Die deutsche Kohorte Langzeitüberlebender nach Krebs im Kindesalter

Due to the considerably improved prognosis of childhood cancer, research regarding the long-term consequences has become highly valuable. The population-based German Childhood Cancer Registry forms the basis of the long-term follow-up of these patients. The cohort comprises over 25,000 patients (with malignant diseases before their 15th birthday) with a current address and who are neither deceased nor lost to follow-up. The current median age is 21 years and 500 individuals are already over 40 years old. All the long-term survivors are contacted every 5 years at the latest and are asked about possible long-term effects. Due to the continued improvement of the prognosis for childhood cancer over the years, such cohorts of long-term survivors have altered in their composition. Corresponding long-term follow-up studies can therefore not easily be compared to one another. This is illustrated by a nested case control study on the possible relationship between the occurrence of second tumors and the therapy undergone for the initial diagnosis. The cohort of long-term survivors in the German Childhood Cancer Registry is highly valuable both for research on long-term effects in Germany as well as for integration into international projects.     

Care for survivors of childhood cancer in the Netherlands

Survival of childhood cancer has improved resulting in an increasing number of survivors who are at high risk of developing treatment-related health problems. The authors emphasize the need for specialized care for survivors of childhood cancer by describing three patients who all developed late effects of treatment. The first patient, a 32-year-old female, who had several late effects caused by treatment for nephroblastoma; the second a 39-year-old female, who developed breast cancer after thoracic irradiation for metastatic nephroblastoma; the third a 45-year-old female diagnosed with a meningioma caused by cranial irradiation for acute lymphoblastic leukaemia. In the Netherlands medical care for survivors is clustered at special outpatient clinics (in Dutch: Langetermijneffecten na kinderkanker (Long-term effects after childhood cancer; LATER)-outpatient clinics). In 2010 a guideline was published with recommendations for optimal follow-up and care for survivors of childhood cancer.     

The PanCareSurFup cohort of 83,333 five-year survivors of childhood cancer: a cohort from 12 European countries

Childhood cancer survivors face risks from a variety of late effects, including cardiac events, second cancers, and late mortality. The aim of the pan-European PanCare Childhood and Adolescent Cancer Survivor Care and Follow-Up Studies (PanCareSurFup) Consortium was to collect data on incidence and risk factors for these late effects among childhood cancer survivors in Europe. This paper describes the methodology of the data collection for the overall PanCareSurFup cohort and the outcome-related cohorts. In PanCareSurFup 13 data providers from 12 countries delivered data to the data centre in Mainz. Data providers used a single variable list that covered all three outcomes. After validity and plausibility checks data was provided to the outcome-specific working groups. In total, we collected data on 115,596 patients diagnosed with cancer from 1940 to 2011, of whom 83,333 had survived 5 years or more. Due to the eligibility criteria and other requirements different numbers of survivors were eligible for the analysis of each of the outcomes. Thus, 1014 patients with at least one cardiac event were identified from a cohort of 39,152 5-year survivors; for second cancers 3995 survivors developed at least one second cancer from a cohort of 71,494 individuals, and from the late mortality cohort of 79,441 who had survived at least 5 years, 9247 died subsequently. Through the close cooperation of many European countries and the establishment of one central data collection and harmonising centre, the project succeeded in generating the largest cohort of children with cancer to date.     

Risk of second malignant neoplasms among childhood cancer survivors treated with radiotherapy: meta-analysis of nine epidemiological studies

In the light of notable advances made in childhood cancer therapies, an understanding of the late effects of treatment is important for continued medical care. We conducted a meta-analysis of studies on the excess relative risk (ERR) of second malignant neoplasm (SMN) among childhood cancer survivors treated with radiotherapy. Relevant studies were retrieved by searching the PubMed database, supplemented by hand-searching of reference lists of already retrieved papers. Nine studies were identified and overall ERR estimates were calculated using a fixed effects model and a random effects model.
The overall ERR per Gy (absorbed dose of ionising radiation) estimates of radiotherapy by a fixed effect model and a random effects model were 0.50 [95% CI 0.20, 1.21] and 0.53 [95% CI 0.22, 1.31] respectively. Heterogeneity among studies was suggested by Cochran's Q statistic (Q = 40.4, d.f. = 8, P  < 0.001). The estimate obtained using a random effects model was far smaller than the corresponding estimate of 1.7 [95% CI 1.1, 2.5] from the study on atomic bomb survivors exposed as young children, suggesting underestimation of ERR estimates among the nine studies compared with the estimates from the study of atomic bomb survivors. In view of the heterogeneity and underestimation in ERR estimates, more studies concerning the risk of SMN among childhood cancer survivors are still needed for further understanding of the carcinogenic effects of radiotherapy on children.     

