Vulvar angiomyofibroblastoma is molecularly defined by recurrent MTG1-CYP2E1 fusions.


Journal

Histopathology
ISSN: 1365-2559
Titre abrégé: Histopathology
Pays: England
ID NLM: 7704136

Informations de publication

Date de publication:
Dec 2022
Historique:
revised: 20 09 2022
received: 03 08 2022
accepted: 22 09 2022
pubmed: 1 10 2022
medline: 11 11 2022
entrez: 30 9 2022
Statut: ppublish

Résumé

Angiomyofibroblastoma (AMF), a rare benign vulvovaginal mesenchymal tumour, poses a diagnostic challenge due to histologic and immunohistochemical overlap with other vulvar mesenchymal tumours. Recently, MTG1-CYP2E1 fusion transcripts were reported in 5/5 AMFs; no other genetic alterations have been described to date. Herein, we sought to investigate the frequency of the MTG1-CYP2E1 fusion and the presence of other potential genetic alterations in a cohort of AMFs (n = 7, patient age range: 28-49 years). Tumours demonstrated classic morphologic features including alternating hypo/hypercellular areas, capillary channels surrounded by epithelioid/spindled tumour cells, and variable amounts of mature adipose tissue. reverse transcription-polymerase chain reaction (RT-PCR) for MTG1-CYP2E1 fusion, performed in all seven cases, showed the fusion transcript in five of six cases (one case with technical failure). Two tumours, including the one lacking the fusion, were subjected to targeted next-generation sequencing (104 genes) and a sarcoma fusion assay (28 genes); the fusion negative AMF also underwent RNA sequencing. No additional mutations, copy number alterations, or fusion genes were identified with the assays employed. We conclude that the majority of AMFs harbour recurrent MTG1-CYP2E1 fusion transcripts and identification of this fusion may aid in the diagnosis.

Identifiants

pubmed: 36177509
doi: 10.1111/his.14813
pmc: PMC10335785
mid: NIHMS1910592
doi:

Substances chimiques

Cytochrome P-450 CYP2E1 EC 1.14.13.-
MTG1 protein, human EC 3.6.1.-

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

841-846

Subventions

Organisme : NCI NIH HHS
ID : P30 CA008748
Pays : United States
Organisme : NCI NIH HHS
ID : P50 CA247749
Pays : United States

Informations de copyright

© 2022 John Wiley & Sons Ltd.

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Auteurs

Baris Boyraz (B)

James Homer Wright Pathology Laboratories, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

Ryosuke Tajiri (R)

Department of Pathology and Oncology, University of Occupational and Environmental Health, Kitakyushu, Japan.

Rofieda R Alwaqfi (RR)

Department of Pathology, University of Iowa, Iowa City, LA, USA.

Arnaud Da Cruz Paula (ADC)

Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

Qiqi Ye (Q)

Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

G Petur Nielsen (GP)

James Homer Wright Pathology Laboratories, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

Yin P Hung (YP)

James Homer Wright Pathology Laboratories, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

Esther Oliva (E)

James Homer Wright Pathology Laboratories, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

Britta Weigelt (B)

Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

Masanori Hisaoka (M)

Department of Pathology and Oncology, University of Occupational and Environmental Health, Kitakyushu, Japan.

Jaclyn C Watkins (JC)

James Homer Wright Pathology Laboratories, Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

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Classifications MeSH