Aspectos clínicos, epidemiológicos e sobrevida de crianças com neuroblastoma: 21 anos de experiência do instituto de oncologia pediátrica, são paulo / Clinical and epidemiological characteristics and survival outcomes of children with neuroblastoma: 21 years of experience at the instituto de oncologia pediátrica, in são paulo, brazil

RESUMO Objetivo: Descrever as características clínicas e epidemiológicas e a sobrevida de crianças com neuroblastoma (NB) atendidas em centro de oncologia pediátrica, no período de 1991 a 2012. Métodos: Estudo retrospectivo, com análise de dados clínicos e epidemiológicos de 258 pacientes com NB atendidos em centro de oncologia pediátrica, no período de 1991 a 2012, por meio de consulta a prontuários médicos. Resultados: A idade média das crianças foi de 40,5±46,4 meses, e a mediana, de 28,9 meses (intervalo interquartil 42,2); relação masculino:feminino 1,3:1, sendo 1% dos pacientes assintomáticos. As manifestações mais frequentes foram: febre (25%), dor abdominal (22%), massa abdominal (19%) e dor óssea (19%). O tempo médio do início dos sintomas até a realização do diagnóstico foi de 3,0±4,8 meses. A localização do tumor mais frequente foi o abdome (63%). As metástases ocorreram na medula óssea (37%) e nos ossos (33%). A sobrevida global (SG) e a sobrevida livre de eventos (SLE) em cinco anos foram de 62 e 52%, respectivamente. A principal causa de óbito foi a progressão da doença (72%). Conclusões: As características clínicas das crianças com NB são variáveis e, em sua maioria, inespecíficas, o que torna o reconhecimento clínico difícil e, em geral, tardio. Em crianças com idade inferior a 5 anos, massa abdominal e/ou dor óssea, irritabilidade e febre de origem indeterminada, o diagnóstico de NB deve ser considerado.

ABSTRACT Objective: To describe the clinical and epidemiological characteristics and survival outcomes of children with neuroblastoma (NB) treated at a pediatric oncology center from 1991 to 2012. Methods: A retrospective study with clinical and epidemiological data from 258 patients with neuroblastoma treated at a pediatric oncology center from 1991 to 2012, using medical records. Results: The average age of the children at diagnosis was 40.5±46.4 months with a median age of 28.9 months (interquartile range 42.2). The male:female ratio was 1.3:1, and 1% of the patients were asymptomatic. The most frequent manifestations were: fever (25%), abdominal pain (22%), abdominal mass (19%), and bone pain (19%). The mean time from symptom onset to diagnosis was 3.0±4.8 months. The most common location of the tumor was the abdomen (63%). Metastases occurred in the bone marrow (37%) and in the bone (33%). Overall survival (OS) and event-free survival (EFS) in five years were 62 and 52%, respectively. The main cause of death was the progression of the disease (72%). Conclusions: The clinical features of children with neuroblastoma are variable and mostly nonspecific, which makes clinical recognition difficult and, in general, too late. In children less than 5 years old, with an abdominal mass and/or bone pain, irritability, and a fever from an unknown cause, neuroblastoma should be considered as a possible diagnosis.

High Risk Neuroblastoma in Uruguay. Where are we? / Neuroblastoma de Alto Riesgo en Uruguay. ¿Dónde estamos? / Neuroblastoma de Alto Risco no Uruguai. Onde estamos?

