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.

[The mammographic images of the irradiated breast after conservative therapy for carcinoma]. / Quadri mammografici della mammella irradiata dopo terapia conservativa per carcinoma.

The mammographic patterns of the patients treated with conservative surgery (quadrantectomy) plus radiotherapy for early breast carcinoma were evaluated to assess treatment-induced changes over time and to improve the differential diagnosis between postirradiation effects and possible tumor recurrences. The mammographic examinations of 79 patients who had undergone quadrantectomy and radiotherapy for breast carcinoma (stage T1-T2) were examined. Skin thickening, edema, fibrosis, distortion and calcifications were considered and classified by comparing the radiographic patterns of the treated breast with those of the contralateral and untreated one. Pattern changes over time were also evaluated and quantified by comparing serial follow-up examinations of the same breast. The percentage of patients with irradiation-induced skin thickening steadily reduced from 100% at 6 months to just above 50% at 4 years. The number of patients showing diffuse irradiation-induced edema decreased from 56% at 6 months to 15% at 1 year and to 0% at 2 years, while the number of cases with localized edema decreased more slowly. The incidence of localized postirradiation breast fibrosis increased to 74% at 4 years, while diffuse fibrosis stabilized around 14%. The patients with no postoperative breast distortion accounted for nearly 33% of the examined cases, while those with minimal distortion approximated 58% and those with gross distortion 9%. In conclusion, the overall results confirmed the value of mammography in the follow-up of the patients treated with QUART.

[The multidisciplinary treatment of rhabdomyosarcoma in childhood. A 10-year multicenter experience (1978-1988) in Liguria]. / Trattamento multidisciplinare del rabdomiosarcoma in età pediatrica. Esperienza pluricentrica decennale (1978-1988) in Liguria.

The authors retrospectively analyzed the therapy results in a large group of children affected by rhabdomyosarcoma and treated from 1978 to 1988 with chemotherapy, radiotherapy and surgery. 39/58 patients (67%) achieved a complete response and 25/39 maintained the NED status. An individualized treatment was given to 13 patients who relapsed, but only 3 of them obtained a second response. Those with primary tumor in the orbit or in the genito-urinary tract had lower recurrence rate than patients with head and neck, extremities, pelvis and trunk diseases. Patients in clinical group I and II (according with I.R.S. stadiation) had a significantly better survival than those with more advanced disease (clinical groups III and IV). Relatively to the primary site of the disease, the late effects of combined treatment are mentioned.

Fractionated total body irradiation in marrow transplantation for leukaemia.

Thirty consecutive patients with leukaemia were prepared for bone marrow transplantation (BMT) with cyclophosphamide (CY) 120 mg/kg followed by total body irradiation (TBI). TBI was delivered in a single dose (sTBI) of 10 Gy, at a dose rate of 0.06-0.08 Gy/min, or in fractionated doses (fTBI) of 3.3 Gy/d, on each of 3 consecutive days, at the same dose rate. Lung shielding was adopted for all patients, in order to obtain a homogeneous dose delivered to the lung and at midline. The first 12 patients were prepared with sTBI and the following 18 with fTBI. Several variables (remission status, mode of irradiation, prevention of graft versus host disease (GvHD) with methotrexate (MTX) or cyclosporin A(CyA) were then analysed for their influence on (a) incidence and mortality of GvHD, (b) incidence and mortality of interstitial pneumonia (IP), (c) 100-d survival, and (d) relapses. Our data suggest that fTBI is the single most important factor associated with a lower incidence of IP (P = 0.002), a lower mortality from GvHD and IP (P = 0.03 and 0.001) and a better 100-d survival (P = 0.03). Remission status had no significant influence on GvHD, IP and acute mortality. When compared to MTX, the use of CyA was associated with less GvHD and IP, but when only patients in remission given fTBI were analysed, this was no longer true. Relapses were mostly influenced by the remission status of the patient at transplant (P = 0.001). Although this is not a randomized study, our data suggest that fTBI can reduce the acute risks of marrow transplantation in leukaemia.

[Acute non-lymphoblastic leukemia in patients treated for Hodgkin's disease. Analysis of 309 cases]. / ANLL in pazienti trattati per morbo di Hodgkin. Analisi di 309 casi.

The analysis of 309 Hodgkin patients treated by radiotherapy alone or in association with chemotherapy, between 1969 and 1979, has shown seven cases of ANLL (acute non lymphoblastic leukemia). The incidence was 2.26% in the overall group and 3.38% in the patients treated with combined therapies. From the frequency and distribution of ANLL in the various treatment groups we can argue that: ANLL may be considered as a second, therapy-induced tumor, correlated with the association of chemotherapy and radiotherapy.