Dental and orthodontic follow-up in nevoid basal cell carcinoma syndrome patient with odontogenic keratocystic tumors

Feghali, Sara; Vi‐Fane, Brigitte; Picard, A.; Kadlub, Natacha

doi:10.1016/j.jormas.2021.07.009

Cited by 3 publications

(4 citation statements)

References 13 publications

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“…Syndromic mBCCs were also significantly associated with jaw cysts ( P < .0001; NNS = 2 [95% CI, 1.1 to 1.4]), which may cause other oral abnormalities such as tooth impaction or agenesis, or dentofacial defomities. 22 , 23 Based on the computed NSS, PaR screening of 10 in every 13-20 mBCCs patients would identify one GGS patient.…”

Section: Discussionmentioning

confidence: 99%

Dental Screening Including Panoramic Radiograph for Gorlin-Goltz Syndrome in Patients With Multiple Basal Cell Carcinomas

et al. 2022

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Purpose To answer the following clinical research question: “Among patients with multiple basal cell carcinomas (mBCCs), can panoramic radiograph (PaR) facilitate the diagnosis of Gorlin-Goltz syndrome (GGS)?” Methods This retrospective study enrolled mBCCs subjects who presented to a German tertiary care center between 1 January 2015 and 31 December 2021. The primary predictor was presence of syndromic mBCCs, and the main outcomes were jaw cysts and odontogenic keratocysts (OKCs). Descriptive, bi- and multivariate statistics, diagnostic test evaluation, and number needed to screen (NNS) were computed at α = 95%. Results The sample comprised 527 mBCCs patients (36.1% females; 6.8% GGS; 5.5% OKCs; mean age, 74.5 ± 15.8 years [range, 15-102]). There was a significant association between syndromic mBCCs and jaw cysts ( P < .0001; NNS = 2 [95% CI, CI, 1.1 to 1.4]). In the adjusted logistic model, PaR identified GGS via radiographic diagnosis of jaw cysts in case of 1) age ≤ 35 years, 2) ≥ 5 BCCs, and 3) ≥ 1 high-risk BCCs. Nearly every jaw cyst identified by PaR was OKCs ( P = .01; 95% CI, 3.1 to 3,101.4; NNS = 1.3 [95% CI, .9 to 2]). The post hoc power was 100%. Conclusions Dental screening with the use of PaR for mBCCs patients, especially those aged ≤35 years, or with ≥5 BCCs, or ≥1 high-risk BCCs, may be helpful in detection and identification of GGS through recognition of OKCs.

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Section: Discussionmentioning

confidence: 99%

Dental Screening Including Panoramic Radiograph for Gorlin-Goltz Syndrome in Patients With Multiple Basal Cell Carcinomas

et al. 2022

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“…Such NBCCS-related OKCs usually do not abate until 30 years of age ( Mustaciuolo et al, 1989 ). They can range from 1 to 30 lesions with an average number of five ( Lo Muzio, 2008 , Feghali et al, 2022 ). Prior to initiating the processes for NBCCS diagnosis, it is important that the cysts are histopathologically diagnosed as OKC.…”

Section: Multiple Odontogenic Keratocystsmentioning

confidence: 99%

“…The overall goal of early diagnosis is to carry out recommended surveillance; to prevent the development of cutaneous BCC due to the use of radiation in patients ( Gorlin, 1999 , Akbari et al, 2018 ); and to educate the patient to avoid UV radiation that may also result in BCC. Early diagnosis also helps with anticipating the development of new OKCs, managing them early and instituting simple orthodontic treatment to promote adequate jaw function ( Fujii and Miyashita 2014 ) and improve occlusal and esthetic outcome ( Feghali et al, 2022 ).…”

Section: Early Diagnosis Of Nbccs With Solitary Okcmentioning

confidence: 99%

“…Early diagnosis of NBCCS is important for carrying out recommended surveillance and preventing iatrogenic induction of BCC during the management of early lesional features in the disease (such as avoidance or lessening of radiation exposure and frequent CT scans at an early age ( Akbari et al, 2018 ), and seeking modalities other than x-irradiation for the treatment of patients with medulloblastoma ( Gorlin 1999 ), -all aimed at preventing the development of BCCs). In addition, early diagnosis and treatment of NBCCS-related OKCs may be crucial for maintaining adequate jaw function ( Fujii and Miyashita 2014 ), followed by the need to institute orthodontic therapy to address the occlusal and esthetic consequences of such treatments, as well as to eliminate or reduce any negative impact on maxillofacial growth ( Feghali et al, 2022 ). Early diagnosis of NBCCS is often hampered by the absence of some important lesions until late adolescence or adulthood ( Lo Muzio, 2008 , Gold et al, 2021 ), no previously diagnosed family members in over a quarter of cases ( Akbari et al, 2018 ), significant rarity (only 1–5 % of NBCCS) of medulloblastoma (an early lesion recently classified as a major criterion) ( Bree and Shah, 2011 , Palacios-Álvarez et al, 2018 ), and variation in presentation of lesions among different racial groups e.g.…”

Section: Introductionmentioning

confidence: 99%

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Pediatric odontogenic keratocyst and early diagnosis of Gorlin syndrome: Clinicopathological aids

Bello

2024

The Saudi Dental Journal

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Conservative management of multiple odontogenic keratocysts in a child with nevoid basal cell carcinoma syndrome: A case report

de Arruda,

de Lima,

de Oliveira Filho

et al. 2024

Special Care in Dentistry

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BackgroundNevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant multisystemic disorder characterized by the presence of multiple odontogenic keratocysts (OKC), which are a hallmark feature of the syndrome. The treatment of these OKC poses challenges due to their high recurrence rates and the myriad of management options available.Case ReportWe describe here a case of NBCCS diagnosed in an 11‐year‐old girl who presented with multiple OKC in the jaws. Chest and cranial radiographs showed no abnormalities in the ribs and the cerebral falx, respectively. Cephalometric analysis indicated mandibular retrusion, a skeletal class II relationship, and a convex profile. The treatment approach involved a personalized strategy tailored for each cyst, comprising marsupialization followed by enucleation. This approach aimed to minimize surgical trauma and to reduce the risk of recurrence. The patient underwent regular follow‐up appointments, demonstrating successful outcomes with no signs of recurrence or de novo OKC observed over a 32‐month period.ConclusionClinicians should consider lesion characteristics and patient cooperation when determining treatment strategies for the optimization of outcomes for children and adolescents with NBCCS and multiple OKC.

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Dental and orthodontic follow-up in nevoid basal cell carcinoma syndrome patient with odontogenic keratocystic tumors

Cited by 3 publications

References 13 publications

Dental Screening Including Panoramic Radiograph for Gorlin-Goltz Syndrome in Patients With Multiple Basal Cell Carcinomas

Dental Screening Including Panoramic Radiograph for Gorlin-Goltz Syndrome in Patients With Multiple Basal Cell Carcinomas

Pediatric odontogenic keratocyst and early diagnosis of Gorlin syndrome: Clinicopathological aids

Conservative management of multiple odontogenic keratocysts in a child with nevoid basal cell carcinoma syndrome: A case report

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