Physical performance limitations and participation restrictions among cancer survivors: a population-based study

PURPOSE: Medical late effects among cancer survivors may result in impairments that limit physical performance and activities necessary for normal participation in daily life. The aim of this analysis was to estimate the prevalence of physical performance limitations and participation restrictions among recent (< 5 years since diagnosis), and long-term (> or = 5 years) cancer survivors. METHODS: Data from the 1999-2002 National Health and Nutrition Examination Survey were analyzed to compare the proportions of physical performance limitations and participation restrictions among 279 recent and 434 long-term cancer survivors, and among 9370 persons with no reported cancer history. Multivariable logistic regression was used to calculate adjusted prevalence odds ratios. RESULTS: Physical performance limitations were 1.5-1.8 times (53% versus 21%) and participation restrictions 1.4-1.6 times (31% versus 13%) more prevalent in cancer survivors than in those with no cancer history. Recent cancer history was associated with increased prevalence of physical performance limitation and participation restriction, particularly in survivors aged 40-49 years. CONCLUSIONS: Over half of the cancer survivors reported physical performance limitations; one third reported participation restrictions. Deficits were present many years following cancer diagnosis, even among survivors who were not elderly. Cancer survivors may benefit from evaluation for rehabilitation services long after treatment for their original disease.     

The Minneapolis-Manchester Quality of Life Instrument: reliability and validity of the Adult Form in cancer survivors

Purpose

Childhood cancer survivors are at risk for deficits in health-related quality of life (HRQL) as they age. Youth (8–12 years) and adolescent (13–20 years) versions of the Minneapolis-Manchester Quality of Life Instrument (MMQL) have been developed to address survivor-specific issues and are currently in use; the MMQL-Adult Form has now been developed to assess HRQL in childhood cancer survivors aged 21–55 years.

Methods

The MMQL-Adult Form was administered to 499 adults: 65 cancer patients on-therapy, 107 off-therapy, and 327 healthy controls. Forty-four percent of patients were under 30 years old at cancer diagnosis. Principal components analysis was performed. We evaluated internal consistency reliability, stability (re-administration of the MMQL-Adult Form 2 weeks later), construct validity (concurrent administration of the SF-36), and known-groups validity (score comparisons across the three groups).

Results

Principal components analysis resulted in retention of 44 items across six scales: social functioning, physical functioning, cognitive functioning, outlook on life, body image, and psychological functioning. Internal consistency (Cronbach’s α) was 0.80–0.90 for individual scales and 0.95 overall. Strong intraclass correlations (0.98 overall) indicated high stability. The MMQL-Adult Form distinguished between known groups; healthy controls scored better than patients on four of six scales. The MMQL-Adult Form scales correlated highly with similar SF-36 scales, demonstrating construct validity.

Conclusions

The MMQL-Adult Form is a reliable and valid self-report instrument for measuring multidimensional HRQL in cancer survivors. Development of this instrument ensures availability of a tool enabling cross-sectional and longitudinal assessment of HRQL in childhood cancer survivors as they age.

