Article and Find Full Text PDFJ Kidney Cancer VHL
December 2024
Department of Urology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA.
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