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1.
Rev. pediatr. electrón ; 17(3): 49-56, oct. 2020. ilus
Article in Spanish | LILACS | ID: biblio-1367347

ABSTRACT

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.


Subject(s)
Humans , Male , Infant, Newborn , Infant , Head and Neck Neoplasms/diagnosis , Neuroblastoma/surgery , Neuroblastoma/diagnosis , Horner Syndrome , Diagnosis, Differential , Airway Obstruction/etiology , Head and Neck Neoplasms/surgery , Head and Neck Neoplasms/complications , Neuroblastoma/complications
2.
Arch. argent. pediatr ; 117(6): 651-654, dic. 2019. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1046714

ABSTRACT

El síndrome de opsoclonus mioclonus es un trastorno poco frecuente en pediatría. El diagnóstico es clínico y se caracteriza por la presencia de, al menos, tres de los siguientes: opsoclonus, mioclonías, ataxia, irritabilidad y trastornos del sueño. En más del 50 % de los casos, se asocia con la presencia de neuroblastoma. Es un trastorno de origen inmunitario y su tratamiento es a base de inmunosupresores, inmunomoduladores y resección tumoral en los casos secundarios a neuroblastoma. Entre el 70 % y el 80 % de los casos pueden tener secuelas neurológicas, dependiendo de la causa, la gravedad inicial de los síntomas y la velocidad de instauración del tratamiento.Se presenta el caso de un varón de 2 años con diagnóstico de síndrome de opsoclonus mioclonus secundario a un neuroblastoma suprarrenal izquierdo, en el que se realizó la resección tumoral y el tratamiento con corticoides, inmunoglobulina y rituximab.


Opsoclonus-myoclonus syndrome is a rare disorder among pediatric patients.The diagnosis is clinical and is characterized by the presence of at least three of the following: opsoclonus, myoclonus, ataxia, irritability and sleep disorders. In over 50 % of cases it is associated with the presence of Neuroblastoma. It is a disorder of immune origin and its treatment is based on immunosuppressants, immunomodulators and tumor resection in cases secondary to Neuroblastoma. Up to 70 % to 80 % of cases may present neurological sequelae, depending on the cause, the initial severity of symptoms and the delay of proper treatment. We present the case of a 2-year-old male with diagnosis of opsoclonus-myoclonus syndrome secondary to a left adrenal Neuroblastoma. Tumor resection and treatment with corticosteroids, immunoglobulin and rituximab were performed.


Subject(s)
Humans , Male , Child, Preschool , Opsoclonus-Myoclonus Syndrome , Neuroblastoma/surgery , Neuroblastoma/drug therapy , Pediatrics , Abdominal Neoplasms
3.
Rev. chil. pediatr ; 90(3): 321-327, jun. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1013840

ABSTRACT

Resumen: Introducción: Las masas suprarrenales en recién nacidos son infrecuentes. El diagnóstico diferen cial incluye masas benignas (hemorragia suprarrenal o secuestro pulmonar extralobar) y malignas (neuroblastoma), y pueden ser un hallazgo durante la ecografía obstétrica. El uso de imágenes com plementarias en el periodo postnatal permite una mejor aproximación diagnóstica, con implicancias en el manejo de estos pacientes. Objetivos: comunicar el caso de una recién nacida portadora de una masa suprarrenal, discutir los diagnósticos diferenciales y el manejo de lesiones suprarrenales en recién nacidos. Caso Clínico: Lactante de 2 meses de edad, derivada para estudio de tumor supra rrenal de diagnóstico antenatal a las 22 semanas de edad gestacional. El estudio imagenológico con ecografía postnatal mostró un tumor compatible con neuroblastoma. Paciente asintomática, estudios de laboratorios sin hallazgos relevantes. Se realizó resección laparoscópica de la lesión. El estudio histológico confirmó un secuestro pulmonar. Conclusión: El secuestro pulmonar extralobar debe ser considerado en el diagnóstico diferencial de una masa suprarenal del recién nacido. La cirugía mínimamente invasiva debiera considerarse como el abordaje de elección en casos como este, donde existe factibilidad técnica y beneficios en la recuperación y secuelas cosméticas del paciente.


