For most diseases, this has been brought about largely as a result of the incorporation of chemotherapy as part of a multimodality approach, including surgery and radiotherapy. Increasingly, collaboration is across European boundaries, with clinical trials coordinated via the International Society of Paediatric Oncology (SIOP).Ĭurrently, approximately two-thirds of children treated for cancer can expect to be long-term survivors. In the UK, treatment is coordinated by the network of twenty-two Children’s Cancer and Leukaemia Group (CCLG) (formerly UKCCSG) paediatric oncology centres. In 1999, these were amalgamated to form the Children’s Oncology Group (COG), the largest paediatric oncology collaborative group in the world. In North America, clinical research has been coordinated via the Paediatric Oncology Group (POG), Children’s Cancer Group (CCG), Intergroup Rhabdomyosarcoma Study Group (IRSG) and National Wilms’ Tumour Study Group (NWTS). Paediatric oncology collaborative groups have been very successful at entering a high proportion of children into clinical trials. The evolution of the multidisciplinary care of children with cancer has been one of the success stories of modern oncology. Table 33.1 Surveillance, epidemiology and end results (SEER) programme registrations 1975–2001, annual incidence rate per 1 000 000 and proportion of children aged 0–14 with cancer Table 33.1 summarizes data on relative incidence of the various tumour types from the US Surveillance, Epidemiology and End Results (SEER) programme. The range of childhood cancers is very different from that seen in the adult population. Approximately one individual child in 500 will develop cancer before the age of 15. In the UK, approximately 1500 children below the age of 15 develop cancer each year. These results also support the view that MMB and medulloblastoma may have common tumorigenic origins, given their similar histologic and molecular features.Cancer in childhood is uncommon. The results of the current study demonstrated the frequent correlation of biphasic nodularity (as determined by MRI or CT) with discrete rhabdomyoblastic and primitive neuroectodermal islands (as revealed by microscopy) in MMB. At a median follow-up of 92 months (range, 23-187 months), 3 patients remain alive with no evidence of disease, 2 patients have died of disease, and 1 patient has died of secondary acute lymphocytic leukemia. All patients underwent macroscopic total resection and subsequently received chemotherapy and craniospinal (five patients) or local conformal (one patient) radiotherapy. All 4 tumors that were tested exhibited alterations in chromosome 17 or c-myc amplification. Large cell/anaplastic (five tumors), nodular/desmoplastic (two tumors), and classic (two tumors) medulloblastoma histologies were encountered either alone (five tumors) or in combination with each other (two tumors). Three tumors contained discrete, magnetic resonance imaging (MRI) T2-weighted-hypointense/computed tomography (CT)-hyperdense enhancing regions and separate hyperintense/hypodense nonenhancing regions, which correlated microscopically with geographic islands of primitive neuroectodermal and rhabdomyoblastic cells. Radiographically, all tumors were cerebellar and exhibited variable enhancement, and 50% of tumors had necrotic foci. The median age at diagnosis was 4.5 years (range, 0.83-7.5 years). A literature review also was conducted and focused on imaging and pathologic findings. Fluorescence in situ hybridization (FISH) data were available for four children. Jude Children's Research Hospital (Memphis, TN) between 19.
The authors conducted a retrospective review of the radiographic and pathologic characteristics, treatment, and clinical outcomes of six children with MMB who were treated at St. It is biphasic by microscopy, containing myoblastic and primitive neuroectodermal components. Medullomyoblastoma (MMB) is a rare cerebellar embryonal neoplasm that occurs almost exclusively in children.
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