Recent clinical research on the application of liquid biopsy in neuroblastoma / 中国当代儿科杂志

Neuroblastoma (NB) is the most common extracranial solid tumor in children and has the features of high recurrence rate and low survival rate, and therefore, early diagnosis, treatment response evaluation, and recurrence monitoring are of great significance for NB patients. Liquid biopsy refers to the detection of cells and nucleic acids in fluid specimens, mainly blood. It is noninvasive and can overcome tumor heterogeneity, thus making it possible to achieve the early diagnosis and dynamic detection of NB. This review introduces the latest advances in clinical research on the application of liquid biopsy in NB.

Neuroblastoma cervical: presentación de casos y revisión actualizada del tema / Cervical neuroblastoma: presentation of cases and updated review of the topic

El neuroblastoma es uno de los tumores sólidos extracraneales más comunes en la edad pediátrica, y se origina en células precursoras del sistema nervioso simpático. La ubicación cervical corresponde a un 2-5% del total de los neuroblastomas y puede tener distintas manifestaciones clínicas, tales como masa cervical, disnea, estridor, síndrome de Horner o disfagia. Esta entidad debe ser considerada dentro del diagnóstico diferencial de una masa cervical pediátrica, especialmente ante la presencia de masas sólidas, laterales o paramedianas, palpables o no al examen físico. El tratamiento específico del neuroblastoma depende de la clasificación de riesgo del paciente, pudiendo ser expectante en casos específicos, exclusivamente quirúrgico, o bien requerir complementarse con otras terapias. En este artículo se presentan 2 casos clínicos de pacientes pediátricos con neuroblastoma cervical tratados de forma exclusiva y exitosa con cirugía, y una revisión del tema.

Neuroblastoma is one of the commonest extracranial solid tumors at pediatric age, originating from sympathetic nervous system precursor cells. Cervical position stands for 2-5% of all neuroblastomas, with variable clinical expression that includes cervical mass, dyspnea, stridor, Horner syndrome and dysphagia. This condition must be considered in the differential diagnosis of a pediatric cervical mass, specially in those solid, lateral/paramedian masses that could be palpable or not at physical examination. The specific treatment in neuroblastoma depends on patient´s risk group, including conservative follow-up in selected cases, surgery alone, or complementary perioperative therapy with chemotherapy and others. In this article, the group report two cases of cervical neuroblastoma exclusively treated with surgery with good results, and a literature review.

Crisis adrenérgica como forma de debut de un neuroblastoma / Adrenergic crisis as a debut form of a neuroblastoma

INTRODUCCIÓN: La forma clínica de presentación más común del neuroblastoma es el de una masa abdominal, pero puede presentarse con sintomatología menos habitual, como es la crisis adrenérgica por liberación de catecolaminas. OBJETIVO: Describir una forma de presentación inusual de neuroblastoma y el amplio diagnóstico diferencial que existe en un lactante con síntomas adrenérgicos. CASO CLÍNICO: Lactante femenina de 7 semanas de vida, consultó por historia de tres semanas de sudoración e irritabilidad a lo que se asoció fiebre de 24 h de evolución y dificultad respiratoria. Al ingreso presentaba mal esta do general, irritabilidad, sudoración, enrojecimiento facial, taquipnea y palidez cutánea, taquicardia sinusal extrema e hipertensión arterial (HTA), interpretadas como sintomatología adrenérgica. Se completó el estudio con una ecografía abdominal y resonancia magnética que mostraron una gran masa retroperitoneal compatible con neuroblastoma. Las catecolaminas en sangre y en orina mostraron altos niveles de dopamina, adrenalina y noradrenalina, probablemente de origen tumoral. Se inició tratamiento antihipertensivo con fármacos alfa bloqueantes con buen control de la tensión arterial. Se resecó quirúrgicamente el tumor sin incidencias y con una adecuada recuperación posterior. La paciente presentó evolución favorable a tres años de seguimiento. CONCLUSIONES: en un lactante con sintomatología adrenérgica como irritabilidad, enrojecimiento, sudoración asociada a HTA, se debe descartar patología cardiaca, metabólica (hipoglucemia), intoxicaciones y/o patología suprarrenal. Dentro de esta última, el neuroblastoma es la primera posibilidad diagnóstica, por ser uno de los principales tumores en la infancia y aunque esta presentación no es habitual puede producir estos síntomas.

