OBJECTIVE: To study the efficacy and safety of intensity-modulated radiotherapy (IMRT) in children with high-risk neuroblastoma (NB). METHODS: A retrospective analysis was performed on the medical data of 24 children with high-risk NB who were diagnosed and treated with IMRT in the Department of Hematology and Oncology, Hunan Provincial People's Hospital, from April 2018 to December 2020. The medical data included age, radiotherapy dose, times of radiotherapy, laboratory examination results, adverse reactions, and survival. RESULTS: All 24 children (14 boys and 10 girls) received IMRT, with a mean age of (65±23) months and a median age of 59 months. The primary tumor was located in the abdomen in 23 children and 1 child had primary tumor in the mediastinum. The median age was 41.5 months at the time of radiotherapy. The radiation dose of radiotherapy ranged from 14.4 to 36.0 Gy, with a mean dose of (22±3) Gy and a daily dose of 1.8-2.0 Gy. The radiotherapy was performed for a total number of 8-20 times, with a mean number of 11.9 times. Among these children, 6 received radiotherapy for the residual or metastatic lesion. Of all the 23 children, 3 experienced cough, 2 experienced diarrhea, and 1 experienced vomiting during radiotherapy. At 2 weeks after radiotherapy, serum creatinine ranged from 2.3 to 70.1 μmol/L and alanine aminotransferase ranged from 9.1 to 65.3 μ/L. Ten children experienced grade Ⅲ bone marrow suppression and 2 experienced grade Ⅳ bone marrow suppression 1 to 2 weeks after radiotherapy. Four children experienced grade Ⅲ bone marrow suppression and 1 experienced grade Ⅳ bone marrow suppression 3 to 4 weeks after radiotherapy. During a median follow-up time of 13.5 months, 23 children (96%) achieved stable disease and 1 died. Up to the follow-up date, second malignant tumor or abnormal organ function was not observed. CONCLUSIONS IMRT can improve the local control rate of NB. IMRT appears to be safe in the treatment of children with NB.
Child ; Child, Preschool ; Female ; Humans ; Male ; Neuroblastoma/radiotherapy* ; Radiotherapy Dosage ; Radiotherapy, Intensity-Modulated/adverse effects* ; Retrospective Studies

PURPOSE: To investigate the patterns of recurrence in patients with neuroblastoma treated with radiation therapy to the primary tumor site. MATERIALS AND METHODS: We retrospectively analyzed patients with high-risk neuroblastoma managed with definitive treatment with radiation therapy to the primary tumor site between January 2003 and June 2017. These patients underwent three-dimensional conformal radiation therapy or intensity-modulated radiation therapy. A total of 14–36 Gy was delivered to the planning target volume, which included the primary tumor bed and the selected metastatic site. The disease stage was determined according to the International Neuroblastoma Staging System (INSS). We evaluated the recurrence pattern (i.e., local or systemic), progression-free survival, and overall survival. RESULTS: A total of 40 patients with high-risk neuroblastoma were included in this study. The median patient age was 4 years (range, 1 to 11 years). Thirty patients (75%) had INSS stage 4 neuroblastoma. At the median follow-up of 58 months, there were 6 cases of local recurrence and 10 cases of systemic recurrence. Among the 6 local failure cases, 4 relapsed adjacent to the radiation field. The other 2 relapsed in the radiation field (i.e., para-aortic and retroperitoneal areas). The main sites of distant metastasis were the bone, lymph nodes, and bone marrow. The 5-year progression-free survival was 70.9% and the 5-year overall survival was 74.3%. CONCLUSION: Radiation therapy directed at the primary tumor site provides good local control. It seems to be adequate for disease control in patients with high-risk neuroblastoma after chemotherapy and surgical resection.
Bone Marrow ; Disease-Free Survival ; Drug Therapy ; Follow-Up Studies ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Neuroblastoma ; Radiotherapy ; Recurrence ; Retrospective Studies

