Visual Universitätsmedizin Mainz

An overview of the most important results from the 2018 yearly report

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15 to 17 year old adolescents

In the annual report 2013/14 we presented analyses for cases aged 15-17, which have been registered since 2009, for the first time. The current report makes data for the last 8 years available. It is becoming clear, that the 16-year olds, and even more so the 17-year olds, are not complete. Some of these cases are treated out- side of pediatric oncology units, where reporting to the GCCR is not mandatory. 

There is not yet sufficient data to present survival probabilities.

I Leukaemias, myeloproliferative and myelodysplastic diseases

These hematological diseases are the most frequent malignant diseases in childhood and adolescence. One child out of 1250 under 15 years is affected, boys ca. 20% more often than girls. About half of the cases are 5 years and below. Children and adolescents show mostly acute forms, whereas adults show mostly chro- nic forms, which are very rare in children. Based on international comparisons we assume completeness is close to 100%. 

The most frequent form, lymphoid leukaemia (used to be ALL), slowly increased until the mid-2000s in Ger- many and Europe (ca. 0.7% p.a.), in Germany we see no further increase, similarly for Europe. Almost 98% of all lymphoid leukaemias are precursor cell leukaemi- as, which makes it the most frequent single diagnosis in childhood and adolescence. It shows a typical age peak at ages 2-4. The prognosis is good (90% long- term survivors for more than 15 years). 

Acute myeloid leukaemias (AML) are much less fre- quent and have a worse prognosis (71% long-term sur- vivors); the improvements in therapy since the 1980s are considerable and keep increasing. 

The myelodysplastic syndrome (MDS) was reclassified as malignant since the 2000s (introduction of ICD-O-3). Numbers of cases and survival are not representative before this. Some MDS cases progress to an AML. There were different approaches of counting such cas- es; as a consequence comparisons over time or across registries are problematic. 

More than 16% of the second and subsequent neo- plasms (SN) within 30 years of diagnosis are AML or MDS.

II Lymphomas and reticuloendothelial neoplasms

Lymphomas (one child in 4000 under 15) occur mostly in adolescents and adults, while they are rare in small children. We assume completeness is close to 100%. Hodg- kin lymphomas are about 50% more frequent in boys. Patients with Hodgkin lymphoma have shown a good prognosis for decades (current long-term survival is 97%), so for this entity late effects are particularly well known. Patients with Hodgkin lymphoma are especially frequently affected by SN (almost 16% within the first 30 years). 

Burkitt lymphomas (BL) are a subgroup of the Non-Hodgkin lymphomas (NHL); they are presented separately for international comparisons. Boys are af- fected by NHL more than twice as often as girls, five times as often by Burkitt lymphoma. The prognosis of NHL is good (NHL/BL 86%/92% long-term survivors). The SN risk after NHL is above average, especially af- ter precursor cell lymphoma (22% risk). 

Unspecified lymphomas are rarely reported, this shows the high quality of diagnosis and reports.

III CNS and miscellaneous intracranial and intraspinal neoplasms

Tumours of the central nervous system (CNS, brain tumours) affect one child in 1600 under 15. They are a heterogeneous group of neoplasms, including malig- nant and non-malignant forms. Based on international comparisons we assume especially the non-malignant forms to be slightly underreported. The observed increase in cases shows improvements in completeness of registration, especially regarding astrocytomas and other gliomas. Boys have an about 20% higher inci- dence. The seemingly worsening prognosis of "other gliomas" since 1990 is due to considerable changes in the composition of this group due to improvements in completeness and classification changes. 

The risk of an SN after intracranial and intraspinal em- bryonal tumours is unusually low after astrocytoma, other gliomas, and craniopharyngiomas, while after Medulloblastoma it is unusually high (34%). 23% of all SN in the 30 years after primary diagnosis are CNS tumours, most of these are meningiomas, followed by astrocytomas.

IV Neuroblastoma and other peripheral nervous cell tumours

Neuroblastomas are embryonal tumours, which are observed mainly in small children. It affects one child in 6000 under 15, boys have an about 40% higher inci- dence than girls. We assume completeness is close to 100%. Overall long-term survival is 76%, but patients with advanced disease (stage IV) still have a rather bad prognosis, although it has improved considerably since the 1980ies. 

A subset of neuroblastomas (especially low stages before the 2nd birthday) is capable of spontaneous regression. During the screening evaluation project 1995-2000 this led to a considerable number of addi- tional cases, which is visible in the trend graphic. How- ever, screening did not lead to the intended drop in mortality, so it was not introduced. The increased atten- tion and the extended usage of ultrasound diagnostics have since led to an increase in the number of reported cases even without screening. 

Subsequent neoplasms are rare after neuroblastoma, which are in turn almost never reported as SN.