INTRODUCTION: The most common clinical presentation of neuroblastoma is an abdominal mass, but it can present with uncommon symptoms, such as adrenergic storm due to catecholamine release. OBJECTIVE: To describe an unusual presentation of neuroblastoma and the wide differential diagnosis that exists in an infant with adrenergic symptoms. CLINICAL CASE: A 7-week old female infant was evaluated due to a 3-week history of sweating and irritability associated with a 24-hour fever and respiratory distress. At admission, she presented poor general condition, irritability, sweating, facial redness, tachypnea and skin paleness, extreme sinus tachycardia, and high blood pressure (HBP), interpreted as adrenergic symptoms. The study was completed with abdominal ultrasound and magnetic reso nance imaging that showed a large retroperitoneal mass compatible with neuroblastoma. Plasma and urinary catecholamines tests showed high levels of dopamine, adrenaline, and noradrenaline, probably of tumor origin. We started antihypertensive treatment with alpha-blocker drugs, showing a good blood pressure control. The tumor was surgically resected without incidents and adequate subsequent recovery. The patient presented a favorable evolution after three years of follow-up. CONCLUSIONS: In an infant with adrenergic symptoms such as irritability, redness, sweating associated with HBP, it should be ruled out pathology heart or metabolic (hypoglycemia) pathology, intoxications, and/or adrenal pathology. Within this last one, neuroblastoma is the first diagnostic possibility, since it is one of the main tumors in childhood and, although this presentation is not usual, it can produce these symptoms.

Neuroblastoma in patients under 18 months: Single institution experience in Argentina

The purpose of the study was to evaluate the outcome of patients under 18 months diagnosed with neuroblastoma. Between April 2006 and December 2013, 45 consecutive patients followed in Hospital de Pediatría Garrahan, were retrospectively reviewed. With a median age of 9.3 months (1-18 months) N-myc amplification was detected in 5 out of 38 patients, 1p deletion (del1p) in 4 patients, and 11q aberration in one patient. With a median follow-up of 53 (range: 6-109 months), at 24 months the event free survival (EFS) of all patients was 83% (SE 6%) and overall survival (OS) of 88% (SE 5%). Significant difference was found in OS and EFS between patients with stages L1, L2 and Ms vs. stage M (p = 0.01 and p = 0.01 respectively). EFS for each stage: L1 85% (SE 7%), L2 100%, MS 100%, vs. M 55% (SE 16%). OS: L1 90% (SE 6%), L2 100%, MS 100%, vs. M 66% (SE 15%). OS and EFS results are similar to those reported in international studies. However, better identification of biological prognostic factors will warr ant accurate staging and consequently an appropriate treatment.

El objetivo del trabajo fue evaluar las características y evolución de pacientes menores de 18 meses de edad, con diagnóstico de neuroblastoma. Se realizó un análisis descriptivo, retrospectivo entre abril/2006 y diciembre/2013, de 45 pacientes diagnosticados en forma consecutiva. La edad media fue 9.3 meses (1-18 meses). La amplificación del gen N-myc fue detectada en 5 pacientes, deleción del cromosoma 1p (del1p) en 4, y aberración de 11q en uno. Con una media de seguimiento de 53 meses (6-109 meses), la supervivencia libre de eventos (SLE) de todos los pacientes, a 24 meses fue 83% (ES 6%) y la supervivencia global (SG) de 88% (ES 5%). Se encontró diferencia significativa en la SG y SLE entre los pacientes con estadios L1, L2 y Ms, y aquellos con estadio M (p = 0.01). La SLE para cada estadio fue: L1 85% (ES 7%), L2 100%, MS 100%, M 55% (ES 16%). SG para cada estadio: L1 90% (ES 6%), L2 100%, MS 100%, y M 66% (ES 15%). Aunque los resultados de SG y SLE son similares a los publicados en estudios internacionales, una mejor identificación de los factores pronósticos biológicos permitirá una estadificación precisa y, en consecuencia, un tratamiento adecuado.

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.

