Bilateral lipomatous hamartoma of the tongue: A case report in a child with oral‐facial‐digital syndrome type VI

A hamartoma is a benign proliferation of typical mature cells specific to a particular anatomical site. In the oral cavity, they may occur as isolated cases or be associated with genetic syndromes. Oral‐facial‐digital syndrome type VI is a rare genetic disorder with an estimated incidence of one in...

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Veröffentlicht in:Special care in dentistry 2024-07, Vol.44 (4), p.1036-1040
Hauptverfasser: Queiroz, Aline, Pina, Paulo Sérgio Souza, Novaes, Myrian Stella Paiva, Dutra, Beatriz, de Sousa, Suzana Cantanhede Orsini Machado, Azevedo, Luciane Hiramatsu
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container_issue 4
container_start_page 1036
container_title Special care in dentistry
container_volume 44
creator Queiroz, Aline
Pina, Paulo Sérgio Souza
Novaes, Myrian Stella Paiva
Dutra, Beatriz
de Sousa, Suzana Cantanhede Orsini Machado
Azevedo, Luciane Hiramatsu
description A hamartoma is a benign proliferation of typical mature cells specific to a particular anatomical site. In the oral cavity, they may occur as isolated cases or be associated with genetic syndromes. Oral‐facial‐digital syndrome type VI is a rare genetic disorder with an estimated incidence of one in 50,000–250,000 newborns. Here, we report a case of a 2‐year‐old boy diagnosed with oral‐facial‐digital syndrome type VI who was referred for evaluation of a bilateral and normochromic to slightly pinkish nodule on the lateral surface of the tongue. Clinically, the child presented hypotonia, low visual acuity, absence of oculocephalic reflex, delay in neuropsychomotor development, and polydactyly in the feet. Excisional biopsies of both sides of the tongue were performed using a 1.5 W high‐power diode laser (wavelength of 980 nm), and histopathological analysis revealed abundant mature adipocytes predominantly arranged in lobules that mainly surrounded the minor salivary gland parenchyma. The surgical sites healed with no complications and the patient remains under follow‐up for 10 months. Due to the limited literature on this syndrome and the frequent presence of tongue hamartomas in children, dentists need to be familiar with them.
doi_str_mv 10.1111/scd.12958
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source MEDLINE; Access via Wiley Online Library
subjects Acuity
Adipocytes
Biopsy
Case reports
Cell proliferation
Child, Preschool
Genetic disorders
Hamartoma - pathology
Humans
Joubert syndrome
lipomatous
Male
Medical diagnosis
Neonates
Neoplasia
Oral cavity
oral‐facial‐digital syndrome
Orofaciodigital Syndromes - diagnosis
Orofaciodigital Syndromes - pathology
Parenchyma
Polydactyly
Salivary gland
Tongue
Tongue Diseases - diagnosis
Tongue Diseases - pathology
tongue hamartoma
title Bilateral lipomatous hamartoma of the tongue: A case report in a child with oral‐facial‐digital syndrome type VI
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