RESUMEN
BACKGROUND: Warm-antibody AIHA is known to complicate solid organ (SOT) and HSCT, the disease maybe refractory to standard therapy. Immunosuppressive therapies as well as IVIG, and rituximab have been the main stay of treatment. Over the past decade, B-lymphocyte targeted, anti-CD-20 antibody has been recognized in the treatment of autoimmune diseases and utilized in AIHA. Bortezomib, a proteasome inhibitor that causes apoptosis of plasma cells, is an appealing targeted therapy in secondary AIHA and has demonstrated efficacy in HSCT patients. From our experience, we advocate for early targeted therapy that combines B cell with plasma cell depletion. CASE REPORT: We describe a 4-year-old-girl with stage III neuroblastoma, complicated with intestinal necrosis needing multivisceral transplant developed warm AIHA 1-year after transplantation, and following an adenovirus infection. She received immunoglobulin therapy, rituximab, sirolimus, plasmapheresis, and long-term prednisolone with no sustained benefit while developing spinal fractures related to the latter therapy. She received bortezomib for intractable AIHA in combination with rituximab with no appreciable adverse effects. Three years later the child remains in remission with normal reticulocyte and recovered B cells. In the interim, she required chelation therapy for iron overload related to blood transfusion requirement during the treatment of AIHA. CONCLUSION: We propose early targeted anti-plasma cell therapy with steroid burst, IVIG, rituximab, and possible plasmapheresis may reduce morbidity in secondary refractory w-AIHA.
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Anemia Hemolítica Autoinmune/terapia , Neuroblastoma/cirugía , Complicaciones Posoperatorias/terapia , Vísceras/trasplante , Antineoplásicos/uso terapéutico , Bortezomib/uso terapéutico , Preescolar , Terapia Combinada , Femenino , Humanos , Terapia de Inmunosupresión/métodos , Necrosis , Neuroblastoma/patología , Plasmaféresis , Rituximab/uso terapéuticoRESUMEN
OBJECTIVE: Children are at high risk of injuries and wounds. The application of medical grade honey is a promising approach to improving the healing of wounds of various origin and severity. However, the use of medical grade honey in young paediatric patients remains limited. The aim of this study is to show the safety, efficacy and usefulness of medical grade honey in abdominal wounds, of different causes, in paediatric patients. METHOD: This was a prospective, observational case series evaluating five young infants with abdominal wounds at the General Hospital in Thessaloniki. All wounds were treated in the same manner with daily medical grade honey applied to the wound area and closely monitored. RESULTS: All treated wounds rapidly presented granulation tissue formation and underwent re-epithelialisation. Peripheral oedema and inflammation decreased upon initial application. Necrotic tissue was effectively debrided when present. Slough was removed and no signs of infection were detected, irrespective of initial wound presentations. Scar formation was minimal and the full range of motion was preserved in all cases. CONCLUSION: Based on this case study, medical grade honey is safe and effective in treating different abdominal wounds, including infected or dehisced wounds as well as burns. The easy application and broad applicability make medical grade honey recommendable as a first-line treatment in paediatric patients.
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Traumatismos Abdominales/terapia , Apiterapia/métodos , Quemaduras/terapia , Miel , Repitelización , Dehiscencia de la Herida Operatoria/terapia , Infección de la Herida Quirúrgica/terapia , Apendicectomía , Apendicitis/cirugía , Ácido Ascórbico/uso terapéutico , Infecciones por Bacteroides/terapia , Quemaduras Químicas/terapia , Niño , Preescolar , Fármacos Dermatológicos/uso terapéutico , Farmacorresistencia Bacteriana Múltiple , Edema , Femenino , Gastrostomía , Grecia , Humanos , Lactante , Recién Nacido , Inflamación , Infecciones por Klebsiella/terapia , Lanolina/uso terapéutico , Masculino , Neuroblastoma/cirugía , Aceites Volátiles/uso terapéutico , Pomadas , Estudios Prospectivos , Neoplasias Retroperitoneales/cirugía , Vitamina E/uso terapéutico , Vitaminas/uso terapéutico , Óxido de Zinc/uso terapéuticoRESUMEN
BACKGROUND: Pediatric patients with cancer undergo repeated painful procedures, including bone marrow aspirations and biopsies (BMABs). Optimal management of procedure-related pain can reduce discomfort, anxiety, and distress. METHODS: Children with neuroblastoma were randomly assigned to 1 of 2 arms on a prospective, single-blind, crossover trial conducted at Memorial Sloan Kettering Cancer Center from October 2016 to January 2018 (www.clinicaltrials.gov, identifier NCT02924324). Participants underwent 2 sequential BMABs: one with general anesthesia (GA) alone, the other with GA plus local anesthesia (LA) (GA + LA). The objective was to assess procedure-related pain and its interference with quality of life (QoL) with GA versus GA + LA. Primary outcome was percentage of participants requiring postprocedural opioids. Secondary outcomes were total opioid and nonopioid analgesics, pain scores, time to first analgesic, QoL, and toxicity. Management of postprocedural pain was standardized. RESULTS: Of 56 participants randomly assigned (3-16.5 years old), 46 completed both procedures. There was no significant difference in percentage of participants requiring opioids with GA versus GA + LA (24% vs 20%, P = .5). Pain scores in the recovery room were significantly lower for GA + LA versus GA (median [IQR]: 0 [0-2] vs 2 [0-4], P = .002). There were no statistically significant differences in total opioid or nonopioid analgesic, 6- and 24-hour pain scores, median time to first analgesic, or pain interference. No adverse events occurred. CONCLUSIONS: LA was associated with significant improvement in pain scores in the immediate recovery period. LA did not reduce postprocedural opioid use, nor did it improve QoL for patients undergoing BMAB with GA.
