[The (German) Center for Cancer Registry Data (ZfKD) at the Robert Koch Institute (RKI) in Berlin]. / Das Zentrum für Krebsregisterdaten (ZfKD) im Robert Koch-Institut (RKI) in Berlin.

Cancer represents the second most common cause of death in Germany. The country's federal states operate regional population-based cancer registries that collect and analyze data on cancer patients. This provides an essential basis for describing the cancer burden in the German population. In order to obtain valid and reliable information on cancer incidence at the national level, the Robert Koch Institute (RKI) set up the Federal Cancer Surveillance Unit in 1983 as a central institution for evaluating this cancer registry data. In August 2009, when the Federal Cancer Registry Data Act (BKRG) came into force, the Center for Cancer Registry Data (ZfKD) at the RKI took over the work of the Cancer Surveillance Unit with a broader remit. In the future, it will also regularly publish findings on survival, prevalence, and tumor stage distribution. A newly established record linkage process will help identify multiple submissions from the federal states. Further innovations and new tasks of the ZfKD include expanding an interactive Internet platform and encouraging a more intensive use of cancer registry data for epidemiological research by providing datasets to external scientists. The range of information available to the interested public is also to be expanded.

[Future cancer incidents in Germany]. / Die zukünftige Entwicklung des Krebsgeschehens in Deutschland.

The future as well as the past development of cancer incidents in Germany is of continuous importance for health policy. Cancer incidence data observed over more than 20 years are analysed by log-linear models with polynomial trend components. The estimated trend models are used for a trend extrapolation until the year 2020. Since cancer registration in Germany is not complete yet and does not cover the whole area of the country national incidences are estimated by the data of the existing complete regional cancer registries. In this way it can be assessed that the number of new cancer diseases increased from 270,000 cases in 1980 to 420,000 cases in 2002. Extrapolating the trends and taking into account the demographic prognosis of the German Federal Statistical Office 570,000 (version 1) or 590,000 (version 9) annual cancer cases are predicted for the year 2020. From 1980 to 2002 a decrease of cancer mortality was observed. If this trend continues until 2020, the number of deaths caused by cancer would decrease from 209,000 in 2002 to 153,000 (or 158,000) in 2020, although the number of cancer cases are predicted to increase. Assuming constant mortality rates the number of deaths caused by cancer would be much higher.

[Federal Cancer Reporting Unit. Surveillance program for cancer registration in Germany]. / Die Dachdokumentation Krebs. Eine Surveillance-Einrichtung der Krebsregistrierung in Deutschland.

For 20 years the Federal Cancer Reporting Unit has been processing and analyzing the comprehensive data of the population-based cancer registries in the Federal Republic of Germany. For this purpose the registries transfer their epidemiological records that have been made anonymous to the Federal Cancer Reporting Unit in the Robert Koch Institute once a year. An essential task of the Federal Cancer Reporting Unit is to check the data coherence and especially the completeness of cancer registration. The completeness of registration is estimated by site- and register-specific log-linear models. Based on data from sufficiently complete registries, national incidence rates and their time trends are estimated. Cancer survival rates, lifetime, and age-conditional probability of developing cancer and risk of dying from cancer are calculated and interpreted. The results are published regularly. With increasing completeness of registration, multiple notification of cases in different registries and their exclusion by matching procedures becomes more important. With increasing completeness of registration, further problems can be addressed such as the incidence of rare cancer sites or the analysis of regional patterns of cancer incidence and their underlying causes. Given the different legal requirements of cancer registration in the federal states of Germany, a national institution such as the Federal Cancer Reporting Unit in the Robert Koch Institute, which is not directly involved in cancer registration, can perform these activities best.

[Cancer incidence estimates for Germany via log-linear models]. / Krebsinzidenzschätzungen für Deutschland mittels log-linearer Modelle.

In Germany presently no nationwide cancer registration exists. To estimate national cancer incidence, Poisson regression models were fitted to incidence/mortality ratios using age and sex specific data of the cancer registry of Saarland, Germany and were then applied to national mortality. The models estimate the absolute number of incident cases at a given point in time and moreover allow the assessment of time trends. Applied to nationwide mortality the models imply a total of 347,000 new cancer cases in Germany for 1998 with 179,000 females and 168,000 males. During the nineties the age-standardised rate (European standard) has slightly decreased for males and slightly increased for females.