V Retinoblastoma

One child in 17,000 under 15 is affected with a Reti- noblastoma. These are embryonal tumours which ra- rely affect older children (10 years or older). Based on international comparisons we assume completeness is high. Retinoblastoma has a known genetic cause and can be inherited, especially bilateral cases. When a case is diagnosed, family members should also be examined. 

Retinoblastomas are very rare as SN.

VI Renal Tumours

Almost all renal tumours in childhood are nephro- blas- tomas (Wilm's tumour). One child under 15 in 7400 is affected, girls about 10% more often. Based on in- ternational comparisons we assume completeness is close to 100%. The prognosis is good (93% long-term survivors). 

The risk of a subsequent neoplasm is below average, nephroblastoma is hardly ever reported as an SN. 

Renal carcinomas, usually observed in adults, are oc- casionally diagnosed in older children and adolescents; boys are affected 40% more often. 

Subsequent neoplasms after renal carcinomas are rare, renal carcinomas as subsequent neoplasms do occur occasionally. 

No unspecified renal tumours were reported, this shows the high quality of diagnoses and reports.

VII Hepatic tumours

Almost all hepatic tumours in childhood (one in 33,000 children until 14 years is affected) are hepatoblasto- mas. Boys have a 40% higher incidence. We assume completeness is good and has visibly improved further since a hepatic tumour registry for children was fund- ed in 2011. The prognosis is moderate (79% long-term survivors) and has been improving considerably since the 1980ies. 

Subsequent neoplasms are rare; hepatoblastomas hardly ever are subsequent neoplasms. Hepatic carcinomas, usually observed in adults, are occasionally diagnosed in older children and adole- scents. 

SN are rarely reported, hepatic carcinomas occasion- ally occur as SN. 

Unspecified hepatic tumours were not reported, this shows the high quality of diagnoses and reports.

VIII Malignant bone tumours

Bone sarcomas (one case in 9700 children under 15) are typical for older children and adolescents. The most frequent forms are osteosarcoma and Ewing sar- comas. Based on international comparisons we assu- me completeness is high. 

5% of all subsequent neoplasms within 30 years of the first neoplasm are bone tumours, mostly osteosarco- ma. 

Unspecified bone tumours are rarely reported, this shows the high quality of diagnoses and reports.

IX Soft tissue and other extraosseous sarcomas

Soft tissue sarcomas occur in all ages in childhood (one child under 15 in 7200). The most frequent type in childhood is rhabdomyosarcoma. Based on interna- tional comparisons we assume completeness is high. Boys have a 20% higher incidence than girls. The prog- nosis is below average (70% long-term survivors). 

Soft tissue sarcoma patients have an average risk of a subsequent neoplasm; about 6% of all subsequent neoplasms within 30 years after a primary neoplasm are soft tissue sarcomas.

X Germ cell tumours, trophoblastic tumours and neoplasms of gonads

Germ cell tumours are a heterogeneous group of neo- plasms; one child under 15 in 13,000 is affected. Some become more frequent as puberty sets in, others are typical for infants, so they are rare from the 4th to 7th year of life. We assume completeness is high. Girls under 15 have about 30% higher incidence. Some in- tracranial forms (localized in the brain) have been re- classified as brain tumours (CNS) since about 2000 (new diagnosis classification ICD-O-3. In general the prognosis is good (93% long-term survivors). 

The risk of an SN is average. Germ cell tumours are rare as SN.

XI Other malignant epithelial neoplasms and malignant melanomas

This is a heterogeneous group of rare cancers. Carci- nomas are usually observed in adults. The most fre- quent among them in childhood are adrenocortical car- cinoma, thyroid carcinoma (improved reporting since 1996), nasopharyngeal carcinoma, and malignant me- lanoma. Some carcinomas in children are clearly un- derreported, though not nasopharyngeal carcinomas and thyroid carcinomas. Appendix carcinoids have been reclassified as malignant in 2011, which explains the sudden considerable increase in reported appen- dix carcinomas since then. The reporting of malignant melanoma has improved over the years, but we as- sume they continue to be underreported. Breast carci- nomas have not been reported as primary neoplasms. Thyroid carcinomas have a good prognosis (94% long- term survivors). 

Carcinoma patients have an above average SN risk. One third of all subsequent neoplasms within 30 years are carcinomas, particularly thyroid carcinoma, skin carcinoma (mostly not malignant melanoma), breast carcinoma and colon carcinoma. Among the thyroid cancer cases under 15, more than 10% are SN

XII Other and unspecified neoplasms

This is a heterogeneous group of very rare neoplas- ms in childhood not classifiable anywhere else (one child under 15 in 250,000). The most frequent tumour among these is pulmonary blastoma.