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Anestesia General/métodos , Anestesia Local/métodos , Médula Ósea/patología , Neuroblastoma/diagnóstico , Neuroblastoma/cirugía , Dolor Postoperatorio/prevención & control , Biopsia/efectos adversos , Biopsia/tendencias , Niño , Preescolar , Estudios Cruzados , Femenino , Humanos , Masculino , Dolor Postoperatorio/etiología , Estudios Prospectivos , Método Simple CiegoRESUMEN
BACKGROUND: In high-risk neuroblastoma patients, response to induction chemotherapy is emerging as an important determinant of overall survival. We sought to determine whether histological changes in the primary tumor following induction therapy could be used as a marker of response. PROCEDURE: Second-look primary tumor specimens from 43 patients were reviewed according to specific morphological features. RESULTS: In the majority, induction therapy resulted in a shift from an intermediate/high to low mitosis-karyorrhexis index (MKI) (P = 0.0009) and from undifferentiated/poorly differentiated to differentiating tumors (P < 0.0001). Following induction therapy, persistence of intermediate/high tumor MKI and ≥90% persistent neuroblastic cells were predictive of a poor outcome (P = 0.001 and 0.03, respectively). Less than 10% tumor necrosis was associated with a trend towards lower survival. CONCLUSIONS: High proliferative activity in the primary tumor following induction therapy portends a poor outcome in patients with high-risk neuroblastoma. If confirmed in a larger cohort, tumor histology at second-look surgery could be used to define a subset of very high risk patients who would benefit from alternative therapies prior to myeloablative dose-intensive transplant.
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Neuroblastoma/tratamiento farmacológico , Neuroblastoma/patología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Humanos , Quimioterapia de Inducción , Lactante , Recién Nacido , Masculino , Índice Mitótico , Terapia Neoadyuvante , Neuroblastoma/cirugía , Pronóstico , Riesgo , Segunda Cirugía , Resultado del TratamientoRESUMEN
Patients undergoing auto-SCT for neuroblastoma present a unique population to study transplant-associated thrombotic microangiopathy (TA-TMA), due to standardized chemotherapy and later exposure to radiation and cis-retinoic acid (cis-RA). We retrospectively analyzed 20 patients after auto-SCT to evaluate early clinical indicators of TA-TMA. A total of 6 patients developing TA-TMA (30% prevalence) were compared with 14 controls. Four of six patients were diagnosed with TA-TMA by 25 days after auto-SCT. Compared with controls, TA-TMA patients had higher average systolic and diastolic blood pressure levels during high-dose chemotherapy and developed hypertension by day 13 after auto-SCT. Proteinuria was a significant marker for TA-TMA, whereas blood and platelet transfusion requirements were not. Serum creatinine did not differ between groups post transplant. However, patients with TA-TMA had a 60% decrease in renal function from baseline by nuclear glomerular filtration rate, compared with a 25% decrease in those without TA-TMA (P=0.001). There was no TA-TMA-related mortality. Significant complications included end-stage renal disease (n=1) and polyserositis (n=3). Patients with TA-TMA were unable to complete cis-RA therapy after auto-SCT. We suggest that careful attention to blood pressure and urinalysis will assist in the early diagnosis of TA-TMA, whereas serum creatinine seems to be an insensitive marker for this condition.
