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Histopathology
Article . 2020 . Peer-reviewed
License: CC BY NC ND
Data sources: Crossref
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Histopathology
Article
License: CC BY NC ND
Data sources: UnpayWall
Histopathology
Article . 2021
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Papillary‐cystic neoplasms of the middle ear are distinct from endolymphatic sac tumours

Authors: Sarina K. Mueller; Marco Santucci; Robert Stoehr; Beatrice Giannoni; Rudi Pecci; Abbas Agaimy; Caterina Fattorini; +4 Authors

Papillary‐cystic neoplasms of the middle ear are distinct from endolymphatic sac tumours

Abstract

AimsPapillary neoplasms of the middle and inner ear are rare and poorly characterised. The current World Health Organization classification divides them into two major subtypes: aggressive papillary tumours (APTs) and endolymphatic sac tumours (ELSTs). The aim of this article is to present two papillary neoplasms of the middle ear that do not fit into either the classic APT category or the classic ELST category, and compare them with three ELSTs.Methods and resultsThe patients were a 48‐year‐old female and a 59‐year‐old male without a history of other neoplasms. Histology showed papillary‐cystic growth of predominantly oncocytic (Case 1) or mucinous (Case 2) cells surrounded by a p63‐positive basal layer. The overall histology was reminiscent of oncocytic sinonasal papilloma (Case 1) and pancreatobiliary or salivary intraductal papillary mucinous neoplasms (Case 2). Ovarian‐type stroma, invasion and malignant features were absent. Immunohistochemistry revealed expression of cytokeratin (CK) 7, but not carbonic anhydrase IX (CAIX) or paired box gene 8 (PAX8) (except for very focal PAX8 expression in Case 1). The TST15 gene panel and HRAS sequencing revealed no pathogenic mutations in BRAF, KRAS, EGFR, AKT1, or HRAS. The TruSight RNA fusion panel revealed an MKRN1–BRAF fusion in Case 1. No fusion was detected in Case 2. The three ELSTs showed classic features of the entity, expressed CK7, epithelial membrane antigen, PAX8, and CAIX, and lacked a basal cell layer.ConclusionThese novel cases suggest that papillary tumours of the ear represent a heterogeneous spectrum of distinct neoplasms unified by a prominent papillary‐cystic pattern rather than a single entity. Future studies should clarify whether the MKRN1–BRAF fusion is a defining recurrent driver event, especially in those cases reported as sinonasal‐type middle ear papillomas.

Country
Italy
Keywords

Aggressive papillary tumour, BRAF, ear, endolymphatic sac tumour, IPMN, oncocytic, papillary mucinous neoplasms, Schneiderian papilloma., Male, Proto-Oncogene Proteins B-raf, Pancreatic Intraductal Neoplasms, aggressive papillary tumour; BRAF; ear; endolymphatic sac tumour; IPMN; oncocytic; papillary mucinous neoplasms; Schneiderian papilloma, Ear, Middle, Middle Aged, Immunohistochemistry, Diagnosis, Differential, Adenocarcinoma, Papillary, Biomarkers, Tumor, Humans, Female, Endolymphatic Sac, Ear Neoplasms

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citations
This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Citations provided by BIP!
popularity
This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.
BIP!Popularity provided by BIP!
influence
This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).
BIP!Influence provided by BIP!
impulse
This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.
BIP!Impulse provided by BIP!
10
Top 10%
Average
Top 10%
Green
hybrid