The study evaluated 139 patients diagnosed with Langerhans' cell histiocytosis (LCH) and enrolled in any protocol of the Italian Association of Pediatric Hematology/Oncology since 1982. Treatment was etoposide (VP-16) only in 50 patients, VP-16 and other drugs with an already established leukemogenic effect in 17 patients, only drugs with leukemogenic effect in 6 patients, other drugs in 35 patients, and surgery only in 31 patients. Median length of follow-up after diagnosis was 65 months (range, 1 to 126 months) for a total of 742.5 person-years at risk (PYRs). Three cases of acute myelogenous leukemia (AML) were reported; only 0.0044 case was expected. The standard incidence ratio (SIR) of AML in this cohort was 680.5 [95% confidence interval (CI), 140.2-1988.5], and the incidence rate per 1000 PYRs was 4.0 (95% CI, 0.8-11.8). For the subgroup treated with single-agent VP-16, the SIR after treatment was 2270.0 (95% CI, 275-8199), and the incidence rate after treatment was 14.7 (95% CI, 1.8-42.8). The study confirms a higher risk of leukemia after LCH and supports the hypothesis of an association between treatment-related acute nonlymphocytic leukemia and single-agent treatment with VP-16.

Antineoplastic Combined Chemotherapy Protocols↗

PubMedDate not supplied

Wilms' tumor after treatment.

Sixty-one Wilms' tumors (WTs) from 59 patients who received preoperative therapy were studied. Twenty-seven WTs from 26 patients who did not receive preoperative treatment were also reviewed as controls. Marked and diffuse morphological changes occurred in treated cases. Necrosis affected mostly undifferentiated and replicating elements and was extensive, up to 90% of tumor mass. Minimal residual tumor, permitting recognition as Wilms', was always spared. Epithelial and rhabdomyoblastic components were more resistant to treatment; moreover, they appeared to be susceptible to differentiation and maturation. Necrosis and muscle cell differentiation seemed to have prognostic implications. Cases with extensive necrosis (greater than 90%) had a better outcome, although the difference was not statistically significant. The rhabdomyoblast/tumor mass ratio, after treatment, appears to carry prognostic meaning. Chemotherapy had no apparent effect on anaplasia.

Adolescent↗

PubMedDate not supplied

Endocrine autoimmunity in young patients with juvenile chronic arthritis.

OBJECTIVE: The aim of our study was to investigate the coexistence of autoimmune diseases (autoimmune thyroid disease and type 1 diabetes mellitus, T1DM) in patients affected by Juvenile Chronic Arthritis (JCA). METHODS: We studied 66 patients affected by JCA, 42 females and 24 males: 42/66 patients had a pauciarticular form of JCA, 13/66 had a polyarticular form and 11/66 had a systemic form. All the patients underwent autoimmune thyroid screening through determination of anti-thyroglobulin (TgA) and anti-peroxidase (TPOA) autoantibodies. Patients with TgA and/ or TPOA, underwent thyroid sonography. T1DM screening included determination of anti-glutamic acid decarboxylase (GADA), anti-insulin (IAA), anti-tyrosine phosphatase-like protein (IA-2A) and anti-islet cell (ICA) autoantibodies. Oral glucose tolerance test (OGTT) was performed only in patients with autoantibody positive values. HLA typing for risk of T1DM was performed in 43 patients. RESULTS: Nine female patients (14%) showed anti-thyroid autoantibodies, in particular: TgA in 3 cases, TPOA in 5, TgA and TPOA in only 1. In 3 of these patients, ultrasound examinations showed thyroid abnormal pattern, suggesting Hashimoto's thyroiditis. As regards T1DM, only 2 patients showed positive levels of GADA. As regards HLA typing, one or more T1DM susceptibility heterodimers were detected in 20 patients (46%) (13 with 1 heterodimer, 7 with 2 heterodimers). CONCLUSION: Our study showed that anti-thyroid autoantibody frequency (9/66, 14%) was higher in JCA than in the general population, while T1DM markers (islet autoantibodies and genetic markers) were not frequent. These results suggest to investigate specific markers of thyroid autoimmunity in patients with JCA, in particular in females with JCA pauciarticular form.