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Trasplante de Células Madre Hematopoyéticas/efectos adversos , Neuroblastoma/cirugía , Microangiopatías Trombóticas/diagnóstico , Antihipertensivos/uso terapéutico , Presión Sanguínea , Estudios de Casos y Controles , Preescolar , Femenino , Humanos , Hipertensión/tratamiento farmacológico , Hipertensión/etiología , Masculino , Proteinuria/etiología , Estudios Retrospectivos , Microangiopatías Trombóticas/complicaciones , Microangiopatías Trombóticas/etiología , Acondicionamiento Pretrasplante , Trasplante AutólogoRESUMEN
Focal nodular hyperplasia (FNH) is a benign, poorly understood hepatic tumor that is rare in children. Although there is no evidence for malignant degeneration, FNH can occur adjacent to a malignancy. Here, the case of a 4-year-old boy with a hepatic mass and history of stage IV neuroblastoma is presented. Initial imaging and core-needle biopsy were consistent with FNH. However, after left lateral segmentectomy, pathologic examination revealed a malignant tumor most consistent with small cell undifferentiated hepatoblastoma as well as 3 foci of FNH in the surrounding parenchyma.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Hiperplasia Nodular Focal/etiología , Hepatoblastoma/diagnóstico , Neoplasias Hepáticas/diagnóstico , Neoplasias Primarias Secundarias/diagnóstico , Neuroblastoma , Neoplasias de las Glándulas Suprarrenales/tratamiento farmacológico , Neoplasias de las Glándulas Suprarrenales/radioterapia , Neoplasias de las Glándulas Suprarrenales/cirugía , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Monoclonales de Origen Murino , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Trasplante de Médula Ósea , Carboplatino/administración & dosificación , Preescolar , Cisplatino/administración & dosificación , Terapia Combinada , Ciclofosfamida/administración & dosificación , Etopósido/administración & dosificación , Fluorouracilo/administración & dosificación , Factor Estimulante de Colonias de Granulocitos/administración & dosificación , Hepatectomía , Hepatoblastoma/complicaciones , Hepatoblastoma/diagnóstico por imagen , Hepatoblastoma/tratamiento farmacológico , Hepatoblastoma/cirugía , Humanos , Ifosfamida/administración & dosificación , Inmunoglobulina G/uso terapéutico , Hallazgos Incidentales , Neoplasias Hepáticas/complicaciones , Neoplasias Hepáticas/diagnóstico por imagen , Neoplasias Hepáticas/tratamiento farmacológico , Neoplasias Hepáticas/cirugía , Masculino , Melfalán/administración & dosificación , Mesna/administración & dosificación , Neoplasias Inducidas por Radiación/etiología , Neoplasias Primarias Secundarias/complicaciones , Neoplasias Primarias Secundarias/patología , Neuroblastoma/tratamiento farmacológico , Neuroblastoma/radioterapia , Neuroblastoma/cirugía , Radioterapia Adyuvante/efectos adversos , Tomografía Computarizada por Rayos X , Vincristina/administración & dosificaciónRESUMEN
Intractable post-operative chylous ascites had been managed successfully using a peritoneovenous shunt (PVshunt). A 4-year-old girl with neuroblastoma originated from the right adrenal gland was admitted to our hospital. Following the preoperative chemotherapy, tumor resection, and lymph node dissection of the abdominal paraaortic region were carried out. Post-operative radiation therapy 9.6 gray to the tumor bed and to the paraaortic region and a high dose chemotherapy supported by auto bone marrow transplantation were completed. Three months later some enlarged lymph nodes along the duodeno-hepatic ligament were detected and these had gradually increased in size. Lymph node dissection along the hepatic artery and the abdominal aorta was carried out. Pathological examination of the specimen showed reactive lymph node swelling. Chylous ascites developed several days after surgery. Despite the medium-chain triglycerides meal or total parental nutrition, the ascites persisted for more than 80 days. Multiple paracenteses were mandatory. A PV shunt was implanted and the ascites was resolved by the fourth post-operative day. Thirty months later, the vascular end tube of the shunt was ligated. As ascites had not accumulated for 2 weeks, the PV shunt was removed. The patient has been doing well without recurrence of ascites or neuroblastoma for 12 years. As PV shunts were mostly used for long lasting disease, it has not been referred as to how to know when the shunt should be removed. If the shunt is inserted for transient management of ascites, less invasive methods of investigation to know when to remove the shunt need to be developed.
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Ascitis Quilosa/cirugía , Derivación Peritoneovenosa , Neoplasias de las Glándulas Suprarrenales/cirugía , Ascitis Quilosa/terapia , Femenino , Humanos , Ligadura , Neuroblastoma/cirugía , Paracentesis , Nutrición Parenteral TotalRESUMEN
OBJECTIVE: The aim of the paper was to improve the prognosis of neuroblastoma (NB) stage III and IV in children through the comprehensive therapy including chemotherapy, delayed tumor resection, autologous stem cell transplantation (ASCT) and inducing differentiation and to analyze the factors affecting the prognosis. METHODS: Newly diagnosed neuroblastoma patients seen from Oct.1998 to Dec.2003 were divided into high, medium and low risk groups depending on clinical stage and age. Comprehensive protocol included accurate staging, delayed and/or second tumor resection for stage III and IV patients, chemotherapy of different intensity mainly composed of cell cycle nonspecific drugs and 13-cis-retinoid for inducing cell differentiation. ASCT was given at the end of therapy for high risk group. RESULT: Forty-five patients, 6 months to 11 years of age, 32 males and 13 females, were analyzed. Of them, 15 were found to have the tumor in adrenal gland, 12 had the tumor extended to the retro-peritoneal space, while in 15 cases the tumor was beside the spinal column in chest and in 3 the tumor was located in other places. Nine cases had stage I, 1 case had stage II, 8 cases had III, 26 cases had stage IV and 1 case had stage IVs of the tumor. Depending on the age and stage of the tumor, 26 cases were aligned into high