Ganglioneuroblastoma

A little more about Haven's type of cancer. As I said previously (or maybe I didn't, can't remember) there are 3 types of Neuroblastoma. They are listed below in the Pathophysiology section. Haven is confirmed to have Ganglioneuroblastoma (the middle type).

Ganglioneuroblastoma is an intermediate tumor arising from nerve tissue. An intermediate tumor is one that is between benign (slow-growing and unlikely to spread) and malignant (fast-growing, aggressive, and likely to spread).

Pathophysiology: The histologic difference between ganglioneuroma, ganglioneuroblastoma, and neuroblastoma is their stage of neuroblast maturation (Shimada, 1999). The less mature tumors, ie, ganglioneuroblastoma and neuroblastoma, are more aggressive and dangerous. These tumors have a higher neuroblast content and tend to occur in young children with the median age of 2 years.

Ganglioneuroblastomas are a mix of malignant neuroblastoma and benign ganglioneuroma. The prognosis for patients with a ganglioneuroblastoma is still relatively good. These tumors can spontaneously regress or mature. Patients with ganglioneuroblastomas often present with pain caused by either the primary tumor or metastatic disease and abdominal distention. These neuroblastic tumors vary widely in their biologic behavior. Features such as DNA content, tumor proto-oncogenes, and catecholamine synthesis influence prognosis, and their presence or absence aids in categorizing patients as high, intermediate, or low risk.

Treatment consists of surgery and, usually, chemotherapy. Despite recent advances in treatment, including bone marrow transplantation, neuroblastoma remains a relatively lethal tumor, accounting for 10% of pediatric cancers but 15% of cancer deaths in children.

Approximately 500–525 new cases of NB are diagnosed each year in the United States, making it the third most common pediatric malignancy, after leukemia and central nervous system tumors.

Causes, incidence, and risk factors:

This rare tumor has a yearly occurrence of less than 5 per 1,000,000 children.Tumors of the nervous system vary in their degree of differentiation. The degree of differentiation determines how the tumors appear under the microscope and whether or not they are likely to spread. Benign tumors are less likely to spread. Malignant tumors are aggressive, grow quickly, and often spread. A ganglioneuroma is a benign tumor, while a neuroblastoma(occurring in children more than a year old) is generally malignant. A ganglioneuroblastoma may be localized to one area or it may be widespread, but it is usually less aggressive than a neuroblastoma. The cause is unknown.

Symptoms: Most commonly, a mass can be felt in the abdomen, but this condition may also occur in other parts of the body.

Treatment: Because these tumors are rare, they should be treated in a specialized center by experts who have experience with them. Depending on the specific nature of the tumor, treatment can consist of surgery, and possibly chemotherapy and radiation therapy.

Locations:
Ganglioneuroblastoma tumors are found in the adrenal medulla (35%), retroperitoneum (30-35%), posterior mediastinum (20%), neck (1-5%), and pelvis (2-3%). Other locations, such as the thymus, lung, kidney, anterior mediastinum, stomach, and cauda equina, are affected less frequently and considered unusual. Approximately 1% of these tumors metastasize.

Mortality/Morbidity:
Patients with either a low- or intermediate-risk tumors have a relatively good prognosis. Children with stages 1, 2, and 4S tumors have a 3-year event-free survival rate of 75-90%. Children younger than 1 year with stage 3 or 4 tumors have a 1-year event-free survival rate of 80-90% and 60-75%, respectively. Children older than 1 year with INSS stage 3 or 4 tumors have a 3-year event-free survival rate of 50% and 15%, respectively (Coldman, 1980).