Biomedical subjects
Publications and source records attributed to G Perilongo.
The authors report the experience of the Italian Cooperative Study AIEOP-CNR RMS-79 concerning 16 children affected by localized paratesticular rhabdomyosarcoma (15 stage I, 1 stage II). The good results obtained by multidisciplinary treatment suggest a less aggressive approach as regards retroperitoneal lymph node biopsy and chemotherapy.
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This report deals with a randomized prospective multicentric clinical trial in childhood rhabdomyosarcoma (RMS) conducted to evaluate the toxicity and the effectiveness of dactinomycin (ACT-D) administered as high, single doses v five-day, divided doses administered in combination with vincristine (VCR) and cyclophosphamide (CYC). Fifty-five group III evaluable patients (pts) less than 15 years of age with tumor size greater than 5 cm in diameter, without high-risk features of CNS involvement, and 15 group IV RMS pts were randomized to receive VAC as primary chemotherapy (CT): VCR, 1.5 mg/m2 intravenously (IV) days 1 and 8; CYC, 275 mg/m2 IV days 1 through 5; and ACT-D, 0.45 mg/m2 IV days 1 through 5 every 28 days for three cycles (33 pts), or VAC-M: CYC, 150 mg/m2 intramuscularly (IM) days 1 through 7; VCR, 2.0 mg/m2 IV day 8; and ACT-D, 1.7 mg/m2 IV day 8 every 21 days for four cycles (37 pts). Major responses (complete plus partial responses [PR]) were obtained in 67% of the VAC pts and in 70% of the VAC-M pts. Toxic effects were low, and no increased toxicity was observed in pts treated with high, single-dose ACT-D. These results confirm the effectiveness and feasibility of single, high doses of ACT-D with the advantage of requiring less pt hospitalization.
The clinical features and the treatment of undifferentiated (embryonal) sarcoma of the liver in 8 patients younger than 19 years old were analyzed. All these cases were registered in the retrospective multicentric study on childhood malignant tumors of the liver, conducted between 1983 and 1985 by the Italian Association of Pediatric Hematology Oncology. The age of the patients ranged from 94 to 190 months (median = 113.5 months); all children were males. An abdominal mass was the common presenting features. Abnormalities in hemogram and common liver tests were rarely reported. Angiography revealed various degrees of vascularization in these tumors. Two patients achieved a surgical complete remission (CR) at diagnosis; one patient achieved surgical CR after primary chemotherapy with vincristine, adriamycin, cyclophosphamide and 5-fluorouracil, which reduced the tumor volume and permitted surgical resection. Two of these patients are still in CR at 14 and 60 months after diagnosis; the third patient died of liver failure without evidence of recurrence 6 months after diagnosis. All of the other patients, who never achieved CR, died of disease. One was lost to follow-up, and one surgical death occurred. Reports of childhood undifferentiated sarcoma are reviewed.
We analyzed the clinical features and treatment of 23 hepatoblastomas (HPBs) and 16 hepatocellular carcinomas (HPCs) occurring in patients less than 19 years old, admitted to the Italian retrospective multicentric study, conducted between 1983 and 1985, on childhood malignant hepatic tumors. The median ages of the patients with HPB and HPC at diagnosis were 22.34 months and 96.23 months, respectively, with a male/female ratio of 0.7 and 1.7, respectively. Fourteen HPBs (61%) and 5 HPCs (31%) achieved surgical complete remission (CR). Of these, 11 HPB and all 5 HPC are still in CR with a median follow-up of 36 months and 3.5 years, respectively. One HPB and 1 HPC became resectable after a primary course of cis-platinum alone in the case of HPB and used with VP-16 in the case of HPC. All of the 9 HPBs and 11 HPCs, who never achieved CR, died of disease at a median interval from diagnosis of 5 and 2 months, respectively. The published therapeutic approaches for these tumors were also reviewed.
A phase II multicenter evaluation of cisplatin (90 mg/m2, Day 1) and etoposide (150 mg/m2, Days 2, 3, and 4) in 4-week cycles was carried out in 21 relapsed children with rhabdomyosarcoma (RMS) and six with non-RMS soft tissue sarcomas (NRSTS). Clinical responses were evaluated after four cycles. There were three complete responses (CRs) and four partial responses (PRs) among the 21 patients with RMS and no responses among the patients with NRSTS. The durations of the three CRs and the four PRs were 11, 9, and 8 months, and 5, 2, 2, and 1 month, respectively. Bone marrow and renal toxicity was cumulative and predictable, but not life threatening. The response rate (CR + PR = 33.3%) to cisplatin and etoposide warrants testing as front-line therapy for RMS.
