Evidence map›Paper›PMID 39815310›Full record

ArticleDiagnostic pathology2025

TFE3-rearranged perivascular epithelioid cell tumors of the head and neck with rare fusion partners: clues to the differential diagnosis between benign and malignant tumors.

Yuka Takahashi, Akihiko Yoshida, Seiichi Yoshimoto, Shigenobu Suzuki, Satsuki Kishikawa, Ayaka Mitsui, Eijitsu Ryo, Yuki Kojima, Kan Yonemori, Yasushi Yatabe and 1 more

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In one paragraph

Article in Diagnostic pathology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Pigmented perivascular epithelioid cell tumors of the orbit withAmerican journal of ophthalmology case reports · 2025
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4 · The record

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PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

11 authors.

Yuka TakahashiDepartment of Diagnostic Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Akihiko YoshidaDepartment of Diagnostic Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Seiichi YoshimotoDepartment of Head and Neck Surgery, National Cancer Center Hospital, Tokyo, Japan.
Shigenobu SuzukiDepartment of Ophthalmic Oncology, National Cancer Center Hospital, Tokyo, Japan.
Satsuki KishikawaDepartment of Diagnostic Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Ayaka MitsuiDepartment of Diagnostic Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Eijitsu RyoDivision of Molecular Pathology, National Cancer Center Research Institute, Tokyo, Japan.
Yuki KojimaDepartment of Medical Oncology, National Cancer Center Hospital, Tokyo, Japan.
Kan YonemoriDepartment of Medical Oncology, National Cancer Center Hospital, Tokyo, Japan.
Yasushi YatabeDepartment of Diagnostic Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Taisuke MoriDepartment of Diagnostic Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan. tamori@ncc.go.jp.

Funding

Japan Society for the Promotion of Science 23K6476
6 · The paper itself

Abstract

backgroundPerivascular epithelioid cell tumors (PEComas) rarely appear in the head and neck region. This case report describes two transcription factor E3 (TFE3)-rearranged PEComa cases, consisting of one in the orbit and one in the nasal cavity. CASE PRESENTATION: Both cases demonstrated sheet-like or focal nested architecture and comprised epithelioid cells with abundant clear to eosinophilic cytoplasm and vascular stroma. The first case exhibited partial pleomorphism, a small necrosis area, and slightly increased mitosis and was classified as malignant. The second case demonstrated mild atypia and no mitosis or necrosis and was categorized as benign. The nasal tumor was initially considered a TFE3-rearranged renal cell carcinoma metastasis. However, a subsequent renal tumor biopsy revealed angiomyolipoma. The RNA sequence revealed ZC3H4::TFE3 and PRCC::TFE3 fusions in the first and second cases, respectively.

conclusionThe fusion partner gene ZC3H4 is uncommon, and this is the third reported PEComa case. The fusion partner gene PRCC is often reported in TFE3-rearranged renal cell carcinoma, and this PEComa case is the second reported in the head and neck region. The initially reported cases with the fusion partner genes ZC3H4 and PRCC were categorized as malignant. These cases were discussed with a literature review.

Indexed as

Basic Helix-Loop-Helix Leucine Zipper Transcription FactorsGene RearrangementPerivascular Epithelioid Cell NeoplasmsAdultBiomarkers, TumorCell Cycle ProteinsDiagnosis, DifferentialFemaleGene FusionHead and Neck NeoplasmsHumansMaleMiddle AgedNeoplasm ProteinsBasic Helix-Loop-Helix Leucine Zipper Transcription FactorsBiomarkers, TumorCell Cycle ProteinsNeoplasm ProteinsPRCC protein, humanTFE3 protein, humanHead and neckPerivascular epithelioid cell tumorPRCCTranscription factor E3ZC3H4

Identifiers

PMID39815310
PMCPMC11734225

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