Gorlin-Goltz syndrome and neoplasms: a case study.

UNLABELLED: Gorlin syndrome is a rare autosomal dominant disorder exhibiting high penetrance and variable expressivity. It is characterized by facial dysmorphism, skeletal anomalies, multiple basal cell carcinomas, odontogenic keratocysts (OKC), palmar and plantar pits, bifid ribs, vertebral anomalies and a variety of other malformations. Various neoplasms, such as medulloblastomas, meningiomas, ovarian and cardiac fibromas are also found in this syndrome. OBJECTIVE: To describe a twelve-year-old patient with Gorlin-Goltz syndrome, with basal cell carcinomas and promyelocytic leukemia developed after receiving craniospinal radiation for a medulloblastoma. Bifid ribs as well as mandibular and maxillar OKC were also diagnosed Conclusion: The patient with Gorlin-Goltz syndrome should receive close follow-up for early detection of malformations nd malignant neoplasias.

Dental abnormalities in children submitted to antineoplastic therapy.

PURPOSE: The purpose of this study was to assess, by panoramic radiographs, the prevalence of morphological dental changes in children with cancer who were submitted for chemotherapy alone or concomitant radiotherapy of the head and neck. METHODS: All patients admitted between March, 1996 and February, 2004 were analyzed and 137 were included in this retrospective, nonrandomized, institutional study. The rates of microdontia, taurodontia, anodontia, macrodontia, blunt root, and tapered root were assessed. RESULTS: The patients were distributed into 2 groups: (1) those with lymphoproliferative neoplasias (61%); and (2) those with solid tumors (39%). Their mean age when treatment began was 5 years and 6 months. Dental abnormalities were found in 39 (29%) patients, while 98 (72%) patients did not present any abnormality. The abnormalities found were: (1) microdontia (7%; N= 10); (2) anodontia (6%; N=8); (3) taurodontia (14%; N=19); (4) macrodontia (5%; N=7); (5) blunted root (2%; N=2); and (6) tapered root (4%; N=5). Of these patients: 22% (N=30) presented 1 abnormality; 4% (N=6) presented 2 abnormalities; and 2% (N=3) presented 3 abnormalities. CONCLUSION: Taurodontia was the most frequent abnormality found in children and adolescents who underwent antineoplastic treatment, and its rate was significantly higher than those found for the healthy Brazilian population. This study's results show that it is necessary for the odontologist to systematically research the dental changes that occur among this special group of patients.

Rhabdomyosarcoma of the oral tissues--two new cases and literature review.

Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm consisting of cells derived from the primitive mesenchyme that exhibit a profound tendency to myogenesis. About 35% of RMS arises in the head and neck, being classified as parameningeal and non-orbital non-parameningeal forms. Parameningeal tumors carry the worst prognosis. The use of contemporary, multi-agent chemotherapy, radiotherapy, and surgery has made treatment of the disseminated disease possible, and has significantly improved overall survival from 25% in 1970 to 70% in 1991. Here, we present the management of two cases of orofacial RMS in adolescents: an 18-year-old, white female that had a 9-month history of a nodule in the left buccal mucosa, and a 19-year-old, white male who had been aware of a nodule in the left, posterior maxillary ridge with progressive growth for 4 months. Before final diagnosis, both cases were previously treated as inflammatory lesions. Their clinicopathological aspects, treatment, and poor survival as a consequence of delays in diagnosis are discussed.

Dental maturity assessment in children with acute lymphoblastic leukemia after cancer therapy.

UNLABELLED: Dental age is largely used in both forensic studies and clinical practice. All over the world, many studies have been made to determine chronological age using dental ages of individuals, but selecting individuals with no chronic or acute sickness as the study group. Cancer is the second most frequent cause of death in children, and acute lymphoblastic leukemia (ALL) is the most common type of cancer in childhood. Most of the children who survived childhood cancer experienced disturbances in dental development due to cancer therapy or to cancer itself. The aim of this study is to assess dental development in children and teenagers who had suffered from childhood leukemia and were submitted to chemotherapy isolated or associated with radiotherapy, by comparing the dental ages with those corresponding features in a healthy control group. Dental development was analyzed using panoramic radiographs of 92 children divided in two groups: 46 children between 5 and 12 years old, treated for ALL at GRAACC-IOP, UNIFESP, and as a control group, 46 healthy children treated for dental reasons at APCD, São Paulo. The dental age of the subjects was estimated using the system of Demirjian et al. [A. Demirjian, H. Goldstein, J.M. Tanner, A new system of dental age assessment, Hum. Biol. 45(2) (1973) 211-227]. A significant difference was found between the chronological and dental age of patients submitted to antineoplasic therapy for ALL, when compared to those of the control group, but there were no significant differences between patients treated with different protocols for ALL. CONCLUSIONS: although the study was within a small group of patients, we could clearly conclude that antineoplastic therapy can interfere in the dental maturity of patients treated for childhood cancer by interfering in dental formation and root development.