PURPOSE: Although the prognosis is generally good in patients with intermediate-risk neuroblastoma, no consensus has been reached on the ideal treatment regimen. This study analyzed treatment outcomes and toxicities in patients younger than 18 months with stage 4 MYCN nonamplified neuroblastoma. METHODS: We retrospectively analyzed 20 patients younger than 18 months newly diagnosed with stage 4 MYCN nonamplified neuroblastoma between January 2009 and December 2015. Patients received 9 cycles of chemotherapy and surgery, with or without local radiotherapy, followed by 12 cycles of differentiation therapy with 13-cis-retinoic acid. Chemotherapy consisted of alternating cycles of cisplatin, etoposide, doxorubicin, and cyclophosphamide (CEDC) and ifosfamide, carboplatin, and etoposide (ICE) regimens. RESULTS: The most common primary tumor site was the abdomen (85%), and the most common metastatic sites were the lymph nodes (65%), followed by the bones (60%), liver (55%), skin (45%), and bone marrow (25%). At the end of induction therapy, 14 patients (70%) achieved complete response, with 1 achieving very good partial response, 4 achieving partial response, and 1 showing mixed response. Nine patients (45%) received local radiotherapy. At a median follow-up of 47 months (range, 17–91 months), none of these patients experienced relapse, progression, or secondary malignancy, or died. Three years after chemotherapy completion, none of the patients had experienced grade ≥3 late adverse effects. CONCLUSION: Patients younger than 18 months with stage 4 MYCN nonamplified neuroblastoma showed excellent outcomes, without significant late adverse effects, when treated with alternating cycles of CEDC and ICE, followed by surgery and differentiation therapy.
Abdomen ; Bone Marrow ; Carboplatin ; Child ; Cisplatin ; Consensus ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Etoposide ; Follow-Up Studies ; Humans ; Ice ; Ifosfamide ; Infant ; Isotretinoin ; Liver ; Lymph Nodes ; Neoplasm Metastasis ; Neuroblastoma ; Prognosis ; Radiotherapy ; Recurrence ; Retrospective Studies ; Skin

OBJECTIVES: Neuroblastoma (NBL) predominantly affects children under 5 years of age. Through multimodal therapy, including chemotherapy, radiotherapy, surgery, and peripheral blood stem cell transplantation, the survival rate in patients with NBL have improved while treatment-related complications have also increased. Treatment-related ototoxicity, mainly from cisplatin, can result in profound hearing loss requiring cochlear implantation (CI). We analyzed the effectiveness and hearing preservation of CI recipients who had treated with multimodal therapy due to NBL. METHODS: Patients who received multimodal therapy for NBL and subsequent CIs were enrolled. A detailed review of the perioperative hearing test, speech evaluation, and posttreatment complications was conducted. Speech performance was analyzed using the category of auditory performance (CAP) score and the postoperative hearing preservation of low frequencies was also compared. Patients who were candidates for electro-acoustic stimulation (EAS) used an EAS electrode for low frequency hearing preservation. RESULTS: Three patients were identified and all patients showed improvement of speech performance after CI. The average of CAP score improved from 4.3 preoperatively to 5.8 at 1 year postoperatively. Two patients who were fitted with the Flex electrode showed complete hearing preservation and the preserved hearing was maintained over 1 year. The one remaining patient was given the standard CI-512 electrode and showed partial hearing preservation. CONCLUSION: Patients with profound hearing loss resulting from NBL multimodal therapy can be good candidates for CI, especially for EAS. A soft surgical technique as well as a specifically designed electrode should be applied to this specific population during the CI operation in order to preserve residual hearing and achieve better outcomes.
Child* ; Cisplatin ; Cochlear Implantation* ; Cochlear Implants* ; Combined Modality Therapy* ; Drug Therapy ; Electrodes ; Hearing Loss* ; Hearing Tests ; Hearing* ; Humans ; Neuroblastoma* ; Peripheral Blood Stem Cell Transplantation ; Radiotherapy ; Survival Rate

