Assessing the feasibility and validity of the Toronto Childhood Cancer Stage Guidelines: a population-based registry study.

BACKGROUND: Cancer stage at diagnosis is crucial for assessing global efforts to increase awareness of childhood cancer and improve outcomes. However, consistent information on childhood cancer stage is absent from population cancer registries worldwide. The Toronto Childhood Cancer Stage Guidelines, compiled through an international consensus process, were designed to provide a standard framework for collection of information on stage at diagnosis of childhood cancers. We aimed to assess the feasibility of implementing the Toronto Guidelines within a national population cancer registry. METHODS: We did a population-based registry study using data from the Australian Childhood Cancer Registry and included data from children aged 0-14 years diagnosed between Jan 1, 2006, and Dec 31, 2010 with one of 16 childhood cancers listed in the Toronto Guidelines (acute lymphoblastic leukaemia, acute myeloid leukaemia, Hodgkin's lymphoma, non-Hodgkin lymphoma, neuroblastoma, Wilms' tumour, rhabdomyosarcoma, non-rhabdomyosarcoma soft tissue sarcoma, osteosarcoma, Ewing's sarcoma, retinoblastoma, hepatoblastoma, testicular cancer, ovarian cancer, medulloblastoma, and ependymoma). We extracted data from medical records, and assigned stage according to the Tier 1 criteria (basic) and Tier 2 criteria (more detailed, requiring data from cytology, imaging, and other diagnostic tests, where available) using computer algorithms derived from the Toronto Guidelines. Additionally, expert reviewers independently assigned Tier 2 stage to a random subsample of 160 cases (ten per malignancy type). Feasibility of the guidelines was assessed on the percentage of cases that could be staged, agreement between stage assigned by the algorithms and the expert reviewers, and the mean time (min) taken to collect the required data. FINDINGS: We obtained data for 1412 eligible children. Stage could be assigned according to Tier 2 criteria for 1318 (93%) cases, ranging from 48 (84%) of 57 cases of non-rhabdomyosarcoma soft tissue sarcoma to 46 (100%) cases of hepatoblastoma. According to Tier 1 criteria, stage could be assigned for 1329 (94%) cases, ranging from 131 (87%) of 151 cases of acute myeloid leukaemia to 46 (100%) cases of hepatoblastoma. By contrast, stage at diagnosis was recorded by the treating physician for 555 (39%) of the 1412 cases. The computer algorithm assigned the same stage as did one or more independent expert reviewers in 155 (97%) of the 160 cases assessed. The mean time taken to review medical records and extract the required data was 18·0 min (SD 9·5 per case). INTERPRETATION: The Toronto Guidelines provide a highly functional framework that can be used to assign cancer stage at diagnosis using data routinely available in medical records for most childhood cancers. Data on staging have the potential to inform interventions targeting improved diagnosis and survival. FUNDING: Cancer Australia.

Surgical resection and long-term survival outcome for non-small cell lung cancer: a comparison of Victorian population-based studies spanning a decade.

AIM: A survey of management of lung cancer diagnosed in Victoria in 2003 was commissioned by the Victorian Cooperative Oncology Group to identify gaps in the management of this disease. Results from a similar survey in 1993 were available to identify differences in the disease, management and outcomes. This paper details results of the surgically managed subset within the larger study. METHODS: All patients diagnosed with lung cancer in the first 6 months of 2003 were identified from the Victorian Cancer Registry. Registry research staff completed a detailed questionnaire using primary source documents from hospitals and consulting rooms. The survey data were then de-identified with respect to patient and treating clinician prior to statistical analysis by the investigators. RESULTS: From eligible cases identified, non-small cell lung cancer was confirmed in 655 cases with a minimum of 6 years of follow-up. Thoracotomy was performed in 145 cases (22%), but only 130 received the intended resection. Compared with 1993, significant differences were increased use of preoperative positron emission tomography (PET) scanning (79% vs 0%), relatively fewer resections (20% vs 25%), lower pneumonectomy rate (14% vs 25%) and higher sub-lobar resection rate (22% vs 11%). The 30-day mortality remained below 2%. Positive resection margin (21%) and abandoned resection rates (10%) were much higher than expected. Overall 5-year survival was 42%, unchanged from 1993. CONCLUSION: Irrespective of widespread introduction of PET scanning, thoracotomy without resection was common. While operative mortality and overall survival were well within benchmark standards, futile thoracotomy and positive resection margin rates were unacceptably high.

Lung cancer in Victoria: are we making progress?

