The leukaemias and lymphomas of childhood account for almost half of the malignant disease seen i... more The leukaemias and lymphomas of childhood account for almost half of the malignant disease seen in this age group in the Western world. The lymphomas represent 10% of the total, with an approximately even division between Hodgkin’s disease and non-Hodgkin’s lymphoma. Hodgkin’s disease was the first of the childhood lymphoid malignancies to become curable and still has the best prognosis of the group. The prognosis for acute lymphoid leukaemias improved substantially in the early 1970s but has recently been overtaken by dramatic advances in the curability of the non-Hodgkin’s lymphomas.
Cancer is primarily a disease of the elderly and the proportion of people affected in childhood i... more Cancer is primarily a disease of the elderly and the proportion of people affected in childhood is very small. Nevertheless, about one person in every 500 will develop malignant disease during the years of childhood and, despite a much improved prognosis, cancer remains the most common disease to kill children. At least one in every 1000 young people entering adult life has been cured of childhood cancer, and the physical and psychological sequelae of the disease and its treatment are therefore matters of concern.
We report a case of a 16 year old girl with orbital rhabdomyosarcoma who during chemotherapy deve... more We report a case of a 16 year old girl with orbital rhabdomyosarcoma who during chemotherapy developed bloody diarrhoea. On investigation she was found to have multiple colonic polyps. Cytogenetic analysis has shown a pattern typical of familial adenomatous polyposis. We present a review of the literature concerning the associations of familial adenomatous polyposis. The association of familial adenomatous polyposis with rhabdomyosarcoma has not been previously reported.
This report describes two children with lymphoblastic lymphoma who relapsed more than 2 1/2 years... more This report describes two children with lymphoblastic lymphoma who relapsed more than 2 1/2 years from diagnosis. Relapses occurred at seven and 20 months after completion of treatment. Their therapy consisted of an intensive pulse chemotherapy program combined with radiation therapy. Initial relapse after two years' treatment has been extremely rare in patients receiving contemporary chemotherapy programs, and two-year survival without disease has been considered a cure. These cases illustrate that late relapses can occur after intensive chemotherapy and that two-year disease-free survival must not be interpreted as a complete cure.
A 14-month-old boy presented with hepatoblastoma, which was completely excised. He had pulmonary ... more A 14-month-old boy presented with hepatoblastoma, which was completely excised. He had pulmonary metastases, diagnosed 1 year later, treated with chemotherapy followed by resection at age 2 1/2 years. At the age of 3 1/2 years a further metastasis was shown on CT scan and this was resected. Two further metastses were resected at ages 4 1/2 and 5 1/2 years. At age 9 1/2 he had a further isolated pulmonary metastasis resected and is again disease-free more than 30 months from the last thoracotomy. Prolonged survival following metastatic hepatoblastoma can be achieved with repeated thoracotomies, especially if the first recurrence is more than 6 months from diagnosis and the number of metastases is small. Follow-up examinations should be both radiological and with serum alpha fetaprotein (S alpha FP).
Abnormal visual evoked potentials (VEPs) have been reported in children treated for acute lymphob... more Abnormal visual evoked potentials (VEPs) have been reported in children treated for acute lymphoblastic leukaemia (ALL), which suggests that VEPs may be useful in screening for toxicity. The authors investigated this by recording flash and pattern VEPs in a control group of 34 siblings of patients, a group of six children studied longitudinally during the early stages of treatment for ALL, and three other follow-up groups. In only three follow-up patients were VEP results outside the normal range and the six ALL patients did not develop new abnormalities during early treatment. Although differences were detected between the groups, there was no evidence of VEPs being a useful means of monitoring the treatment of individual patients.
A boy suffering from the Beckwith-Wiedemann syndrome (BWS) was found to have partial trisomy of t... more A boy suffering from the Beckwith-Wiedemann syndrome (BWS) was found to have partial trisomy of the short arm of chromosome 11 [46,XY,der(5)t(5;11)(p15.2;p14)]. Both his parents were phenotypically normal, but his father carried a balanced translocation between chromosomes 5 and 11 [46,XY,t(5;11)(p15.2;p14)]. DNA analysis of polymorphic markers on 11p15 confirmed the paternal origin of the duplicated material in the child. This case is the sixth report of paternal duplication of 11p15 in BWS. These results are discussed in relation to the possible role of genomic imprinting in BWS and in Wilms' tumor.
The therapeutic and prognostic implications of relapse and clonal evolution of leukaemia are subs... more The therapeutic and prognostic implications of relapse and clonal evolution of leukaemia are substantially different from those of secondary (induced) malignancy. This report documents the case of a patient who presented with apparent acute non-lymphocytic leukaemia (ANLL) following therapy for acute (T-cell) lymphoblastic leukaemia (ALL) 4 years previously. Morphologically and cytochemically the cells were of myeloid type, but the cell markers showed a T cell lineage. Cytogenetic studies confirmed that this was a relapse of T cell ALL with a phenotypic change, rather than a second malignancy induced by chemotherapy. A 14q deletion present at initial diagnosis recurred at relapse, with the addition of cells with complete deletion of chromosome 14, indicating clonal evolution.
The records of 121 children presenting with acute lymphoblastic leukaemia between 1969 and 1982 w... more The records of 121 children presenting with acute lymphoblastic leukaemia between 1969 and 1982 were reviewed. The overall relapse free survival rate was 50%. However, girls presenting with a total white blood cell count of 20 X 10(9)/l or less (35% of all patients) had a particularly favourable prognosis with an 80% relapse-free survival rate at 12 years. The difference in prognosis between the sexes is confirmed but this difference is confined to the low initial white cell count group. We suggest that girls in this group should be excluded from the more intensive arms of new treatment protocols.