Introdução: El neuroblastoma es el tumor sólido extracraneal más frecuente en niños. Aproximadamente el 50 % de los pacientes son clasificados como de alto riesgo, con base en características clínicas, biológicas e histológicas. Objetivo: Describir a la población asistida en el Centro Hemato-Oncológíco Pediátrico (CHOP) del Centro Hospitalario Pereira Rossell (CHPR) con diagnóstico de neuroblastoma de alto riesgo, su tratamiento y sobrevida. Método: Estudio descriptivo y retrospectivo de todos los pacientes con neuroblastoma de alto riesgo diagnosticados en el CHOP entre el 2001 y el 2015. En el CHOP se ubica el Registro Nacional de Cáncer Pediátrico, así como también el Archivo de Historias Clínicas de todos los pacientes. Los datos son recolectados y analizados por el sector de estadística. Resultados: Se diagnosticaron 35 pacientes, de los cuales 20 (57%) eran varones con mediana de edad de 36,6 meses (5-93), localización suprarrenal 23 (66%) y 100% estadio IV. Metástasis, médula ósea y hueso: 27 (71%). Treinta y tres pacientes recibieron autotransplante de progenitores hematopoyéticos (TPH) (94%). Estatus previo a TPH, remisión completa: 19 (58 %), remisión parcial: 14 (42%). Mortalidad relacionada al tratamiento: 15 % y de recaídas: 68 %. Mediana de tiempo de recaída: 15 meses (3-52). La probabilidad de sobrevida global y sobrevida libre de eventos a 5 años fue de 37,8% ± 8,4 y 23,8% ± 7,3 (mediana de seguimiento 40 meses). Conclusión: A pesar del tratamiento intensivo y de las medidas de soporte adecuadas, el pronóstico en los niños con neuroblastoma de alto riesgo sigue siendo pobre en nuestro país. Es necesario incorporar nuevas estrategias terapéuticas aún no disponibles en nuestro medio.

Introduction: Neuroblastoma is the most common extracranial solid tumor in children. Approximately 50% of patients are classified as high risk on the basis of clinical, biological, and histological characteristics. Objective: To describe the population of patients diagnosed with high-risk neuroblastoma at the Centro Hemato-Oncológíco Pediátrico (CHOP, Center for Pediatric Hematology and Oncology) of the Centro Hospitalario Pereira Rossell (CHPR, Pereira Rossell Hospital), in terms of their treatment and survival. Method: Descriptive, retrospective study of all patients diagnosed with highrisk neuroblastoma at the CHOP between 2001 and 2015. The National Registry of Pediatric Cancer is located at the CHOP, as is the Archive of Patient Clinical Histories. The data are collected and analyzed by the statistics sector. Results: Among the 35 patients diagnosed, 20 (57%) were men, the median age was 36.6 months (range, 5-93 months), and the tumor had an adrenal location in 23 (66%). All of the tumors were classified as stage IV. Metastasis to the bone marrow or bone was seen in 27 (71%). Thirty-three patients (94%) received autologous hematopoietic stem-cell transplantation (HSCT). The status prior to HSCT was complete remission in 19 (58%) and partial remission in 14 (42%). The treatment-related mortality rate was 15%, and the relapse rate was 68%. The median time to relapse was 15 months (3-52 months). The probability of overall survival and 5-year event-free survival was 37.8% ± 8.4 and 23.8% ± 7.3 (median follow-up of 40 months), respectively. Conclusion: Despite intensive treatment and adequate support measures, the prognosis for high-risk neuroblastoma in children remains poor in Uruguay. There is a need to incorporate new therapeutic strategies not yet available in our country.

Introducción:O neuroblastoma é o tumor sólido extracraniano mais frequente em crianças. Aproximadamente 50% dos pacientes são classificados como de alto risco considerando as características clínicas, biológicas e histológicas. Objetivo: Descrever a população atendida no Centro Hemato-Oncológíco Pediátrico (CHOP) do Centro Hospitalario Pereira Rossell (CHPR) com diagnóstico de neuroblastoma de alto risco, seu tratamento e sobrevida. Método: Estudo descritivo, retrospectivo, de todos os pacientes com neuroblastoma de alto risco diagnosticados no CHOP, no período entre 2001 e 2015. O Registro Nacional de Câncer Pediátrico está localizado no CHOP, bem como o Arquivo de Histórias Clínicas de todos os pacientes. Os dados são coletados e analisados pelo setor estatístico. Resultados: Foram diagnosticados 35 pacientes. Vinte (57%) eram do sexo masculino. Mediana de idade: 36,6 meses (5-93). Localização suprarrenal: 23 (66%). Estádio IV 100%. Metástases, medula óssea e osso: 27 (71%). Trinta e três pacientes receberam transplante de células-tronco hematopoiéticas (TCTH) (94%). Status prévio a TCTH, remissão completa: 19 (58%), remissão parcial: 14 (42%). Incidência de mortalidade relacionada ao tratamento: 15% e de recaídas: 68%. Mediana do tempo de recaída: 15 meses (3-52). A probabilidade de sobrevida global e sobrevida livre de eventos aos cinco anos foi de 37,8% ± 8,4 e 23,8% ± 7,3 (mediana de seguimento 40 meses). Conclusão: Apesar do tratamento intensivo e medidas de suporte adequadas, o prognóstico em crianças com neuroblastoma de alto continua sendo pobre no Uruguai. É necessário incorporar novas estratégias terapêuticas que ainda não estão disponíveis em nosso meio

