BACKGROUND:Therapeutic approaches in pediatric populations are based on adult data because there is a lack of appropriate data for children. Consequently, there are many controversies regarding the proper treatment of pediatric patients.OBJECTIVE:The present study was designed to evaluate patients with differentiated thyroid carcinoma diagnosed before 20 years of age and to determine the factors associated with the response to the initial therapy.METHODS:Sixty‐five patients, treated in two tertiary‐care referral centers in Rio de Janeiro between 1980 and 2005 were evaluated. Information about clinical presentation and the response to initial treatment was analyzed and patients had their risk stratified in Tumor‐Node‐ Metastasis; Age‐Metastasis‐Extracapsular‐Size; distant Metastasis‐Age‐Completeness of primary tumor resection‐local Invasion‐Size and American‐Thyroid‐Association classificationRESULTS:Patients ages ranged from 4 to 20 years (median 14). The mean follow‐up was 12,6 years. Lymph node metastasis was found in 61.5% and indicated a poor response to initial therapy, with a significant impact on time for achieving disease free status (p = 0.014 for response to initial therapy and p<0,0001 for disease‐free status in follow‐up). Distant metastasis was a predictor of a poor response to initial therapy in these patients (p = 0.014). The risk stratification systems we analyzed were useful for high-risk patients because they had a high sensitivity and negative predictive value in determining the response to initial therapy.CONCLUSIONS:Metastases, both lymph nodal and distant, are important predictors of the persistence of disease after initial therapy in children and adolescents with differentiated thyroid cancer.

Unlike their cutaneous counterparts, head and neck mucosal malignant melanomas (HNMM) are more aggressive, and their prognostic markers have not been fully elucidated. This study, comprising 28 patients with HNMM, aimed to establish the relationship between different mutations and outcome, define the incidence of KIT mutations in HNMM, and identify the correlation among therapeutic options, histopathological findings, demographic data, and clinical response. Clinical analysis included patient characteristics, staging, primary and palliative treatments, and disease-free survival and overall survival (OS). Progression-free survival and OS were analyzed. Paraffin blocks were selected following histologic analyses, enabling DNA extraction. PCR amplification of exons 9, 11, 13, and 17, with different DNA concentrations, was performed. Patients were predominantly females (57%) and aged 27–85 years. All patients underwent surgery; 17 received adjuvant radiotherapy, and recurrences occurred in 82% patients. Oncologic mutations in KIT were found in 7 of 7 tumors, 3 in exon 9, 3 in exon 11, and 1 in exon 13. Predictive factors for recurrence were mitotic rate, vascular invasion, and perineural spread. There were no significant differences in DFS and OS according to KIT mutation. Our study results suggest that some patients might benefit from appropriate targeted therapy with kinase inhibitors.

Mucosal melanomas of the head and neck are very rare malignancies that present with aggressive behavior and poor prognosis. Usually diagnosed at advanced stages, thus presenting macroscopically as aggressive nodular neoplasms arising from the mucosa; few cases are detected in situ. Tumor staging for mucosal melanoma remains a challenge. Several staging systems have been suggested, including tumornodal-metastases (TNM) staging systems, but none are frequently used. There is no clear consensus on the management of head and neck mucosal melanoma, which reflects the rare nature of the disease and complexity of the anatomic site. The late diagnosis, frequently presenting at an advanced stage, denotes the aggressive nature of the disease. Currently, early detection and surgical excision is considered the primary method of treatment. The multidisciplinary team approach can help reduce morbidity and mortality once optimize treatment, reduce costs and minimize adverse events, while maximizing the chances of recovery.

