Late dental and bone alterations in patients after orbital rhabdomyosarcoma treatment.

PURPOSE: Orbital rhabdomyosarcoma is a rare soft tissue sarcoma in childhood but with a good prognosis. Treatment usually includes surgery, chemotherapy, and radiotherapy. This study aimed to evaluate long-term alterations in teeth and cranial bones in children, adolescents, and young adults after oncologic treatment for childhood orbital rhabdomyosarcoma. METHOD: This was a cross-sectional study that evaluated patients treated for orbital rhabdomyosarcoma between 1988 and 2011. Demographic, clinical, and treatment data were collected during the study period; also, panoramic radiographs, cephalometric study, and photographs of the face were taken. RESULTS: Eight long-term survivors were studied. Of those, 50% were male, 75% had less than 5 years of treatment, and 88% had only one of the orbits affected by the tumor. Regarding treatment, 50% received 50.4 Gy of radiotherapy in the orbit; the chemotherapy included vincristine, actinomycin D, and cyclophosphamide in 75% of the cases and also ifosfamide and etoposide in 25%. The children presented craniofacial alterations, mainly when radiotherapy occurred between 0 and 5 years old (p = 0.01). The mandibles also showed dental alterations, probably due to chemotherapy. CONCLUSION: In conclusion, orbital RMS patients treated with chemoradiotherapy, important dental, and facial bone alterations were found. The most significant were in the maxilla and close to the irradiation field. Dental and mandibular bone alterations were also found, indicating the probable chemotherapy action, as this region was not included in the irradiation field.

Epidemiological characteristics and survival outcomes of children with medulloblastoma treated at the National Cancer Institute (INCA) in Rio de Janeiro, Brazil.

BACKGROUND: Medulloblastoma (MB),the most common malignant brain tumor of childhood has survival outcomes exceeding 80% for standard-risk and 60% for high-risk patients in high-income countries (HICs). These results have not been replicated in low- and middle-income countries (LMICs), where 80% of children with cancer live. METHODS: This is a retrospective review of 114 children aged 3-18 years diagnosed with MB from 1997 to 2016 at National Cancer Institute (INCA). Sociodemographic, clinical, and treatment data were extracted from the medical records and summarized descriptively. Overall survival (OS) and progression-free survival (PFS) were calculated using the Kaplan-Meier method. RESULTS: The male-to-female ratio was 1.32 and the median age at diagnosis was 8.2 years. Headache (83%) and nausea/vomiting (78%) were the most common presenting symptoms. Five-year OS was 59.1% and PFS was 58.4%. The OS for standard-risk and high-risk patients was 69% and 53%, respectively. The median time to diagnosis interval was 50.5 days and the median time from surgery to radiation therapy initiation was 50.4 days. Patients who lived >40 km from INCA fared better (OS = 68.2% vs. 51.1%, p = .032). Almost 20% of families lived below the Brazilian minimum wage. Forty-five patients (35%) had metastatic disease at admission. Gross total resection was achieved in 57% of the patitents. CONCLUSIONS: Although there are considerable barriers to deliver effective MB treatment in countries like Brazil, the OS seen in the present study demonstrates that good outcomes are not only feasible but can and should be increased with appropriate interventions.

Human Epidermal Growth Factor Receptor 2 (HER2) Expression by Immunohistochemistry and Its Clinical Significance in Hepatocellular Carcinoma: A Single-Center Analysis.