The leukaemias and lymphomas of childhood account for almost half of the malignant disease seen i... more The leukaemias and lymphomas of childhood account for almost half of the malignant disease seen in this age group in the Western world. The lymphomas represent 10% of the total, with an approximately even division between Hodgkin’s disease and non-Hodgkin’s lymphoma. Hodgkin’s disease was the first of the childhood lymphoid malignancies to become curable and still has the best prognosis of the group. The prognosis for acute lymphoid leukaemias improved substantially in the early 1970s but has recently been overtaken by dramatic advances in the curability of the non-Hodgkin’s lymphomas.
Cancer is primarily a disease of the elderly and the proportion of people affected in childhood i... more Cancer is primarily a disease of the elderly and the proportion of people affected in childhood is very small. Nevertheless, about one person in every 500 will develop malignant disease during the years of childhood and, despite a much improved prognosis, cancer remains the most common disease to kill children. At least one in every 1000 young people entering adult life has been cured of childhood cancer, and the physical and psychological sequelae of the disease and its treatment are therefore matters of concern.
We report a case of a 16 year old girl with orbital rhabdomyosarcoma who during chemotherapy deve... more We report a case of a 16 year old girl with orbital rhabdomyosarcoma who during chemotherapy developed bloody diarrhoea. On investigation she was found to have multiple colonic polyps. Cytogenetic analysis has shown a pattern typical of familial adenomatous polyposis. We present a review of the literature concerning the associations of familial adenomatous polyposis. The association of familial adenomatous polyposis with rhabdomyosarcoma has not been previously reported.
This report describes two children with lymphoblastic lymphoma who relapsed more than 2 1/2 years... more This report describes two children with lymphoblastic lymphoma who relapsed more than 2 1/2 years from diagnosis. Relapses occurred at seven and 20 months after completion of treatment. Their therapy consisted of an intensive pulse chemotherapy program combined with radiation therapy. Initial relapse after two years' treatment has been extremely rare in patients receiving contemporary chemotherapy programs, and two-year survival without disease has been considered a cure. These cases illustrate that late relapses can occur after intensive chemotherapy and that two-year disease-free survival must not be interpreted as a complete cure.
A 14-month-old boy presented with hepatoblastoma, which was completely excised. He had pulmonary ... more A 14-month-old boy presented with hepatoblastoma, which was completely excised. He had pulmonary metastases, diagnosed 1 year later, treated with chemotherapy followed by resection at age 2 1/2 years. At the age of 3 1/2 years a further metastasis was shown on CT scan and this was resected. Two further metastses were resected at ages 4 1/2 and 5 1/2 years. At age 9 1/2 he had a further isolated pulmonary metastasis resected and is again disease-free more than 30 months from the last thoracotomy. Prolonged survival following metastatic hepatoblastoma can be achieved with repeated thoracotomies, especially if the first recurrence is more than 6 months from diagnosis and the number of metastases is small. Follow-up examinations should be both radiological and with serum alpha fetaprotein (S alpha FP).
Abnormal visual evoked potentials (VEPs) have been reported in children treated for acute lymphob... more Abnormal visual evoked potentials (VEPs) have been reported in children treated for acute lymphoblastic leukaemia (ALL), which suggests that VEPs may be useful in screening for toxicity. The authors investigated this by recording flash and pattern VEPs in a control group of 34 siblings of patients, a group of six children studied longitudinally during the early stages of treatment for ALL, and three other follow-up groups. In only three follow-up patients were VEP results outside the normal range and the six ALL patients did not develop new abnormalities during early treatment. Although differences were detected between the groups, there was no evidence of VEPs being a useful means of monitoring the treatment of individual patients.
A boy suffering from the Beckwith-Wiedemann syndrome (BWS) was found to have partial trisomy of t... more A boy suffering from the Beckwith-Wiedemann syndrome (BWS) was found to have partial trisomy of the short arm of chromosome 11 [46,XY,der(5)t(5;11)(p15.2;p14)]. Both his parents were phenotypically normal, but his father carried a balanced translocation between chromosomes 5 and 11 [46,XY,t(5;11)(p15.2;p14)]. DNA analysis of polymorphic markers on 11p15 confirmed the paternal origin of the duplicated material in the child. This case is the sixth report of paternal duplication of 11p15 in BWS. These results are discussed in relation to the possible role of genomic imprinting in BWS and in Wilms' tumor.
The therapeutic and prognostic implications of relapse and clonal evolution of leukaemia are subs... more The therapeutic and prognostic implications of relapse and clonal evolution of leukaemia are substantially different from those of secondary (induced) malignancy. This report documents the case of a patient who presented with apparent acute non-lymphocytic leukaemia (ANLL) following therapy for acute (T-cell) lymphoblastic leukaemia (ALL) 4 years previously. Morphologically and cytochemically the cells were of myeloid type, but the cell markers showed a T cell lineage. Cytogenetic studies confirmed that this was a relapse of T cell ALL with a phenotypic change, rather than a second malignancy induced by chemotherapy. A 14q deletion present at initial diagnosis recurred at relapse, with the addition of cells with complete deletion of chromosome 14, indicating clonal evolution.
The records of 121 children presenting with acute lymphoblastic leukaemia between 1969 and 1982 w... more The records of 121 children presenting with acute lymphoblastic leukaemia between 1969 and 1982 were reviewed. The overall relapse free survival rate was 50%. However, girls presenting with a total white blood cell count of 20 X 10(9)/l or less (35% of all patients) had a particularly favourable prognosis with an 80% relapse-free survival rate at 12 years. The difference in prognosis between the sexes is confirmed but this difference is confined to the low initial white cell count group. We suggest that girls in this group should be excluded from the more intensive arms of new treatment protocols.
Uploads
Papers by Martin Mott