OBJECTIVE: Neuroblastoma is a very common pediatric malignant tumor and sometimes involves the spinal cord to result in neurological deficits. The authors perform a retrospective analysis of the 12 cases of pediatric neuroblastoma with spinal involvement to assess the characteristics and surgical outcome. METHODS: We retrospectively reviewed the 12 cases of pediatric neuroblastoma with spinal involvement which underwent surgery from 1988 to 2002 in our hospital. All the cases were confirmed by pathologic diagnosis and reviewed about initial presentation, the location of tumor, treatment, outcome and complication. RESULTS: The ratio of male to female was 1: 2 and mean age was 3.5 years(0.3-13.6). The chief complaint was motor weakness in 7 cases, mass in 2, urinary incontinence in one and 2 cases were asymptomatic. The tumor involved thoracic level in 5 cases, thoracolumbar level in 3, cervicothoracic level in 2, cervical and lumbar levels in one case each. All the cases underwent surgery, four had chemotherapy and four had both chemotherapy and radiotherapy. After 33.9 months of mean follow up, in all of the 4 cases whose intraspinal tumor was gross-totally resected, neurological status improved. Of the 4 cases with subtotal resection, all except one showed progression or no neurologic improvement. Postoperative spinal deformity has developed in 5 cases. Three cases expired because of chemotherapy complication and tumor progression. CONCLUSION: Active surgery about the spinal involvement of pediatric neuroblastoma shows neurological improvement regardless of the survival. It seems to be helpful to the quality of life in pediatric patients.
Congenital Abnormalities ; Diagnosis ; Drug Therapy ; Female ; Follow-Up Studies ; Humans ; Male ; Neuroblastoma* ; Quality of Life ; Radiotherapy ; Retrospective Studies ; Spinal Cord ; Urinary Incontinence

PURPOSE: This study was done to evaluate whether risk-directed treatment can improve survival of patients with high risk neuroblastoma (NBL). METHODS: Forty two patients with NBL were newly diagnosed and treated at Samsung Seoul Hospital from June 1997 to December 2000. Patients were divided into high risk or low risk group according to 3 important prognostic factors. Poor prognostic factors were defined as follows; amplification of N-myc oncogene, age at diagnosis higher than 1 years, and INSS stage 4. Patients with 2 or more poor prognostic factors were defined as high risk patients. While conventional treatment including surgery, radiotherapy, and pre and post-operative chemotherapy was applied to low risk patients, intensive multimodality treatment including single or double high dose chemotherapy (HDCT) and autologous peripheral blood stem cell transplantation (PBSCT) followed by immunotherapy using interleukin-2 (IL-2) and differentiating therapy using 13-cis-retinoic acid (CRA) was applied to high risk patients. RESULTS: Among 42 patients, 30 patients were high risk, 10 patients were low risk, and 2 patients were impossible to classify. Forty five HDCTs and PBSCTs were applied to 28 high risk patients and 2 low risk patients. All of the low risk patients are alive without relapse. Three year event free survival (EFS) after diagnosis in high risk patients was 54.8%. EFS after diagnosis in patients with 2 or 3 risk factors were 81.3%, 39.3% (P=0.0292) respectively. EFS after HDCT was 65.1%. EFS after HDCT in patients with 2 or 3 risk factors were 85.7%, 47.1% (P=0.0527) respectively. CONCLUSION: Risk-based grouping of patients and risk-directed treatment are necessary for better outcome. Multimodality treatment including HDCT and autologous PBSCT followed by immunotherapy using IL-2 and differentiatin therapy using CRA can improve survival in high risk patients.
Diagnosis ; Disease-Free Survival ; Drug Therapy ; Humans ; Immunotherapy ; Interleukin-2 ; Isotretinoin ; Neuroblastoma* ; Oncogenes ; Peripheral Blood Stem Cell Transplantation ; Radiotherapy ; Recurrence ; Risk Factors ; Seoul