risk protocol, 10 into medium risk and 9 into low risk groups. Thirty nine cases were treated as planned. Eleven of them received ASCT including 2 cases who received second ASCT. Of the thirty-nine patients, 31 achieved complete remission (CR) and 8 partial remission (PR) after surgery and/or chemotherapy. During up to 21 months median following up period (range 14 to 64 months), 24 cases (62%) kept CR (median 22 months) and 4 survived with stable disease. The survival rate (SR) was 72%. Eleven cases died of relapse and disease progression. No death occurred from treatment complication. Statistical analysis showed that the age older than 18 months, and stage III and IV of the tumor were the factors predicting poor prognosis (P = 0.04 and 0.003, respectively). Patients who had the tumor originated from the retroperitoneal space, who had incomplete tumor resection, and those who did not receive ASCT had poorer prognosis, but the differences were not significant (P = 0.092, 0.55 and 0.60, respectively). CONCLUSION: The comprehensive protocol seemed to be reasonable. Age older than 18 months, and stage III and IV were the factors suggesting poor prognosis. The origin of the tumor, completeness of tumor resection, and use of ASCT had no significant impact on the prognosis.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Trasplante de Células Madre Hematopoyéticas , Neuroblastoma/diagnóstico , Neuroblastoma/terapia , Trasplante Autólogo , Factores de Edad , Quimioterapia Adyuvante , Niño , Preescolar , Femenino , Estudios de Seguimiento , Trasplante de Células Madre Hematopoyéticas/métodos , Humanos , Lactante , Masculino , Recurrencia Local de Neoplasia/prevención & control , Recurrencia Local de Neoplasia/cirugía , Recurrencia Local de Neoplasia/terapia , Estadificación de Neoplasias , Neuroblastoma/tratamiento farmacológico , Neuroblastoma/mortalidad , Neuroblastoma/patología , Neuroblastoma/cirugía , Pronóstico , Inducción de Remisión/métodos , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Factores de Tiempo , Trasplante Autólogo/métodos , Resultado del TratamientoRESUMEN
BACKGROUND/PURPOSE: Gross total resection of the primary tumor in treatment of high-risk neuroblastoma remains controversial. Furthermore, there are few reports of the effect of primary tumor resection on local control as opposed to overall survival. The authors reviewed their institutional experience to assess the effect of primary tumor resection on local control and overall survival. METHODS: A total of 141 patients were treated on protocol between November 1, 1979 and June 25, 2002 and are the subject of this report. Gross total resection was assessed by review of operative notes, postoperative computerized axial tomograms, and postoperative meta-iodobenzyl guanidine (MIBG)1 scans when available. RESULTS: The median age was 3.3 years, and all patients were International Neuroblastoma Staging System (INSS) stage 4 with 79% having metastases to cortical bone. The primary site was the adrenal gland in 74%, the central abdominal compartment in 13%, the posterior mediastinum in 7%, and other sites in 6%. Gross total resection was accomplished in 103 (73%) but was more than 90% for the last 3 protocols. Five kidneys were lost overall. The probability of local progression was 50% in unresected patients compared with 10% in patients undergoing gross total resection (P <.01). Overall survival rate in resected patients was 50% compared with 11% in unresected patients (P <.01). CONCLUSIONS: Our data indicate that local control and overall survival rate are correlated with gross total resection of the primary tumor in high-risk neuroblastoma. Gross total resection should be part of the management of stage 4 neuroblastoma in patients greater than 1 year of age.
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Neoplasias Abdominales/tratamiento farmacológico , Neoplasias Abdominales/cirugía , Neoplasias de las Glándulas Suprarrenales/tratamiento farmacológico , Neoplasias de las Glándulas Suprarrenales/cirugía , Neoplasias del Mediastino/tratamiento farmacológico , Neoplasias del Mediastino/cirugía , Neuroblastoma/tratamiento farmacológico , Neuroblastoma/cirugía , Neoplasias Abdominales/mortalidad , Neoplasias Abdominales/patología , Neoplasias Abdominales/radioterapia , Adolescente , Neoplasias de las Glándulas Suprarrenales/mortalidad , Neoplasias de las Glándulas Suprarrenales/patología , Neoplasias de las Glándulas Suprarrenales/radioterapia , Adulto , Análisis de Varianza , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Preescolar , Progresión de la Enfermedad , Humanos , Lactante , Radioisótopos de Yodo , Neoplasias del Mediastino/mortalidad , Neoplasias del Mediastino/patología , Neoplasias del Mediastino/radioterapia , Estadificación de Neoplasias , Neuroblastoma/mortalidad , Neuroblastoma/patología , Neuroblastoma/radioterapia , Modelos de Riesgos Proporcionales , Radioterapia Adyuvante , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
HISTORY AND CLINICAL FINDINGS: A 21-year-old patient was admitted to the hospital because of massively enlarged cervical lymph nodes. Additionally, a left-sided facial and brachial edema was visible. Auscultation of the left lung was remarkable for diminished breath sounds. EXAMINATIONS: Diagnostic imaging showed an extensive thoracic tumor and enlarged mediastinal and cervical lymph nodes. The diagnosis of neuroblastoma was established by biopsy. TREATMENT AND CLINICAL COURSE: The patient was treated with a polychemotherapy protocol according to the pediatric neuroblastoma study NB97. Subsequently, the patient underwent partial tumor resection, received two further chemotherapy courses and irradiation of the remaining tumor. Because of residual vital tumor cells, a second surgical tumour reduction followed by high-dose chemotherapy with autologous stem-cell support was performed. Two cycles of high-dose retinoic acid followed. Six months after the end of therapy, the patient is in a good condition despite of the presence of residual tumor. CONCLUSION: Neuroblastoma is a very rare tumor in adult patients. Therapy is multimodal and should follow pediatric guidelines for neuroblastoma treatment.