Primary cutaneous melanoma. Identification of prognostic groups and estimation of individual prognosis for 5093 patients.

BACKGROUND: Numerous investigations have examined prognostic factors for patients with primary cutaneous melanoma. However, only a few studies have been published on the definition of prognostic groups. The first aim of the present study was to determine the relative importance of different prognostic factors in a large collective study. The second aim was to define prognostic groups of patients based on combinations of prognostic factors and to define a model that allows the estimation of individual survival probability. METHODS: Long term follow-up of 5264 patients with invasive primary cutaneous melanoma was performed from 1970 to 1988 at four German University Departments of Dermatology (Berlin-Steglitz, Münster-Hornheide, Tübingen, and Würzburg). The multivariate Cox model was used to analyze 5093 patients, and 4371 patients with complete information were included in a classification and regression tree analysis (CART). RESULTS: Tumor thickness, sex, anatomic location, and level of invasion were highly significant prognostic factors according to the multivariate analysis (P < 0.0001). However, histologic subtype and age influenced prognosis less significantly (P < 0.05). The CART analysis resulted in 12 groups defined mainly by tumor thickness, sex, and anatomic location, which were combined into five prognostic groups. The prognostic stratification defined by the five groups was superior compared with the standard TNM model. Ten-year survival rates of the five groups ranged from 97% to 14% (P < 0.0001), and an equation was used to calculate individual survival probabilities based on the significant factors of the Cox model. CONCLUSIONS: Consideration of all significant prognostic factors of patients with primary cutaneous melanoma investigated in the present study allows for the definition of prognostic groups with a more reliable estimation of prognosis than by previous staging systems and also enables calculation of individual survival probabilities.

Primary cutaneous melanoma. Prognostic classification of anatomic location.

BACKGROUND: Anatomic location has been identified by several investigators as a significant prognostic factor for patients with primary cutaneous melanoma (CM). However, the best determination of higher and lower risk sites is still controversial, and the biologic significance of tumor site in the course of primary CM is unknown. The aim of the present study was to identify higher and lower risk sites based on multivariate analysis. METHODS: A series of 5093 patients with invasive primary cutaneous melanoma followed from 1970 to 1988 at four university centers in Germany was investigated using the multivariate Cox proportional hazard model to analyze the importance of anatomic location for survival probability. RESULTS: The anatomic location was found to be a highly significant prognostic factor for patients with primary melanoma by multivariate analysis (P < 0.0001). An optimized classification into sites of higher and lower risk with respect to survival was evaluated by multivariate analysis controlling for the possible confounding effects of the other significant prognostic factors. Relative to the lower leg as the prognostically favorable baseline, the following locations were associated with a significantly higher risk of death caused by primary cutaneous melanoma: back and breast (thorax), upper arm, neck, and scalp (TANS regions). The lower trunk, thigh, lower leg, foot, lower arms, hands, and face were identified as lower risk sites. CONCLUSIONS: Anatomic location was confirmed as an independent prognostic factor for patients with primary cutaneous melanoma. The TANS regions were identified as high risk sites, and the lower trunk, thigh, lower leg, foot, lower arms, hands, and face were identified as intermediate sites.

Primary cutaneous melanoma. Optimized cutoff points of tumor thickness and importance of Clark's level for prognostic classification.

