Chemo- and radiosensitivity testing in a patient with ataxia telangiectasia and Hodgkin disease

Treatment of Hodgkin disease (HD) in ataxia telangiectasia (AT) patients is hampered by hypersensitivity to radiation and chemotherapy. Most patients die, due to toxicity or rarely, to progressive disease. The authors report on a 9-year-old girl with stage IIA HD and AT She was treated with a tailored combined modality approach. No unacceptable toxicity was found, but the girl died of a relapse outside the irradiation field. In comparison with fibroblasts of non-AT patients, the fibroblasts of the patient were 3 times as sensitive for radiotherapy but just 1.2 times as sensitive for doxorubicin. A good correlation was shown between in vitro radio and chemosensitivity testing and the observed clinical toxicity. The authors suggest, therefore, treating AT patients as much as possible according to standard protocols by adjusting the radiotherapy delivery and the chemotherapy regimen to individual doses derived from in vitro radio and chemosensitivity testing

Renal function after unilateral nephrectomy for Wilms' tumour:The influence of radiation therapy

The effect of therapy on renal function after unilateral nephrectomy for Wilms' tumour was studied. In the second year following unilateral nephrectomy, glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) were estimated simultaneously by measuring I-125-iothalamate clearance and I-131-hippurate clearance. Of 41 evaluable patients, 29 received chemotherapy as sole treatment modality following nephrectomy (group 1); 12 patients additionally received radiation therapy to a field that included the remaining kidney (group 2). Results were expressed as standard deviation scores (z-scores). In group 1, mean z-score for GFR was -0.27 (94.6% of normal) and in group 2 mean z-score was -1.51 (72.7% of normal for two kidneys) (P = 0.022, Mann-Whitney U-test). Mean z-score for ERPF was -0.09 (97.0%) in group 1 and -1.53 (73.8%) in group 2 (P = 0.039). It was concluded that the combination of chemotherapy and radiation therapy, in contrast to chemotherapy alone, negatively affects the ability of the remaining kidney to adjust its function after the loss of its counterpart

Phase II study of intensive chemotherapy with autologous bone marrow transplantation in patients in complete remission of disseminated breast cancer

Background: This trial studied the disease-free survival after high-dose chemotherapy in patients in complete remission of metastatic breast cancer. Patients and methods: Thirty women, mean age 42.2 years (range 33-55) with metastatic breast cancer, received high-dose chemotherapy in a phase II study. Patients were eligible if they were less than or equal to 55 years of age, had achieved complete remission within 6 months of the initiation of chemotherapy, and had a WHO performance scale of 0 or 1. The high-dose regimen consisted of melphalan 180 mg/m(2) and mitoxantrone 60 mg/m(2) both divided over 3 days. On day 7 bone marrow and/or peripheral stem cells were infused. After bone marrow recovery, external beam radiation was administered to sites of previous metastatic disease in 15 patients. Results: Apart from leuko- and thrombocytopenia, mucositis was the major side effect. One patient died during the bone marrow transplant period due to an aspergillus infection. The median follow-up since high-dose chemotherapy is 25 months (range 13 to 56 months). The median disease-free survival since high-dose chemotherapy is 27 months and the disease free survival is still 43% with an overall survival of 53% at 3 years. In two patients tumor relapse occurred only in the brain; in one patient the only relapse sign was a meningeal carcinosis. At the moment 17 patients are disease-free (13+-56+) months after high-dose chemotherapy. Conclusion: Until now this high-dose regimen in selected patients with complete remission after induction chemotherapy for metastatic breast cancer has a promising disease free survival

AGGRESSIVE FIBROMATOSIS (NONFAMILIAL DESMOID TUMOR) - THERAPEUTIC PROBLEMS AND THE ROLE OF ADJUVANT RADIOTHERAPY

Aggressive fibromatosis is a rare soft tissue tumour with a high tendency to local recurrence, even after apparently adequate resection. Wide local excision with a margin of at least 3 cm, depending on the anatomical location, should be performed to improve rates of recurrent disease. There is no consensus concerning the role of radiotherapy in the treatment of these lesions. The clinical findings of 39 cases diagnosed between 1972 and 1991 were reviewed retrospectively. Local control was effected in 19 of 32 patients treated with surgery alone after a median (range) follow-up of 72 (18-236) months. There were 40 cases of recurrent fibromatosis in 15 patients. Local control was obtained in 13 of 14 patients who received radiotherapy using a wide-field technique and doses of more than 50 Gy over a period of 5 weeks after marginal or incomplete resection of primary or recurrent lesions (P <0.001). The results suggest that in a selected group of patients with aggressive fibromatosis radiotherapy may effectively achieve control of residual disease after surgery without marked disfigurement and loss of function

Pulmonary function after high-dose chemotherapy with autologous bone marrow transplantation and radiotherapy in patients with advanced loco-regional breast cancer

Our aim was to study the extent of pulmonary toxicity after high-dose chemotherapy and radiotherapy in breast cancer patients. In a retrospective study the pulmonary symptoms and chest X-rays were analysed before, during and after treatment in 17 patients, treated with loco-regional radiotherapy to the breast/chest wall and the regional lymph nodes after the completion of high-dose chemotherapy and autologous bone man ow transplantation for locally advanced breast cancel: Lung function was evaluated between 15 and 46 months after completion of the irradiation. Nine patients (53%) had pulmonary symptoms during and/or within two months from irradiation. Radiographic changes were seen in twelve patients (71%) and a decreased diffusion capacity occurred in nine patients (53%). The spirometric values (VC and FEV1) were abnormal in two patients (12%). There is a high incidence of radiation pneumonitis, although no consistent correlation between the various parameters (pulmonary symptoms, chest X-rays and lung function tests) could be found