Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies

Zanoletti, Elisabetta; Girasoli, Laura; Borsetto, Daniele; Opocher, Giuseppe; Mazzoni, Antonio

doi:10.14639/0392-100x-1402

Cited by 24 publications

(19 citation statements)

References 31 publications

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“…Emanating from the posterior aspect of the petrous ridge, ELSTs disrupt the petrous bone and can expand into the middle ear, otic capsule, middle cranial fossa, and posterior cranial fossa. 4 Our review is consistent, with many tumors invading the posterior cranial fossa, labyrinth, jugular bulb, middle ear, or limited only to the endolymphatic sac. Advanced tumors can compress the jugular bulb, carotid artery, and cerebellopontine angle.…”

Section: Tumor Characteristics and Behaviorsupporting

confidence: 81%

“…The translabyrinthine approach is indicated in patients without useful hearing secondary to advanced tumors extending through the labyrinth and/or into the posterior fossa dura. 4,18 For extensive intracranial disease, combined approaches are employed based on the structures involved with tumor. For VHL patients with bilateral disease and a preserved cochlea, early cochlear implantation has been proposed as an alternative to help mitigate the sequelae of hearing loss.…”

Section: Treatment and Outcomesmentioning

confidence: 99%

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Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes

Tang

Grady

Nickel

et al. 2022

Otolaryngol.--head neck surg.

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Objective Endolymphatic sac tumors are rare neoplasms originating in the endolymphatic sac. Current literature is limited to case reports and small case series. The objective of this study was to systematically review the literature to better describe clinical presentation, treatment options, and outcomes in endolymphatic sac tumors. Data Source PubMed, Embase, and Cochrane Library. Review Methods A systematic review was performed following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines describing human endolymphatic sac tumors. Risk of bias was assessed using a validated critical appraisal checklist for case series. Studies without inclusion of individual patient characteristics, corresponding treatments, and outcomes were excluded. Heterogeneity of data precluded meta-analysis. Results A total of 82 studies met inclusion criteria, and 253 discrete tumors were analyzed. A total of 23.4% of patients had von Hippel-Lindau disease. Von Hippel-Lindau–associated tumors affected females to males in a 2.4:1 ratio. Patients with von Hippel-Lindau disease displayed earlier average age at diagnosis compared to the sporadic cohort. Surgery was the primary treatment modality and was performed in 88.9% of cases. Adjuvant radiation therapy was employed in 18.7% of cases; 16.2% cases recurred, and 10.6% had progression of residual disease after treatment. Mean time to recurrence or progression was 53.1 ± 52.4 months with a range of 3 to 240 months. Conclusion Endolymphatic sac tumors require a high degree of suspicion for early diagnosis. Complete resection is the standard of care. No strong evidence supports routine use of adjuvant radiation therapy. Given the high rate of recurrence and wide-ranging time to recurrence, long-term follow-up is necessary.

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Section: Tumor Characteristics and Behaviorsupporting

confidence: 81%

Section: Treatment and Outcomesmentioning

confidence: 99%

Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes

Tang

Grady

Nickel

et al. 2022

Otolaryngol.--head neck surg.

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“…The estimated prevalence of ELST in VHL disease is between 11% and 25%. 4,5 Though distant metastasis has not been reported, "drop metastasis" to the spinal column has been reported. 6,7 Radiologically, a preoperative diagnosis of an ELST is challenging.…”

Section: Discussion and Evaluationmentioning

confidence: 99%

“…A histopathology exam revealed papillary and cystic structures, lined by a single layer of cuboidal cells (►Fig. [5][6] with areas of hemorrhage, fibrosis, and cholesterol clefts. Hemosiderin-laden macrophages and cystic glandular spaces were observed filled with a colloid-like material, reminiscent of thyroid tissue (►Fig.…”

Section: Postoperative Coursementioning

confidence: 99%

Endolymphatic Sac Tumour: A Case Report and Review of the Literature

Patankar

Aiyer

Chamarajan

et al. 2021

Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery

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Introduction Endolymphatic sac tumor (ELST) is a slow-growing, low-grade, locally-infiltrative tumor arising from the endolymphatic sac/duct, which is located in the posterior part of the petrous temporal bone. It may be sporadic in origin, or may be associated with Von-Hippel Lindau (VHL) syndrome. Case description A 40-year-old female patient with an ELST without VHL syndrome who was treated successfully by microsurgical extirpation of the tumor. Discussion We discuss the radiological features and the histopathology of this rare tumor and review the relevant literature. Conclusion The case herein reported adds to the previously-reported cases of this rare tumor.

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“…The different parts of our body are derived from three different germ layers. Similarly, the membranous labyrinth and endolymphatic sac (ELS) are derived from the ectoderm and are found postero-medial to the temporal bone [ 1 , 2 ]. The endolymphatic duct connects with the proximal part of the ELS and the posterior segment of the ELS contiguous with the posterior cranial fossa [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

Papillary Mucinous Adenocarcinoma of the Endolymphatic Sac: A Rare Middle Ear Neoplasm

Tahir

Frick

Tranesh

2021

Cureus

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Glandular neoplasms of the temporal-mastoid region and endolymphatic sac (ELS) are rare, and it is quite challenging to differentiate between an adenoma and an adenocarcinoma. ELS tumors (ELST) usually present with papillary, follicular, or solid patterns and can be further distinguished histologically and through immunohistochemistry. The microscopic features and clinical course of this neoplasm have been comprehensively explained by Heffner, who considered it "low-grade adenocarcinoma of likely ELS origin." The papillary form more commonly affects females, and it is a more aggressive form of ELST that is destructive and exhibits extensive local spread. The tumor usually has a close association with von Hippel-Lindau (VHL) disease, but 11%-30% of the ELST cases develop in individuals without a VHL mutation. ELSTs manifest with headaches, hearing loss, ear discharge, and cranial nerve palsies. Currently, the only available curative therapeutic intervention consists of wide local excision and long-term follow-up. Because of the sensitive location of this tumor, the adjuvant radiotherapy options are still questionable. In this case report, the author presents a 74-year-old woman with a past medical history of Schneiderian papilloma and was diagnosed with papillary mucinous adenocarcinoma of the ELS not associated with VHL disease.Categories: Pathology, Oncology Keywords: endolymphatic sac tumor, papillary mucinous adenocarcinoma, glandular neoplasm, middle ear tumor, neoplasm of temporal region and mastoids air cells ELS tumors (ELSTs) are extremely uncommon, with only around 300 cases published in the literature [1]. The purpose of this case report is to discuss the histopathological presentation, diagnosis, and treatment of this rare entity. Case PresentationA 74-year-old female patient came in for a follow-up with an extensive past medical and surgical history. During the visit, her vital signs were as follows: blood pressure, 133/83 mmHg; heart rate, 88 beats/min; temperature, 97.5°F; respiratory rate, 16/min; and body mass index, 27.16. Her medications were the following: vitamin D, potassium, magnesium, aspirin 81 mg/day (calcium supplement), glucosamine capsule, and prednisone 20 mg as needed.

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Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies

Cited by 24 publications

References 31 publications

Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes

Systematic Review of Endolymphatic Sac Tumor Treatment and Outcomes

Endolymphatic Sac Tumour: A Case Report and Review of the Literature

Papillary Mucinous Adenocarcinoma of the Endolymphatic Sac: A Rare Middle Ear Neoplasm

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