Neurosurgical aspects of the treatment of neuroblastoma patients

Abstract

Neuroblastoma (NB) is the most common extracranial solid tumor in children. The neoplasm grows from progenitor cells of the sympathetic nervous system and can be detected anywhere along the sympathetic neurological circuit: retroperitoneally, mediastinally, cervically, and pelvically. Examination of children with suspected neuroblastoma is comprehensive and performed in strict compliance with a therapeutic protocol. A decision on the treatment regimen is made based on the tumor staging and the risk group of the patient. The diagnosis and treatment of NB patients are comprehensive and can be fully carried out only at the pediatric oncology department. In 10-15% of cases, an hourglass tumor spreads to the intervertebral foramina or spinal canal at one or more levels. A tumor node is always located extradurally with respect to the spinal cord. Symptoms of spinal cord compression of various severity are observed in 5-7% of patients. We present several cases of patients with neuroblastoma with intraspinal extension. Despite apparent benefits of primary surgical decompression of the spinal cord, modern experience of treatment of children with intraspinal tumor extension does not reveal advantages of surgery over chemotherapy. Neurological disorders of various nature and severity persist in the majority of patients in the long-term period, regardless of primary treatment. A higher level of spinal deformities after surgical tumor resection is observed. The issue of spinal cord decompression should be discussed by the neurosurgeon and pediatric oncologist, and the most common method of choice may be chemotherapy. The article discusses the indications and contraindications for neurosurgical interventions in NB patients and addresses the issues of NB metastasis to the brain and cranial bones as well as the opsoclonus-myoclonus syndrome.

Case report: Turcot syndrome type 2 in a developing country within the Caribbean

“…To date, there are over 150 cases of TS accounted for in literature. Recordings of the actual number of the cases have been limited by prevalence, the need for genetic testing and the use of various alternative terms such as the more recently suggested substitution of TS with brain tumor polyposis syndrome (BTPS) ( 13 , 17 , 19 ).…”

“…This accounts for approximately 10% of diagnoses and is found mainly in girls with peak incidence between 10-12 years of age, as seen in our patient. MB-Wnt has a low tendency to metastasize and patients under 16 years of age have an excellent prognosis due to its prognostic status in comparison to other subgroups ( 8 , 17 , 19 , 21 , 23 ).…”

“…Medulloblastoma are mostly sporadic in nature, however there is an inherited predisposition in TS2 ( 17 , 21 , 23 ). In TS2, the primary brain tumor of medulloblastoma is associated with a germ line variant in the APC gene which is a tumor suppressor gene associated with CRC due to FAP ( 17 , 21 ).…”

“…Medulloblastoma are mostly sporadic in nature, however there is an inherited predisposition in TS2 ( 17 , 21 , 23 ). In TS2, the primary brain tumor of medulloblastoma is associated with a germ line variant in the APC gene which is a tumor suppressor gene associated with CRC due to FAP ( 17 , 21 ). There can be inactivation of the second copy of the gene when there is a germline variant of APC, which appears to be a brain tumorigenesis factor.…”

Comparison of mediastinal and non-mediastinal neuroblastoma and ganglioneuroblastoma associated with opsoclonus-myoclonus syndrome: a systematic review and meta-analysis

“…The median age at diagnosis is 16 months, and 95% of cases are diagnosed before 7 years of age ( 1 ). The neoplasm grows from progenitor cells of the sympathetic nervous system and can be detected anywhere along the sympathetic neurological circuit: retroperitoneally (65%), in the adrenal glands (40%), mediastinally (15%), cervically (11%), and pelvically (3%) ( 2 ). Children with opsoclonus-myoclonus syndrome (OMS) are typically presented with acute or subacute ataxia between 6 and 36 months of age and they are unable to walk and/or sit ( 3 ).…”