Health status of long-term survivors after cancer in childhood. Results of an uniinstitutional study in Italy.

PURPOSE: This study aims at defining the frequency and severity of late effects in a series of 288 long-term survivors of childhood cancer treated from 1962 to 1982 at the Giannina Gaslini Children's Research Hospital of Genoa, Italy. PATIENTS AND METHODS: All cases with a diagnosis of malignancy in childhood and a minimum of 2.5 years from discontinuation of treatment were considered eligible. For all cases the study included physical, endocrinological, and psychological examination. Groups of patients selected according to treatment underwent cardiac, pulmonary, orthopedic, and ophthalmologic evaluation. The sequelae observed were scored according to a grading system in which asymptomatic subclinical defects are distinguished from those that are sufficiently symptomatic to require some type of corrective measure. RESULTS: Overall, 200 of 288 cases (69.4%) presented with some kind of abnormality. Symptomatic changes were present in 92 cases (42%); in these, severe and life-threatening late toxicity was reported in 61 (21.2%) and 12 cases (4.2%), respectively. The major risk factors appeared to be irradiation, type of tumor, and whether the patient had received therapy before 1974. CONCLUSIONS: In our experience, this study demonstrates that there was a true excess of morbidity caused by the disease and its treatment in long-term survivors from almost any kind of childhood cancer. It also sheds light on how to prevent, diagnose, and adequately treat these patients and proposes specific criteria for the evaluation of the severity of delayed toxicity in long-term survivors of cancer in childhood.

Langerhans' cell histiocytosis: a case report.

The authors describe the morphologic features of Langerhans' cell histiocytosis and discuss the main prognostic factors which divide patients into two groups requiring different treatment. They then describe a case of multifocal Langerhans' cell histiocytosis due to pelvic osteolysis and early diabetes insipidus, emphasizing the effectiveness of the combination of surgery and chemotherapy and the rapid reparative new bone formation.

Height of children off therapy after acute lymphoblastic leukemia.

A total of 290 children off therapy after acute lymphoblastic leukemia, in continuous complete remission for at least 2 years, were evaluated for height at the onset of the disease and at the most recent clinical visit (median time after suspension of treatment 4 years 4 months, range 2 years-11 years 3 months). All patients had been treated with multidrug schedules; intrathecal drugs had been given to 84% of the patients for prevention of CNS involvement, associated with radiotherapy. The height percentiles at the most recent examination were shifted downward significantly compared with the expected pattern (p less than 0.001). The effect on stature was much more marked in girls, with a reduction of height percentiles at most recent examination from expected and from diagnosis; in males there was a reduction from diagnosis to latest follow-up, but the values were within the limits of normal. The short stature was mostly observed in pubertal girls and in patients who had undergone radiotherapy.

Health status of young children with cancer following discontinuation of therapy.

This paper reports late effects and health status of 198 children who had cancer or leukemia diagnosed under 2 years of age and their therapies electively withdrawn. This series (92 neuroblastoma [NBL], 57 Wilms' tumor [WT], 46 acute lymphoblastic leukemia [ALL], and 3 non-Hodgkin's lymphoma) was followed for 1-12 years after discontinuation of therapy. Thirty-three children were diagnosed before 1973, 92 between 1973 and 1977, and 73 after 1977 in 16 Italian Pediatric Oncology Centers. As of December 1983, 176 children were reported to be alive and without evidence of primary cancer by physicians responsible for their care. One child died from a second primary tumor, two from late recurrences of the primary cancer, and three from other causes; eight were alive with evidence of primary cancer; and eight were lost to follow-up. Kyphoscoliosis was found in 22 children and other musculoskeletal anomalies in 8. Neurological sequelae were observed in 8 out 35 children with ALL treated with radiotherapy (RT) and intrathecal methotrexate. All but one were in continuous complete remission when they developed seizures (three cases), leukoencephalopathy (three cases), or intracerebral calcifications (two cases). One child had cardiomyopathy and subsequently died from cardiac failure: he had received doxorubicin (400 mg/m2) and mediastinal RT (13 Gy) for NBL. Growth impairments were observed in children with NBL and WT.

[Skeletal changes in acute lymphoblastic leukemia in children. Incidence and prognostic significance]. / Alterazioni scheletriche nella leucemia linfoblastica acuta del bambino. Incidenza e significato prognostico.

119 children with acute lymphoblastic leukemia at onset underwent radiography of the whole body in order to determine whether initial radiological bone lesions were correlated with subsequent clinical course. Radiological findings were divided into three groups: 101 children (84.8%) had no bone involvement (group 0); 10 had bone changes compatible with acute lymphoblastic leukemia, such as slight metaphyseal transverse lucent bands with or without diffuse demineralization (group 1); 8 had bone changes, such as periosteal reaction with or without intramedullary osteolytic mottling. Bone involvement was not correlated with prognostic factors. Furthermore there was no significant correlation between bone changes and complete remission or survival.

[2 cases of osteomyelitis in acute leukemia in the induction phase of treatment]. / Due casi di osteomielite in fase di induzione di leucemia acuta.

Whereas children with Acute Leukemia are highly susceptible to infectious complications, the occurrence of acute osteomyelitis is extremely rare in these patients. The authors describe two such cases in children at onset of an acute lymphoblastic and of a myelomonocytic leukemia, respectively. In the former case, the clinical course has been characterized by the progressive involvement of several joints and bones. A citrobacter Freundii was isolated in the synovial fluid of an involved knee. This complication was successfully treated with proper antimicrobic agents and surgical toilet, while the patient was vigorously treated for her leukemia, achieving a complete remission. The latter case developed a right humerus osteomyelitis from an Enterobacter. The patient failed to respond to antibiotics, and his leukemia also turned refractory to antiblastic therapy. The difficulty in the differential diagnosis among the X-graphic aspects of leukemic, inflammatory and degenerative disease of bones are discussed by the authors. Some pathogenetic hypothesis of leukemic osteomyelitis are also presented.