Tahir · Cureus 2021 · case report · n=1

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

Cited 4 times in the scientific literature.

Level 4 - case-series / case-control

Single case report

PubMed 34401213 · doi:10.7759/cureus.16413 · record verified 2026-08-26

What was done

This is a case report of a 74-year-old woman with a medical history of Schneiderian papilloma diagnosed with papillary mucinous adenocarcinoma of the endolymphatic sac (ELS) without von Hippel-Lindau (VHL) disease association.

What was found

The abstract details the clinical context of ELS tumors (noting that 11%-30% develop without VHL mutations) and the presentation of this individual case, but reports no specific numerical clinical measurements, follow-up timelines, or outcome metrics.

Why it matters

It documents a rare case of non-VHL-associated aggressive papillary mucinous adenocarcinoma of the ELS, adding to the sparse literature on differentiating and managing these middle ear neoplasms.

Limits

As a single-patient case report (n = 1), findings cannot establish causality, treatment efficacy, or generalized prognosis. The abstract lacks specific data on treatment interventions, pathology quantification, and clinical follow-up.

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