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To evaluate the treatment results and outcome of T4a and T4b oral squamous cell carcinoma (OSCC) at a single institution. METHODS: The charts of 251 consecutively untreated T4 OSCC patients (reclassified by AJCC 2002) eligible for treatment (surgery plus RT, exclusive RT, RT plus QT) were retrospectively analyzed. Factors with possible impact on survival were analysed. Survival rates were calculated according to the Kaplan-Meier method. RESULTS: 196 patients were classified T4a and 55 as T4b. 49% percent of patients underwent radical surgery plus RT, 30.6% received RTϩ/-CT and 17.2% had palliative RT. The 5-year disease-free (DFS) and overall (OS) survival rates for those who had surgery plus RT were 49.5% and 42.5%, respectively, in comparison with 30.4% and 37.3% for patients undergoing RTϩ/-CT, respectively. Univariate analysis revealed that age, N-stage, and type of treatment (pϭ.003, pϭ.006, and pϽ.001, respectively) were predictors for local control in T4a and T4b patients. In multivariate analysis, type of treatment was independent predictor for DFS and OS (pϭ.0001, and pϭ.0001, respectively). CONCLUSIONS: Radical surgery plus radiotherapy was shown to be the best therapeutic option for T4a OSCC patients. This study also emphasized the role of TNM classification as a reliable prognostic indicator.

Dedico esta obra, especialmente, ao meu pai, Dr. Manoel Elpídio Toscano de Mendonça, assim como eu, era médico, dedicou toda a sua vida aos mais carentes. Hoje chego ao final dessa estrada graças a ele. Infelizmente, você nos deixou antes do tempo, mas sei que, de onde você estiver, você estará comemorando comigo. À minha mãe, Sônia, que me ensinou os valores necessários para que eu pudesse alcancar sucesso e felicidade na vida pessoal e profissional. A minha esposa e companheira, Cristiane, pela compreensão, me apoiando nas longas jornadas de trabalho e me estimulando a procurar sempre o melhor. A meu filho, Rafael, que, apesar do pouco tempo de vida, me ensinou a batalhar pela vida. Aos meus irmãos, Gavroche, Sandino, Ludmylla, pelos momentos felizes da nossa infância. A minha sogra, Maria de Fátima, pelo carinho e pela presteza com a minha família.

To analyze the influence of the unique percentage of skin carcinomas with skull base invasion on the choice of the facial surgical approach. Design: Multi-institutional retrospective analysis of the medical charts of all patients who had undergone oncological craniofacial operations from 1981 to 2005. Data were collected on demographic distribution, location of the primary tumor, histologic type, type of operation, reconstruction, complications, and outcome. Special attention was directed toward the choice of facial approach. Setting: Two major tertiary care centers. Patients: A total of 484 patients who had undergone major skull base operations. Intervention: Frequency of atypical facial approaches. Main Outcome Measures: Impact on the need for more sophisticated reconstructions and on surgical morbidity. Results: During this 25-year period, 484 patients underwent major skull base operations in the 2 centers; data concerning 467 cases were available for analysis. The median age of the patients was 52.8 years (range, 4-88 years), and the male-female ratio was 1.9:1.0. The initial location of the tumor was the craniofacial skin in 63.5% of cases, ethmoid in 10.8%, maxilla in 2.3%, orbit in 1.9%, and other origins, including endocranial, in 19.4%. The histologic type of the lesions was basal cell carcinoma in 42.0% of cases, squamous cell carcinoma in 29.5%, esthesioneuroblastoma in 5.3%, adenocarcinoma in 3.9%, adenoid cystic carcinoma in 2.8%, and other types in 16.5%. Owing to this high prevalence of advanced skin carcinomas, the most commonly employed facial approach was atypical, tailored to encompass all compromised skin and underlying tissues, in 55.5% of cases, followed by the Weber-Ferguson approach, with all its variations (eg, nasal swing) in 17.8%, lateral rhinotomy in 12.2%, facial translocation in 3.8%, and other facial techniques in 7.7%. No facial approach was required in 1.5% of cases. Conclusion: In most situations, head and neck surgeons chose an atypical surgical approach to properly resect all facial structures invaded by very advanced skin cancers.