A fifty-year review of soft tissue sarcomas in Jamaica: 1958-2007 / Cincuenta años de revisión de los sarcomas de tejidos blandos en Jamaica: 1958-2007

OBJECTIVE: To determine the distribution of histologic subtypes of soft tissue sarcomas (STS) in Kingston and St Andrew, Jamaica, according to age and topography. METHODS: From the Jamaica Cancer Registry (JCR) archives, all cases of STS diagnosed between 1958 and 2007 were extracted. For each case, age, gender, histological diagnosis and anatomical site of tumour were recorded. Patients were categorized according to age at diagnosis as: children (0-14 years) and adults (> 14 years), and the distribution of histologic diagnoses with respect to age and anatomical site were analysed. RESULTS: There were 432 cases (67 children, 364 adults, one person of unknown age) of STS recorded in the JCR over the 50-year period (218 males, 214 females). The commonest STS in adults were "sarcoma, not otherwise specified [NOS]" (20.1%), malignant fibrous histiocytoma [MFH] (17.9%), fibrosarcoma (12.4%), liposarcoma (10.7%) and malignant peripheral nerve sheath tumour [MPNST] (10.2%). In children, they were neuroblastoma (38.8%), rhabdomyosarcoma (23.9%), "sarcoma, NOS" (9%), fibrosarcoma (6%) and MFH (6%). In adults, the lower limb was the commonest location, followed by trunk and/or upper limb for MFH, fibrosarcoma and liposarcoma, and head and neck for MPNST. In children, head and neck was the commonest site for rhabdomyosarcoma, head and neck and upper limb for MFH, retroperitoneum for neuroblastoma and trunk for fibrosarcoma. CONCLUSION: A high proportion of soft tissue sarcomas in Jamaica are unclassified and the anatomical distribution of common classified sarcomas shows some differences with the literature. Limited access to immunohistochemistry/molecular diagnostics and increasing core biopsy diagnosis may contribute to these phenomena.

OBJETIVO: Determinar la distribución de subtipos histológicos de sarcomas de tejido blando (STB) en Kingston y Saint Andrew, Jamaica, según la edad y la topografía. MÉTODOS: De los archivos del Registro de Cáncer de Jamaica, se extrajeron todos los casos de STB diagnosticados entre 1958 y 2007. Para cada uno de los casos, se registró la edad, el género, el diagnóstico histológico, y el sitio anatómico del tumor. Los pacientes fueron clasificados de acuerdo con la edad en el momento del diagnóstico, bajo las categorías de niños (0-14 años) y adultos (> 14 años), y se analizó la distribución de diagnósticos histológicos con respecto a la edad y el sitio anatómico. RESULTADOS: Se registraron 432 casos de STB (67 niños, 364 adultos, una persona de edad desconocida) en el JCR en un período de 50 años (218 varones, 214 hembras). Los STB más comunes en los adultos fueron "el sarcoma no especificado [NE]" (20.1%), el histiocitoma fibroso maligno [HFM] (17.9%), el fibrosarcoma (12.4%), el liposarcoma (10.7%), y el tumor maligno de la vaina del nervio periférico [TMVNP] (10.2%). En los niños, se trató de los neuroblastomas (38.8%), los rabdomiosarcomas (23.9%), "los sarcomas NE" (9%), los fibrosarcomas (6%), y los HFM (6%). En los adultos, los miembros inferiores fueron el lugar más común, seguido del tronco y/o los miembros superiores para el HFM, el fibrosarcoma y el liposarcoma; y la cabeza y el cuello para el TMVNP. En los niños, la cabeza y el cuello fueron el sitio más común para el rabdomiosarcoma; la cabeza, el cuello y los miembros superiores para el HFM; el retroperitoneo para el neuroblastoma; y el tronco para el fibrosarcoma. CONCLUSIÓN: Una proporción alta de sarcomas de tejidos blandos en Jamaica no están clasificados, y la distribución anatómica de sarcomas clasificados comunes muestran algunas diferencias con la literatura. El acceso limitado a los diagnósticos moleculares/inmunohistoquímicos, y el aumento de los diagnósticos centrales, pueden contribuir a estos fenómenos.