BACKGROUND: Maximum tumor thickness and level of invasion are known to be the most important prognostic factors for patients with primary cutaneous melanoma. However, the classification of tumor thickness and the question of whether the combination of tumor thickness and level of invasion provides a better prognostic classification than tumor thickness alone are still matters of debate. The present study examined the relationship between tumor thickness and survival probability to define cutoff points of tumor thickness. Secondly, it investigated the prognostic value of the combination of tumor thickness and level of invasion as proposed in the current TNM classification system. METHODS: A series of 5093 patients with invasive primary cutaneous melanoma followed from 1970 to 1988 at four University centers in Germany (Departments of Dermatology in Tübingen, Würzburg, Berlin-Steglitz, and at the Fachklinik) were analyzed by multivariate Cox models. RESULTS: The relationship between tumor thickness and relative risk of death caused by melanoma was found to be almost linear to a tumor thickness of 6 mm. For tumors greater than 6 mm, no further marked increase in relative risk was observed. The stratification of tumor thickness with endpoints at 1, 2, and 4 mm resulted in the best fit to the authors' data among all classifications with three endpoints, but differences were only slight. By multivariate analysis, the combination of tumor thickness and level of invasion as proposed by the current TNM classification were found to be prognostically less significant than tumor thickness alone. The prognostic influence of level of invasion was proved statistically only for tumor thickness less than or equal to 1 mm. CONCLUSIONS: The proposed stratification of tumor thickness with cutoff points at 1, 2, and 4 mm was supported by multivariate statistical analysis. The analysis of the current TNM staging system indicates the precedence of tumor thickness for the staging of patients with primary cutaneous melanoma in the case of discordance between tumor thickness and level of invasion.

Malignant melanoma in southern Germany: different predictive value of risk factors for melanoma subtypes.

The presented case-control study with 204 melanoma patients and 200 control persons assesses the significance of melanoma risk factors for an ethnically homogeneous population from a geographically small region. In a multivariate analysis of the data the total number of benign naevi proved to be the most predictive parameter with a relative risk (RR) of 14.9 (total number of naevi higher than 50). The constitutional factors red hair colour and skin type 1 were less predictive with an RR of 2.9 and 4.9, respectively. Occupational and recreational sun exposure were of ancillary importance (RR 1.8 and 2.1). The assessment of the risk for the subtypes of melanoma however showed a clear difference in the predictive value of the mentioned risk factors. The risk of developing superficial spreading melanoma is nearly exclusively defined by the number of benign naevi (RR 24.8), red hair colour was of subordinate importance (RR 4.2), whereas the risk of lentigo maligna melanoma is dependent on skin type 1 (RR 12.9) and sun exposure (RR 3.4).

Incidence and mortality of malignant melanoma in Berlin (West) from 1980 to 1986.

Newly diagnosed melanomas were investigated utilizing the histological reports from the 4 Departments of dermatology as well as from 3 Departments of pathology in Berlin (West) during the years 1980-86. The study included 960 melanomas and documented the histological features, age, gender and nationality of the patients involved. 936 patients were Germans (379 males, 557 females), and the mean age-adjusted incidence rate (for the European standard population) was 7.1 cases per 100,000 inhabitants and year for both genders with an increase between 1980-81 and 1985-86 in men from 6.0 to 9.8 and in women from 5.8 to 7.8/100,000 and year. Thus a 49% increase in incidence was observed for both genders combined during a 5-year period. In this study, a preponderance of male incidence rates was observed for the first time in Germany. Interestingly, the age-adjusted incidence rate for the Turkish population, which is the largest foreign population with more than 100,000 inhabitants in Berlin, was only 1.3/100,000 and year. 162 men and 145 women died of melanoma in the time period examined. From 1980-81 to 1985-86, the age-adjusted mortality rate changed from 3.5 to 2.6 for men and from 1.2 to 1.6 for women per 100,000 and year, thus revealing a slight decrease in mortality for both genders combined.

[Risk factors for the development of malignant melanoma in West Germany. Results of a multicenter-case control study]. / Risikofaktoren für die Entwicklung maligner Melanome in der Bundesrepublik Deutschland. Ergebnises einer multizentrischen Fall-Kontroll-Studie.

In this multicenter case-control study (1,079 melanoma patients, 778 control persons), the significance of the well-known risk factors for the development of malignant melanoma (MM) was assessed for a German population. The multifactorial analysis of the data confirmed the total number of melanocytic nevi (MCN) as the most robust indicator for an increased melanoma risk. For persons with more than 50 MCN the relative risk was 4.8 times as high as for persons with fewer than 10 MCN. Hair color was found to be another valuable indicator. In persons with red hair the risk of MM was 4.7 that in individuals with black hair. Remarkably fair persons with skin type 1 (always sunburn, never tanning) had two times the risk of that in persons with skin type 4 (never sunburn, always tanning). The habit sun bathing for recreation showed no influence on the development of melanoma. A 2.7 x increased melanoma-risk was detected in persons with occupational sun exposure and out of doors work.