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Neuroblastoma/diagnóstico , Neuroblastoma/terapia , Neoplasias Torácicas/diagnóstico , Neoplasias Torácicas/terapia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Quimioterapia Adyuvante , Terapia Combinada , Diagnóstico Diferencial , Humanos , Imagen por Resonancia Magnética , Masculino , Neuroblastoma/cirugía , Radioterapia Adyuvante , Reoperación , Trasplante de Células Madre , Neoplasias Torácicas/cirugía , Trasplante Autólogo , Tretinoina/administración & dosificaciónRESUMEN
BACKGROUND: We reviewed the utility of different treatment modalities in a large series of adolescents/adults with neuroblastoma (NB). PROCEDURE: The 30 adolescents/adults (median age, 19 years) had stage 2B (n = 1), 3 (n = 2), or 4 (n = 27) NB. Treatments included conventional and myeloablative therapy; local radiotherapy (RT); immunotherapy with anti-G(D2) 3F8 monoclonal antibody +/- granulocyte-macrophage colony-stimulating factor (GM-CSF); and 3F8 alternating with low-dose oral etoposide. RESULTS: Seven patients are in first (n = 4) or second (n = 3) complete/very good partial remission (CR/VGPR) at 9+ to 181+ (median, 45+) months. Among 13 newly diagnosed or minimally prior-treated patients, no major responses were seen in 4/4 treated with N4/N5 chemotherapy, but 6/9 treated with the higher dose N6/N7 regimens and surgery had major responses, and immunotherapy produced CR in BM in three patients. Among 17 patients referred because of resistant NB, favorable responses occurred in 6/12 treated with high-dose cyclophosphamide-based salvage therapy, including one patient who is in CR 170+ months after myeloablative consolidation and five patients who achieved CR/VGPR after 3F8/GM-CSF (n = 4) or 3F8/oral etoposide (n = 1). With a median follow-up of 32+ months post-RT, no local relapses occurred in 10/10 patients who received hyperfractionated 21 Gy RT to prevent regrowth of soft tissue masses that had been resected. CONCLUSIONS: High-dose chemotherapy and surgery can achieve a minimal disease state in >50% of newly diagnosed older NB patients. In that setting, local RT, and the use of agents with recently confirmed anti-NB activity, including anti-G(D2) antibodies, and cis-retinoic acid, may improve the poor prognosis of these patients reported to date.
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Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neuroblastoma/tratamiento farmacológico , Neuroblastoma/cirugía , Adolescente , Adulto , Niño , Terapia Combinada , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Inmunoterapia , Masculino , Pronóstico , Terapia Recuperativa , Resultado del TratamientoRESUMEN
PURPOSE: To assess the effect of local radiation administered to primary disease sites in children with high-risk neuroblastoma. MATERIALS AND METHODS: A total of 539 eligible patients were entered on protocol CCG-3891, consisting of chemotherapy, primary surgery, and 10 Gy of external beam radiation therapy (EBRT) to gross residual disease, followed by randomized assignment to continuation chemotherapy (CC) or autologous bone marrow transplantation (ABMT). ABMT patients received total body irradiation (TBI). RESULTS: Estimated event-free survival and overall survival at 5 years were 25% +/- 2% and 35% +/- 2%, respectively. Estimated 5-year locoregional recurrence rates were 51% +/- 5% and 33% +/- 7% for CC and ABMT patients (p = 0.004). For patients who received 10 Gy of EBRT to the primary, the addition of 10 Gy of TBI and ABMT decreased local recurrence compared with CC (22% +/- 12% and 52% +/- 8%, p = 0.022). EBRT did not increase acute toxicity, except for increased total parenteral nutrition administration. CONCLUSIONS: In combination with EBRT to the primary tumor site, the addition of 10 Gy of TBI as a component of high-dose chemotherapy with ABMT improved local control compared with CC without TBI. Results suggest a dose-response relationship for local EBRT. Short-term toxicity of local EBRT is limited.
