Aggressive high-grade papillary-patterned renal cell carcinomas harboring FLCN mutations: expanding the clinicopathological spectrum in Birt-Hogg-Dubé syndrome and sporadic cases.

doi:10.1007/s00428-026-04693-x

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Article

Aggressive high-grade papillary-patterned renal cell carcinomas harboring FLCN mutations: expanding the clinicopathological spectrum in Birt-Hogg-Dubé syndrome and sporadic cases.

Virchows Archiv : an international journal of pathology - 1 Sept 2026

Wang Xiao-Tong, Fang Ru, Ye Sheng-Bing, Wang Xuan, Li Rui, He Yan, Zhang Ru-Song, Xia Qiu-Yuan, Zhao Ming, Rao Qiu

Abstract excerpt

Folliculin (FLCN)-mutated renal tumors, in the setting of Birt-Hogg-Dubé (BHD) syndrome or sporadically, typically show low-grade eosinophilic morphology and follow an indolent clinical course; however, rare high-grade variants with more aggressive behavior remain poorly defined and underrecognized. To better characterize this unusual subset, we identified six high-grade renal cell carcinomas (RCCs) and performed...

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Topics

Humans
Birt-Hogg-Dube Syndrome
Carcinoma, Renal Cell
Kidney Neoplasms
Proto-Oncogene Proteins

Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

Tumor Suppressor Proteins

Male

Female

Middle Aged

Aged

Mutation

Biomarkers, Tumor

Neoplasm Grading

Phenotype

Adult

DNA Mutational Analysis

Source

Open in PubMed

Identifiers

PMID: 42687010
DOI: 10.1007/s00428-026-04693-x

Genes

FLCN
FH
GPNMB
TFEB

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 22 Oct 2026.

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