Abstract: Introduction: Adrenal masses are uncommon in newborns. The differential diagnosis includes be nign masses (adrenal hemorrhage, extralobar pulmonary sequestration) and malignant ones (neuro blastoma) that may be a finding during an obstetric ultrasound. The use of complementary imaging methods allows a better diagnosis approach during the postnatal period, with implications for the management of these patients. Objective: To report the case of a female newborn with diagnosis of an adrenal mass, and to discuss differential diagnoses and management alternatives of adrenal lesions in newborns. Case report: Two-month-old female infant, referred for adrenal tumor study diagnosed at 22 weeks gestational age. Postnatal ultrasound showed a tumor compatible with neuroblastoma. The patient was asymptomatic, and the laboratory studies showed no relevant findings. The lesion was excised by laparoscopy. A histological study confirmed pulmonary sequestration. Conclusions: Extralobar pulmonary sequestration should be considered in the differential diagnosis of an adrenal mass in the newborn. Minimally invasive surgery should be the preferred surgical technique choice in these cases, given the technical feasibility and benefits in the recovery and cosmetic issues of the patient.


Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Infant , Bronchopulmonary Sequestration/diagnosis , Adrenal Gland Neoplasms/diagnostic imaging , Neuroblastoma/diagnostic imaging , Ultrasonography, Prenatal , Ultrasonography , Laparoscopy/methods , Adrenal Gland Neoplasms/surgery , Minimally Invasive Surgical Procedures , Diagnosis, Differential , Neuroblastoma/surgery
4.
São Paulo med. j ; 137(1): 104-106, Jan.-Feb. 2019. tab, graf
Article in English | LILACS | ID: biblio-1004749

ABSTRACT

ABSTRACT CONTEXT: Neuroblastoma is the most common extracranial malignant solid tumor that occurs during childhood. It arises from primitive cells and is seen in the adrenal medulla and sympathetic ganglia of the sympathetic nervous system. CASE REPORT: We present a rare case of a 40-year-old man who was diagnosed with the onset of neuroblastoma arising in the mediastinum. He was treated by means of surgical resection in the superior mediastinum after neoadjuvant chemotherapy. The patient's surgical outcome was satisfactory. CONCLUSION: There are still no standard treatment guidelines for adult neuroblastoma patients. Although they have a poor prognosis, the main treatment option should be complete surgery at an early stage. This situation may become clarified through biological and genetic studies in the future.


Subject(s)
Humans , Male , Adult , Mediastinal Neoplasms/surgery , Neuroblastoma/surgery , Tomography, X-Ray Computed , Treatment Outcome , Chemoradiotherapy, Adjuvant
5.
ABCD (São Paulo, Impr.) ; 30(2): 88-92, Apr.-June 2017. tab, graf
Article in English | LILACS | ID: biblio-885719

ABSTRACT

ABSTRACT Background: The treatment of neuroblastoma is dependent on exquisite staging; is performed postoperatively and is dependent on the surgeon's expertise. The use of risk factors through imaging on diagnosis appears as predictive of resectability, complications and homogeneity in staging. Aim: To evaluate the traditional resectability criteria with the risk factors for resectability, through the radiological images, in two moments: on diagnosis and in pre-surgical phase. Were analyzed the resectability, surgical complications and relapse rate. Methods: Retrospective study of 27 children with abdominal and pelvic neuroblastoma stage 3 and 4, with tomography and/or resonance on the diagnosis and pre-surgical, identifying the presence of risk factors. Results: The mean age of the children was 2.5 years at diagnosis, where 55.6% were older than 18 months, 51.9% were girls and 66.7% were in stage 4. There was concordance on resectability of the tumor by both methods (INSS and IDRFs) at both moments of the evaluation, at diagnosis (p=0.007) and post-chemotherapy (p=0.019); In this way, all resectable patients by IDRFs in the post-chemotherapy had complete resection, and the unresectable ones, 87.5% incomplete. There was remission in 77.8%, 18.5% relapsed and 33.3% died. Conclusions: Resectability was similar in both methods at both pre-surgical and preoperative chemotherapy; preoperative chemotherapy increased resectability and decreased number of risk factors, where the presence of at least one IDRF was associated with incomplete resections and surgical complications; relapses were irrelevant.