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Neuroblastoma/radioterapia , Radioterapia Adyuvante , Irradiación Corporal Total , Adolescente , Antineoplásicos/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Purgación de la Médula Ósea , Trasplante de Médula Ósea , Niño , Preescolar , Cisplatino/administración & dosificación , Terapia Combinada , Ciclofosfamida/administración & dosificación , Fraccionamiento de la Dosis de Radiación , Doxorrubicina/administración & dosificación , Enfermedades Hematológicas/inducido químicamente , Enfermedad Veno-Oclusiva Hepática/etiología , Humanos , Lactante , Isotretinoína/uso terapéutico , Tablas de Vida , Recurrencia Local de Neoplasia , Neoplasias Inducidas por Radiación/etiología , Neoplasias Primarias Secundarias/epidemiología , Neuroblastoma/tratamiento farmacológico , Neuroblastoma/cirugía , Neuroblastoma/terapia , Radioterapia Adyuvante/efectos adversos , Inducción de Remisión , Riesgo , Análisis de Supervivencia , Tasa de Supervivencia , Trasplante Autólogo , Irradiación Corporal Total/efectos adversosRESUMEN
BACKGROUND: The N7 protocol for poor-risk neuroblastoma uses dose-intensive chemotherapy (as in N6 protocol [Kushner et al.: J Clin Oncol 12:2607-2613, 1994] but with lower dosing of vincristine) for induction, surgical resection and 2100 cGy hyperfractionated radiotherapy for local control, and for consolidation, targeted radioimmunotherapy with 131I-labeled anti-GD2 3F8 monoclonal antibody and immunotherapy with unlabeled/unmodified 3F8 (400 mg/m2). PROCEDURE: The chemotherapy consists of: cyclophosphamide 70 mg/kg/d x 2 and a 72-hr infusion of doxorubicin 75 mg/m2 plus vincristine 2 mg/m2, for courses 1, 2, 4, and 6; and cisplatin 50 mg/m2/d x 4 and etoposide 200 mg/m2/d x 3, for courses 3, 5, and 7. 131I-3F8 is dosed at 20 mCi/kg, which is myeloablative and therefore necessitates stem-cell support. RESULTS: Of the first 24 consecutive previously untreated patients more than 1 year old at diagnosis, 22 were stage 4 and two were unresectable stage 3 with MYCN amplification. Chemotherapy achieved CR/VGPR in 21 of 24 patients. Twenty patients to date have completed treatment with 131I-3F8, and 15 patients have completed all treatment. With a median follow-up of 19 months, 18 of 24 patients remain progression-free. CONCLUSIONS: Major toxicities were grade 4 myelosuppression and mucositis during chemotherapy, and self-limited pain and urticaria during antibody treatment. Late effects include hearing deficits and hypothyroidism.
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Anticuerpos Monoclonales/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Inmunoconjugados/uso terapéutico , Inmunoglobulina G/uso terapéutico , Radioisótopos de Yodo/uso terapéutico , Neuroblastoma/terapia , Radioinmunoterapia , Anticuerpos Monoclonales de Origen Murino , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Biomarcadores de Tumor/sangre , Enfermedades de la Médula Ósea/inducido químicamente , Quimioterapia Adyuvante , Niño , Preescolar , Aberraciones Cromosómicas , Cisplatino/administración & dosificación , Cisplatino/efectos adversos , Terapia Combinada , Ciclofosfamida/administración & dosificación , Ciclofosfamida/efectos adversos , Supervivencia sin Enfermedad , Fraccionamiento de la Dosis de Radiación , Doxorrubicina/administración & dosificación , Doxorrubicina/efectos adversos , Etopósido/administración & dosificación , Etopósido/efectos adversos , Amplificación de Genes , Genes myc , Humanos , Hipotiroidismo/etiología , Inmunización Pasiva , Inmunoconjugados/efectos adversos , Radioisótopos de Yodo/efectos adversos , Proteínas de Neoplasias/sangre , Estadificación de Neoplasias , Neuroblastoma/tratamiento farmacológico , Neuroblastoma/mortalidad , Neuroblastoma/radioterapia , Neuroblastoma/cirugía , Radioinmunoterapia/efectos adversos , Radioterapia Adyuvante , Inducción de Remisión , Factores de Riesgo , Análisis de Supervivencia , Resultado del Tratamiento , Vincristina/administración & dosificación , Vincristina/efectos adversosRESUMEN
El neuroblastoma es el tumor maligno más frecuente del período neonatal. Su detección mediante ecografía obstétrica, permite realizar un diagnóstico y tratamiento precoz, lo que condicionará un mejor pronóstico. Presentamos un caso de neuroblastoma quístico suprarrenal, detectado mediante ecografía obstétrica en la 34 semana de gestación, así como su seguimiento postnatal (AU)
Asunto(s)
Adulto , Embarazo , Femenino , Humanos , Recién Nacido , Ultrasonografía Prenatal , Neuroblastoma , Neuroblastoma/embriología , Neoplasias de la Corteza Suprarrenal , Pronóstico Clínico Dinámico Homeopático , Pronóstico , Diagnóstico Diferencial , Neuroblastoma/cirugía , Neoplasias de la Corteza Suprarrenal/cirugíaRESUMEN
UNLABELLED: Adult's retroperitoneal tumours of nervous origin are infrequent clinical entities with characteristic evolution and treatment. We review 13 cases, dealt with in N. Gh. Lupu Surgical Clinic between 1975-1994 and representing 20% of the retroperitoneal tumour admitted in our clinic during the same period. Most of the patients were males (sex ratio 10:3). The main symptom was the abdominal pain (84.6% of all cases) and all the tumours were extremely big (15-30 cm diameter). The surgical approach always tried to remove the tumour, which succeeded, even if in 4 cases some of the surrounding organs had to be removed also. The two postoperative complications consisted in a severe wound infection and an acute pancreatitis; the second evolved poorly and the patient was lost. No complementary therapy was used. None of the five patients followed up for five years presented clinical evidence of recurrent tumour. CONCLUSIONS: Due to an unexplained evolutive tolerance, adult's retroperitoneal tumours of nervous origin reach large dimensions and have late clinical expression, as the complications occur. Preoperative imagistic findings supply valuable informations for the subsequent surgical treatment. The surgical approach must aim to the ablation of the tumour, with or without the sacrifice of other organs which the tumours can include. This aggressive surgical conduite is sustained by the satisfying postoperative results.