     

A population based pediatric oncology registry in Southern Sweden: the BORISS registry

A population based registry, with the acronym BORISS, was established. It contains all individuals (0–18 years of age at diagnosis) diagnosed with cancer from 1970–01–01 until 2016–12–31 in Southern Sweden. The treatment data has been entered into the registry after confirmation of the diagnosis by the Swedish national cancer registry and updates on vital status from the Swedish population registry. The number of individuals with a pediatric cancer diagnosed during these 46 years are 2928. Of these, 2065 are currently alive and 1882 individuals are 5-year survivors. Data on treatment and malignancy of the 5–year survivors has been collected from medical records and entered into the database. Treatment data contains surgical procedure, target organ of radiation therapy including dose and fractionation, and cytostatic treatment with dose (mg) per body surface area (m²) for all cytostatic agents. Data on individuals receiving stem cell treatment is included. The database is unique in that it is population based, contains all individuals and detailed treatment data on all 5-year survivors after childhood cancer in Southern Sweden since 1970. The database has contributed to several academic theses in the field of late effects after childhood cancer. BORISS also supports the Late Effect Clinic at Skåne University Hospital in Lund, Sweden with treatment details enabling a stratified surveillance.     

Awareness of diagnosis,treatment and risk of late effects in Chinese survivors of childhood cancer in Hong Kong

BackgroundFor survivors of childhood cancer, awareness of personal health risks is a critical component of long‐term health management.ObjectiveTo evaluate the awareness of the diagnosis, treatment and risk of late effects among survivors of childhood cancer in Hong Kong.MethodsBetween June 2019 and March 2020, this cross‐sectional study recruited 155 adult survivors (mean age = 26.9, standard deviation [SD] = 6.4 years) and 45 parents of paediatric survivors (mean age = 11.1, SD = 3.6 years) from a long‐term follow‐up clinic. At >10 years post‐treatment (mean = 13.4, SD = 7.6 years), they completed a structured questionnaire to report their cancer‐specific knowledge. Multiple linear regression analysis was conducted to identify clinical, socioeconomic and behavioural factors associated with poor awareness.ResultsThe majority of participants accurately recalled their diagnoses (73.5%) and major treatment modalities (chemotherapy 92.4%, radiation 82.9% and surgery 88.2%). However, less than half (45%) of the participants recognized more than 25% of the total late effects for which they were at risk. The highest levels of awareness were reported for endocrine problems (49%), neurocognitive impairment (44%) and secondary cancers (43%), and the lowest for peripheral neuropathy (21%) and vision problems (23%). Compared with survivors of haematological malignancies, those of central nervous system (CNS) tumours (standardized estimate [B] = −9.33, 95% confidence interval [95% CI]: −13.41 to −5.26) and non‐CNS solid tumours (B = −8.47, 95% CI: −12.39 to −4.94) had less knowledge about their diagnosis. Retaining medical records (P < .0001) and better medical information‐seeking habits (P = .048) were associated with better awareness.ConclusionsSurvivors of childhood cancer in Hong Kong have deficient awareness of their personal health risks. They may benefit from the provision of a survivorship care plan and personalized education regarding treatment‐related late effects.Patient ContributionPatients contributed in designing the study tools. Results were presented at a non‐governmental organization.     

A pooled analysis of second primary pancreatic cancer

Studies of pancreatic cancer in the setting of second primary malignant neoplasms can provide etiologic clues. An international multicenter study was carried out using data from 13 cancer registries with a registration period up to year 2000. Cancer patients were followed up from the initial cancer diagnosis, and the occurrence of second primary malignant neoplasms was compared with expected values derived from local rates, adjusting for age, sex, and period of diagnosis. Results from individual registries were pooled by use of a fixed-effects model. People were at higher risk of developing pancreatic cancer within 10 years of a diagnosis of cancers of the pharynx, stomach, gallbladder, larynx, lung, cervix, corpus uteri, bladder, and eye and 10 years or later following a diagnosis of cancers of the stomach, colon, gallbladder, breast, cervix, placenta, corpus uteri, ovary, testis, bladder, kidney, and eye, as well as Hodgkin's and non-Hodgkin's lymphomas. Pancreatic cancer was connected with smoking-related cancers, confirming the etiologic role of tobacco. The associations with uterine and ovarian cancers suggest that reproductive factors might be implicated in pancreatic carcinogenesis. The elevated pancreatic cancer risk in young patients observed among several types of cancer implies a role of genetic factors. Radiotherapy is also suggested as a risk factor.     