Adolescent↗

PubMedDate not supplied

[Wilms' tumor, polycythemia, ichthyosis and ear malformations: a new syndrome?].

Wilms' tumor (WT) is frequently associated to congenital malformations, i.e. aniridia (0.8%), hemihypertrophy (2.5%), and genitourinary malformations. Ear malformations have been suggested to be a sign of genitourinary malformations. On the other hand WT cases associated to ichthyosis has never been reported. The authors present a case of Wilms' tumor (WT) associated to ear malformations, ichthyosis and polycythemia, and suggest that such a malformation pattern might represent a new syndrome.

Child↗

PubMedDate not supplied

Cytogenetics of infantile leukemias and its correlations with bio-clinical features. The "G. Gaslini" Children's Hospital experience over a 9-year period.

BACKGROUND AND METHODS: Infantile leukemia is a rare disorder, and few cytogenetic studies have been performed on this condition. RESULTS AND CONCLUSIONS: The authors present the cytogenetic analyses performed on 14 cases of infantile leukemia. The most frequent chromosomal changes are rearrangements involving 11q (4 cases) and gains of one or more chromosomes 21. Patients with chromosomal rearrangements show a worse prognosis than those with only hyperdiploidy or a normal karyotype, although the difference was not statistically significant due to the small size and short median follow-up.

Chromosome Aberrations↗

PubMedDate not supplied

[Plasminogen activator activity of the synovial fluid as an indicator of activation phenomena in degenerative joint diseases].

Activity of plasminogen activator in synovial fluids of patients with osteoarthritis was determined by a radial diffusion assay. Synovial fluid from human knee joints was obtained by joint lavage with 35 ml physiological saline containing 0.5% hydroxyethyl starch. Synovial fluids from contralateral, healthy knee joints served as controls. The activity of plasminogen activator in synovial fluid from activated (painful) osteoarthritis is significantly higher than in cases of latent osteoarthritis, whereas the activity of controls is significantly lower as in latent osteoarthritis. The findings are helpful for the discovery of patients with enhanced plasminogen activator levels in synovia which should be treated therapeutically.

Electrophoresis, Polyacrylamide Gel↗

PubMedDate not supplied

Very high dose cis-platinum (450 mg/sq m) in an infant with rhabdomyosarcoma.

The toxic effects of CDDP, erroneously given at 450 mg/sq m, are described. Life threatening gastrointestinal and myelotoxicity was observed as well as prolonged severe renal tubular acidosis without renal failure. No significant oto (brain-stem evoked responses) or neurotoxicity (EEG, neurologic examination and nerve conduction velocity) was detected indicating major age-related differences in the toxicity of this drug.

Cisplatin↗

PubMedDate not supplied

[Ewing's sarcoma in preschool children. Description of 6 cases].

Ewing's sarcoma (ES) is rarely diagnosed in the pre-school age; these few cases however present a number of difficult diagnostic and therapeutic problems. The Authors describe a series of six such cases aged less than 4 years diagnosed in the period 1974-1987. Standard treatment was modified with the purpose to reduce acute toxicity and late sequelae. These modifications are described in details. Four/6 patients are presently alive disease-free from 29 to 87 months from diagnosis (median, 34 months). Two patients died from acute toxicity. The Authors suggest that young children with ES may be treated successfully with proper adjustments of current protocols. Increased aggressiveness of chemotherapy regimens may compensate for reduced radiotherapy; however, the high susceptibility to infection of youngsters has to be taken into account and makes mandatory a careful monitoring during phases of profound myelodepression.

Antineoplastic Combined Chemotherapy Protocols↗