Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children's Cancer Group 921 randomized phase III study.
PURPOSE: From 1986 to 1992, "eight-drugs-in-one-day" (8-in-1) chemotherapy both before and after radiation therapy (XRT) (54 Gy tumor/36 Gy neuraxis) was compared with vincristine, lomustine (CCNU), and prednisone (VCP) after XRT in children with untreated, high-stage medulloblastoma (MB). PATIENTS AND METHODS: Two hundred three eligible patients with an institutional diagnosis of MB were stratified by local invasion and metastatic stage (Chang T/M) and randomized to therapy. Median time at risk from study entry was 7.0 years. RESULTS: Survival and progression-free survival (PFS) +/- SE at 7 years were 55%+/-5% and 54%+/-5%, respectively. VCP was superior to 8-in-1 chemotherapy, with 5-year PFS rates of 63%+/-5% versus 45%+/-5%, respectively (P = .006). Upon central neuropathology review, 188 patients were confirmed as having MB and were the subjects for analyses of prognostic factors. Children aged 1.5 to younger than 3 years had inferior 5-year estimates of PFS, compared with children 3 years old or older (P = .0014; 32%+/-10% v 58%+/-4%, respectively). For MB patients 3 years of age or older, the prognostic effect of tumor spread (MO v M1 v M2+) on PFS was powerful (P = .0006); 5-year PFS rates were 70%+/-5%, 57%+/-10%, and 40%+/-8%, respectively. PFS distributions at 5 years for patients with M0 tumors with less than 1.5 cm2 of residual tumor, versus > or = 1.5 cm2 of residual tumor by scan, were significantly different (P = .023; 78%+/-6% v 54%+/-11%, respectively). CONCLUSION: VCP plus XRT is a superior adjuvant combination compared with 8-in-1 chemotherapy plus XRT. For patients with M0 tumors, residual tumor bulk (not extent of resection) is a predictor for PFS. Patients with M0 tumors, > or = 3 years with < or = 1.5 cm2 residual tumor, had a 78%+/-6% 5-year PFS rate. Children younger than 3 years old who received a reduced XRT dosage had the lowest survival rate. (+info)
Hemangioblastoma mimicking tentorial meningioma: preoperative embolization of the meningeal arterial blood supply--case report.
A 72-year-old male presented with a primary hemangioblastoma of the posterior fossa with unusual dural attachment and meningeal arterial blood supply from the external carotid artery and marginal tentorial artery. Preoperative embolization facilitated complete resection of the tumor with no resultant neurological deficit. Hemangioblastoma must be included in the differential diagnosis of tumors with dural involvement. Preoperative embolization is very useful in such tumors. (+info)
Activation of the CD95 (APO-1/Fas) pathway in drug- and gamma-irradiation-induced apoptosis of brain tumor cells.
Chemotherapeutic agents and gamma-irradiation used in the treatment of brain tumors, the most common solid tumors of childhood, have been shown to act primarily by inducing apoptosis. Here, we report that activation of the CD95 pathway was involved in drug- and gamma-irradiation-induced apoptosis of medulloblastoma and glioblastoma cells. Upon treatment CD95 ligand (CD95-L) was induced that stimulated the CD95 pathway by crosslinking CD95 via an autocrine/paracrine loop. Blocking CD95-L/receptor interaction using F(ab')2 anti-CD95 antibody fragments strongly reduced apoptosis. Apoptosis depended on activation of caspases (interleukin 1beta-converting enzyme/Ced-3 like proteases) as it was almost completely abrograted by the broad range caspase inhibitor benzyloxycarbonyl-Val-Ala-Asp-fluoromethyl ketone. Apoptosis was mediated by cleavage of the receptor proximal caspase FLICE/MACH (caspase-8) and the downstream caspase CPP32 (caspase-3, Apopain) resulting in cleavage of the prototype caspase substrate PARP. Moreover, CD95 was upregulated in wild-type p53 cells thereby increasing responsiveness towards CD95 triggering. Since activation of the CD95 system upon treatment was also found in primary medulloblastoma cells ex vivo, these findings may have implications to define chemosensitivity and to develop novel therapeutic strategies in the management of malignant brain tumors. (+info)
Cicatricial fibromatoses usually occur in the anterior abdominal wall or in the extremities, but rarely in the scalp or the soft tissues of the neck. We report a case of desmoid fibromatosis that developed in a 15-year-old boy 8 months after surgery for cerebellar medulloblastoma. (+info)
Unexpected stomach uptake of technetium-99m-MDP.
Two pediatric cases are described in which the results of each patient's bone scan demonstrated abnormal stomach uptake. There have been a number of reports in the literature describing stomach uptake of bone agents, however, it is an uncommon finding. (+info)
Clinical features and outcomes in patients with non-acoustic cerebellopontine angle tumours.
OBJECTIVES: Non-acoustic tumours of the cerebellopontine angle differ from vestibular schwannomas in their prevalence, clinical features, operative management, and surgical outcome. These features were studied in patients presenting to the regional neuro-otological unit. METHODS: A retrospective analysis of clinical notes identified 42 patients with non-acoustic tumours of the cerebellopontine angle. Data were extracted regarding presenting clinical features, histopathological data after surgical resection, surgical morbidity and mortality, and clinical outcome (mean 32 months follow up). RESULTS: The study group comprised 25 meningiomas (60%), 12 epidermoid cysts/cholesteatomata (28%), and five other tumours. In patients with meningiomas, symptoms differed considerably from patients presenting with vestibular schwannomas. Cerebellar signs were present in 52% and hearing loss in only 68%. Twenty per cent of patients had hydrocephalus at the time of diagnosis. After surgical resection, normal facial nerve function was preserved in 75% of cases. In the epidermoid group, fifth, seventh, and eighth nerve deficits were present in 42%, 33%, and 66% respectively. There were no new postoperative facial palsies. There were two recurrences (17%) requiring reoperation. Overall, there were two perioperative deaths from pneumonia and meningitis. CONCLUSIONS: Patients with non-acoustic lesions of the cerebellopontine angle often present with different symptoms and signs from those found in patients with schwannomas. Hearing loss is less prevalent and cerebellar signs and facial paresis are more common as presenting features. Hydrocephalus is often present in patients presenting with cerebellopontine angle meningiomas. Non-acoustic tumours can usually be resected with facial nerve preservation. (+info)
Medullomyoblastoma: A case report.
Medullomyoblastoma is a rare tumour seen in childhood. We report a medullomyoblastoma occurring in the cerebellar vermis of a 4 year old boy. The light microscopic features, immunohistochemistry and histogenesis are described. (+info)
Choroid plexus papilloma of cerebellopontine angle with extension to foramen magnum.
A case of choroid plexus papilloma resembling meningioma of cerebellopontine (CP) angle with its extension to foramen magnum is presented. Occurrence of this tumour in CP angle is very rare. Its extension towards foramen magnum is further rare. It was a real diagnostic enigma preoperatively as the tumour was resembling meningioma upto some extent on radiological study. Retromastoid craniectomy with microsurgical excision of tumour and its extension was achieved in toto. Tumour was attached to few rootlets of lower cranial nerves which were preserved. Attachment of the tumour with lower cranial nerves again caused diagnostic confusion with neurofibroma intraoperatively. (+info)