The risk of nonocular cancer among survivors of retinoblastoma has been investigated in a populationbased study in Denmark, 1943 to 1984. None of the survivors had been treated with chemotherapeutic ...drugs. Forty‐eight patients were treated with x‐rays, and 102 patients were treated primarily with surgical removal of the eye(s). The overall relative risk (RR) for a new primary cancer was 4.2 (95% confidence limits, 1.1–11.5). In the subgroup of genetic retinoblastoma the risk was 15.4 (95% confidence limits, 2.6–50.8) and in the group of nonhereditary cancer the risk was 1.7 (95% confidence limits, 0.1–8.5). For all retinoblastoma patients the RR of bone cancer was 100 (95% confidence limits, 17–330). Parents not having retinoblastoma themselves were not at increased risk for nonocular cancer.
A population-based study was carried out on 5790 tumours in children (aged 0-14 years) diagnosed in the period 1943-1984 in Denmark. Cases were identified from the files of the high-quality National ...Cancer Registry in which codes for tumours were based solely on topography until the end of 1977. To achieve a uniform data set following the outlines of the International Classification of Diseases for Oncology (ICD-O) coding system used by the Cancer Registry today, all cases of childhood cancer diagnosed prior to 1978 were re-evaluated, and an ICD-O code was applied. Tumours were aggregated into diagnostic groups suitable for analysis and presentation using an internationally agreed scheme, which was designed by the Manchester Children's Tumour Registry and modified recently by the International Agency for Research on Cancer. The average incidence rates for all histological types of childhood cancer combined were 137 per million boys and 111 per million girls, which are close to those reported from the USA but higher than most of the overall figures reported from Europe. The proportions of specific tumours were similar to those observed in other industrialized countries. The well known excess of cancer cases among boys compared to girls was due mainly to the occurrence of 90% more lymphomas, 30% more leukaemias and 15% more tumours of the central nervous system (CNS) among boys. Although significant increases were seen in the subgroups of CNS neoplasms and neuroblastomas (both sexes) and of lymphomas (boys only), no overall increase in childhood cancer was observed during the 42-year period of registration. While the increase in the incidence of CNS tumours was explained at least partly by better cancer surveillance, no interpretation can be offered for the increases seen for neuroblastomas and lymphomas. Our descriptive data suggest that environmental exposures do not play any significant role in the aetiology of the majority of childhood cancers.
Radioimmunotherapy in indolent NHL is a new and promising therapy which adds a new therapeutic tool to the treatment palette. Recent advantages in RIT are described and perspectives discussed.
Today several monoclonal antibodies, including the anti-CD20 antibody (rituximab), the anti-CD52 antibody (alemtuzumab) and the anti-CD33 antibody (gemtuzumab ozogamacin) are all integrated in the ...therapeutic armamentarium of patients with malignant lymphoma, chronic lymphocytic leukaemia and acute myelogenous leukaemia, respectively. Rituximab has also been shown to be highly effective in the treatment of refractory autoimmune haemolytic anemias, idiopathic thrombocytopenia, and relapsing thrombotic thrombocytopenic purpura. New signal transduction inhibitors, dasatinib and nilotinib, are being used in patients with chronic myelogeneous leukaemia who develop resistance to imatinib. Thalidomide, lenalidomide and bortezomib have all been shown to be highly effective in multiple myeloma, and JAK2-inhibitors have entered phase II studies of patients with JAK2-positive primary myelofibrosis and related diseases.
: Extracellular proteolytic enzymes of the urokinase‐type plasminogen activator (uPA) system and the family of metalloproteases play a crucial role in the matrix degradation and tissue remodelling ...processes characteristic of malignant disorders. The receptor for urokinase plasminogen activator (uPAR) serves to localise and intensify the action of uPA and is expressed on the surface of malignant as well as tumour stromal cells including fibroblasts. A soluble form of uPAR (suPAR) cleaved from its glycosyl‐phosphatidylinositol anchor is detected in plasma from healthy individuals and increased levels of suPAR have been found in advanced malignancy, suggesting that suPAR may be a marker of extensive tissue remodelling. In an attempt to clarify whether suPAR might be a marker for bone marrow tissue remodelling we measured plasma suPAR levels in a patient cohort comprising 17 with myelofibrosis (MF), 17 with polycythaemia vera (PV), 15 with essential thrombocythaemia (ET), one with a transitional myeloproliferative disorder evolving from PV and 30 controls. Compared with controls suPAR levels were significantly higher in the patients (P < 0.0001) (median 3.35 vs. 2.32 µg L−1). Moreover, in subgroup analyses including patients with MF, PV, and ET, respectively, suPAR levels differed significantly with the highest levels found in patients with MF and PV (MF vs. PV vs. ET; P = 0.0003). When comparing suPAR levels of the individual patient subgroups with controls, only suPAR levels of PV and MF patients were significantly increased (P < 0.0001). Sixty‐five percent of patients with PV and MF (22/34) had suPAR plasma values that were above the mean +2 standard deviations (SD) of controls. The concentration of suPAR was significantly correlated to plasma lactate dehydrogenase, thrombomodulin, and complex of tPA:PAI‐1 in the patients. There was no difference between patients and controls when comparing plasma uPA levels. Increased plasma suPAR levels in patients with chronic myeloproliferative disorders may reflect increased uPAR production in the bone marrow, leading to enhanced bone marrow remodelling.
The increased risk of nonocular cancer seen consistently in studies of survivors of retinoblastoma may be caused in part by the presence of a retinoblastoma gene that also predisposes to other ...cancers. It has been claimed that this gene also increases the risk for cancer among unaffected relatives of genetic retinoblastoma probands. We report here a population-based study of the risk of nonocular cancer in parents and siblings of persons notified to the Danish Cancer Registry with retinoblastoma during 1943-84. No excess was observed among first degree relatives of 61 genetic retinoblastoma probands, whereas a slight (10%) excess was seen among the parents of 115 nongenetic probands. The latter was the result of significant excesses of malignant melanoma (4 observed, 0.4 expected), multiple myeloma (2 observed, 0.2 expected) and osteogenic sarcoma (1 observed, 0.03 expected). The observed risk pattern cannot be explained by the presence of the retinoblastoma gene.
PDF