PURPOSE: Despite the use of improved therapeutic modalities, long-term survival remains poor in patients in stage lll or lV of neurobalstoma and for over 1 year olds at diagnosis. We evaluated the long-term survival rate and related prognostic factors in advanced neuroblastoma. METHODS: Twenty-nine children diagnosed with stage lll or lV neuroblastoma after 1 year of age, between May 1987 and December 1996, were enrolled. Therapy included surgery, radiotherapy, and chemotherapy with cyclophosphamide, DTIC, and vincristine (CDV) for stage lll, and CDV or intensified regimen for stage IV. RESULTS: Of the 25 patients, 18 male and 7 female, 23 (92%) were<5 years of age at diagnosis. The most commonly appearing initial presentation was abdominal mass, which was observed in 14 patients. Abdominal primary was found in 21 patients (84%) including 17 (68%) of adrenal origin and mediastinal primary in 4 (16%). The 5Y-EFS was 44% in all study patients with 80% for stage lll and 20% for stage lV. The age< years, mediastinal primary, low serum NSE, and low serum ferritin were associated with better outcome. In stage lll, progression of disease was observed in only two, both with unfavorable prognostic factors. In stage lV, no difference in long-term survival was noted between those treated with CDV regmen and those with intensified chemotherapeutic regimen. CONCLUSIONS: Long-term event free survival (EFS) rate for stage III was 80% with poor outcome for stage lll including certain unfavorable prognostic factors and for stage lV, which might need more effective innovative therapies, such as autologous bone marrow transplantation or peripheral blood stem cell transplantation.
Bone Marrow Transplantation ; Child* ; Cyclophosphamide ; Dacarbazine ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Female ; Ferritins ; Humans ; Male ; Neuroblastoma ; Peripheral Blood Stem Cell Transplantation ; Radiotherapy ; Survival Rate* ; Therapies, Investigational ; Vincristine

Neuroblastoma originate in neural crest and is one of the most frequent tumor next to leukemia and brain tumor in child. The tumor is most highly malignant tumor due to early metastasis at diagnosis. It could be diagnosed by various diagnostic modalities such as roentgenologic examination, laboratory data and confirmed by pathologic diagnosis. Management of neuroblastoma is surgery, radiotherapy and chemotherapy. We experienced a case of adrenal neuroblastoma at age of 20-month male child and report with a brief review of literature
Adrenal Glands ; Brain Neoplasms ; Child ; Diagnosis ; Drug Therapy ; Humans ; Leukemia ; Male ; Neoplasm Metastasis ; Neural Crest ; Neuroblastoma* ; Radiotherapy

Fine needle aspiration has been effectively being applided to pediatric tumors since it renders a rapid diagnosis with minimal intervention. This measure is especially required for the large pediatric mass, which needs preoperative chemotherapy or radiotherapy to shrink the tumor to an operable size. A case of neuroblastoma of mediastinum, stage IV diagnosed by CT-guided FNA is described.
Biopsy, Fine-Needle* ; Diagnosis ; Drug Therapy ; Mediastinum* ; Neuroblastoma* ; Radiotherapy

Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blood count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VCR) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden ganglioneuroblastoma in 22-month-old girl.
Blood Cell Count ; Bone Marrow ; Brachiocephalic Trunk ; Catecholamines ; Creatinine ; Dacarbazine ; Diagnosis ; Drug Therapy ; Electrolytes ; Epilepsies, Myoclonic* ; Eye Movements ; Female ; Follow-Up Studies ; Ganglioneuroblastoma* ; Head ; Hospitalization ; Humans ; Infant ; Lymph Nodes ; Myoclonus ; Nervous System ; Neural Crest ; Neuroblastoma ; Neurologic Manifestations ; Paraneoplastic Syndromes* ; Radiotherapy ; Thorax ; Tomography, X-Ray Computed ; Upper Extremity ; Urinalysis