Astrocytoma and Pineoblastoma Arising Sequentially in the Fourth Ventricle of the Same Patient

Abstract

The sequential appearance of two different brain tumors in the same patient without intervening radiation or chemotherapy is a rare event, most often seen in hereditary cancer syndromes. We present one such case of sequential tumors, along with their molecular analysis. A 17-year-old male presented with a pilocytic astrocytoma arising in the fourth ventricle at the pontomedullary junction. Six and one half years later, a pineoblastoma was discovered in the fourth ventricle, rostral to the first tumor site. Both tumors were treated by gross-total surgical resection. Following resection of the pineoblastoma, the patient underwent craniospinal irradiation and systemic chemotherapy. Single-strand conformation polymorphism analysis showed that the patient had neither a germ-line mutation nor a somatic tumor mutation in the p53 tumor suppressor gene. Coupled with the lack of a family history of cancer, these data suggest that these were not manifestations of Li-Fraumeni syndrome, but rather two sporadic tumors which arose via a p53-independent mechanism.

The long‐term postsurgical prognosis of patients with pineoblastoma

“…Our search resulted in 109 publications 1, 3, 4, 7, 10, 17‐61. amounting to 299 nonduplicated, diaggregated patients who were treated for pineoblastoma (see Fig.…”

Comparative genomic hybridization in pineal parenchymal tumors

“…Cytogenetic and molecular data on pineal parenchymal tumors are sparse and have only been reported for nine pineocytomas (Rainho et al, 1992;Bello et al, 1993;Tsumanuma et al, 1995;Nozaki et al, 1998), and eight pineoblastomas or their cell lines (Sreekantaiah et al, 1989;Kees et al, 1994;Tsumanuma et al, 1995;Brockmeyer et al, 1997;Kees et al 1998). To date, no data are available for pineal parenchymal tumors of intermediate differentiation.…”

“…To date, no cytogenetic data exist on pineal parenchymal tumors of intermediate differentiation. Pineal parenchymal tumors have also been investigated for mutations of the TP53 tumor suppressor gene; however, no mutations were detected in any of the six pineocytomas (Tsumanuma et al, 1995;Nozaki et al, 1998) and five pineoblastomas examined (Tsumanuma et al, 1995;Brockmeyer et al, 1997). Furthermore, no amplifications of the proto-oncogenes MYC and MYCN were found in three pineoblastoma cell lines despite enhanced protein expression (Kees et al, 1994(Kees et al, , 1998.…”

Clinical, Pathological, and Surgical Outcomes for Adult Pineoblastomas

“…Diffusion restriction was demonstrated in both cases that had diffusion-weighted images available. Diffusion restriction Gener,6 has been suggested as a possible distinguishing characteristic at diagnosis (9) and a possible tumor marker at posttreatment follow-up (13).…”