RESUMO Racional: O tratamento do neuroblastoma é dependente de estadiamento primoroso, realizado no pós-cirúrgico e dependente da expertise do cirurgião. O uso de fatores de risco através da imagem ao diagnóstico surge como preditivo de ressecabilidade, complicações e homogeneidade no estadiamento. Objetivos: Avaliar o critério de ressecabilidade tradicional com os fatores de risco para ressecabilidade, através das imagens radiológicas, em dois momentos no diagnóstico e no pré-cirúrgico analisando a ressecabilidade, complicações cirúrgicas e índice de recidiva. Métodos: Estudo retrospectivo em 27 crianças com neuroblastoma estádios 3 e 4 em abdome e pelve, e com tomografia e/ou ressonância no diagnóstico e pré-cirúrgico, identificando-se a presença de fatores de risco. Resultados: A idade média das crianças foi de 2,5 anos ao diagnóstico, onde 55,6% estavam acima dos 18 meses, 51,9% eram meninas e 66,7% tinham estádio 4. Houve concordância da ressecabilidade do tumor pelos dois métodos avaliados (INSS e IDRFs) e em ambos os momentos da avaliação, ao diagnóstico (p=0,007) e pós-quimioterapia (p=0,019). Desta forma todos pacientes ressecáveis por IDRFs no pós-quimioterapia tiveram ressecção completa; já nos irressecáveis, 87,5% tiveram ressecção incompleta. Houve remissão em 77,8%, 18,5% recaíram e 33,3% morreram. Conclusões: Aressecabilidade foi semelhante em ambos os métodos tanto no diagnóstico como no pré-cirúrgico. A quimioterapia pré-operatória aumentou a ressecabilidade e diminuição do número de fatores de risco, onde a presença de ao menos um IDRF associou-se às ressecções incompletas e complicações cirúrgicas. As recidivas foram irrelevantes.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Abdominal Neoplasms/surgery , Abdominal Neoplasms/diagnostic imaging , Neuroblastoma/surgery , Neuroblastoma/diagnostic imaging , Postoperative Complications/etiology , Prognosis , Retrospective Studies , Risk Factors , Risk Assessment , Neoplasm Recurrence, Local/diagnostic imaging
6.
Rev. bras. anestesiol ; 67(2): 210-213, Mar.-Apr. 2017. tab, graf
Article in English | LILACS | ID: biblio-843373

ABSTRACT

Abstract Neuroblastoma is the most common, non-central nervous system tumor of childhood. It has the potential to synthesize catecholamines. However, the presences of hypertension are uncommon. We report the perioperative management of a 15-month-old infant with giant abdominal neuroblastoma who presented severe hypertension. The pathophysiological alterations of neuroblastoma are reviewed and perioperative management presented.


Resumo Neuroblastoma é o tumor mais comum do sistema nervoso não central na infância. Esse tumor tem o potencial de sintetizar catecolaminas; entretanto, a presença de hipertensão é rara. Relatamos o manejo perioperatório de uma criança de cinco meses com neuroblastoma abdominal gigante que apresentou hipertensão grave. As alterações fisiopatológicas do neuroblastoma foram revistas e o manejo perioperatório é apresentado.


Subject(s)
Humans , Male , Infant , Anesthesia , Abdominal Neoplasms/surgery , Abdominal Neoplasms/pathology , Neuroblastoma/surgery , Neuroblastoma/pathology
7.
Arch. argent. pediatr ; 114(5): e343-e345, oct. 2016. ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-838279

ABSTRACT

La Rothia mucilaginosa forma parte de la flora normal y no suele causar una enfermedad invasiva. Los pacientes inmunodeprimidos tienen mayor riesgo de infección grave. En este artículo, presentamos el caso de un paciente con neuroblastoma recidivante hospitalizado por neumonía. Después de la mejoría clínica, los síntomas respiratorios del paciente empeoraron de nuevo. En el hemocultivo, se aisló la bacteria Rothia mucilaginosa. El empeoramiento de los síntomas respiratorios puede explicarse por la diseminación hematopoyética de la bacteria. Se le administró tratamiento con meropenem y vancomicina durante 14 días, cuyos resultados fueron satisfactorios. Se sabe que esta bacteria poco frecuente tiene una alta tasa de mortalidad si no se trata de forma adecuada, y debe considerarse especialmente en los pacientes inmunodeprimidos con neoplasias malignas.