BACKGROUND: Human epidermal growth factor receptor 2 (HER2) is a member of the tyrosine kinase receptor family. It has been identified as an oncogene and is associated with poor outcomes in multiple tumor types. In hepatocellular carcinoma (HCC), there are contradictory data regarding the HER2 expression and its role in tumor development and progression. Some studies have identified HER2 expression as an early event during tumorigenesis, which decreases with progression and metastasis. Additional data provided evidence that treatment with anti-HER2 therapy resulted in local response and reduction in the metastasis rate in HCC mice models. METHODS: Patients with histological diagnoses of HCC between 2010 and 2020 were included. HER2 staining was performed by immunohistochemistry (IHC), and scoring was done in accordance with the gastric cancer guidelines as 0, 1+, 2+, and 3+. Clinicopathological features were accessed by medical records. This study aims to evaluate HER2 expression by IHC in HCC and to correlate this expression with some clinicopathological features such as Barcelona Clinic Liver Cancer (BCLC) staging, number of hepatic lesions, alpha-fetoprotein level, underlying liver disease, presence of liver cirrhosis, Child-Pugh score, and tumor recurrence. RESULTS: A total of 57 specimens from 54 patients were included. Of the patients, 85% were men, and the median age at diagnosis was 71 years (interquartile range: 59-75 years). Regarding stage, 61% were at stage 0-A of BCLC. Of the patients, 57% had a solitary HCC nodule. Concerning treatment, surgery was performed in 50% of the patients. HER2 expression was identified in seven patients: five in the membrane and two in the cytoplasm. Concerning the membrane staining, HER2 expression was scored as 1+/2+ in 7.4% (n = 4 patients). Of the patients with HER2 expression, four had a BCLC stage of 0-A and a single HCC nodule; alpha-fetoprotein was <400 ng/mL in all cases. There was no correlation to clinicopathological features. In one patient with HER2 2+ expression at diagnosis, this expression was not identified at tumor progression. Median disease-free survival in HER2 with IHC scores 1+/2+ and cytoplasmatic was 38 months versus 22 months in HER2 with a score of 0 (p = 0.604). CONCLUSIONS: HER2 expression is a rare event in HCC. It was not possible to identify any relation to clinicopathological features. However, when we relate our data to previous trials, HER2 appears to be an early event in the course of HCC.

HER2-Positive Inflammatory Breast Cancer Challenges of Clinical Practices.

HER2-positive inflammatory breast cancer (IBC) is associated with poor overall survival. Targeted therapies have led to improved outcomes. IBC is underrepresented in clinical trials due to its rareness. This case reports a 52-year-old woman diagnosed with IBC of 119x89mm, axillar node-positive, hormone receptor-negative, HER2 positive. The patient underwent neoadjuvant chemotherapy with dual HER2 blockage. Mastectomy histology showed pathological complete response. After two cycles of adjuvant trastuzumab, the patient developed asymptomatic cardiotoxicity leading to the therapeutic suspension. Early recurrence and persisting cardiac alterations prevented treatment with anti-HER2 therapy. At the time of brain recurrence, with cardio-oncology collaboration, it was possible to start TDM-1, with a reduction of 71% of brain lesions size, after two cycles. This case highlights the effectiveness of anti-HER therapy in IBC and the importance of multidisciplinary discussion in treatment choice and toxicity management.

Do Not Forget Poly (Adenosine Diphosphate-Ribose) Polymerase Inhibitors in Ovarian Carcinosarcoma.

Ovarian carcinosarcoma (OCS) is a rare entity with a poor prognosis and without evidence-based therapy. Here, we report the case of a 55-year-old woman with a germline BRCA1 mutation and a stage IV OCS who was proposed olaparib maintenance therapy after three platinum-based chemotherapies in relapsed disease. Currently, the patient has an overall survival of 102 months and progression-free survival of 60 months with olaparib, with a good quality of life and not experiencing any adverse events. Despite the lack of evidence for the use of poly (adenosine diphosphate-ribose) polymerase inhibitors in OCS, our case report proves that patients with a potential biomarker of response to these drugs (such as BRCA mutation and platinum-sensitive disease) derive great benefits from it.

Long lasting complete response with immunotherapy in a metastatic bladder carcinoma: a case report.

At diagnosis, approximately 25% of urothelial carcinoma are invasive and only 15% of stage IV are alive at 5-years. We report a case of a 69-year-old woman with oligometastatic bladder cancer, treated with Atezolizumab in first-line, achieving a complete response after 4 cycles. Presently, the patient has an overall survival and progression free survival of 26 months with an improvement in her quality of life. Therefore, immunotherapy seems to be a promising treatment in advanced urothelial carcinoma. The previously performed radiotherapy, in association with a good performance status and oligometastatic disease, might have contributed to this admirable outcome.

Dental and craniofacial alterations in long-term survivors of childhood head and neck rhabdomyosarcoma.