Using Medicare claims to identify second primary cancers and recurrences in order to supplement a cancer registry

BACKGROUND AND OBJECTIVE: The purpose of this study was to use Medicare claims to develop models to assist cancer registries in identifying cancer patients with second primaries or recurrences (an "event"). METHODS: Medicare inpatient and Part B data were merged with a cancer registry for patients first diagnosed in 1993-1994. Logistic regression was used to model the occurrence of an event at least 1 year after initial diagnosis, and to identify factors that could discriminate between recurrences and second primaries. RESULTS: Predictors of an event included an inpatient cancer diagnosis, cancer diagnosis different from the initial diagnosis from any source, and radiation or surgery claims in Part B. The ROC curve area was 0.90 with all Medicare data; 0.84 when restricted to inpatient data. A cancer diagnosis different from the initial diagnosis or having surgery predicted a second primary; regional or distant stage disease, diagnosis of secondary malignancy, or an inpatient diagnosis of primary cancer in a position other than principal predicted recurrence. CONCLUSIONS: Although Medicare claims have not been evaluated as a stand-alone system to identify second primaries and recurrences, Medicare claims may be useful tools for Cancer Registries in case ascertainment and follow-up.     

诸城市1998～2007年恶性肿瘤发病情况回顾性分析

目的 了解中小城市恶性肿瘤发病的基本情况,为肿瘤防治工作提供基本数据和参考依据.方法 以潍坊地区诸城市二级医院经病理确诊的恶性肿瘤患者为研究对象,初步分析这一地区人群恶性肿瘤的发病趋势、发病病因.结果 10年间经病理确诊的恶性肿瘤计8490例,男女之比1.57∶ 1,排序前5位的分别是胃癌、肺癌、乳腺癌、食道癌、结肠癌.胃癌、肺癌分别占居第1位和第2位.吸烟、饮酒分别是肺癌、食道癌的主要致病因素.癌前病变与相关肿瘤发病相关.结论 中小沿海发达城市肿瘤发病情况不容乐观,政府应加大肿瘤防治工作的资金投入.     

Malignancies among adolescents and young adults in the province of Trieste,Italy, 1972-1993

We analysed incidence, survival and mortality from cancer among people aged 15-24 years resident in the province of Trieste, Italy, during 1972-1993, and evaluated the quality of the local diagnostic facilities and of the care provided by local hospital departments. We compared the results with those previously published on childhood cancer. We recorded 118 new cases of cancer (96% microscopically verified) corresponding to a rate, age-standardized to the world population, of 162.3 (standard error SE = 15.0) per million person-years. The diagnostic group with the highest rate was that of carcinomas (54.5; SE = 8.7; 40 cases). The diagnosis was reached at hospitals in the province of Trieste for 107 cases, with a median time of nine days (25th-75th percentile = 5-23) between admission and diagnosis. Among patients with leukaemias, lymphomas and brain tumours, this interval was longer than in children affected by the same neoplasms. One girl with cancer of the uterine cervix refused all treatment. The therapy of the other 117 cases were co-ordinated by 29 different departments of hospitals located in Trieste for 86 cases, in other Italian hospitals for 26 cases and in European hospitals for five cases. On the other hand, out of 123 childhood cancers 107 were co-ordinated by three departments in Trieste, seven by other Italian hospitals and nine by foreign hospitals. The 10-year survival probabilities of children with acute lymphoblastic leukaemia and non-Hodgkin lymphomas were higher than those of patients aged 15-24 years: 66.7% (SE = 9.1) vs 14.3% (SE = 13.2) and 77.8% (SE = 13.9) vs 40.0% (SE = 21.9), respectively.     

Decreased risk of prostate cancer after skin cancer diagnosis: a protective role of ultraviolet radiation?