Rothia muciloginosa is a member of normal flora and rarely causes invasive disease. Immunosupressed patients have increased risk for severe infection. Here, we report a male patient with relapsed neuroblastoma hospitalized for pneumonia. After clinical improvement, patient's respiratory symptoms worsened again. Rothia muciloginosa was isolated from blood culture. The worsening of respiratory symptoms can be explained by hematogenous spread of bacteria. He was successfully treated with meropenem and vancomycin for 14 days. This rarely seen bacterium is known to have high mortality rates unless treated appropriately and should be considered especially in patients with malignancy due to their immunsupressed situation.


Subject(s)
Humans , Male , Child, Preschool , Postoperative Complications/microbiology , Actinomycetales Infections/complications , Bacteremia/microbiology , Pneumonia, Bacterial/complications , Hematopoietic Stem Cell Transplantation , Micrococcaceae , Neuroblastoma/surgery
8.
Rev. medica electron ; 35(6): 622-627, nov.-dic. 2013.
Article in Spanish | LILACS | ID: lil-696706

ABSTRACT

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.


Subject(s)
Humans , Female , Infant, Newborn , Head and Neck Neoplasms , Neuroblastoma/surgery , Neuroblastoma/diagnosis , Case Reports
9.
Rev. chil. pediatr ; 83(2): 170-174, abr. 2012. ilus
Article in Spanish | LILACS | ID: lil-639753

ABSTRACT

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.


Subject(s)
Humans , Female , Adolescent , Thoracic Neoplasms/surgery , Neuroblastoma/surgery , Thoracic Neoplasms/diagnosis , Thoracic Neoplasms/drug therapy , Thoracic Neoplasms/radiotherapy , Neuroblastoma/diagnosis , Neuroblastoma/drug therapy , Neuroblastoma/radiotherapy , Treatment Outcome
10.
Rev. cuba. med ; 50(1): 101-106, ene.-mar. 2011.
Article in Spanish | LILACS | ID: lil-584810

ABSTRACT

Se presentó un caso raro de ganglioneuroblastoma sacro en un paciente de 32 años de edad, con evolución tórpida. Se presentan datos clínicos, estudios imaginológicos e histológicos. Se realiza revisión del tema


This is a uncommon case of sacral ganglioneuroblastoma in a patient aged 32 with a torpid evolution. Clinical data, imaging and histological studies are showed. A review of this subject is carried out


Subject(s)
Humans , Ganglioneuroblastoma/pathology , Abdominal Neoplasms , Neuroblastoma/surgery , Sacrum/physiopathology , Tomography/methods
12.
Cir. & cir ; 77(5): 397-401, sept.-oct. 2009. ilus, tab
Article in Spanish | LILACS | ID: lil-566467

ABSTRACT

Introducción: El neuroblastoma es una neoplasia común en la infancia, pero extremadamente rara en el adulto. Se origina del sistema nervioso simpático y su localización más común es abdominal. Su estadificación y tratamiento se han estandarizado en niños y adultos, aunque el pronóstico es muy distinto debido a un comportamiento más agresivo y menor sobrevida en los segundos. Caso clínico: Hombre de 31 años de edad evaluado por dolor abdominal inespecífico y constipación, a quien se le diagnosticó gran neuroblastoma retroperitoneal estadio III. Al no tolerar quimioterapia se realizó cirugía. Se presenta la evaluación, manejo y seguimiento, así como una revisión de la literatura. Conclusiones: El neuroblastoma en el adulto es una enfermedad poco común que cursa con una evolución inicial insidiosa y la presentación suele ser en estadios avanzados. A diferencia del comportamiento en la infancia, en el adulto es más agresivo y con menor sobrevida a pesar de realizar el mismo tratamiento.