OBJECTIVES: To identify areas to improve patient management in lung cancer, which remains the greatest cause of death from cancer in Australia. DESIGN AND SETTING: Retrospective survey of all cases of lung cancer reported to the Victorian Cancer Registry from 1 January to 30 June 2003 and followed up for 5 years. MAIN OUTCOME MEASURES: Patient and disease characteristics, investigations, staging, treatment, cause of death, survival. RESULTS: 841 patients were included. Smoking data were available for 799, of whom 63 (7.9%) had never smoked. Of 655 non-small cell lung cancer (NSCLC) cases, 198 (30.2%) were treated with curative intent, 125 (19.1%) by surgery and 73 (11.1%) by radiotherapy with or without chemotherapy. Only 7 (6.9%) of surgical patients with complete R0 resection had adjuvant chemotherapy. Of 101 small cell lung cancer (SCLC) cases, a third had limited stage disease which was mostly treated with curative intent by chemotherapy with or without radiotherapy. Patients whose cases were discussed at a multidisciplinary meeting (MDM) were significantly more likely to receive anticancer treatment and had longer survival; on multivariate analysis, MDM discussion was an independent prognostic factor. Compared with a similar survey 10 years earlier, the median age of patients diagnosed with lung cancer had increased by almost 3 years, the proportion of affected men decreased and adenocarcinoma was more frequent, while 10% of patients continued to have no pathologically confirmed diagnosis and 26% continued to receive no anticancer treatment. The number of patients with NSCLC who went on to a definitive surgical procedure fell with no detriment to survival, which likely reflected better staging with the introduction of positron emission tomography scanning. CONCLUSIONS: Opportunities to improve patient management included increasing the proportion with a pathologically confirmed diagnosis and greater use of postsurgical adjuvant chemotherapy. A high proportion of patients received no treatment, with underuse of chemotherapy and radiotherapy. Critically, the low rate of case discussions at MDMs needs to increase. However, effective strategies are required to identify cases early, as over two-thirds currently present with incurable disease.

Pancreatic cancer: surgical management and outcomes after 6 years of follow-up.

OBJECTIVE: To describe the management and outcomes of a population-based cohort of patients with pancreatic cancer in Victoria, Australia. DESIGN, SETTING AND PATIENTS: Retrospective study based on questionnaires completed from medical histories of patients diagnosed with pancreatic cancer during 2002-2003 in Victoria who were identified from the Victorian Cancer Registry and followed up for 6 years. MAIN OUTCOME MEASURES: Proportion of patients receiving each form of treatment, 30-day mortality, median survival, and 5-year and 6-year survival. RESULTS: Of 1044 patients with pancreatic cancer identified, 927 were eligible for the study, and questionnaires were completed for 830 (response rate, 89.5%); 67 patients with ampulla of Vater and neuroendocrine tumours were excluded. Of the 763 remaining patients (median age, 72 years), notification of death was available for 747 (97.9%). Most patients (n = 548) had tumours in the head and neck of the pancreas. Resection was performed in a total of 87 patients (11.4%). Patients managed with Whipple resection (n = 75) had a 30-day mortality rate of 5.3% and median survival of 16.3 months. A relatively large number of surgeons (n = 31) each performed a modest number of Whipple resections during the study period. Jaundice was palliated with biliary stents (n = 240) and bypass surgery (n = 99). Survival was shortest in those treated with best supportive care (median, 2.3 months for those with head and neck of pancreas tumours, and 3.4 months for body and tail of pancreas tumours). Of the 20 patients who survived to 5 years, 10 did not have histological confirmation of carcinoma and were presumably false-positive diagnoses, and three of the 10 patients who did have positive histological results had experienced recurrent disease by 6-year follow-up. CONCLUSIONS: Most outcomes in Victoria compared favourably with other studies. Prognosis for patients with carcinoma of the pancreas is grim, with few long-term survivors. Six-year survival appears to be a better proxy for cure than 5-year survival.

Gender-specific activity of chemotherapy correlates with outcomes in chemosensitive cancers of young adulthood.

Good evidence indicates that adolescents and young adults (AYAs) with cancer do badly compared with children with similar cancers. The reasons are poorly understood. Australian registry data on 14,824 cancers of adolescence and young adulthood seen between 1982 and 2002 were reviewed. A detailed substudy of clinical characteristics was analyzed from 179 AYAs with Hodgkin lymphoma (HL), Ewing sarcoma (ES) or osteosarcomas (OS) treated at a single institution. Despite significant improvements in survival for both groups over the period in question, for acute lymphoblastic leukaemia, rhabdomyosarcoma, ES, OS and HL, survival for AYAs was worse than for children. For ES, OS and HL, the survival gap occurred almost entirely in males (Hazard ratios compared with female AYAs of 1.8 [p < 0.01], 1.4 [p = 0.03] and 1.5 [p < 0.01] respectively). Survival outcomes from ES, OS and HL for female AYAs were not significantly different from children of either sex. For brain tumors and thyroid cancers, which are primarily treated surgically, there were no gender-related differences in outcomes. Although no differences in tumor stage or compliance were identified, male AYAs experienced less toxicity and lower response rates to chemotherapy (p = 0.008). Young males account almost entirely for excess mortality from chemosensitive cancers of adolescence and young adulthood compared to children, which may be due to relative underdosing with current chemotherapy dosing algorithms.

