Pheochromocytomas and extra-adrenal paragangliomas are rare tumors arising from neural crest tissue that develops into sympathetic and parasympathetic paraganglia throughout the body.
The most recent World Health Organization classification utilizes the term pheochromocytoma exclusively for tumors arising from the adrenal medulla, and the term extra-adrenal paraganglioma for similar tumors that arise from other locations.
Incidence and Mortality
The incidence of pheochromocytoma is 2...
Attempting aggressive surgical resection is the first step in the management of newly diagnosed CNS PNETs, although studies have yet to demonstrate that the extent of resection is predictive of outcome.[1,2,3]
CNS PNETs are often amenable to resection; in reported case series, 50% to 60% of patients were totally or near-totally resected.[1,2]
After surgery, children with CNS PNETs usually receive treatment similar to that received by children with high-risk medulloblastoma.
Conventionally, patients are treated with radiation to the entire neuraxis with local boost radiation therapy, as given for medulloblastoma. However, the local boost radiation therapy may be problematic because of the size of the tumor and its location in the cerebral cortex. Also, there is no definitive evidence that craniospinal radiation therapy is superior to radiation to the primary tumor site alone in children with nondisseminated lesions.[1,2,3]
The chemotherapeutic approaches during and after radiation therapy are similar to those used for children with high-risk medulloblastoma. Three-year to 5-year overall survival rates of 25% to 50% have been noted.[1,2,3]; [Level of evidence: 2A]; [Level of evidence: 3iiiB]
Children Aged 3 Years and Younger
Standard treatment options
Treatment of children aged 3 years and younger with CNS PNETs is similar to that outlined for children aged 3 years and younger with medulloblastoma. (Refer to the medulloblastoma Children Aged 3 Years and Younger section of this summary for more information).
With the use of chemotherapy alone, outcome has been variable, with survival rates at 5 years ranging between 0% and 50%.[6,7,8]; [Level of evidence: 2Di]
Treatment options under clinical evaluation
The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.
COG-ACNS0334 (NCT00336024)(Combination Chemotherapy Followed By Peripheral Stem Cell Transplant in Treating Young Patients With Newly Diagnosed Supratentorial PNETs or High-Risk Medulloblastoma): This Children's Oncology Group phase III randomized trial is studying children aged 3 years and younger with high-risk medulloblastoma or CNS PNETs. Patients are randomly assigned to intensive induction chemotherapy with or without methotrexate followed by consolidation with hematopoietic stem cell rescue.