Asunto(s)
Ganglioneuroma/diagnóstico , Neurilemoma/diagnóstico , Neuroblastoma/diagnóstico , Neoplasias Retroperitoneales/diagnóstico , Dolor Abdominal/diagnóstico , Dolor Abdominal/etiología , Dolor Abdominal/cirugía , Adulto , Femenino , Ganglioneuroma/complicaciones , Ganglioneuroma/cirugía , Humanos , Complicaciones Intraoperatorias/epidemiología , Masculino , Persona de Mediana Edad , Neurilemoma/complicaciones , Neurilemoma/cirugía , Neuroblastoma/complicaciones , Neuroblastoma/cirugía , Complicaciones Posoperatorias/epidemiología , Neoplasias Retroperitoneales/complicaciones , Neoplasias Retroperitoneales/cirugíaRESUMEN
Pretreatment with [131I] metaiodobenzylguanidine (MIBG) followed by surgical resection in advanced neuroblastoma (stage 3 and 4) has been studied in relation to resectability, morbidity and mortality, survival rate after two years, control of distant metastasis and serum levels of LDH as prognostic factors. Twenty-one patients with advanced neuroblastoma were primarily treated with MIBG radiotherapy, followed by surgical resection. Sixteen patients had stage 4 disease. Between 2 and 6 courses of MIBG treatment were given per patient. In 17 patients gross complete resection was achieved. Two patients developed complications directly related to the operation, one died as a result of this. The overall mortality was 38%. MIBG therapy resulted in partial response in 13 patients and in stable disease in 8 patients. Two years survival in the group with partial response was 86% and in the group with stable disease 28%. Because of the resulting excellent general condition of the patients the interval between pretreatment with MIBG and surgery could be very short. Follow-up till December 1994 showed that 13 children were alive for 3 to 47 months. Seven had no evidence of disease. Preoperative MIBG de novo treatment in advanced neuroblastoma is equal to induction chemotherapy, but less toxic.
Asunto(s)
Neoplasias de las Glándulas Suprarrenales/radioterapia , Antineoplásicos/uso terapéutico , Radioisótopos de Yodo/uso terapéutico , Yodobencenos/uso terapéutico , Neuroblastoma/radioterapia , Neoplasias del Sistema Nervioso Periférico/radioterapia , 3-Yodobencilguanidina , Adolescente , Neoplasias de las Glándulas Suprarrenales/mortalidad , Neoplasias de las Glándulas Suprarrenales/patología , Neoplasias de las Glándulas Suprarrenales/cirugía , Niño , Preescolar , Terapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Estadificación de Neoplasias , Neuroblastoma/mortalidad , Neuroblastoma/patología , Neuroblastoma/cirugía , Neoplasias del Sistema Nervioso Periférico/mortalidad , Neoplasias del Sistema Nervioso Periférico/patología , Neoplasias del Sistema Nervioso Periférico/cirugía , Radioterapia Adyuvante , Tasa de Supervivencia , Sistema Nervioso Simpático/patología , Sistema Nervioso Simpático/efectos de la radiación , Sistema Nervioso Simpático/cirugíaRESUMEN
PURPOSE: To test the efficacy of a protocol for poor-risk neuroblastoma that builds on the following: (1) our favorable previously reported results with dose-intensive use of cyclophosphamide; (2) our retrospective analysis of neuroblastoma chemotherapy reports, which supported the value of high-dose cisplatin and etoposide (VP-16); and (3) the Goldie-Coldman hypothesis that rapid cytoreduction plus the use of non-cross-resistant chemotherapy combinations will decrease the risk of drug resistance. PATIENTS AND METHODS: The N6 protocol included seven courses of high-dose chemotherapy plus surgical resection of bulk disease. Courses 1, 2, 4, and 6 consisted of 6-hour intravenous infusions of cyclophosphamide 70 mg/kg/d on days 1 and 2 (ie, 140 mg/kg per course), a 72-hour intravenous infusion of doxorubicin 75 mg/m2 and vincristine 0.1 mg/kg beginning day 1, and vincristine 1.5 mg/m2 intravenous bolus on day 9. Courses 3, 5, and 7 consisted of 2-hour intravenous infusions of VP-16 200 mg/m2/d on days 1 to 3 (ie, 600 mg/m2 per course), and 1-hour intravenous infusions of cisplatin 50 mg/m2/d on days 1 to 4 (ie, 200 mg/m2 per course). Courses were to start after neutrophil counts reached 500/microL and platelet counts reached 100,000/microL. Response was defined by international criteria. RESULTS: Among 24 consecutive previously untreated patients diagnosed with stage 4 neuroblastoma at more than 1 year of age, 21 patients achieved a complete or very good partial remission; one patient had no evidence of disease except by iodine-131-metaiodobenzylguanidine (MIBG) scan, which was markedly improved; and one patient had resolution of extensive metastatic disease, but still had an incompletely resected primary tumor. The sole patient to have a poor response had clinical features at diagnosis that are atypical for neuroblastoma, namely, 8 years of age and an unknown primary tumor. Severe toxicities included myelosuppression, mucositis, and hearing deficits. CONCLUSION: The N6 approach reliably achieves significant cytoreduction against stage 4 neuroblastoma. This may eventuate in an improved cure rate, since consolidative treatments using myeloablative therapy, immunotherapy, or biologic response modifiers such as cis-retinoic acid are most likely to be effective against minimal residual disease.