Neuroblastoma y citometría de flujo multiparamétrica, una nueva y posible herramienta diagnóstica: Caso clínico / Multi-parametric Flow Cytometry for Neuroblastoma, a new and possible diagnostic tool: Case report

El neuroblastoma es el tumor sólido pediátrico extracraneal más frecuente, que representa un 5,6% según el Registro On-copediátrico Hospitalario Argentino. Se requieren, para su diagnóstico, varios métodos complementarios (radiológicos, biológicos y bioquímicos), entre los que la citometría de flujo multiparamétrica (CFM) surge con un potencial rol, aún no explorado. La CFM es una metodología que permite obtener información sobre el tamano, la complejidad y la expresión antigénica de la célula mediante el uso de un láser y anticuerpos monoclonales fluorescentes. Existe un creciente número de trabajos en la literatura que dan cuenta de la relevancia de la aplicación de la CFM en el diagnóstico y seguimiento de tumores sólidos. El objetivo de esta presentación es destacar el rol fundamental que tuvo la CFM en el caso de una paciente con neuroblastoma, en la cual un diagnóstico precoz permitió administrar rápidamente un adecuado tratamiento inicial.

Neuroblastoma is the most frequent extracranial solid tumor in childhood, representing 5.6% according to the "Registro Oncopediátrico Hospitalario Argentino". For its diagnosis, several complementary methods (radiological, biological and biochemical) are required, and Multi-parametric Flow Cytometry (MFC) arises as a potential diagnostic method, despite not having been so far extensively explored. MFC is a method that allows to obtain several information about size, internal complexity and antigenic expression by the use of a laser and fluorescent monoclonal antibodies. There is an increasing number of reports in the literature which reveal the importance of using MFC for diagnosis and monitoring of solid tumors. The aim in this presentation is to highlight the fundamental role that MFC had in the case of a patient affected by neuroblastoma, in whom an early diagnosis using this methodology allowed prompt administration of adequate treatment.

Neuroblastoma: a clinico-epidemiological study in Al-Khadhimyia teaching hospital

Background: Neuroblastoma is the third most common childhood cancer, after leukemia and brain tumors, and is the most common solid extra cranial tumor in children. The term neuroblastoma is commonly used to refer to a spectrum of neuroblastic tumors [including neuroblastomas, ganglioneuroblastomas, and ganglioneuromas] that arise from primitive sympathetic ganglion cells

Objective: The aim of this research is to study the epidemiological and clinical feature of neuroblastoma in a group of Iraqi infants and children who were admitted to the pediatric ward of Al-Khadhimyia Teaching Hospital

Patients and methods: The study was done over a period of three months from 1[st] of Feb. 2010 to the end of Apr. 2010. In reviewing all files, 18 cases were collected from the Pediatric Hemato-Oncology Consultation Clinic in Al-Khadhimyia Teaching Hospital, they were diagnosed and treated consequently in the pediatric ward / hemato-oncology unit over a period of 9 years [2002-2010]. Data regarding age, sex, residence, site of primary disease and clinical presentation were taken from the recording files in the pediatrics Hemato-Oncology Clinic. Methods used to diagnose our patients includes, fine needle aspiration of tumor mass, Bone marrow aspiration and biopsy of different sites. Chemotherapy was the main line of treatment

Results: The total number of studied cases was eighteen cases. The median age was 15 months with male to female ratio of [0.63:1], 9 cases [50%] were below 1 year. Abdominal mass was the commonest site of tumor 12 cases [67%], weight loss was the most common symptom 18 cases [100%] followed by abdominal distention 14 cases [78%], abdominal mass represent the most common sign in 12 cases [67%]. Stage IV was noticed in 9 cases [50%], 10 cases [56%] were a high risk group, 7 cases [38.8%] had bone marrow metastases, 6 cases [34%] finished treatment and survive, 4 cases [22%] relapsed, 2 cases [11%] died

Conclusion: The majority of cases were recognized during the first two years of age. Female were involved more than male. The outcome of treated cases was good and accepted. Abdominal mass is the commonest site, weight loss is the commonest symptoms

Thoracic neuroblastoma presenting as recurrent empyema.

Neuroblastoma is the most common intra-abdominal and extracranial solid tumour in children, accounting for 7%–8% of all childhood cancers. It is a malignant tumour of the autonomic nervous system derived from the neural crest. Most children with neuroblastoma have distant metastatic disease at the time of diagnosis. Pulmonary metastasis at the time of diagnosis is rare, and rarer is the presence of associated pleural effusion. We present the case of a child with recurrent empyema, who was diagnosed to have a thoracic neuroblastoma.

