IV. Hodgkin's disease

There is no consensus on the treatment of Hodgkin's disease in children, with the exception of stage IV disease, which is primarily treated with chemotherapy. The role of staging laparotomy is still questionable, and splenectomy is contraindicated in young children because of fatal infectious complications. Chemotherapy rather than RT is generally preferred for all stages of disease. The alternation of MOPP and ABVD regimens is frequently recommended rather than either regimen alone. In children, local field rather than extended field radiation is also preferred.

Brain tumors

I. Epidemiology

Brain tumors in children are associated with certain underlying diseases, including neurofibromatosis, tuberous sclerosis, and von Hippel-Lindau angiomatosis. Family clusters of CNS tumors have occasionally been reported.

II. Pathology and natural history

A. Pathology

The vast majority of CNS neoplasms in children are primary tumors of the brain; the single exception is meningeal metastases, which are very common with leukemia and lymphoma. Astrocytomas are the most frequent type (30% of all cases). Medulloblastomas account for 25 percent of cases; ependymomas, 9 percent; and glioblastomas, 9 percent.

B. Sites of disease

Brain tumors in children tend to occur along the central neural axis (i.e., near the third or fourth ventricle or along the brainstem). The majority of brain tumors that occur during the first year of life are supratentorial. Between 2 and 12 years of age, 85 percent are infratentorial. After the age of 12 years, the relative incidence of supratentorial tumors increases.

III. Symptoms and signs

A. Symptoms

The most common symptoms include headaches, irritability, vomiting, and gait abnormalities. Morning headaches are most characteristic, but drowsiness and abnormal behavior are also quite common. Symptoms may be intermittent, particularly in very young children, who have open fontanelles.

B. Physical findings

Physical findings include enlarged or bulging fontanelles in very young children, and cerebellar abnormalities, papilledema, and sixth cranial nerve abnormalities in older children.

IV. Treatment and survival

Survival rates for patients with low-grade astrocytomas are high if the tumor can be surgically removed (>90% at five years) and low if the tumor is high grade (<10% at five years). Survival for medulloblastoma depends on both local recurrence (<25% with RT and surgery) and spinal metastases (approximately 35% incidence without prophylactic spinal irradiation); this tumor is invariably recurrent when treated with surgery alone.

Chemotherapy is now being used more frequently in children with brain tumors in an attempt to improve survival and to reduce the use of radiation, which has devastating effects in young children. RT is being deferred in children under three years of age.

Neuroblastoma

I. Epidemiology and etiology

Neuroblastoma is the most common congenital tumor and the most common tumor to occur during the first year of life. It rarely occurs in individuals more than 14 years of age. The incidence is 10 cases per million among children 0 to 4 years of age, and 4 per million among children 5 to 10 years of age. Rarely, family clusters are reported.