OBJECTIVE: Rhabdomyosarcoma (RMS) represents the most common soft tissue sarcoma that affects children. Treatment involves chemoradiotherapy. This study aimed at evaluating the long-term alterations to teeth and cranial bones in children, teenagers, and young adults after oncologic treatment. STUDY DESIGN: We conducted a cross-sectional study of patients undergoing treatment for head and neck RMS between 1988 and 2011. We evaluated demographic, clinical, and treatment data and performed panoramic radiography, cephalometry, and photography. RESULTS: We evaluated 27 long-term survivors, most of whom had been treated between ages 0 to 5 years (51.9%). The total radiation dose applied was 50.4 Gy, and the chemotherapy combination included vincristine, actinomycin D, and cyclophosphamide in 51.9% of the cases. We observed 603 dental alterations, among which 377 (62.7%) occurred in patients ages 0 to 5 years, and root shortening was the most frequent alteration observed (24.2%). With regard to facial bones, 74% of the patients had some level of facial asymmetry, 70.4% had reduced facial depth, 48.4% had mandibles of short size, and 77.8% had reduced facial height. CONCLUSIONS: Children submitted to RMS treatment involving chemotherapy and radiotherapy displayed significant dental and craniofacial alterations, especially when treatment occurred between ages 0 and 5 years.

Análise Epidemiológica, Clínica e Patológica de Crianças com Neoplasias do Sistema Nervoso Central Tratadas com Radioterapia no Instituto Nacional de Câncer / Epidemiological, Clinical, and Pathological Analysis of Children with Central Nervous System Neoplasms Treated with Radiotherapy at the National Cancer Institute / Análisis Epidemiológico, Clínico y Patológico de Niños con Neoplasias del Sistema Nervioso Central Tratados con Radioterapia en el Instituto Nacional de Cáncer

Introdução: As neoplasias de sistema nervoso central representam 21% do câncer infantojuvenil, sendo a principal causa de morte nessa população em países desenvolvidos. Avanços na neuroimagem têm melhorado seu diagnóstico, e a combinação de terapias tem permitido a cura, com maior preservação neurológica. A cirurgia é o principal tratamento, frequentemente combinada com radioterapia e quimioterapia. Objetivo: Descrever achados socioeconômicos, sinais e sintomas, e aspectos histológicos dos tumores cerebrais pediátricos, tratados com radioterapia. Método: Estudo transversal e descritivo, utilizando prontuários de 257 crianças e adolescentes portadores de neoplasias de sistema nervoso central, tratados com radioterapia no Instituto Nacional de Câncer, no Rio de Janeiro, de 2012 a 2020. As variáveis foram divididas em sociodemográficas, clínicas e patológicas. Resultados: Crianças brancas do sexo masculino foram as mais prevalentes, com mediana de idade de 7,5 anos no diagnóstico e de 8,0 anos na radioterapia. A maioria possuía casa própria com infraestrutura básica e renda familiar de até um salário-mínimo. Os sintomas mais comuns foram cefaleia, vômitos e alterações na marcha, variando de acordo com a localização do tumor e a idade da criança. A radioterapia foi utilizada em 64% dos pacientes, principalmente para meduloblastoma, tumores de tronco cerebral e ependimoma anaplásico. Conclusão: Estudos específicos sobre crianças e adolescentes com neoplasias cerebrais tratados com radioterapia são raros na literatura. Quando comparados com a população em geral, apresentam perfis sociodemográficos e clínicos semelhantes, com diferenças notáveis apenas na frequência dos tipos tumorais

Introduction: Central nervous system neoplasms account for 21% of pediatric cancer cases and are the leading cause of death in this population in developed countries. Advances in neuroimaging have improved diagnosis, and combination therapies have ensured the cure with greater neurological preservation. Surgery is the primary treatment, often combined with radiation therapy and chemotherapy. Objective: To describe socioeconomic findings, signs and symptoms, and histological aspects of pediatric brain tumors treated with radiation therapy. Method: A cross-sectional and descriptive study was conducted with 257 children and adolescents utilizing medical records with central nervous system neoplasms treated with radiotherapy at the National Cancer Institute, Rio de Janeiro, from 2012 to 2020. The variables were divided into sociodemographic, clinical, and pathological categories. Results: White male children were the most prevalent, with a median age at diagnosis of 7.5 years and at radiotherapy, of 8.0 years. The majority had their own home with basic infrastructure and family income of up to one minimum wage. The most common symptoms were headache, vomiting, and gait disorders, varying according to tumor location and child's age. Radiation therapy was used in 64% of patients, mainly for medulloblastoma, brainstem tumors, and anaplastic ependymoma. Conclusion: Specific studies on children and adolescents with brain neoplasms treated with radiotherapy are rare in the literature. When compared with the general population, they present similar sociodemographic and clinical profiles, with remarkable differences only in the frequency of tumor types