Hyperostotic Esthesioneuroblastoma: Rare Variant and Fibrous Dysplasia Mimicker

A 65-year-old male presented with a 3-year history of orbital symptoms. An imaging-based diagnosis of fibrous dysplasia involving the skull base was made at another institution. CT showed a diffuse sinonasal mass and ground-glass appearance of the bones of the anterior skull base with bony defects and mucocele formation. MRI demonstrated an accompanying intracranial and orbital rind of soft tissue mass along the hyperostotic bones. FDG-PET showed corresponding intense hypermetabolism. Small cysts were observed at the tumor-brain interface. Biopsy revealed esthesioneuroblastoma with bone infiltration that is compatible with the hyperostotic variant of esthesioneuroblastoma. There are a few cases of hyperostotic esthesioneuroblastoma reported in the literature.

Neuroblastoma congénito cervical en recién nacido: presentación de un caso / Cervical congenital neuroblastome in a newborn: presentation of a case

Los neuroblastomas congénitos cervicales son muy raros. Esta forma de cáncer infantil se forma en el tejido nervioso y por lo general suele presentarse con mayor frecuencia en las glándulas suprarrenales. Aunque puede aparecer prenatalmente, es más frecuente que se diagnostique en el primer año de vida. Son tumores agresivos con una alta mortalidad. En casi todos los casos (50-60 por ciento de los mismos), para cuando se detecta un neuroblastoma, ya se ha diseminado a otras partes del cuerpo. Se presenta un caso de recién nacido que las primeras 24 horas, muestra una historia de compromiso de vías respiratorias y digestivas asociado a una masa cervical sólida y parálisis de XII par craneal.

Cervical congenital neuroblastomes are very rare. This form of infantile cancer forms in the nervous tissue and generally it uses to appear more frequently in the suprarenal glands. Although they may appear prenatally, they are more frequent in the first year after birth. They are aggressive tumors with a high mortality. In almost all the cases (50/60 percent of them), when a neuroblastome is detected, it is already disseminated to other parts of the body. We present the case of a newborn who shows a history of respiratory and digestive tracts compromise associated to a solid cervical mass and XII cranial par paralysis during the first 24 hours after birth.

Neuroblastoma en niños menores de 18 meses. Experiencia de 10 años en Centro Hematooncológico Pediátrico del Centro Hospitalario Pereira Rossel / Neuroblastome in children younger than 18 months. Ten years experience at the Pediatric Hemato Oncological Center of the Centro Hospitalario Pereira Rossell

El neuroblastoma es el tumor maligno más frecuente en los lactantes. Su curso clínico es variable, desde la regresión espontánea a la progresión maligna, y los factores pronósticos son múltiples, como edad, estadio, amplificación de N-myc y ploidía tumoral. Se describen las características de todos los pacientes con neuroblastoma menores de 18 meses asistidos en CHOP. Pacientes y métodos: estudio observacional, descriptivo y retrospectivo en el período entre 31 de enero de 2000 y 31 de enero de 2011. El diagnóstico se realizó por histología y aspirado de médula ósea. Los pacientes se estadificaron por INSS; el tratamiento se decidió según estadio y riesgo. Resultados: se incluyeron 22 pacientes menores de 18 meses (52% de todos los neuroblastomas), con una media de edad de 9,6 meses. Once pacientes se encontraban en estadio 4. La localización más frecuente fue suprarrenal; presentaban metástasis 13 pacientes. Quince niños recibieron poliquimioterapia y 20 fueron tratados quirúrgicamente. La amplificación del genN-myc se demostró en tres pacientes. La sobrevida global fue de 77% y la sobrevida libre de enfermedad fuede 77%. Discusión y conclusiones: la mayor parte de los casosfueron diagnosticados en niños menores de 9 meses. Fueron más frecuentes los estadios 4 y 1. No se pudo demostrar asociación entre N-myc y estadio de enfermedad. La sobrevida fue excelent...

