Gorlin-Goltz syndrome (GGS), also known as nevoid basal cell carcinoma syndrome, is an autosomal dominant inherited disorder with high penetrance and variable expressivity. The classic triad originally described by Gorlin and Goltz in 1960 is composed of multiple nevoid basal cell carcinomas (NBCCs), odontogenic keratocysts (OKCs) in the jaws and bifid ribs. in 1977, this triad was modified by Rayner et al, and to GGS diagnosis, the OKCs had to appear in combination with calcification of the cerebellar falx or palmar and plantar pits. It may occur that although GGS syndrome is a well-known condition, only the specific symptom could be observed by different specialists. Therefore, the patient cannot be placed in an always complex clinical panel. The authors introduce an example in this report. In the present case, the patient had NBCCs, OKCs, and probably other signs of GGS since 1998, and has been treated for this conditions separated, without a diagnosis of a syndromic condition. A 54-year-old white woman was referred to the oral medicine service due to cyst located in the right mandibular body. She had history of skin cancer and undergone surgeries and radiotherapies for the lesions treatment, scars on the skin face due to the lesions removed, and a new ulcerated lesion on the back of was diagnosed. In addition, the patient presented frontal and parietal bossing leading to increased cranial circumference, hypertelorism, strabismus, broad base, and mandibular prognathism. To the image examination, skull radiography revealed calcification of the falx cerebri; on chest X-ray bifid rib was observed and spine radiography showed vertebral osteophytes. Panoramic radiograph showed a well-defined bilocular radiolucent image located in posterior and anterior mandibular region. The whole elements induced us to investigate the patient's past medical history, which revealed that since 1998 had the diagnosis of NBCC and OKC. A multidisciplinary approach becomes necessary for the diagnosis and follow-up of patients with GGS, considering the complexity of the clinical manifestations. Therefore, it is of primary importance for dental surgeons and dermatologists to know the signs and symptoms of GGS to perform early diagnosis and to avoid progression of the oral cysts or metastasis of the skin lesions.
Objetivo: analizar retrospectivamente resultados y factores pronósticos de pacientes con cáncer cérvico uterino estadios IIB-IIIB tratados con radioquimioterapia concomitante.Materiales y métodos: desde septiembre 1999 a diciembre del 2002, 164 pacientes ingresaron con el diagnóstico de cáncer de cérvix, seleccionándose 83 en estadios IIB-IIIB, las que fueron tratadas con radioterapia y cisplatino semanal por cinco semanas.Resultados: el 89,15% completó cinco ciclos de quimioterapia. La mediana de sobrevida global fue de 41 meses, con una sobrevida global (SG), sobrevida libre de enfermedad (SLE) y sobreviva libre de recurrencia loco-regional (SLRLR) a tres años de 68,8%, 66,3% y 83,1%, respectivamente. Factores pronósticos de sobrevida fueron etapa IIIB, diámetro tumoral clínico ≥ 7 cm, no realización de braquiterapia y enfermedad biparametrial.Conclusiones: el tratamiento con radioterapia y quimioterapia concomitante con cisplatino en pacientes con cáncer cervicouterino estadios IIB-IIIB, es posible de realizar en el Hospital Carlos Van Buren perteneciente al sistema de salud público chileno, con buena tolerancia, resultados y factores pronósticos similares a los de la literatura internacional.
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