Risk of thyroid neoplasms in patients with 22q11.2 deletion and DiGeorge-like syndromes: an insight for follow-up.

22q11 deletion syndrome DiGeorge genetic syndrome thyroid cancer thyroid nodules

Journal

Frontiers in endocrinology
ISSN: 1664-2392
Titre abrégé: Front Endocrinol (Lausanne)
Pays: Switzerland
ID NLM: 101555782

Informations de publication

Date de publication:
2023
Historique:
received: 20 04 2023
accepted: 14 07 2023
medline: 29 8 2023
pubmed: 28 8 2023
entrez: 28 8 2023
Statut: epublish

Résumé

The chromosome 22q11.2 deletion syndrome comprises phenotypically similar diseases characterized by abnormal development of the third and fourth branchial arches, resulting in variable combinations of congenital heart defects, dysmorphisms, hypocalcemia, palatal dysfunction, developmental or neuropsychiatric disorders, and impairment of the immune system due to thymic dysfunction. Other genetic syndromes, often called DiGeorge-like, share clinical and immunological features with 22q11.2 deletion syndrome. This syndrome has been rarely associated with malignancies, mainly hematological but also hepatic, renal, and cerebral. Rarely, malignancies in the head and neck region have been described, although no aggregate of data on the development of thyroid neoplasms in patients with this clinical phenotype has been conducted so far. To characterize this possible association, a multicenter survey was made. Thus, we present a case series of five pediatric patients with 22q11.2 deletion syndrome or DiGeorge-like syndrome who were occasionally found with confirmed or highly suspected neoplasms of the thyroid gland during their follow-up. In three cases, malignancies were histologically confirmed, but their outcome was good due to an early recognition of suspicious nodules and precocious surgery. This study underlines for clinicians the higher risk of neoplasms in the head and neck district for patients affected by these syndromes. It also emphasizes the importance of a prolonged clinical and ultrasound follow-up for patients with this clinical and immunological phenotype.

Identifiants

pubmed: 37635986
doi: 10.3389/fendo.2023.1209577
pmc: PMC10450035
doi:

Types de publication

Case Reports Multicenter Study Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

1209577

Informations de copyright

Copyright © 2023 Sarli, Ricci, Lodi, Cavone, Pacillo, Giancotta, Ubertini, Baroncelli, Cancrini, Azzari and Stagi.

Déclaration de conflit d'intérêts

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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Auteurs

Walter Maria Sarli (WM)

Department of Health Sciences, University of Florence, Florence, Italy.
Paediatric Immunology Division, Meyer Children's Hospital IRCCS, Florence, Italy.

Silvia Ricci (S)

Department of Health Sciences, University of Florence, Florence, Italy.
Paediatric Immunology Division, Meyer Children's Hospital IRCCS, Florence, Italy.

Lorenzo Lodi (L)

Department of Health Sciences, University of Florence, Florence, Italy.
Paediatric Immunology Division, Meyer Children's Hospital IRCCS, Florence, Italy.

Federica Cavone (F)

Pediatrics Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.

Lucia Pacillo (L)

Unit of Clinical Immunology and Vaccinology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.
Research Unit of Primary Immunodeficiency, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.
Department of Systems Medicine, University of Rome "Tor Vergata", Rome, Italy.

Carmela Giancotta (C)

Unit of Clinical Immunology and Vaccinology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.

Graziamaria Ubertini (G)

Unit of Endocrinology and Diabetology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.

Giampiero Baroncelli (G)

Pediatrics Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.

Caterina Cancrini (C)

Unit of Clinical Immunology and Vaccinology, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.
Research Unit of Primary Immunodeficiency, Bambino Gesù Children's Hospital IRCCS, Rome, Italy.
Department of Systems Medicine, University of Rome "Tor Vergata", Rome, Italy.

Chiara Azzari (C)

Department of Health Sciences, University of Florence, Florence, Italy.
Paediatric Immunology Division, Meyer Children's Hospital IRCCS, Florence, Italy.

Stefano Stagi (S)

Department of Health Sciences, University of Florence, Florence, Italy.
Auxoendocrinology Division, Meyer Children's Hospital IRCCS, Florence, Italy.

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