Asunto(s)
Neoplasias Abdominales/tratamiento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neuroblastoma/tratamiento farmacológico , Neoplasias Torácicas/tratamiento farmacológico , Neoplasias Abdominales/patología , Neoplasias Abdominales/cirugía , Antibacterianos/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Niño , Preescolar , Cisplatino/administración & dosificación , Cisplatino/efectos adversos , Terapia Combinada , Ciclofosfamida/administración & dosificación , Ciclofosfamida/efectos adversos , Doxorrubicina/administración & dosificación , Doxorrubicina/efectos adversos , Etopósido/administración & dosificación , Etopósido/efectos adversos , Femenino , Factor Estimulante de Colonias de Granulocitos/administración & dosificación , Enfermedades Hematológicas/inducido químicamente , Enfermedades Hematológicas/terapia , Humanos , Infecciones/inducido químicamente , Infecciones/terapia , Masculino , Estadificación de Neoplasias , Neuroblastoma/secundario , Neuroblastoma/cirugía , Transfusión de Plaquetas , Inducción de Remisión/métodos , Estudios Retrospectivos , Neoplasias Torácicas/patología , Neoplasias Torácicas/cirugía , Vincristina/administración & dosificación , Vincristina/efectos adversosRESUMEN
BACKGROUND: Encouraging results have been reported with high dose chemotherapy and total body radiation followed by bone marrow autotransplantation in children with advanced neuroblastoma; however, relapse remains a significant problem. METHODS: The authors treated 22 children with advanced neuroblastoma with high dose chemotherapy, surgery, intraoperative radiation, and a bone marrow autotransplant (treated in vitro to remove tumor cells) followed by 13-cis-retinoic acid. RESULTS: The 3-year relapse rate was 25% (95% confidence interval [CI], 6-44%). The 3-year disease free survival rate was 72% (95% CI, 52-92%). Toxicities included hemolytic uremic syndrome, herpes infection, and hepatic venoocclusive disease. CONCLUSION: These data suggest that this treatment strategy offers an increased rate of 3-year disease free survival. The nonrandomized nature of this study and its use of multiple modalities precludes the analysis of the specific contribution of each treatment component and comparison with conventional therapy.
Asunto(s)
Trasplante de Médula Ósea , Neuroblastoma/cirugía , Purgación de la Médula Ósea , Trasplante de Médula Ósea/efectos adversos , Niño , Preescolar , Terapia Combinada , Femenino , Humanos , Lactante , Masculino , Trasplante Autólogo , Resultado del TratamientoAsunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Purgación de la Médula Ósea/métodos , Trasplante de Médula Ósea/métodos , Lectinas , Neuroblastoma/terapia , Proteínas de Soja , Niño , Preescolar , Terapia Combinada , Femenino , Factor Estimulante de Colonias de Granulocitos y Macrófagos/uso terapéutico , Humanos , Masculino , Melfalán/administración & dosificación , Neuroblastoma/tratamiento farmacológico , Neuroblastoma/radioterapia , Neuroblastoma/cirugía , Lectinas de Plantas , Dosificación Radioterapéutica , Glycine max , Trasplante Autólogo/métodos , Vincristina/administración & dosificaciónRESUMEN
This study evaluates the effect of intraoperative photodynamic therapy (PDT) using the multiline argon laser (488-514 nm) or the argon-dye laser (630 nm) combined with surgical resection compared with surgical resection alone in reducing the incidence of C1300 neuroblastoma recurrence in mice. In the control groups, surgical resection alone resulted in 86% +/- 12% tumor recurrence. Surgical resection and intraoperative lasing without photosensitizer resulted in 75% +/- 27% tumor recurrence with the argon-dye laser and 55% +/- 18% recurrence with the multiline argon laser. In the treatment groups, surgical resection and intraoperative PDT at 630 nm resulted in 56% +/- 19% tumor recurrence whereas surgical resection and intraoperative PDT at 488-514 nm resulted in 21% +/- 7% tumor recurrence. The cause for the decrease in local recurrence in the control group using the multiline argon laser is unknown, but could it be due in part to hyperthermic effects. Intraoperative PDT was an effective adjunct to surgical resection in preventing local recurrence in this tumor model.