Second neoplasms in pediatric patients with primary central nervous system tumors

Abstract

BACKGROUNDDetails on second neoplasms (SNs) following pediatric central nervous system (CNS) tumors are scant, because of the rarity of such SNs. The goal of the current study was to investigate and characterize these rare SNs.METHODSThe authors reviewed clinical and treatment data on all institutional patients age < 22 years at diagnosis of a primary CNS tumor who developed any type of SN. Patients with neurofibromatosis type 1 were excluded. Cumulative incidence rates were estimated, and putative risk factors were analyzed.RESULTSThe SNs investigated in the current study included 10 gliomas (42%), 5 meningiomas (21%), 2 desmoid tumors, 2 myelodysplastic syndromes, 2 basal cell carcinomas, 1 leukemia, 1 malignant fibrous histiocytoma, and 1 thyroid carcinoma. Twenty‐one patients had previously received radiotherapy, and 12 patients had received chemotherapy. The SN was related to a genetic cause in 7 patients (29%). Eleven patients died of their SNs, including 8 patients with glioma and 2 patients with myelodysplastic syndromes. The estimated 15‐year cumulative incidence rate for malignant SNs was 4%. Children with choroid plexus tumors had an estimated 10‐year cumulative incidence rate of 20.2%; 2 of those patients had germline TP53 mutations. Age ≤ 2 years was a significant risk factor (P = 0.016) for development of an SN only when patients with genetic conditions were included in the analysis. No significant difference in the estimated cumulative incidence of SNs was found among patients who had received different types of therapy.CONCLUSIONSThe risk of lethal SNs after pediatric CNS tumors is small. Young patients and patients with choroid plexus tumors appear to have an increased risk of SNs that is associated with genetic factors. Cancer 2004. © 2004 American Cancer Society.

Second brain tumors following central nervous system radiotherapy in childhood

“…[1][2][3][4][5] The cranial irradiation, especially the radiation dose and the field size, is known as the significant risk factor for developing second brain neoplasms. 1,[6][7][8][9][10][11] However, details of this relationship are still not clear.…”

A Novel <i>TP53</i> Germline Mutation in a Family with a History of Multiple Malignancies: Case Report and Review of the Literature

“…Since their 3 cases, there have been 17 additional cases, including this report, of primary CPC, occurring in patients with LFS/LFL, germline TP53 mutations, or both [12, 13, 15, 29,45,46,47,48,49,50]. The number of such cases may be underestimated since many reports do not distinguish between types of CNS tumors or divide CPTs into papillomas and carcinomas [1,2,3,4,5, 23, 25, 26, 51, 52].…”

Misdiagnosing recurrent medulloblastoma: the danger of examination and imaging without histological confirmation

“…We know that pe diatric patients with primary malignant brain tumors who received radiation therapy have an increased risk of devel oping a secondary malignant neoplasm, the majority of which are brain tumors and include meningioma, glioblas toma, and anaplastic astrocytoma. 4,6 Studies have shown a high rate of mortality associated with secondary gliomas, but a high survival rate of secondary meningiomas. 4 The utility of screening for recurrent medulloblasto ma remains controversial, especially in the absence of es tablished effective salvage therapies; however, as current protocols still recommend routine surveillance scanning, the physician must be prepared to critically interpret posi tive results.…”

“…4,6 Studies have shown a high rate of mortality associated with secondary gliomas, but a high survival rate of secondary meningiomas. 4 The utility of screening for recurrent medulloblasto ma remains controversial, especially in the absence of es tablished effective salvage therapies; however, as current protocols still recommend routine surveillance scanning, the physician must be prepared to critically interpret posi tive results. As we have illustrated, caution must be taken when basing the diagnosis of recurrent medulloblastoma on symptomatology and MRI alone.…”