Ultraviolet radiation causes skin cancer but may protect against prostate cancer. The authors hypothesized that skin cancer patients had a lower prostate cancer incidence than the general population. In the southeastern part of the Netherlands, a population-based cohort of male skin cancer patients diagnosed since 1970 (2,620 squamous cell carcinomas, 9,501 basal cell carcinomas, and 1,420 cutaneous malignant melanomas) was followed up for incidence of invasive prostate cancer until January 1, 2005, within the framework of the Eindhoven Cancer Registry. The incidence rates of prostate cancer among skin cancer patients were compared with those in the reference population, resulting in standardized incidence ratios. Skin cancer patients were at decreased risk of developing prostate cancer compared with the general population (standardized incidence ratio (SIR) = 0.89, 95% confidence interval (CI): 0.78, 0.99), especially shortly after diagnosis. The risk of advanced prostate cancer was significantly decreased (SIR = 0.73, 95% CI: 0.56, 0.94), indicating a possible antiprogression effect of ultraviolet radiation. Patients with a skin cancer in the chronically ultraviolet radiation-exposed head and neck area (SIR = 0.84, 95% CI: 0.73, 0.97) and those diagnosed after the age of 60 years (SIR = 0.86, 95% CI: 0.75, 0.97) had decreased prostate cancer incidence rates. These results support the hypothesis that ultraviolet radiation protects against prostate cancer.     

Lymphomas and bone tumors: clinical presentation, management, and potential late effects of current treatment strategies

While many pediatric malignancies are seen predominantly in pre-school children, many cases of childhood non-Hodgkin's lymphoma and most cases of Hodgkin's disease and bone tumors are seen in the older child and adolescent. This review focuses on current knowledge concerning the epidemiology, histopathology, molecular biology, clinical presentation, diagnosis, staging, treatment, and prognosis for older children and adolescents diagnosed with lymphoma or either of the two commonly seen childhood bone tumors, namely osteosarcoma and Ewing's sarcoma. Survival figures for all of these childhood malignancies have increased markedly in the past two decades. We now have the relatively new experience of having an increasingly large population of childhood cancer survivors to study and, unfortunately, are beginning to see the long-term consequences of these more successful treatments. This review concludes with an overview of the potential late effects of cancer therapy, effects that may first be detected by the primary care physician caring for the adolescent who is a cancer survivor.     

Improved access to mental health evaluation for patients with developmental disabilities using telepsychiatry

The Cedars-Sinai Telepsychiatry Clinic uses a collaborative-care model to treat patients with developmental disabilities. We examined its practice in four areas: patient characteristics, clinical care, symptom severity and diagnostic outcomes to describe the care provided and the population seen in the telepsychiatry clinic. In a chart review, 45 out of 126 cases were selected and evaluated at three times: initial evaluation, year one and year three. Most of the patients (84%) had an intellectual disability, 55% had a pervasive developmental disorder and 71% spoke approximately 50 words or less. Prior to the initial assessment, none of the patients were diagnosed with anxiety or mood disorders, while almost one-third of patients received one of these diagnoses in the telepsychiatry clinic. Patients were seen six times on average in the first year and three times in the second and third years. The telepsychiatrist recommended a change in the patient's medication for 82% of patients at initial assessment, 41% at year one and 46% at year three. The review suggests that telepsychiatry evaluations can be valuable for patients with developmental disabilities, providing diagnostic clarity and specific recommendations that can be implemented by the primary care physician.     

Polymorphous low-grade adenocarcinoma of the accessory salivary glands--a case report

A case of polymorphous low-grade adenocarcinoma of minor salivary gland is reported. This tumor was first described in two clinical case series in 1983. Before that time most of these neoplasms were diagnosed as benign salivary gland neoplasms (pleomorphic adenomas) or salivary malignant conditions (malignant pleomorphic adenomas, adenoid cystic carcinomas, papillary adenocarcinomas and adenocarcinoma not otherwise stated). This neoplasm, with few exceptions, originates in minor salivary gland tissues of the palates or buccal mucosa. It is characteristically slow to enlarge. Clinical reports show the neoplasm present for many years before diagnosis. The tumor have a variety of morphological patterns, a cytological uniformity and an infiltration into adjacent structures. The treatment is a wide local excision. Recurrences and lymph node metastases are rare.     

泌尿系统多发性原发性恶性肿瘤16例临床分析

目的探讨泌尿系统多原发恶性肿瘤(MPMNs)的临床特点。方法回顾性分析该院2001年1月—2011年12月年收治的16例MPMNs临床资料。结果本组MPMNs占同期恶性肿瘤的1.04%,平均发病年龄为72岁,性别比(男:女)为2.2:1,第一癌与第二癌的发病平均间隔时间为15.2个月。结论 MPMNs在泌尿系统中的发病率逐渐升高,应引起足够重视。