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Biomedical subjects

M Guglielmi

Publications and source records attributed to M Guglielmi.

At least 55 records · Page 3Linked to original sources
PubMed1987

Therapeutic approach to primary malignant epithelial tumors of the liver in childhood: results of the Italian retrospective study and literature survey.

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.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1983

The role of surgery in non-localized neuroblastoma. Analysis of 59 cases.

The authors considered 59 stage III and IV neuroblastoma cases, which were observed from January 1967 to June 1979. 13 patients (13/59 = 22%) are surviving in complete remission 3 years after surgery: 9/20 (45%) with stage III and 4/39 (10%) with stage IV disease. The children subjected to complete surgical excision are all alive irregardless of age; the children who were not operated on or subjected to a biopsy, have all died. Of the 33 patients who underwent a reductive excision, 6 (all under 2 years of age) are in complete remission (18%). A better prognosis was found in those patients in whom the site of the primary tumor was at the mediastinal level. These data show the importance of the role of the surgeon in the multidisciplinary treatment of stage III and IV neuroblastomas; our experience is also favorable to the practice of second-look surgery.

Adrenal Gland Neoplasms↗
PubMed1976

Pancreatic ascites in the first year of life.

A case of pancreatic ascites in a 4-month-old baby girl is reported. To the best of our knowledge only 4 previous cases occurring in the first year of life have been reported in the pediatric literature. In patients with unexplained ascites, amylase determination should be performed on ascitic fluid and serum. The baby was first treated by external drainage and later by internal drainage of the pseudocyst which had formed along the fistula.

Ascites↗
PubMedDate not supplied

[Soft tissue sarcoma in childhood: local treatment in patients under 3 years of age. Analysis of the RMS-88 multicenter study].

The purpose of this report is to describe the treatment and the outcome of patients affected by soft tissue sarcomas and enrolled in the Italian Cooperative Study RMS 88, whose age was less than 36 months. In particular the role of local treatment is discussed, considering that RT was not recommended by the protocol. The 104 patients, 70 affected by RMS and 34 by NR-STS, were grouped according to the Intergroup Rhabdomyosarcoma Staging (IRS) system. The general guidelines of the study for the local treatment included surgery in Gr I, surgery + radiotherapy (40 Gy) in Gr II, surgery (1 or more procedures) + radiotherapy (45-54 Gy) in Gr III, but RT was not recommended for patients < 3 years. RMS: The 5 patients in Gr I did not receive RT: 4/5 are alive NED and 1 is lost at FU. Among the 5 patients in Gr II, 5 did not receive RT and are alive NED; 4 were given RT and 2 of these died of disease. In the 56 patients in Gr III, RT was utilized in 25: 5 died of disease and 6 manifested distant sequelae due to RT. 10/31 patients who were not given RT, did not undergo any other local treatment and died of disease; 4 patients died after surgery only. NRSTS: 7/9 Gr I patients are alive (1 was treated with RT and did not present sequelae); 1/9 died and 1 is lost at FU. 3/6 Gr II patients are alive (1 after RT), 2 died of disease and 1 is lost at FU. 2/19 Gr III patients were given RT and 1 died of disease; 3 of the not irradiated patients died without any local treatment, 4 after surgery only. CONCLUSION: The local treatment was important for patients' outcome: only 28% of the RMS and 36% of the NRSTS patients who were not treated with surgery a/o RT are in CR. Surgery was not particularly aggressive (6 mutilating procedures). RT was utilized in 33/104 patients and sequelae were 7. The difference in overall survival between patients less than 3 and patients more than 3 is not statistically significant.

Child, Preschool↗
PubMedDate not supplied

[Role of mutilating surgery in the treatment of non-chemosensitive pediatric soft tissue sarcomas. Experience of the Italian Cooperative Group Studies RMS-79 and RMS-88].

UNLABELLED: Aim of the study was to evaluate the role of mutilating surgery in the patients with non chemosensitive soft tissue sarcomas (STS) registered in the Italian Studies. Between 1979 and 1995, 114 patients (pts) with "non chemosensitive" localized STS were enrolled in the cooperative studies RMS-79 (33) and RMS-88 (81). Median age: 89 months (range 1-194). HISTOLOGY: fibrosarcoma 29, Malignant Perpheral Nerve Sheath Tumors (MPNST) 40, malignant fibrous histiocytoma 5, hemangiopericytoma 6, leiomyosarcoma 4, others 20, STS nos 10. The cases were classified according to the IRS staging system as Gr. I 46, Gr. II 24, Gr. III 44. Twelve out of 114 pts (7%), 5/33 (14%) in the first study and 7/81 (8%) in the second, underwent mutilating surgery: 8 pts (of whom 3 were < 2 y of age) had a fibrosarcoma and 4 a MPNST. The mutilating procedure was carried out at diagnosis in 6 cases (4 in RMS-79 and 2 in RMS-and 88) and achieved radicality in 5/6 cases. It was performed after ineffective chemotherapy (CT) in 5 pts (1 in RMS-79 and 4 in RMS-88). One pt (RMS-88) underwent mutilation after multiple local recurrences treated with CT, radiotherapy and conservative excisions (RMS-88) and died. The procedures consisted of 5 amputations, 4 demolitive excisions of extremities with functional impairment, 1 nephrectomy, 1 excision of deferents with spermatic vesicae and 1 mastectomy. OUTCOME: At present 6/12 pts, 5 with fibrosarcoma and 1 with MPNST, are alive with no evidence of disease (NED), 4 of the first and 2 of the second study. Of the 5 Gr. I patients, 4 are alive (NED) and 1 died of 2nd tumor; 1 Gr. II pt is alive NED; of the 5 Gr. III pts 1 is alive NED and 4 died (3 of metastatic spread and 1 of 2nd tumor); the pt amputated after repeated local relapses (Gr. II) is dead of pulmonary metastases. CONCLUSIONS: In the RMS-79 study the mutilations were frequent and were performed at diagnosis in several cases; this trend decreased in the 2nd study in which chemotherapy was attempted in most of the patients. Probably the timing of mutilating procedures was not always adequate (too early or too late). The outcome of pts undergone mutilations is similar to that of the whole group of 114 pts, whose overall survival at 10 years is 64% (C.I. 60-77). Only fibrosarcomas and MPNST probably requires a more aggressive surgical behaviour. At present we avoid initial demolitive surgery; it should be planned without delay after the first local relapse or after initial ineffective CT and RT.

Adolescent↗