Seventy-two successfully treated patients with acute lymphocytic leukemia, all in first complete remission and all off therapy, who had received CNS prophylaxis (radiotherapy, 2,400 rad, plus intrathecal methotrexate), were studied by computed tomography (CT) of the brain, EEGs, and neurologic evaluations 3 to 9 years after the end of prophylaxis. Thirty-five patients showed CT brain scan abnormalities: intracranial calcifications (twelve); widening of the subarachnoid spaces (eight); isolated dilatation of ventricular spaces (three) and with frontal periventricular hypodensity (two); dilatation of ventricular and subarachnoid spaces (nine); and a hypodense area (one). Only 17 patients showed aspecific EEG abnormalities which were never linked to CT scan findings. None of our patients presented major motor deficits at the neurologic examination. A stepwise logistic regression technique showed that age less than 5 years at the time of prophylaxis was the most important risk factor (p = 0.008) of CT brain scan abnormalities followed by neurets (p = 0.037) and sex (p = 0.10). Furthermore, the multivariate analysis pointed out that the interactions between these variables were not significant and the effects were only of the first order.
Between 1975 and 1989, 45 children with newly diagnosed intracranial ependymomas were treated at the authors' institution. Patients were managed with aggressive surgical resection, followed by postoperative CT or MRI scans to evaluate the extent of resection. Most patients received involved-field radiation therapy, however 10 were treated with craniospinal axis irradiation for disseminated disease, or malignant histology. Beginning in 1983, all patients were also treated with adjuvant chemotherapy with 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU), vincristine, and cisplatinum. Four patients died in the immediate postoperative period for an operative mortality of 8.8%. The 5-year progression-free survival for the remaining 41 patients was 36%. Tumor location had little effect on survival, as the 5-year PFS for supratentorial tumors was 26%, compared with 40% for the posterior fossa lesions (ns). Neither histologic degree of malignancy, nor use of adjuvant chemotherapy impacted on survival. The extent of surgical resection, as determined by operative reports and postoperative imaging studies, was a major determinant of outcome, as patients with total or near-total resections experienced a 5-year PFS of 60%, compared with 21% for children with partial resection or biopsy (p less than 0.01). It is concluded that the use of adjuvant chemotherapy with CCNU, vincristine, and cisplatinum does not improve progression-free survival in childhood ependymoma, and that the extent of surgical resection is a major determinant of outcome in this disease.
We describe a young patient with combined hamartoma of the retina and retinal pigment epithelium who developed bilateral acoustic neuromas and meningiomas of the cervical-medullary junction and fifth cranial nerve. This case illustrates the association between combined hamartoma of the retina and retinal pigment epithelium and neurofibromatosis type 2. The authors recommend that children with a combined hamartoma of the retina and retinal pigment epithelium be screened for neurofibromatosis type 2.
We present a patient with a Wilms' Tumor recurring after 7 years from the primitive surgical excision. After the relapse, surgery and conventional chemotherapy (with Actinomycin-D, Cyclophosphamide and Vincristine) permitted to achieve a second complete remission. Now the patient is alive without evidence of disease after 4 years from the relapse. The non-complete surgical excision of the tumor and the particular biological activity of the residual cells could explain the late relapse.
The authors discuss the CT findings of the late brain damage by the radiochemotherapic treatment for the prophylaxis of meningeal leukemia in 50 patients, 1 divided by 15 years old. The more frequent CT findings are: enlargement of the subaracnoid spaces; ventricular dilatation; hypodensity of cerebral parenchima; calcifications. These findings correspond to two clinical and anatomo-pathological conditions: subacute leucoencephalopathy and mineralizing microangiopathy. A greater damage on central nervous system was demonstrated in younger children (5 years old) at the time of prophylaxis of meningeal leukemia, that were irradiated with NSD = 950 ret.
From March, 1990 to February, 1992, we administered fluconazole as antifungal prophylaxis at doses of 3-5 mg/kg/day to 40 patients with prolonged and severe neutropenia following intensive chemotherapy. Fungemia was observed in 3 out 40 patients, and all three of them were due to Candida non-albicans strains: two Candida parapsilosis and one Candida guilliermondi. In vitro sensitivity tests showed that all three isolated strains were susceptible to amphotericin. In one case, Candida guilliermondi was tested for sensitivity to fluconazole and found to be resistant. We conclude that fluconazole prophylaxis proved effective in preventing Candida albicans infections, but it could also contribute to the emergence of Candida non-albicans strains. It might be possible that fluconazole at higher doses could prevent the selection of less susceptible Candida strains.