The management of primary cutaneous melanoma in Victoria in 1996 and 2000.

OBJECTIVE: To describe tumour characteristics and clinical management of melanomas newly diagnosed in 1996 and in 2000--before and after publication of the clinical practice "Guidelines for the management of cutaneous melanoma" by the Australian Cancer Network (1997), and their endorsement by the National Health and Medical Research Council (NHMRC) and republication (1999). DESIGN AND SETTING: Survey of clinicians involved in the management of patients with melanoma sampled from the Victorian Cancer Registry. The Registry is notified of all cases of cancer diagnosed by pathology laboratories and hospitals in both the public and private health sectors in the state of Victoria. PATIENTS: People with a cutaneous melanoma newly diagnosed in 1996 and 2000. All invasive melanomas > 1.50 mm in thickness were included, and for each year random samples were selected of 100 each of invasive melanomas 0.76-1.50 mm in thickness, invasive melanomas < or = 0.75 mm, and in-situ melanomas, plus 50 melanomas of unknown thickness. MAIN OUTCOME MEASURES: Biopsy method, adequacy of pathology reporting, adequacy of definitive excision (compared with margins recommended by the Guidelines), and follow-up procedures. RESULTS: The use of partial biopsies increased between 1996 and 2000. Recommended margins of definitive excision were used in only 33.6% of cases. Margins were smaller than recommended for 36% of in-situ melanomas, risking recurrence of primary melanoma. Documented follow-up examinations for subsequent primary skin malignancy were uncommon (6%). CONCLUSIONS: Many aspects of the management of primary cutaneous melanoma appear not to meet the recommendations of the published Guidelines. Further studies to explore the reasons for failure to meet the Guideline recommendations are needed.

Management of superficial bladder cancer in Victoria: 1990 and 1995.

BACKGROUND: A retrospective survey of medical practitioners was undertaken to describe the tumour characteristics, management and outcomes of all superficial bladder cancers newly diagnosed in 1990 and in 1995 in Victoria. METHODS: Cases were identified from the population cancer registry. The survey was conducted in 1999 and the cohort followed up until 2000 to obtain at least 5 years of follow-up data on all patients, in particular to identify recurrence of tumour as assessed at surveillance cystoscopy and progression to muscle invasive cancer. RESULTS: Tumour recurrence was observed in 390/610 patients (63.9%), of whom 56.9% had their recurrence noted at the first check cystoscopy. Ultimately 43 (6.3%) of patients progressed to invasive disease, with this subgroup demonstrating 5-year overall survival of 35% (95% confidence interval (CI) 21-49%). Ultimately survival was proportional to the extent of tumour invasion, being greater in low-risk patients (76%, 95% CI 72-80%, mucosal disease only) than in high-risk patients (46%, 95% CI 36-56%, lamina propria invasion noted at diagnosis). CONCLUSIONS: In low-risk subgroups of patients with superficial transitional cell carcinoma, the frequency of surveillance cystoscopy may be able to be reduced to levels in accordance with established European guidelines without a likely impact on patient survival. Where progression to muscle invasive disease does ensue, more aggressive management may be warranted in order to try to improve survival.

Cancer in adolescents and young adults: treatment and outcome in Victoria.

OBJECTIVES: To describe the location of treatment, recruitment to clinical trials and outcomes for adolescents and young adults treated for cancer in Victoria. DESIGN AND SETTING: Retrospective review of all adolescents and young adults aged 10-24 years diagnosed with cancer between 1992 and 1996, identified from the Victorian Cancer Registry. MAIN OUTCOME MEASURES: Treatment regimen (clinical trial, treatment protocol or neither), compliance with treatment and 5-year survival. RESULTS: Questionnaires were completed for 576 of 665 eligible adolescents and young adults (87% response rate). Recruitment into clinical trials decreased with increasing age. Adolescents aged 10-19 years were more likely to be recruited to a clinical trial if treated at a paediatric hospital. For all cancers, 5-year survival was similar across the age groups and was not influenced by the place of treatment. Only 1% of adolescents and young adults failed to complete planned therapy due to non-compliance. CONCLUSIONS: Despite a similar incidence of cancer to that in younger children, adolescents and young adults with cancer are poorly recruited into clinical trials in Victoria. Establishment of a cancer resource network in Victoria may provide information to both paediatric and adult oncologists about currently available clinical trials.