BACKGROUND: Neuroblastoma is a common malignancy in infancy but extremely rare in adults. These tumors, commonly found in the abdomen, originate in the sympathetic nervous system. Staging and management are standardized in children and adults, although their prognosis is very different, being more aggressive and with a poorer outcome in the adult. CLINICAL CASE: We present the case of a 31-year-old male with non-specific abdominal pain and constipation. After several studies, a stage III giant retroperitoneal neuroblastoma was diagnosed. We discuss here the evaluation, management and follow-up of the patient. A literature review is presented as well. CONCLUSIONS: Adult neuroblastoma is an unusual disease with an insidious presentation and is usually diagnosed in advanced stages. Unlike its behavior in young patients, in the adult it is more aggressive and with a poor prognosis.


Subject(s)
Humans , Male , Adult , Retroperitoneal Neoplasms/diagnosis , Neuroblastoma/diagnosis , Age of Onset , Combined Modality Therapy , Constipation/etiology , Abdominal Pain/etiology , Imaging, Three-Dimensional , Neoplasm Staging , Retroperitoneal Neoplasms/drug therapy , Retroperitoneal Neoplasms/pathology , Retroperitoneal Neoplasms , Retroperitoneal Neoplasms/surgery , Neuroblastoma/complications , Neuroblastoma/drug therapy , Neuroblastoma/pathology , Neuroblastoma , Neuroblastoma/surgery , Prognosis , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Remission Induction , Severity of Illness Index , Tomography, X-Ray Computed
13.
Neurosciences. 2008; 13 (1): 49-52
in English | IMEMR | ID: emr-89190

ABSTRACT

To assess the outcome of children older than one year with neuroblastoma treated at King Abdul-Aziz Medical City, Jeddah, Kingdom of Saudi Arabia. We retrospectively reviewed the files of 52 children older than one year with neuroblastoma [NBL] treated at our center between September 1987 and May 2003. Treatment consisted of OPEC chemotherapy regimen [vincristine, cisplatin, etoposide, and cyclophosphamide] or alternating OPEC/OJEC [carboplatin in place of cisplatin], surgical resection +/- radiotherapy [RT]. No patient received high dose therapy [HDT]. Thirty-four patients [65%] were stage 4, 12 [23%] stage 3, and 6 [11%] stage 2. Three stage 2 patients were treated with surgery only, all are alive in complete remission [CR]. All stage 3 and 4 patients were treated with chemotherapy and surgery +/- RT. After induction chemotherapy, CR was achieved in 17 patients [32%] and partial remission in 10 [19%]. Complete surgical resection was possible in 11 patients [22%]. Disease recurrence or progression occurred in 27 patients [51%]. With a median follow-up of 24 months [range 4-120], the 2-year event free survival was 10%, 82%, and 87% and the overall survival was 12%, 83%, and 100% for stage 4, 3, and 2. Children older than one year with localized NBL have good prognosis compared to those with stage 4. The use of HDT may improve the outcome in the latter group. Toxicity was significant, and adoption of risk-stratified treatment may help to reduce treatment complications


Subject(s)
Humans , Male , Female , Neuroblastoma/mortality , Neuroblastoma/surgery , Prognosis , Survival Analysis , Treatment Outcome , Neoplasm Staging , Recurrence , Age Factors , Clinical Protocols , Retrospective Studies
14.
Sohag Medical Journal. 2007; 11 (2): 110-127
in English | IMEMR | ID: emr-124186

