Whole genome sequencing and single-cell transcriptomics identify KMT2D inactivation as a potential new driver for pituitary tumors: a case report

Abstract The pituitary gland is a main component of the endocrine system and a master controller of hormone production and secretion. Unlike neoplastic formation in other organs, Pituitary Neuroendocrine Tumors (PitNETs) are frequent in the population (16%) and, for unknown reasons, almost never met...

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Main Authors: Maxime Brunner, Jenny Meylan-Merlini, Maude Muriset, Sergey Oreshkov, Andrea Messina, Mahmoud Messerer, Roy Daniel, Ekkehard Hewer, Jean Phillipe Brouland, Federico Santoni
Format: Article
Language:English
Published: Nature Portfolio 2025-06-01
Series:BJC Reports
Online Access:https://doi.org/10.1038/s44276-025-00155-0
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author Maxime Brunner
Jenny Meylan-Merlini
Maude Muriset
Sergey Oreshkov
Andrea Messina
Mahmoud Messerer
Roy Daniel
Ekkehard Hewer
Jean Phillipe Brouland
Federico Santoni
author_facet Maxime Brunner
Jenny Meylan-Merlini
Maude Muriset
Sergey Oreshkov
Andrea Messina
Mahmoud Messerer
Roy Daniel
Ekkehard Hewer
Jean Phillipe Brouland
Federico Santoni
author_sort Maxime Brunner
collection DOAJ
description Abstract The pituitary gland is a main component of the endocrine system and a master controller of hormone production and secretion. Unlike neoplastic formation in other organs, Pituitary Neuroendocrine Tumors (PitNETs) are frequent in the population (16%) and, for unknown reasons, almost never metastatic. So far, few genes have been identified as drivers for PitNETs, such as GNAS in somatotroph tumors and USP8 in corticotroph tumors. Using whole genome sequencing, we uncover a potential novel driver, the histone methyltransferase KMT2D, in a patient in his 50s suffering from a mixed somato-lactotroph tumor. Coverage ratio between germline and tumor revealed extensive chromosomal alterations. Single-cell RNA sequencing of the tumor shows up-regulation of known tumorigenic pathways compared to a healthy reference, as well as a different immune infiltration profile compared to other PitNETs, more closely resembling the profile of carcinomas than adenomas. Genome-wide DNA methylation analysis identified 796 differentially methylated regions, including notable hypomethylation in the promoter of SPON2, an immune-related gene. Our results show that tumors considered quiet and non-aggressive can share drivers, features, and epigenetic alterations with metastatic forms of cancer, raising questions about the biological mechanisms controlling their homeostasis.
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spelling doaj-art-595679aa99f746aeb19bac02abf8dd242025-08-20T03:47:24ZengNature PortfolioBJC Reports2731-93772025-06-01311910.1038/s44276-025-00155-0Whole genome sequencing and single-cell transcriptomics identify KMT2D inactivation as a potential new driver for pituitary tumors: a case reportMaxime Brunner0Jenny Meylan-Merlini1Maude Muriset2Sergey Oreshkov3Andrea Messina4Mahmoud Messerer5Roy Daniel6Ekkehard Hewer7Jean Phillipe Brouland8Federico Santoni9Department of Endocrinology, Diabetology and Metabolism, University Hospital of LausanneDepartment of Endocrinology, Diabetology and Metabolism, University Hospital of LausanneDepartment of Endocrinology, Diabetology and Metabolism, University Hospital of LausanneDepartment of Endocrinology, Diabetology and Metabolism, University Hospital of LausanneDepartment of Endocrinology, Diabetology and Metabolism, University Hospital of LausanneFaculty of Biology and Medicine, University of LausanneFaculty of Biology and Medicine, University of LausanneFaculty of Biology and Medicine, University of LausanneFaculty of Biology and Medicine, University of LausanneDepartment of Endocrinology, Diabetology and Metabolism, University Hospital of LausanneAbstract The pituitary gland is a main component of the endocrine system and a master controller of hormone production and secretion. Unlike neoplastic formation in other organs, Pituitary Neuroendocrine Tumors (PitNETs) are frequent in the population (16%) and, for unknown reasons, almost never metastatic. So far, few genes have been identified as drivers for PitNETs, such as GNAS in somatotroph tumors and USP8 in corticotroph tumors. Using whole genome sequencing, we uncover a potential novel driver, the histone methyltransferase KMT2D, in a patient in his 50s suffering from a mixed somato-lactotroph tumor. Coverage ratio between germline and tumor revealed extensive chromosomal alterations. Single-cell RNA sequencing of the tumor shows up-regulation of known tumorigenic pathways compared to a healthy reference, as well as a different immune infiltration profile compared to other PitNETs, more closely resembling the profile of carcinomas than adenomas. Genome-wide DNA methylation analysis identified 796 differentially methylated regions, including notable hypomethylation in the promoter of SPON2, an immune-related gene. Our results show that tumors considered quiet and non-aggressive can share drivers, features, and epigenetic alterations with metastatic forms of cancer, raising questions about the biological mechanisms controlling their homeostasis.https://doi.org/10.1038/s44276-025-00155-0
spellingShingle Maxime Brunner
Jenny Meylan-Merlini
Maude Muriset
Sergey Oreshkov
Andrea Messina
Mahmoud Messerer
Roy Daniel
Ekkehard Hewer
Jean Phillipe Brouland
Federico Santoni
Whole genome sequencing and single-cell transcriptomics identify KMT2D inactivation as a potential new driver for pituitary tumors: a case report
BJC Reports
title Whole genome sequencing and single-cell transcriptomics identify KMT2D inactivation as a potential new driver for pituitary tumors: a case report
title_full Whole genome sequencing and single-cell transcriptomics identify KMT2D inactivation as a potential new driver for pituitary tumors: a case report
title_fullStr Whole genome sequencing and single-cell transcriptomics identify KMT2D inactivation as a potential new driver for pituitary tumors: a case report
title_full_unstemmed Whole genome sequencing and single-cell transcriptomics identify KMT2D inactivation as a potential new driver for pituitary tumors: a case report
title_short Whole genome sequencing and single-cell transcriptomics identify KMT2D inactivation as a potential new driver for pituitary tumors: a case report
title_sort whole genome sequencing and single cell transcriptomics identify kmt2d inactivation as a potential new driver for pituitary tumors a case report
url https://doi.org/10.1038/s44276-025-00155-0
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