Objective:To explore the clinical manifestations, diagnosis, treatment, pathological types and prognosis of primary cervical neuroblastoma in children. Methods:The clinical data of 11 children with primary cervical neuroblastoma admitted to the Department of Otolaryngology and Head and Neck Surgery at Shanghai Children's Hospital from April 2015 to April 2022 were retrospectively analyzed. The clinical characteristics, imaging findings, pathological types, treatment methods, and outcomes of these 11 patients were examined in conjunction with a review of the literature. Results:The cohort of 11 neuroblastoma patients ranged in age from 28 days to 88 months （median age: 24 months）, including 3 males and 8 females. Among the 11 patients, 4 had tumors located in the carotid sheath area, 4 in the cervical space, and 3 in the parapharyngeal and retropharyngeal spaces. The clinical manifestations primarily included painless cervical masses and laryngeal stridor. There were 3 cases of primary cervical tumors with cervical lymph node metastasis and 1 case with bone marrow metastasis. Pathological findings revealed neuroblastoma in 8 cases and ganglionic neuroblastoma in 3 cases. In this group, 7 patients underwent surgery combined with chemotherapy, 2 patients received surgery combined with chemoradiotherapy, and 2 patients underwent surgery alone. Surgical resection was performed via a cervical approach in 7 cases, while 3 cases were treated using a transoral endoscopic approach. Additionally, one patient underwent a transoral endoscopic approach initially and a cervical approach subsequently. All patients completed their treatment and were followed up regularly, with follow-up durations ranging from 6 to 79 months （median: 34 months）. Nine patients achieved complete remission, 2 patients achieved partial remission, and none experienced disease progression. Conclusion:Primary cervical neuroblastoma exhibits a high degree of heterogeneity and presents at a younger age, primarily with cervical masses. Compared to external approaches, endoscopy-assisted transoral resection of parapharyngeal tumors offers advantages such as reduced damage to surrounding tissue and no visible neck scars, providing a new method for clinical treatment. Regular follow-up is essential for children with neuroblastoma, along with the establishment of specific disease management protocols and comprehensive care to improve survival quality.
Humans ; Female ; Male ; Neuroblastoma/surgery* ; Child, Preschool ; Infant ; Retrospective Studies ; Child ; Head and Neck Neoplasms/pathology* ; Prognosis

ObjectiveThis study was to review the efficacy of surgical resections in different clinical situations for a better understanding of the meaning of surgery in the treatment of neuroblastoma (NB).

Data SourcesThe online database ScienceDirect (201-2018) was utilized. The search was conducted using the keywords "neuroblastoma," "neuroblastoma resection," "neuroblastoma surgery," and "high-risk neuroblastoma."

Study SelectionWe retrospectively analyzed of patients who underwent surgical resections in different clinical situations. The article included findings from selected relevant randomized controlled trials, systematic reviews, and meta-analyses or good-quality observational studies. Abstracts only, letters, and editorial notes were excluded. Full-text articles and abstracts were extracted and reviewed to identify key articles discussing surgery management of NB, which were then selected for critical analysis.

ResultsA total of 7800 English language articles were found containing references to NB (201-2018). The 163 articles were searched which were related to the surgical treatment of NB (201-2018). Through the analysis of these important articles, we found that the treatments of NB at low- and intermediate-risk groups were basically the same. High-risk patients remained controversial.

ConclusionsNB prognosis varies tremendously based on the stage and biologic features of the tumor. After reviewing the relevant literature, patients with low-risk disease are often managed with surgical resection or observation alone with tumors likely to spontaneously regress that are not causing symptoms. Intermediate patients are treated with chemotherapy with the number of cycles depending on their response as well as surgical resection of the primary tumor. High-risk patients remain controversial. Multidisciplinary intensive treatment is essential, especially for patients who received subtotal tumor resection. Minimally invasive surgery for the treatment of NBs without image-defined risk factors in low- to high-risk patients is safe and feasible and does not compromise the treatment outcome. We conclude that ≥90% resection of the primary tumor is both feasible and safe in most patients with high-risk NB. New targeted therapies are crucial to improve survival.

