The initial approach to the patient is to evaluate the following parameters:
Detection of a monoclonal (or myeloma) protein (M protein) in the serum or urine.
Detection of more than 10% of plasma cells on a bone marrow examination.
Detection of lytic bone lesions or generalized osteoporosis in skeletal x-rays.
Presence of soft tissue plasmacytomas.
Serum albumin and beta-2-microglobulin levels.
Detection of free kappa and lambda serum immunoglobulin light...
Attempting aggressive surgical resection is the first step in the management of newly diagnosed central nervous system primitive neuroectodermal tumors (CNS PNETs), although studies have yet to demonstrate that the extent of resection is predictive of outcome.[1,2,3] After surgery, children with CNS PNETs usually receive treatment similar to that received by children with poor-risk medulloblastoma. Best results have been obtained after 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. The chemotherapeutic approaches during and after radiation therapy are similar to those used for children with poor-risk medulloblastoma. Three- to 5-year overall survival (OS) rates of 40% to 50% have been noted.[1,2,3]; [Level of evidence: 2A]; [Level of evidence: 3iiiB] Patients with disseminated disease at the time of diagnosis have poorer OS, with reported survival rates at 5 years ranging from 20% to 30%.[1,2,3]
Children Aged 3 Years and Younger
Standard treatment options
Treatment of children aged younger than 3 years with CNS PNETs is similar to that outlined for children with medulloblastoma. 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(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 including or excluding methotrexate followed by consolidation with hematopoietic stem cell rescue.
Current Clinical Trials
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood supratentorial primitive neuroectodermal tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Cohen BH, Zeltzer PM, Boyett JM, et al.: Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a Childrens Cancer Group randomized trial. J Clin Oncol 13 (7): 1687-96, 1995.
Reddy AT, Janss AJ, Phillips PC, et al.: Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy. Cancer 88 (9): 2189-93, 2000.
Timmermann B, Kortmann RD, Kühl J, et al.: Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91. J Clin Oncol 20 (3): 842-9, 2002.
Chintagumpala M, Hassall T, Palmer S, et al.: A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET. Neuro Oncol 11 (1): 33-40, 2009.
Johnston DL, Keene DL, Lafay-Cousin L, et al.: Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report. J Neurooncol 86 (1): 101-8, 2008.
Geyer JR, Sposto R, Jennings M, et al.: Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children's Cancer Group. J Clin Oncol 23 (30): 7621-31, 2005.
Marec-Berard P, Jouvet A, Thiesse P, et al.: Supratentorial embryonal tumors in children under 5 years of age: an SFOP study of treatment with postoperative chemotherapy alone. Med Pediatr Oncol 38 (2): 83-90, 2002.
Grill J, Sainte-Rose C, Jouvet A, et al.: Treatment of medulloblastoma with postoperative chemotherapy alone: an SFOP prospective trial in young children. Lancet Oncol 6 (8): 573-80, 2005.
Fangusaro J, Finlay J, Sposto R, et al.: Intensive chemotherapy followed by consolidative myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) in young children with newly diagnosed supratentorial primitive neuroectodermal tumors (sPNETs): report of the Head Start I and II experience. Pediatr Blood Cancer 50 (2): 312-8, 2008.