Neuroblastoma

Outcome

The overall survival for children with favorable histology is 90%. Children with favorable histology, Stage I lesions have a 97% long-term survival. This drops to 60% for children with favorable histology, Stage IV lesions.

Overall survival for children with unfavorable histology is 50%. Children with unfavorable histology, Stage I lesions have an 89% long-term survival. This drops to 14-55% for children with unfavorable histology and any other Stage disease.

Prognostic factors include recurrent disease, histology, DNA content, gene expression and growth factors. Risk factors for local recurrence include Stage III disease, intraoperative tumor spill (refers to a break in the tumor capsule during operative removal), unfavorable histology and absence of lymph node biopsy. The two year survival after local recurrence is 43%.

Congenital mesoblastic nephroma is a benign lesion with an excellent prognosis.

Neuroblastoma (also known as an "infantile neuroblastoma," and "neuroepithelioma") is the most common extracranial solid cancer in childhood and the most common cancer in infancy. The term “neuro” refers to nerves, while “blastoma” refers to a cancer that affects immature or developing cells.

It is a neuroendocrine tumor, arising from any neural crest element of the sympathetic nervous system (SNS). It most frequently originates in one of the adrenal glands, but can also develop in nerve tissues in the neck, chest, abdomen, or pelvis.

Note! Neuroblastoma is one of the few human malignancies known to demonstrate spontaneous regression from an undifferentiated state to a completely benign cellular appearance.

Neuroblastoma is a tumor derived from neuroblasts. A neuroblast is a dividing cell that will develop into a neuron often after a migration phase. Neuroblasts are derived from neural crest cells and migrate during fetal development to form the autonomic nervous system (autonomic nervous system - part of the nervous system also controls body functions we are rarely aware of, such as heart rate, breathing, blood pressure, digestion, and other functions).

There are two paths of migration: (1) along developing nerves to form the sympathetic plexuses, where they form ganglion cells and (2) to the adrenal gland to form the medulla.

Tumors of the neuroblasts can be either malignant or benign.

There is a wide spectrum of how neuroblastomas can behave. Some grow and spread quickly, while others grow slowly. Sometimes, in very young children, the cancer cells die without any cause and the tumor goes away on its own. In other cases, the cells sometimes mature on their own into normal ganglion cells and stop dividing. This causes the tumor to become a ganglioneuroma

Дата добавления: 2014-10-15