Apresentação clínica dos tumores sólidos mais comuns na infância e adolescência / Clinical presentation of the most common solid tumors in childhood and adolescent

O câncer infanto-juvenil é uma doença relativamente rara, sendo responsável por 0,5-3% do total de casos de câncer. Entretanto, ainda é causa de grande morbimortalidade em pacientes nessa faixa etária em nosso meio. Diagnóstico precoce e tratamento em centros especializados são essenciais para aumento da sobrevida em longo prazo. Objetivo: alertar o pediatra a respeito da importância do reconhecimento precoce das principais neoplasias malignas da infância e adolescência, dando ênfance à epedemiologia e apresentação clínica dos tumores sólidos. Métodos: foram selecionados 26 referências bibliográficas em pesquisa realizada nas base de dados Medline e no LILACS, em português, espanhol e inglês, utilizando-se as palavras-chave câncer infanto-juvenil, apresentação clínica, sobrevida.

Aspectos clínicos, terapêuticos e variáveis de prognóstico em crianças maiores de um ano portadoras de neuroblastoma não disseminado / Clinical features, treatment and prognostic variables in children older than one year with non-advanced neuroblastoma

Objetivo: estudar retrospectivamente o quadro clínico, aspectos epidemiológicos, características laboratoriais, genéticas e histológicas em crianças maiores de 1 ano, portadoras de neuroblastoma não disseminado, correlacionando-os com a evolução clínica e tentando definir fatores de risco que possam influir na sobrevida.Casuística e métodos: as informações foram obtidas a partir de 38 prontuários médicos...

Neuroblastoma / Neuroblastoma

O trabalho tece consideraçoes gerais sobre o conceito e características dos neuroblastomas, estuda sua incidência, etiologia e quadro clínico do tumor primário e das metástases, analisa os critérios diagnósticos, estadiamento do tumor, fatores prognósticos e tramento

Primitive peripheral neuroectodermal tumors Mayagüez Medical Center experience

Primitive neuroectodermal or neuroepithelial tumors are names used to describe neoplasias composed of undifferentiated cells resembling germinal cells of the embryonic neural tube. These tumors are small round cell malignancies of the neural crest origin arising outside the central and sympathetic nervous system. They are described as peripheral and central neuroectodermal tumors related to the original malignant cell. A great number of tumors are described under this classification in spite of the fact that there is no an universal acceptance that these small-cell neoplasms, regardless of their primary site, are derived from immature neuroectoderm tissue. Because one tumor resembles others in terms of its phenotypic expression, multiple specific studies such as clinical profile, ultrastructural, immunocytochemical, and cytogenetic features should be studied, since no single clinical or laboratory marker is by itself diagnostic. However, there is a chromosomal reciprocal translocation, t(11;22)(q24;q12), which is unique to Primitive Neuroectodermal Tumor (PNET)

Diagnóstico ultra-sonográfico pré-natal de neuroblastoma / Prenatal ultrasound diagnosis of neuroblastoma

O neuroblastoma de adrenal é o tumor sólido mais comum na infância. Todavia, é uma patologia relativamente rara e de baixa incidência. O prognóstico da doença está intimamente relacionado com a época do diagnóstico. Em vista disso, o diagnóstico ultra-sonográfico pré-natal de neuroblastoma de adrenal se apresenta como o método de escolha, pois permite detecçao o precoce nao-invasiva e sem periculosidade para o feto, bem como avaliaçoes seriadas e acuradas da doença. Este artigo faz uma análise que objetiva reunir os dados mais recentes da literatura para auxiliar na sistematizaçao da abordagem diagnóstica, para enfatizar a epidemiologia, os padroes ecográficos e o diagnóstico diferencial, e ainda revisar a etiopatogenia da doença.

