✓Radiation-induced glioblastoma multiforme (GBM) is a rare complication of radiotherapy. The authors report such a case occurring 10 years after treatment of cerebellar medulloblastoma. The patient was a 15-year-old boy who had undergone a gross-total removal of a medulloblastoma and received radiation therapy at the age of 5 years. He had experienced no tumor recurrences for 10 years until a new enhancing mass was found at the original site of the medulloblastoma. Following its resection the new lesion was found to be a GBM and there was no evidence of a medulloblastoma. The second tumor developed at the same site as the previous one after a sufficient latent period and fulfilled the criteria for a radiation-induced neoplasm. The original tumor cells expressed synaptophysin without p53 overexpression, a characteristic feature of medulloblastomas. In contrast, cells from the later tumor expressed glial fibrillary acidic protein and p53 but not synaptophysin. A sequence analysis of the p53 gene showed deletion at codon 233 and a C to G transition at codon 278 in the GBM but no mutation in the medulloblastoma. A GBM specimen revealed no amplification of the epidermal growth factor receptor compared with a normal control specimen. In conclusion, the clinical features of a radiation-induced GBM are similar to that of the primary GBM, whereas its genetic alterations render it a secondary GBM. These findings indicate that radiation-induced GBM should be considered a distinct clinical entity.
Seung-Yeob Yang, Kyu-Chang Wang, Byung-Kyu Cho, Young-Yim Kim, Su-Young Lim, Sung-Hye Park, Il Han Kim and Seung-Ki Kim
Kyung Sun Song, Ji Hoon Phi, Byung-Kyu Cho, Kyu-Chang Wang, Ji Yeoun Lee, Dong Gyu Kim, Il Han Kim, Hyo Seop Ahn, Sung-Hye Park and Seung-Ki Kim
Glioblastoma is the most common primary malignant brain tumor; however, glioblastoma in children is less common than in adults, and little is known about its clinical outcome in children. The authors evaluated the long-term outcome of glioblastoma in children.
Twenty-seven children were confirmed to have harbored a glioblastoma between 1985 and 2007. The clinical features and treatment outcomes were reviewed retrospectively. All patients underwent resection; complete resection was performed in 12 patients (44%), subtotal resection in 12 patients (44%), and biopsy in 3 patients (11%). Twenty-four patients (89%) had radiation therapy, and 14 (52%) patients received chemotherapy plus radiation therapy. Among the latter, 5 patients had radiation therapy concurrent with temozolomide chemotherapy. Four patients with small-size recurrent glioblastoma received stereotactic radiosurgery.
The median overall survival (OS) was 43 months, and the median progression-free survival was 12 months. The OS rate was 67% at 1 year, 52% at 2 years, and 40% at 5 years. The median OS was significantly associated with tumor location (52 months for superficially located tumors vs 7 months for deeply located tumors; p = 0.017) and extent of removal (106 months for completely resected tumors vs 11 months for incompletely resected tumors; p < 0.0001).
The prognosis of glioblastoma is better in children than in adults. Radical resection followed by concurrent chemoradiation therapy may be the initial treatment of choice.
Ji Yeoun Lee, Bo Sung Kim, Ji Hoon Phi, Hyoung Jin Kang, Sung-Hye Park, Kyu-Chang Wang, Il Han Kim, Byung-Kyu Cho and Seung-Ki Kim
Primary sarcomas of the CNS are rare and are sometimes associated with chronic subdural effusion (SDE). Approximately 10 patients with such presentations have been reported. The authors report the case of a 5-year-old boy with multiple subdural masses and SDE. An SDE had been diagnosed when the patient was 2 months old, and he received a subduroperitoneal shunt when he was 5 months old. Since then, he had been clinically stable and well for 5 years. When he presented with acute headache, nausea, and vomiting, a newly developed tumor was found. Near-total resection of the tumor was performed, and the mass was diagnosed as an embryonal-type rhabdomyosarcoma. The child was given radiation therapy and 13 cycles of chemotherapy and is still free of disease 13 months after surgery.
Ji Hoon Phi, Seung-Ki Kim, Joongyub Lee, Chul-Kee Park, Il Han Kim, Hyo Seop Ahn, Hee Young Shin, In-One Kim, Hee-Won Jung, Dong Gyu Kim, Sun Ha Paek and Kyu-Chang Wang
Intracranial germ cell tumors (GCTs) frequently present with bifocal lesions in both the suprasellar and pineal areas. The pathogenesis of these bifocal GCTs has been the subject of controversy. Bifocal GCTs may be caused by synchronous tumors or by metastatic spread of tumor cells from one site to the other. The prognosis associated with bifocal GCTs has also been a cause of concern.
The authors constructed a single-institution patient cohort comprising 181 patients with intracranial GCTs. The clinical characteristics of bifocal GCTs were compared with those of suprasellar and pineal GCTs.
Bifocal GCTs were observed in 23 patients (12.8%). Eighteen patients presented with bifocal GCTs that were diagnosed as germinomas, but 5 patients exhibited mixed GCTs. Analyses of age distributions and comparisons of tumor sizes were compatible with a model of a metastatic origin of bifocal GCTs. Eleven patients (47.8%) presenting with bifocal GCTs exhibited tumor seeding at presentation. Tumor seeding was significantly associated with bifocal lesions (p < 0.001). Patients with bifocal germinomas showed significantly shorter event-free survival and overall survival than did those presenting with germinomas from a single site of origin.
Bifocal GCTs are not restricted to germinomas, as had been previously reported, but do include mixed GCTs. The authors hypothesize that bifocal GCTs may result from the metastatic spread of suprasellar or pineal GCTs. The bifocal presentation of germinomas may be a poor prognostic sign and should alert clinicians to the possibility of a disseminated disease.