Brain Neoplasms ; surgery ; Humans ; Neuroblastoma ; surgery ; Neurosurgical Procedures ; methods ; Prognosis ; Randomized Controlled Trials as Topic ; Retrospective Studies ; Treatment Outcome

12E7 Antigen ; Adolescent ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Diagnosis, Differential ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Lymph Node Excision ; Lymphatic Metastasis ; Lymphoma ; metabolism ; pathology ; Male ; Neoplastic Cells, Circulating ; Nephrectomy ; Neuroblastoma ; metabolism ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; metabolism ; pathology ; surgery ; Synaptophysin ; metabolism ; Venae Cavae ; pathology ; Vimentin ; metabolism ; Wilms Tumor ; metabolism ; pathology

OBJECTIVETo investigate the efficacy of combined modality therapy for neuroblastoma in children associated with opsoclonus-myoclonus syndrome (OMS-NB).

METHODFrom May 2011 to December 2013, 6 consecutive patients (4 boys and 2 girls) diagnosed as OMS-NB underwent surgery and chemotherapy in the First Hospital, Peking University. The median age of onset was 19.5 months (range 13-24 months) and misdiagnosis occurred 7.5 months (range 2-14 months) ago. A retrospective analysis for the location, stage, pathological type, treatment way and outcome of neuroblastoma was done.

RESULT(1) All patients were misdiagnosed as simply opsoclonus-myoclonus syndrome (OMS) at the time of onset. They had been receiving treatment with adrenocorticotropic hormone and intravenous immunoglobulin within 1-13 months.OMS-NB was diagnosed by means of enhanced abdominal CT image which was delayed to be given after the poor efficacy or relapse. (2) The primary tumors were almost all small, stage I-II, located in adrenal, retroperitoneal or pelvis. The pathology of tumors included ganglioneuroblastoma (5/6) and neuroblastoma (1/6). (3) All these cases underwent surgery, 4/6 cases with complete tumor resection, 2/6 cases with tumor around the aorta and induced local residue. Preoperative and postoperative chemotherapy was given to 2 and 5 cases, respectively. (4) The patients were followed up for 3-31 months, except 1 patient lost, the other 5 are currently surviving disease-free (3 having been at the end of chemotherapy, 1 still in chemotherapy, and another had local recurrence and is receiving radiotherapy and chemotherapy after the second operation and now also stopped taking the medicine). The symptoms of nervous system have been significantly improved during postoperative chemotherapy.

CONCLUSIONTo reduce the misdiagnosis, regular CT imaging of the abdomen or pelvic should be ordered for all cases with OMS. The children with OMS-NB need to be actively treated with the combined modality therapy including surgery, chemotherapy or radiotherapy, to reduce recurrence and reduce the symptoms of nervous system.

Adrenal Gland Neoplasms ; complications ; diagnosis ; surgery ; therapy ; Antineoplastic Agents ; administration & dosage ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Biomarkers ; analysis ; Chemotherapy, Adjuvant ; Child, Preschool ; Combined Modality Therapy ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Neoplasm Recurrence, Local ; surgery ; therapy ; Neoplasm Staging ; Neuroblastoma ; complications ; diagnosis ; surgery ; therapy ; Opsoclonus-Myoclonus Syndrome ; diagnosis ; etiology ; therapy ; Retroperitoneal Neoplasms ; complications ; diagnosis ; surgery ; therapy ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome

OBJECTIVETo study the clinicopathologic characteristics of peripheral neuroblastic tumors and to evaluate the prognostic significance of these features.

METHODSThe clinical and pathologic findings were retrospectively reviewed in 121 cases of peripheral neuroblastic tumor. The clinical outcomes of patients were evaluated. The three-year event-free survival rate was analyzed, with respect to age of patients, Evan's staging, International Neuroblastoma Pathology Classification and mitosis-karyorrhexis index.

RESULTSThe median age at diagnosis was 2.7 years; and 96 cases (79.3%) occurred in patients younger than 5 years old. The number of cases in Evan's staging I, II, III, IV and IVs was 24, 39, 24, 29 and 5, respectively. There were 82 cases of neuroblastoma (NB) (including 2 cases of undifferentiated NB, 52 cases of poorly differentiated NB and 28 cases of differentiating NB), 9 cases of ganglioneuroblastoma, intermixed type (GNBi), 19 cases of ganglioneuroma, maturing type (GN) and 11 cases of ganglioneuroblastoma, nodular type (GNBn). Forty-nine cases were in the favorable histology subgroup and 72 cases in the unfavorable histology subgroup. The overall three-year event-free survival rate of the 121 cases was 73.0% ± 4.3%. The three-year event-free survival rates were associated with age (P = 0.002), Evan's staging (P = 0.000), histologic category (P = 0.000), mitosis-karyorrhexis index (P = 0.043), prognostic subgroup (P = 0.000).

