Complex care by multiple medical and dental specialists of a patient with aggressive Gorlin–Goltz syndrome

Abstract

Gorlin-Goltz syndrome is a genetically predisposed disease characterised by multiple basal cell carcinomas, odontogenic keratocysts and ectopic calcifications. The aim of this study was to show successful treatment of a 37-year-old male patient by cooperation between different dental and medical specialists. Because of the recurrence of a large basal cell carcinoma after multiple operations and a total dose telecobalt irradiation of 66 Gy, the patient's nose was ablated, with resection of the upper lip and part of the maxilla. The intraoral prosthetic treatment helped to restore the subtotal edentulousness. In order to enhance the application of the dental prosthesis, an Abbe plasty was performed at the second stage of surgery. As previous irradiation of the area precluded the use of facial implants immediately after the intraoral treatment, a temporary artificial nose prosthesis was created. The patient tolerated the procedures well and is completely disease-free 12 months after the surgery. Besides presenting a rare and complicated case of Gorlin-Goltz syndrome, the main purpose of this report is to show that, if different specialists in a dental-clinical team (maxillofacial surgeon, oncologist, radiation oncologist, prosthodontist and psychiatrist) combine their skills and expertise, successful management is possible even in a challenging complex case.

Gorlin-Goltz syndrome – a medical condition requiring a multidisciplinary approach

“…Therefore, genetic tests are a matter of key importance for formulating adequate diagnosis, in particular during the first years of lives of the youngest patients. In most cases, however, GGS is detected using clinical criteria such as the presence of 2 symptoms of high importance or 1 of high and 2 or 3 symptoms of little importance (Table 4) [4,5,16,21,58,59]. …”

“…If there is a suspicion of NBCCS in an adult patient, they too need to be carefully examined (Table 5) [1,4,5,10,16,21,35,59]. …”

“…Aggressive forms of basal cell carcinomas, which infiltrate the facial bones, hardly ever occur [20]. The above-mentioned lesions are extremely challenging for therapists but, thanks to the combined efforts of various medical specialists such as maxillofacial surgeons, plastic surgeons, laryngologists, oncologists, radiation oncologists, restorative dental specialists and psychologists, the patients have a chance to recover and regain their regular social functions [8,15,21]. …”

Clinical and oral findings in an Afro‐Brazilian family with Gorlin‐Goltz syndrome: case series and literature review

“…An exception occurs when oral keratocysts lead to jaw swelling and patients first visit a dental clinic with this chief complaint as happened with our index case. GGS is then, first diagnosed by the dentist in many instances . This report presented a rare GGS diagnosis in an Afro‐Brazilian family, which was made by dentists despite the numerous clinical and radiographic characteristics presented by the index case and her mother.…”

Multidisciplinary oral rehabilitation of an adolescent suffering from juvenile Gorlin-Goltz syndrome – a case report

“…In order to avoid extensive lesions, frequent dental check-ups are recommended during the growth period [14, 33–35]. A treatment including multiple medical disciplines is necessary for the rehabilitation of patients suffering from tumors [33, 36]. On the other hand, the patient’s compliance is inevitable due to the time consuming and stressful therapy.…”