Studying the multiple faces of nevoid basal-cell carcinoma syndrome: A case series

Saxena, Shikha; Sundaragiri, Krishna Sireesha; Bhargava, Akshay; Sankhla, Bharat

doi:10.4103/jomfp.jomfp_358_19

Cited by 2 publications

(1 citation statement)

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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%