Presence of Differentiating Neuroblasts in Bone Marrow is a Favorable Prognostic Factor for Bone Marrow Metastatic Neuroblastoma at Diagnosis

BACKGROUND: The prognostic impact of the presence of differentiating neuroblasts in bone marrow (BM) remains unclear in BM metastatic neuroblastoma (NB). We aimed to identify the prognostic impact of differentiating neuroblasts in BM at diagnosis and after chemotherapy. METHODS: A total of 51 patients diagnosed with BM metastatic NB at Asan Medical Center between January 1990 and July 2005 were enrolled. BM histology and laboratory data along with overall survival (OS) were compared with regard to the differentiation status of neuroblasts in BM at diagnosis and after chemotherapy. RESULTS: Among the 51 patients, 13 (25.5%) exhibited differentiating neuroblasts in BM at diagnosis and 17/51 (33.3%) exhibited them after chemotherapy. The only significant difference among patient groups was the improved OS in patients with differentiated neuroblasts in BM at diagnosis (P=0.021). In contrast, the differentiation status of neuroblasts in BM after chemotherapy did not affect OS (P=0.852). CONCLUSIONS: Our study is the first report describing the presence of differentiating neuroblasts in BM. The presence of differentiating neuroblasts in BM at diagnosis may be a favorable prognostic factor for patients with BM metastatic NB; however, the same phenomenon after chemotherapy is irrelevant to prognosis.

Causa infrecuente de hipertensión arterial en lactantes: neuroblastoma congénito quístico suprarrenal: caso clínico / A rare cause of hypertension in childhood: congenital cystic adrenal neuroblastoma: clinical case

The frequency of hypertension (HBP) in children has increased significantly over the past decade. The younger the patient the greater the likelihood of having secondary HBP. Thus, the main causes of hypertension in new-borns are of renovascular or parenchymatous origin. objective: To present the case of an infant with hypertension caused by a congenital cystic neuroblastoma (NB). Case History: Newborn with prenatal diagnosis of adrenal cyst, who evolved with significant hypertension unresponsive to medical therapy. Neuroblastoma was suspected on the basis of magnetic resonance imaging findings and resection of the lesion was able to resolve the hypertension and to confirm the diagnosis by anatomo-pathological study. Conclusion: Most cases of neonatal hypertension are of renal origin, with the 2 largest categories being renovascular and renal parenchymal diseases. NB is the most common neonatal malignancy. It usually presents as an abdominal mass of antenatal diagnosis, being the hypertension an unusual form of presentation.

La frecuencia de hipertensión arterial (HTA) en niños ha aumentado significativamente en la última década. A menor edad del paciente mayor es la probabilidad de que la HTA sea secundaria. Así, las principales causas de HTA en recién nacidos son de origen renovascular o parenquimatoso. objetivo: Presentar el caso de un lactante hipertenso por neuroblastoma (NB) congénito quístico. Caso Clínico: Recién nacido con diagnóstico prenatal de quiste suprarrenal, quien evolucionó con HTA por sobre el percentil 99 para edad, sexo y talla, sin respuesta a terapia farmacológica. La resonancia magnética permitió realizar el diagnóstico presuntivo de neuroblastoma congénito y la resección de la lesión permitió resolver la HTA y confirmar el diagnóstico. Conclusión: La HTA en recién nacidos generalmente se debe a causas secundarias. El NB es el tumor maligno neonatal más frecuente que se puede presentar como una masa abdominal de diagnóstico antenatal, siendo la HTA una forma infrecuente de presentación.

Neuroblastoma torácico en la adolescencia: caso clínico / Thoracic neuroblastoma in adolescence: clinical case

Neuroblastoma is predominantly a tumor of early childhood, most cases occur in children under 5 years old. It originates in the adrenal gland and paravertebral ganglion cells (neural crest-derived), being the most common an extracranial solid tumor in children. It is characteristic a spontaneous regression, However in some cases it shows progression and dissemination to other organs. Objetive: To show a neuroblastoma in adolescence, with poor response to chemotherapy and radiotherapy, requiring surgery treatment. Clinical case: A 16 y.o. Female patient, previously asymptomatic, who after a body temperature rise up to 39 ° C, was found to have a tumor in the right hemithorax. Biopsy was compatible with neuroblastoma. Surgical removal of a large 20 x 19 cm tumor was achieved, the only complication presented 10 days postop was recurrent pneumothorax. Conclusion: Despite little or no response to standard treatment, surgical resection of this large tumor achieved complete remission for this patient.

