NEUROBLASTOMA

NEUROBLASTOMA:
Objective: To understand the clinical presentations , diagnosis , epidemiology , pathology , prognosis and treatment.
Neuroblastomas are embryonal cancers of the peripheral sympathetic nervous system with heterogeneous clinical presentation and course, ranging from tumors that undergo spontaneous regression to very aggressive tumors unresponsive to very intensive multimodal therapy.
The causes of most cases remain unknown, and the outcomes for aggressive forms of neuroblastoma remain poor.
EPIDEMIOLOGY:
Neuroblastoma is the most common extracranial solid tumor in children and the most commonly diagnosed malignancy in infants. Neuroblastoma accounts for >15% of the mortality from cancer in children. The median age of children at diagnosis of neuroblastoma is 22 mo, and 90% of cases are diagnosed by 5 yr of age. The incidence is slightly higher in boys and in whites.
PATHOLOGY:
Neuroblastoma tumors, which are derived from neural crest cells, form a spectrum with variable degrees of neural differentiation,ranging from tumors with primarily undifferentiated small round cells (neuroblastoma) to tumors consisting of mature and maturing Schwannian stroma with ganglion cells (ganglioneuroblastoma or ganglioneuroma).
The tumors may resemble other small round blue cell tumors, such as rhabdomyosarcoma, Ewing sarcoma, and non- Hodgkin lymphoma. The prognosis of children with neuroblastoma varies with the histologic features of the tumor, and prognostic factors include the presence and amount of Schwannian stroma, the degree of tumor cell differentiation, and the mitosis-karyorrhexis index.