Ovarian cancer: patterns of care in Victoria during 1993-1995.

OBJECTIVE: To describe the management of and outcomes in patients with newly diagnosed ovarian cancer during 1993, 1994 and 1995 in Victoria. DESIGN AND SETTING: Retrospective cohort study conducted by surveying doctors involved in managing incident ovarian cancer cases identified from the population-based Victorian Cancer Registry. The survey was conducted in 1997 and the cohort was followed up until the end of 1999 to obtain at least four years of follow-up data on all patients. PATIENTS: All women with invasive epithelial ovarian cancer diagnosed during 1993, 1994 and 1995. MAIN OUTCOME MEASURES: Reported management in terms of staging, treatment and survival. RESULTS: Management details were obtained for 84.5% (562/665) of eligible patients. Median age at diagnosis was 66 years (range, 22-98 years). Surgery was the primary therapy in 77.2% of women (434/562). Only one in three women had adequate surgery, which was less likely to be performed by general gynaecologists and general surgeons than gynaecological oncologists (21.3% [35/164] v 13.3% [8/60] v 52% [105/202]). After surgery 78.6% of women (341/434) received chemotherapy, usually with platinum-based regimens. The overall five-year relative survival was 46% for women treated surgically; poor survival was related to increasing age, later tumour stage, presence of ascites, residual disease > 2 cm and poorer histological differentiation of the tumour. CONCLUSIONS: For optimal care a preoperative carcinoma antigen (CA)-125 assay, chest x-ray and pelvic ultrasound should be performed, and early referral to a multi-disciplinary unit for definitive surgery is advised. Every effort should be made to adequately stage or debulk the tumour. Women with high-risk early-stage and advanced disease should be considered for platinum-based chemotherapy.

Rectal cancer in Victoria in 1994: patterns of reported management.

BACKGROUND: A retrospective survey of medical practitioners was conducted to describe the management of patients newly diagnosed with rectal cancer in 1994, prior to the publication of best practice guidelines. METHODS: A sample of 908 patients with rectal cancer diagnosed between 1 January and 31 December 1994 was identified from the Victorian Cancer Registry. Questionnaires were then sent to the treating doctor(s) for completion. The topics covered by the questionnaires included: reported management by method of diagnosis; staging investigations; and treatment by surgery, chemotherapy and radiotherapy. RESULTS: Seven hundred and twenty-six (80%) of 908 eligible patients were surveyed. Surgery was the primary treatment in 681 (93.8%) with curative intent in 483 (70.9%) of these cases. One- third (163; 33.7%) of curative cases were pathologically staged as Dukes' C. Almost all patients (96%) were symptomatic, and three-quarters were referred by general practitioners to 166 surgeons. One-third (221; 32.5%) underwent liver computed tomography or ultrasound, and only three cases had transrectal ultrasound. Restorative anterior resection was the most common surgical procedure (431; 63.3%) with 160 (23.5%) and 34 (5.0%) patients being managed with abdominoperineal resections and local excision, respectively. Chemotherapy and radiotherapy were administered as part of the initial management to 216 (31.7%) and 171 (25.1%) patients, respectively. In five of the 171 cases, radiotherapy was given preoperatively. CONCLUSIONS: There was considerable variation in preoperative assessment. Staging was less complete than expected by today's standards. The diversity of surgical techniques observed may reflect both the lack of clinical trials and disparity in surgical training and experience. Referral to stomal therapists, and medical and radiation oncologists was lower than would now be expected, as was the use and timing of adjuvant therapies. These findings will be useful as a baseline for comparison with subsequent surveys conducted since the introduction of evidence-based guidelines.

Survival from colorectal cancer in Victoria: 10-year follow up of the 1987 management survey.

BACKGROUND: In 1987, the Victorian Cancer Registry identified a population-based sample of patients who underwent surgery for colorectal cancer for an audit of management following resection. Over 10 years have passed since this survey, and data on the survival of these patients (incorporating various prognostic indicators collected at the time of the survey) are now discussed in the present report. METHODS: Relative survival analysis was conducted for each prognostic indicator separately and then combined in a multivariate model. RESULTS: Relative survival at 5 years for patients undergoing curative resections was 76% compared with 7% for those whose treatment was considered palliative. Survival at 10 years was little changed (73% and 7% respectively). Survival did not differ significantly by sex or age irrespective of treatment intention. In the curative group, only stage was a significant predictor of survival. Multivariate analysis was performed only for the curative group. Adjusting for all variables simultaneously,stage was the only -significant predictor of survival. Patients with Dukes' stage C disease were at a significantly greater risk (OR 5.5 (1.7-17.6)) than those with Dukes' A. Neither tumour site, sex, age, surgeon activity level nor adjuvant therapies made a significant contribution to the model.