CONCLUSIONSMost of the peripheral neuroblastic tumors occur in the children younger than 5 years old. It is composed of NB, GNBi, GN and GNBn. The three-year event-free survival rate is approximately 70%. Significant prognostic parameters include age of patients, Evan's staging, International Neuroblastoma Pathology Classification and mitosis-karyorrhexis index.

Age Factors ; Antigens, Nuclear ; metabolism ; Child ; Child, Preschool ; Disease-Free Survival ; Female ; Ganglioneuroblastoma ; metabolism ; pathology ; surgery ; Ganglioneuroma ; metabolism ; pathology ; surgery ; Humans ; Infant ; Infant, Newborn ; Male ; Neoplasm Staging ; Nerve Tissue Proteins ; metabolism ; Nestin ; metabolism ; Neuroblastoma ; metabolism ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; Retrospective Studies ; S100 Proteins ; metabolism

OBJECTIVETo study the clinicopathologic characteristics of peripheral neuroblastic tumors and to investigate the prognostic significance of International Neuroblastoma Pathology Classification (INPC).

METHODSOne hundred and thirty-five cases of peripheral neuroblastic tumors encountered in Shanghai Children's Medical Center were enrolled into the study. All the cases were classified according to INPC and International Neuroblastoma Staging System (INSS). The follow-up data were analyzed.

RESULTSThe consensus diagnoses of the 135 cases were as follows: 80 cases (59.2%) of neuroblastoma, 24 cases (17.8%) of ganglioneuroblastoma, intermixed, 17 cases (12.6%) of ganglioneuroma and 14 cases (10.4%) of ganglioneuroblastoma, nodular. The cases were subdivided into 2 subgroups: favorable histology (number = 90, 66.7%) and unfavorable histology (number = 45, 33.3%). According to INSS, the number of cases in stages I, II, III and IV was 22 (16.3%), 24 (17.8%), 34 (25.2%) and 55 (40.7%), respectively. The survival of peripheral neuroblastic tumors correlated with histologic diagnosis, INPC and INSS (P < 0.05).

CONCLUSIONDiagnostic categorization of peripheral neuroblastic tumors according to INPC is of prognostic value.

Adolescent ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Ganglioneuroblastoma ; pathology ; surgery ; Ganglioneuroma ; pathology ; surgery ; Humans ; Infant ; Infant, Newborn ; Male ; Neoplasm Staging ; Neuroblastoma ; classification ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; classification ; pathology ; surgery ; Retrospective Studies ; Survival Rate

Adult ; Axilla ; Cell Transformation, Neoplastic ; pathology ; Diagnosis, Differential ; Epithelioid Cells ; metabolism ; pathology ; Fibrosarcoma ; metabolism ; pathology ; Humans ; Male ; Neurilemmoma ; metabolism ; pathology ; surgery ; Neuroblastoma ; metabolism ; pathology ; S100 Proteins ; metabolism ; Soft Tissue Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Adult ; Diagnosis, Differential ; Follow-Up Studies ; Humans ; Infant ; Keratins ; metabolism ; Male ; Mandibular Neoplasms ; metabolism ; pathology ; surgery ; Melanoma ; metabolism ; pathology ; Neuroblastoma ; metabolism ; pathology ; Neuroectodermal Tumor, Melanotic ; metabolism ; pathology ; surgery ; Rhabdomyosarcoma, Embryonal ; metabolism ; pathology ; Synaptophysin ; metabolism

12E7 Antigen ; Adult ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Desmin ; metabolism ; Desmoplastic Small Round Cell Tumor ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Humans ; Neoplasm Recurrence, Local ; Neuroblastoma ; metabolism ; pathology ; Ovarian Neoplasms ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; Rhabdomyosarcoma, Embryonal ; metabolism ; pathology ; Sarcoma, Ewing ; metabolism ; pathology ; Vimentin ; metabolism

OBJECTIVEThe 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.

METHODSNewly 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.

RESULTForty-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).

CONCLUSIONThe 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.

Age Factors ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Chemotherapy, Adjuvant ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation ; methods ; Humans ; Infant ; Male ; Neoplasm Recurrence, Local ; prevention & control ; surgery ; therapy ; Neoplasm Staging ; Neuroblastoma ; diagnosis ; drug therapy ; mortality ; pathology ; surgery ; therapy ; Prognosis ; Remission Induction ; methods ; Retrospective Studies ; Severity of Illness Index ; Survival Rate ; Time Factors ; Transplantation, Autologous ; methods ; Treatment Outcome