ABSTRACT

Neuroblastoma is the most common intra-abdominal malignancy of infancy and the most common extra cranial solid tumor of childhood. Compared to any other tumour, this, tumour has varied clinical presentation and has great differences in distribution and respond to therapy. Although there have undoubtedly been major advances in therapy over recent decades, there is still room for significant improvements in outcome to be made. To review the clinical behaviors, management options and outcome of pediatric retroperitoneal neuroblastoma, treated at Sohag university hospital and to highlight on some prognostic factors affecting the outcome. Thirteen children with abdominal neuroblastoma were diagnosed and treated between April 1997 and April 2004 at surgery department of Sohag university Hospital. The data included the age, sex, clinical presentation, duration of symptoms, primary anatomic site, size, stage of the tumour, metastatic site if present, histopathology, modalities of treatment and prognostic factors. Patients were stratified as stage 1, 2A, 2B, 3, 4, 4S according to the post surgical International Neuroblastoma Staging System [INSS]. Thirteen patients were encountered. There were seven males and six females. Their ages ranged from 9 months to 5 years. The mean age was 1.8 years. Clinical presentation included painless abdominal swelling in7patients [54%], abdominal pain in 5 [38%], vomiting in 5 [38%], fever in 4 [30%], diarrhea in 2 [15%] and loss of weight in 2 [15%]. Duration of symptoms was from 2 weeks to 8 months, the mean duration was 1 1.5 weeks. Primary sites of involvement included: general retroperitoneal space in 8 patients [62%], the pelvis in 3[23%] and adrenal in 2 patients [15%], site of metastasis was the liver in 3, bone marrow in 4, cortical bone in 2 and distant lymph nodes in 3 patients. According to [INSS], stage 2B in 3, stage 3in 3, stage 4in 3 and stage 4s in 4 patients. Pathological features showed: neuroblastoma in 9 patients [70%], gangiloneuroblastoma in 3 [23%] and Ganglloneuroma in one patient [7%]. All cases were subjected to surgery, where complete excision was done in 7, total nephrectomy in 2. partial exision in 2 and only biopsy in 2 patients. All patients were subjected to postoperative chemotherapy. 6 patients to postoperative radiotherapy. Overall survival rates at 3 years at different stages were as followed: Stage 2: 100%; Stage 3: 33.3%; Stage 4: 0%: and Stage 4S: 100%. The overall survival rate during 3years follow up was 61.5% [5 out of 13 patients] died. Mortality rate is 38.5%. The tumour growth rates of neuroblastoma in age < two years are quicker than in age > two years. Wide local excision with retroperitoneal lymphadenectomy is the treatment of choice in operable cases. Neuroblastoma in pediatrics age group patients is more sensitive to chemotherapy than in older children. Factors of favorable prognosis are infants and children less than two years, stage 1, 2, and .48, the tumour site at the pelvis or retroperitoneal, low level of N.S.E and favorable histology


Subject(s)
Humans , Male , Female , Retroperitoneal Neoplasms , Child , Neuroblastoma/surgery , Neuroblastoma/radiotherapy , Chemotherapy, Adjuvant , Prognosis , Follow-Up Studies , Survival Rate
15.
Pediatría (Santiago de Chile) ; 2(2)ago. 2005. tab, graf
Article in Spanish | LILACS | ID: lil-416698

ABSTRACT

El Neuroblastoma es uno de los tumores sólidos malignos más frecuentes en niños y puede aparecer en cualquiera de los sitios anatómicos a lo largo de la cadena ganglionar simpática, así como en la glándula suprarrenal. A diferencia de otros tumores embrionarios de niños, el pronóstico en este tipo de tumor sigue siendo incierto y está influido por una gran variedad de factores. El peor pronóstico de sobrevida se observa en estadio IV y en presencia de metástasis óseas corticales. El sitio de tumor primario también podría ser considerado predictivo de sobrevida, según algunos investigadores. Objetivo: determinar si existe relación entre la forma inicial de presentación clínica de aquellos pacientes en etapa IV con compromiso óseo y las posibilidades de tratamiento quirúrgico primario. Además, determinar la relación entre la localización del tumor primario y el pronóstico. Material y métodos: se realizó una revisión retrospectiva de 22 fichas de pacientes con neuroblastoma tratados en el Hospital Roberto del Río entre 1994 – 2004. Los datos se analizaron estadísticamente mediante pruebas Fisher- Irwing. Resultados: en 22 pacientes estudiados, la forma inicial de presentación clínica más frecuente fue la de aquellos tumores abdominales y pélvicos, seguida por tumores mediastínicos. La mayoría de los pacientes, al momento del diagnóstico, se encontraban en etapa I o en etapa IV (45 por ciento de los casos para cada etapa). De los casos que se diagnosticaron en la etapa I, la mayoría de los neuroblastomas se hallaban ubicados en mediastino. De los casos que se diagnosticaron que se encontraban en etapa IV, la mayoría tenían localización abdominal. Como tratamiento inicial a la mayoría de los casos, 36 por ciento (8), se le efectuó una biopsia del tumor; con similar frecuencia se realizó resección total del tumor como primer tratamiento (32 por ciento); en 4 casos se decidió comenzar tratamiento con quimioterapia y a 3 casos se le practicó resección parcial del tumor en primera instancia. Conclusiones: no se encontró relación estadísticamente significativa (p=>0.05) entre aquellos casos que tenían neuroblastoma en etapa IV con metástasis óseas corticales y efectuar la resección primaria del tumor.


