| Popis: |
Medulloblastoma is a rare disease in adult patients, it accounts for 6 % of the Posterior Fossa Tumors. Large, trials are often impossible and also access to biospecimens may be problematic Two years ago, during the spring meeting of AINO in Turin we planned an online web-data base on rare tumours of CNS. We decided to start our work with medulloblastoma, as example of multidisciplinary treatment for a rare tumour without codified guidelines. To simplify data collection we chose to include in the data base only the patients treated over the past five years Of the 225 clinicians- centres emailed; 32 (14 %) responded, 27 respondents identified their medical specialties: 11 medical neuro-oncologists, 8 radiation oncologists, 7 neurosurgeons, 1 neurologist 11 centres entered the data in the dbase During the last 5 years, 11 centres reported treated patients for a total of 53 patients 31 Male. Median age at surgery was 35 (17–80). Symptomatic hydrocephalus was found in 50 % of patients. Postoperative residual tumour was found in 10 patients, in 6 pts the residual tumor was less than 1.5 cm square, In 2 pts more than 1,5 cm square Fore other two patients the residue volume was missing. VPS was placed on 3 pts. Positive CFS was found in 3 patients but post op spinal tap was performed only in 20 pts. In eight patients the histological definition was limited to the diction of medulloblastoma non other specified In six patients SHH mutation was found High-risk disease defined as residual disease, metastatic dissemination or large cell- anaplastic histology we defined 14 patients but we have incomplete staging or missing information in 5 pts. After surgery Craniospinal radiotherapy was given to 92 % patients and only 2 had only cranial radiotherapy due to their low PS and the 85 years old patient was not treated. A boost into the posterior fossa was given to almost all patients. X Patients were treated also by chemotherapy and regimens used varied considerably. Recurrence take place in 25 patients, in 10 out of these patients as leptomenigeal dissemination. At recurrence 4 Pts were re-operated on. The radiotherapy re-treatment was administered in one patient. Six Patient were treated with Temozolomide as salvage treatment, plus intrathecal chemotherapy. The data collected on patients and their disease characteristics were consistent with the literature. Similar also relative incidence of different histological subtypes, risk status and tumour surgery. The data are partial due to quite low number of patients included at the moment in d-base. We have extended the observation period up to 10 years and we also have started to insert patients in a prospective way opening the study to the SIN and SINCH members. |
