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UNLABELLED: Medulloblastoma is an aggressive posterior fossa tumor which is rare in adult. We aim to study the clinical features and outcome of adult medulloblastoma patients, and to identify poor outcome predictors. PATIENTS AND METHODS: From January 1995 to December 2006, 35 adult patients (>15 years of age at the time of diagnosis) underwent surgery for medulloblastoma followed by full dose of radiotherapy (54 Gy), with or without systemic chemotherapy. RESULTS: The mean age was 24 years (maximum 52). There were 19 male and 16 female patients. CSF spread was documented in 23 patients. Eight patients developed relapse. Four patients developed isolated CNS relapse, two patients developed isolated lung metastasis and 2 patients developed both lung metastasis and CNS relapse. The craniospinal radiation dose ranged from 34 to 36 Gy and the total posterior fossa dose ranged from 54 to 56 Gy. The 10 year disease free survival rate reached 65% +/- 10. The relapse free survival was significantly different according to the M stage (M0, M1, M2 & 3), as none of the patients in the M0 group had relapsed while 4 patients in the M1 group and 4 patients in the M2&3 group relapsed (p=0.0026). CONCLUSION: The distinction between adult and pediatric medulloblastoma is not clear. Overall, M stage is an important prognostic factor. Although chemotherapy is a part of standard practice in pediatric treatment protocols, its role in older patients should be further evaluated in clinical studies.
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INTRODUCTION: Anaplastic Thyroid Cancer (ATC) is one of the most lethal malignancies with very short survival and extremely poor treatment outcome. ATC accounts for 2-5% of all thyroid cancers worldwide with an annual incidence of about 2 million. OBJECTIVE: To review the natural history and treatment outcome of ATC patients treated at King Faisal Specialist Hospital and Research Centre (KFSH and RC) located at Riyadh, Saudi Arabia. MATERIAL AND METHODS: Retrospective review of 120 Saudi cancer patients collected from registry data at KFSH & RC from 1976-2008. Search key words included: thyroid cancer, anaplastic, undifferentiated and not otherwise specified. Search was not restricted to particular age, gender, treatment or tumor size. Demographic information, baseline patient characteristics including date of diagnosis, type of treatment and date of death were obtained from KFSH & RC registry data and Saudi death registry. RESULTS: A total of 120 cases were identified at our cancer centre from 1976 to 2008. Of these total, 73 were females (60.8%) and 47 were males (39.2%). The average age at diagnosis was 63.34 +/- 12.8 years. Thirty-four patients underwent surgery (28.3%), 52 had a palliative radiation treatment (43.3%) and only 5 had chemotherapy (4.2%). The median survival was 53 days (0-457). CONCLUSION: Our review proves that ATC is rapidly fatal cancer and is unresponsive to currently available therapeutic options. More research is needed to understand the tumor biology and novel treatment options. KEYWORDS: thyroid cancer, anaplastic, undifferentiated, radiation treatment, chemotherapy, median survival, Riyadh and Saudi Arabia.
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AIMS: Aggressive fibromatosis is a locally aggressive infiltrative low-grade tumour, although pathologically benign, and it does not metastasise, yet it can cause serious local distressing symptoms by virtue of local destruction and impairment of local function. The aim of this study was to emphasise the role of radiotherapy and adequate surgery in the treatment of fibromatosis in patients presenting with newly diagnosed or recurrent disease and to analyse our treatment results over 15 years for this rare tumour type. MATERIALS AND METHODS: Fifty-four patients with confirmed diagnosis of aggressive fibromatosis treated at King Faisal Specialist Hospital between 1990 and 2006 were identified from our local cancer registry. Forty-seven patients had surgery: complete resection (R0) in 20 patients, incomplete surgery (R1/2) in 27 patients, and seven patients had biopsy only. Forty-five patients were treated with radiotherapy: 38 patients were treated with postoperative radiotherapy, three patients were treated with preoperative radiotherapy and four patients had radiotherapy as the only treatment. The radiotherapy dose ranged between 45 and 60Gy (median 50.4Gy). Three patients did not receive any form of treatment apart from biopsy, but were still included in the final analysis. RESULTS: Fifty-two per cent (28/54 patients) of our patient population had tumour recurrence when first presented to King Faisal Specialist Hospital. The median age was 29.5 years (range 2-63 years). The most common site of involvement was the extremities (28 patients). Among the 54 patients (with primary and recurrent presentation) there were 10 local recurrences, all of which were within the original primary site. The 5-year progression-free survival and overall survival rates for the whole group were 75 and 95%, respectively. Univariate and multivariate Cox regression analysis showed that the depth of invasion significantly affected progression-free survival. CONCLUSION: Aggressive fibromatosis is effectively treated with surgery and postoperative radiotherapy. Patients first presenting with tumour recurrence may still have local tumour control comparable with newly diagnosed patients.