Introducción: Las neoplasias del sistema nervioso central representan el 21% del cáncer infantil y juvenil, siendo la principal causa de muerte en esta población en países desarrollados. Los avances en neuroimagen han mejorado su diagnóstico y la combinación de terapias viene permitiendo la cura, con una mayor preservación neurológica. La cirugía es el tratamiento principal, a menudo combinada con radioterapia y quimioterapia. Objetivo: Describir los hallazgos socioeconómicos, los signos y síntomas, y los aspectos histológicos de los tumores cerebrales pediátricos tratados con radioterapia. Método: Estudio transversal y descriptivo utilizando las historias clínicas de 257 niños y adolescentes con neoplasias del sistema nervioso central, que fueron tratados con radioterapia en el Instituto Nacional del Cáncer en Río de Janeiro entre 2012 y 2020. Las variables se dividieron en sociodemográficas, clínicas y patológicas. Resultado: Los niños blancos y de sexo masculino fueron los más prevalentes, con una mediana de edad de 7,5 años en el momento del diagnóstico y de 8,0 años en el momento de la radioterapia. La mayoría poseía una vivienda propia con infraestructura básica y un ingreso familiar de hasta un salario mínimo. Los síntomas más comunes fueron dolor de cabeza, vómitos y alteraciones en la marcha, que variaban según la ubicación del tumor y la edad del niño. Se utilizó radioterapia en el 64% de los pacientes, principalmente para meduloblastoma, tumores del tronco cerebral y ependimoma anaplásico. Conclusión: Los estudios específicos sobre niños y adolescentes con neoplasias cerebrales tratados con radioterapia son raros en la literatura. Cuando se comparan con la población en general, presentan perfiles sociodemográficos y clínicos similares, con diferencias notables solo en la frecuencia de los tipos tumorales

Dinâmica da Implantação de Humanização no Serviço de Radioterapia Pediátrica do Instituto Nacional de Câncer José Alencar Gomes da Silva, Brasil / Dynamics of Implementation of Humanization in the Pediatric Radiotherapy Service of the National Cancer Institute José Alencar Gomes da Silva, Brazil / Dinámica de la Implementación de la Humanización en el Servicio de Salud Radioterapia Pediátrica del Instituto Nacional de Cáncer José Alencar Gomes da Silva, Brasil

Introdução: O tratamento do câncer infantil é multidisciplinar, e a radioterapia faz parte dessa abordagem, sendo, em geral, um procedimento crítico em crianças. A necessidade de permanecerem sem acompanhante e imóveis no aparelho de tratamento, aliada ao receio da natureza dessa terapia, faz com que o uso de anestesia seja comum. Para que a criança pudesse se sentir mais confortável e confiante com esse tratamento, foram introduzidas medidas de humanização na rotina da radioterapia. Objetivo: Descrever a dinâmica do processo de humanização utilizado. Método: Estudo descritivo sobre a implantação de medidas de humanização, entre maio/2016 e dezembro/2017, no Serviço de Radioterapia do Instituto Nacional de Câncer José Alencar Gomes da Silva, RJ, Brasil. Resultado: A customização das máscaras de tratamento, a utilização de fantasias correspondentes, o certificado de coragem, as comemorações de datas festivas e as melhorias no ambiente foram as principais intervenções de humanização realizadas. Houve uma transformação no enfrentamento das adversidades inerentes ao tratamento, com evidente benefício para o ambiente e bem-estar da criança. Uma relação de confiança foi estabelecida entre a criança, sua família e a equipe profissional. Maior adesão, redução do absenteísmo e aparente redução de anestesia para o tratamento foram os resultados constatados. Conclusão: As ações implementadas trouxeram inequívoco ganho para o bem-estar geral dos envolvidos no processo de humanização. Aponta-se a necessidade de estudo quantitativo sobre a melhoria no fluxo de atendimento nesse Serviço, seja por meio da redução do uso de anestesia para o tratamento ou no tempo diário no aparelho de radioterapia