Analytical study of 253 cases of central nervous system tumours

This is an analytical study of 253 cases of central nervous system space occupying lesions which were received and diagnosed histologically at the histopathology department at the laboratory section in the specialized surgical hospital at Saddam's Medical City for the period between June 1990 till the end of December 1992. Out of these 253 cases, 231 cases of intracranial and intraspinal tumours were confirmed histologically and 22 cases were miscellaneous. 109 cases were reported among male patients and 122 cases among female. The highest peak incidence was reported in the fifth decade. Of these tumours Glioma constituded the highest incidence [39.4%], and the majority were of high grade malignancy and poor prognosis. This was found to be similar to other previous Iraqi studies and other similar studies reported in literatures

Tumores en recién nacidos / Tumors in newborn infants

Se presenta una serie de 51 casos de tumores registrados en el período neonatal, entre 1.180 pacientes pediátricos con tumores, atendidos entre los años 1969 y 1989 en un hospital pediátrico del área metropolitana de Santiago. Los tumores neonatales más frecuentes fueron los teratomas (n:30), seguidos de los tumores de origen vascular (n:6), neuroblastomas IV-S (n:5), hamartomas hepáticos (n:4), los tumores renales (n:3), sarcoma de partes blandas (n:2) y melanoma melanocítico (n:1). Se destaca el comportamiento diferente de los procesos neoplásticos durante esta etapa de la vida y la importancia que tiene el tratamiento quirúrgico

Clinical Studies of Abdominal Tumors in Infants and Chi1dren: Ten Year Review

We have clinically observed 39 patients with abdominal tumors who were admitted in Severance Hospital during the past 10 years from April 1960 to March 1970. Unfortunately follow-up study couldn't be made. The following results were obtained. 1) Of the total number observed, 25 patients were male and 14 patients female. 2) The majority of the patients, 34 (87%) were under 6 years of age and 20 (51%) were between 1 to 3 years of age. The youngest patient was a 3-month-old female with Wilms' tumor. 3) In our series, Wilms' tumor (15 cases, 38.5%) was the most common and neuroblastoma (8 cases) was next most common tumor in the abdominal cavity. In addition we found another 4 cases of neuroblastoma in other sites. We observed hepatoma (8cases), ovarian tumor (3cases), lymphosarcoma (2cases), mesenteric cyst (one case) and adenoma of the left adrenal gland (one case) in that order of frequency. 4) In 15patients with Wilms' tumor, hypertension was observed in 8 patients and hematuria in 4 patients. Compared with Wilms' tumor, no hematuria was noted in the cases with neuroblastoma and hypertension occurred in 3 of these. 5) On abdominal X-ray, calcification was present in 4 cases with neuroblastoma (50%) and was present in only one of 15 patients with Wilms' tumor. 6) Comparing the sites of metastasis between Wilms' tumor and neuroblastoma, 4 of 15 patients with Wilms' tumor had lung metastasis and 4 of 8 patients of neuroblastoma had bony metastasis. Liver metastasis was found at autopsy in one case of neuroblastoma. 7) Three cases of hepatoblastoma were under 2 years of age and three cases of hepatocarcinoma were over 12 years of age. 8) Three cases of ovarian tumor were benign cystic teratoma. Tortion of the ovarian pedicle occurred in one of 3 patients with severe low abdominal pain. All patients were discharged in good condition after salphingo-oophorectomy. 9) One case of lymphosarcoma of the ileum was associated with intussusception. 10) A one year o1d female with adenoma of the left adrenal gland had typical Cushing's syndrome.