El neuroblastoma es predominantemente un tumor de la infancia temprana que en la mayoría de los casos se presenta en menores de 5 años. Se origina en la glándula suprarrenal y células ganglionares paravertebrales (derivadas de la cresta neural), siendo el tumor sólido extracraneal más común en pediatría, presenta regresión espontánea en algunos casos y en otros progresión y diseminación a otros órganos. Objetivo: Analizar el caso de una adolescente portadora de un neuroblastoma resistente a quimioterapia y radioterapia, que requiere tratamiento quirúrgico. Caso clínico: Paciente asintomática, edad 16 años, que a raíz de alza febril se le detecta un tumor en hemitórax derecho. La biopsia fue compatible con un neuroblastoma. Se realizo la extirpación quirúrgica de un gran tumor con diámetro de 20 cm x 19 cm. A los 10 días se complica con neumotórax recidivante. Conclusión: A pesar de la poca o nula respuesta a las armas terapéuticas habituales: quimioterapia y radioterapia, la cirugía logró la resección del tumor torácico con remisión completa.

Assessment of Serologic Immunity to Diphtheria-Tetanus-Pertussis After Treatment of Korean Pediatric Hematology and Oncology Patients

The aim of this study was to investigate the diphtheria-tetanus-pertussis antibody titers after antineoplastic treatment and to suggest an appropriate vaccination approach for pediatric hemato-oncologic patients. A total of 146 children with either malignancy in remission after cessation of therapy or bone marrow failure were recruited. All children had received routine immunization including diphtheria-tetanus-acellular pertussis vaccination before diagnosis of cancer. The serologic immunity to diphtheria, tetanus and pertussis was classified as: completely protective, partially protective, or non-protective. Non-protective serum antibody titer for diphtheria, tetanus and pertussis was detected in 6.2%, 11.6%, and 62.3% of patients, respectively, and partial protective serum antibody titer for diphtheria, tetanus and pertussis was seen in 37%, 28.1%, and 8.9% of patients. There was no significant correlation between the severity of immune defect and age, gender or underlying disease. Revaccination after antineoplastic therapy showed significantly higher levels of antibody for each vaccine antigen. Our data indicates that a large proportion of children lacked protective serum concentrations of antibodies against diphtheria, tetanus, and pertussis. This suggests that reimmunization of these patients is necessary after completion of antineoplastic treatment. Also, prospective studies should be undertaken with the aim of devising a common strategy of revaccination.

Opsoclonus-mioclonus: uma manifestação clínica aguda de neuroblastoma / Opsoclonus-myoclonus: an acute clinical neuroblastoma's manifestation

Objetivo: Descrever o caso de uma criança do sexo masculino que apresenta sintomas de forma súbita de Opsoclonus-mioclonus associada a tumor da crista neural. Descrição: Os autores relatam um caso de uma criança do sexo masculino de 1 ano e 10 meses que apresenta, subitamente, instabilidade de tronco e membros, tremores e ataxia. Três dias depois aparece opsoclonus, caracterizando a Síndrome de Opsoclonus-mioclonus. Uma massa tumoral retroperitoneal compatível com neuroblastoma foi encontrada em tomografia computadorizada de abdome. Discussão: Quando é feito o diagnóstico de Opsoclonus-mioclonus, a pesquisa de tumores deve periodicamente ser pesquisada, uma vez que estes podem se manifestar agudamente ou tardiamente...

Objective: To describe the case of a male child who presents sudden symptoms of Opsoclonus-myoclonus associated to a neural crest tumor. Description: The authors report a case of a 22-month-old male child that suddenly presents instability from trunk and limbs, trembling and ataxia. Three days later, the opsoclonus appeared and opsoclonus-myoclonus Syndrome was diagnosed. A retroperitoneal tumor, compatible with neuroblastoma was found through an abdominal computed tomography scan. Discussion: When the Opsoclonus-myoclonus is diagnosed, the search for tumors must persist periodically, since these tumors can present acute or late manifestation...

Abnormal brain MRI in a case of acute ataxia as the only sign of abdominal neuroblastoma

Ataxia is a movement disorder that may manifest an acute, intermittent, non progressive or chronic progressive course. Ataxia alone is rare as a paraneoplastic sign, especially if it is due to neuroblastoma [abdominal or chest]. We report an abdominal neuroblastoma in a two-year-old girl presenting with only acute ataxia and abnormal neuroimaging. Brain MRI showed abnormal signal finding in the medulla, pons, corticospinal tract and the periventricular space. In the abdominal CT, a mass was detected in the right adrenal gland with calcification and the histopathologic examination re-vealed neuroblastoma. We suggest in children with acute ataxia, with or without opsoclonus-myoclonus, neuroblastoma should be considered