Introduction: The treatment of childhood cancer is multidisciplinary and radiotherapy is part of this approach, being, in general, a critical procedure in children. The need to remain unaccompanied and motionless in the treatment apparatus, together with the fear of the nature of this therapy, makes the use of anesthesia common. Humanization measures were introduced in the radiotherapy routine to help children to feel more comfortable and confident with this treatment. Objective: To describe the dynamics of the humanization process. Method: A descriptive study addressing the implementation of humanization measures was developed, between May 2016 and December 2017, in the Radiotherapy Service of the National Cancer Institute José Alencar Gomes da Silva, RJ, Brazil. Result: The customization of the treatment masks, the use of matching costumes, the certificate of courage, the celebrations of festive dates and the improvements in the environment were the main humanization interventions carried out. There was a transformation in coping with the adversities inherent to the treatment, with clear benefits for the environment and the child's well-being. A trusting relationship was established between the child, his family and the professional team. Greater adherence, reduced absenteeism and an apparent reduction in the use of anesthesia for treatment were found. Conclusion: The actions implemented brought an unambiguous gain in the general well-being of those involved in the humanization process. The necessity of quantitative study on improving the flow of care in the Radiotherapy Service, either by reducing the use of anesthesia for treatment or the daily time spent in the radiotherapy device is highlighted

Introducción: El tratamiento del cáncer infantil es multidisciplinario y la radioterapia forma parte de este abordaje, siendo, en general, un procedimiento crítico en los niños. La necesidad de permanecer sin compañía e inmóvil en el aparato de tratamiento, combinada con el miedo a la naturaleza de esta terapia, hace que el uso de anestesia sea común. Para que el niño se sintiera más cómodo y seguro con este tratamiento, se introdujeron medidas de humanización en la rutina de radioterapia. Objetivo: Describir la dinámica del proceso de humanización utilizado. Método: Estudio descriptivo sobre la implementación de medidas de humanización, entre mayo/2016 y diciembre/2017, en el Servicio de Radioterapia del Instituto Nacional del Cáncer José Alencar Gomes da Silva, RJ, Brasil. Resultado: La personalización de las máscaras de tratamiento, el uso de los trajes correspondientes, el certificado de valentía, las celebraciones de fechas festivas y las mejoras en el entorno fueron las principales intervenciones de humanización realizadas. Se produjo una transformación en el afrontamiento de las adversidades inherentes al tratamiento, con evidentes beneficios para el medio ambiente y el bienestar del niño. Se estableció una relación de confianza entre el niño, su familia y el equipo profesional. Se encontrón mayor adherencia, menor absentismo y una aparente reducción en el uso de anestesia para el tratamiento. Conclusión: Las acciones implementadas aportaron una ganancia inequívoca en el bienestar general de los involucrados en el proceso de humanización. Es necesario un estudio cuantitativo sobre la mejora del flujo de atención en esto Servicio, ya sea reduciendo el uso de anestesia para el tratamiento o el tiempo diario en el dispositivo de radioterapia

Rabdomiossarcoma Embrionário: Relato de Caso com 15 Anos de Sobrevida e Revisão de Literatura / Embryonal Rhabdomyosarcoma: Case Report with a 15-year Survival and Literature Review

O rabdomiossarcoma de cabeça e pescoço é o sarcoma mais comum de tecido mole em crianças. O planejamento do tratamento depende da localização do tumor, extensão da doença e presença ou não de metástases.O tratamento pode causar diversas sequelas tardias na região de cabeça e pescoço, principalmente na cavidade oral. A prevenção e o controle dessas sequelas proporcionam uma melhor qualidade de vida para o paciente. Relato de caso: Este relato descreve um caso de rabdomiossarcoma embrionário parameníngeo em região de parótida diagnosticadoem um paciente do sexo masculino aos 3 anos de idade, tratado com quimioterapia e radioterapia. Esse paciente recebeu atendimento odontológico como parte integrante do tratamento multidisciplinar. Apresentou boa resposta ao tratamento, permanecendo em controle clínico sem evidência de doença por 15 anos. As sequelas tardias em face e cavidade oral do tratamento oncológico foram: fechamento precoce das raízes; rizogênese incompleta em todos os elementos dentários; agenesias dentárias; múltiplos dentes inclusos; hipoplasia dos ossos da face e trismo. Entretanto,teve qualidade de vida satisfatória, com manutenção da capacidade mastigatória, tendo frequentado a escola edesenvolvido um bom convívio social. Conclusão: O rabdomiossarcoma de cabeça e pescoço está associado a sequelas tardias decorrentes tratamento. A abordagem multidisciplinar é importante para a prevenção e o controle das sequelas e obtenção de uma melhor qualidade de vida para os pacientes...