Subject(s)
Humans , Child , Neuroblastoma , Neuroblastoma/surgery , Neuroblastoma/diagnosis , Neuroblastoma/therapy , Chile
16.
Bol. Asoc. Méd. P. R ; 93(1/12): 23-25, Jan.-Dec. 2001.
Article in English | LILACS | ID: lil-411252

ABSTRACT

Presacral location of neuroblastoma is rare. Resection entails an abdomino-sacral approach. This case report discusses clinical, imaging and successful surgical management of a presacral neuroblastoma in a one-year-old male child


Subject(s)
Male , Infant , Humans , Neuroblastoma , Pelvic Neoplasms , Age Factors , Magnetic Resonance Imaging , Pelvic Neoplasms/diagnosis , Pelvic Neoplasms/surgery , Pelvic Neoplasms , Neuroblastoma/diagnosis , Neuroblastoma/surgery , Neuroblastoma , Prognosis , Sacrococcygeal Region
18.
Rev. cir. infant ; 9(4): 197-204, dic. 1999. graf
Article in Spanish | LILACS | ID: lil-278400

ABSTRACT

Fueron evaluados 79 casos de neuroblastoma tratados en un período de 15 años,con los siguientes objetivos:determinar el papel de la cirugía en el manejo del neuroblastoma,sus complicaciones y la influencia de los factores histológicos en el pronóstico del tumor.En estos pacientes hubo predominio del sexo masculino(56,9 por ciento)del grupo étnico blanco(1,7:1)de localización abdominal(70,9 por ciento) y de estadios avanzados(81 por ciento)El tiempo de sobrevida medio fue de 30 meses y ocurrieron 40 fallecimientos(50,6 por ciento),Parece existir una correspondencia entre la evaluación histológica del tumor, la chance de obtener una resección quirúrgica completa y el pronóstico aunque el número de pacientes con revisión histológica no fue suficiente en términos de significancia estadística


Subject(s)
Child , General Surgery , Drug Therapy , Neuroblastoma/surgery , Pediatrics
19.
Rev. cir. infant ; 9(3): 176-8, sept. 1999. ilus
Article in Spanish | LILACS | ID: lil-256557

ABSTRACT

El neuroblatoma IVs es en general un tumor de buen pronóstico.Existe un grupo de pacientes(10-25 por ciento)con esta enfermedad que tienen mal pronóstico debido a que progresan a un estadio IV a que asocian a hepatomegalia masiva.Presentamos la táctica quirúrgica seguida en un niño con neuroblatoma IVs asociado a hepatomegalia masiva


Subject(s)
Infant, Newborn , Hepatomegaly , Neuroblastoma/surgery , Pediatrics
20.
Pediatr. mod ; 35(8): 624-7, ago. 1999. ilus, tab
Article in Portuguese | LILACS | ID: lil-254967

ABSTRACT

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


Subject(s)
Humans , Male , Infant , Neuroblastoma/surgery , Neuroblastoma/diagnosis , Neuroblastoma/etiology , Neuroblastoma/drug therapy , Neuroblastoma/radiotherapy , Neuroblastoma/epidemiology , Neoplasm Staging , Neoplasm Metastasis , Prognosis
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