[Malignant melanoma in German-speaking countries in the 80's. Initial results of the central registry for malignant melanoma of the Germany Society of Dermatology in cooperation with the federal health office]. / Das maligne Melanom im deutschsprachigen Raum in den 80er Jahren. Erste Ergebnisse des Zentralregisters malignes Melanom der Deutschen Dermatologischen Gesellschaft in Verbindung mit dem Bundesgesundheitsamt.

From July 1983 to April 1987, 2834 reports of malignant melanoma (MM) were received by the Central Registry of the German Dermatological Society in Berlin from 22 departments of dermatology in the German-speaking countries. Of these, 2277 reports were documented in a data bank and evaluated. Compared to earlier reports from the 1960s and 1970s the percentage of male patients with MM was found to be increased: 41% males, 59% females. Also, the tumor occurs today at a slightly younger age: 63% of all males and 56% of all females with MM were adults 30-60 years of age. In 10% of all MM patients, MM metastases were found during their first visit (stages II-IV). In addition, 37% of all patients in stage I without metastasis had MMs that were thicker than 1.5 mm. This indicates that there is a necessity for further information and early recognition of the MM in German-speaking countries. Regarding the therapeutic approach, considerable differences were noted among the various departments. Half of the primary MM excisions were conducted under local anesthesia, the rest with general anesthesia. In about 40% of all cases, a second surgical procedure was performed for larger excisions: half of the patients were operated on with safety margins of less than 3 cm. Twenty-seven percent of the patients in stage I MM received some type of adjuvant therapy, including chemo- and/or immunotherapy. However, postsurgical management varied greatly among the different departments. The additional results regarding possible risk factors, such as sun exposure and preexisting pigmented moles, are remarkable.

[Malignant melanoma: increase in incidence and mortality in West Germany]. / Malignes Melanom: Zunahme von Inzidenz und Mortalität in der Bundesrepublik Deutschland.

Based on official statistics of causes of deaths and data from regional cancer registers as well as clinical epidemiological data from our clinic, incidence and mortality of malignant melanoma of the skin (MM) in Germany has been analysed. Between 1970 and 1980, the mortality rate of MM according to age rose by 20% for both men and women; the regional incidence in the Saarland, for example, increased by more than 50% (men: 3.4 to 4.6; women: 3.2 to 6.0 per 100,000 inhabitants a year). The earlier development of the MM mortality can be estimated by analysing the mortality of skin cancers in general. In the age groups between 30 and 69 years, the skin cancer mortality was nearly exclusively caused by MM; the mortality rates according to age increased between 1955 and 1980 by more than 100% for men and by more than 75% for women. According to all available data, the MM incidence in West Germany in 1980 was estimated to be 5 per 100,000 inhabitants for men and 8 per 100,000 inhabitants for women. Assuming a continuous increase of incidences, we expect 5,500 new MM cases in 1985.

Trigeminal neuralgia due to dolichoectasia: angiographic and CT findings in a patient with the EEC syndrome.

Ectrodactyly (lobster claw deformity), ectodermal dysplasia and clefting of the lip or palate, the EEC syndrome, is a rare genetic disorder involving both mesodermal and ectodermal derivatives. Cerebral arterial dolichoectasia, a pathological dilatation and elongation of intracranial vessels, was found in a patient with the EEC syndrome. Her hypertensive cardiovascular disease, mild dementia and trigeminal neuralgia were all related to her dolichoectasia. Although the association of dolichoectasia has not been previously observed with the EEC syndrome, it may be more frequent than currently recognized. Cerebral arterial dolichoectasia and its attendant sequelae could account for some of the unexplained abnormalities described in other case reports of patients with the EEC syndrome.