0000000000172133
AUTHOR
Freimut H. Schilling
Lead-time and overdiagnosis estimation in neuroblastoma screening.
In Germany, neuroblastoma is the most frequent extracranial solid childhood tumour. Its properties made it seem an ideal candidate for screening. A German trial assessed the effect of screening at one year of age from 1995-2001 in a nationwide project. We present here the methods developed for the estimation of lead-time and overdiagnosis in this project. Follow up on 1.5 million screened children and 2.1 million control children is currently available until June 2002. Ascertainment of control cohort cases and false negative cases is complete up to this date. A method for determining an empirical lead-time distribution and overdiagnosis estimate from comparing the age specific incidences in…
Wirbelsäulenmanifestationen der chronischen rekurrierenden multifokalen Osteomyelitis (CRMO)
Chronic recurrent multifocal osteomyelitis (CRMO) is a systemic osteo-articular disease that is characterized by a sterile, primarily chronic osteomyelitis with various distribution patterns of the individual lesions. In this article, we describe the "axial type" with predominant involvement of the spine, which represents 13 of our 41 CRMO cases of different age groups. The important element of its diagnosis is the typical lympho-plasmacellular spondylitis that can be detected and staged by scintigraphy, MRI and conventional radiography. Potentially affected are all vertebrae from the mid-cervical spine to the sacrum. One or several segments can be involved, sometimes as transient inflammat…
Children may not benefit from neuroblastoma screening at 1 year of age. Updated results of the population based controlled trial in Germany
Neuroblastoma is the second most frequent malignancy in childhood. We investigated whether screening for neuroblastoma at 1 year of age reduces the incidence of metastatic disease or mortality. Screening was offered in 6 of the 16 German states from 1995 to 2000 with the remaining states serving as controls. We studied 2,581,188 children in the screening area born between 1994 and 1999 and 2,117,600 in the control area. We compared mortality from neuroblastoma and the incidence of disseminated disease in the two groups. The screened group and the control group had similar rates of stage 4 neuroblastoma and mortality due to neuroblastoma. Comparison of the screened group and the control area…
The genetic tumor background is an important determinant for heterogeneous MYCN ‐amplified neuroblastoma
Amplification of MYCN is the signature genetic aberration of 20–25% of neuroblastoma and a stratifying marker associated with aggressive tumor behavior. The detection of heterogeneous MYCN amplification (hetMNA) poses a diagnostic dilemma due to the uncertainty of its relevance to tumor behavior. Here, we aimed to shed light on the genomic background which permits hetMNA in neuroblastoma and tied the occurrence to other stratifying markers and disease outcome. We performed SNP analysis using Affymetrix Cytoscan HD arrays on 63 samples including constitutional DNA, tumor, bone marrow and relapse samples of 26 patients with confirmed hetMNA by MYCN‐FISH. Tumors of patients ≤18m were mostly an…
Die chronisch rekurrierende multifokale Osteomyelitis (CRMO)
Chronic recurrent multifocal osteomyelitis (CRMO) is an unusual clinical entity. More than 200 cases are described in the literature and it is presented here with special reference to its radiological aspects. It is an acquired disease of the skeleton which occurs predominantly during childhood and adolescence. About ten per cent of cases begin in early or, rarely, in later adult life. This variant is described here for the first time and is discussed as "adult CRMO". The underlying pathology is a bland, predominantly lympho-plasma cellular osteomyelitis which is self-limiting and leads to bone sclerosis (Garre). It probably involves an abnormal immune process which follows an infection but…