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Giorgio Perilongo

Publications and source records attributed to Giorgio Perilongo.

26 records · Page 2Linked to original sources
PubMed2002

Spinal low-grade neoplasms with extensive leptomeningeal dissemination in children.

OBJECTIVE: The aim of this study is to re-appraise paediatric leptomeningeal disseminated spinal low-grade neoplasms putting forward the hypothesis that these tumours may represent a possible distinctive clinico-pathological entity. CASE REPORT: Three children affected by these rare neoplasms are reported, 2 boys and 1 girl aged 40 months, 7 and 12 years respectively. RESULTS: Despite treatment, 2 died at 9 months and 9 years, while 1 has survived with stable disease at 24 months after diagnosis. Histologically, these tumours shared the generic histological appearance of low-grade neuroectodermal neoplasms, without any features that allowed them to be included in a specific classification niche. The magnetic resonance imaging of the leptomeningeal dissemination took the form of a thick, diffuse subarachnoidal enhancement involving the brain as well the spine in 2 patients; in 1 patient there was an extensive enhancement along the surface of the brain and of the spinal cord and multiple small cysts, creating the impression of a diffuse microcystic meningoencephalopathy. DISCUSSION: The patients described here and the similar ones found in the literature cannot be easily placed into the present WHO system of classification for central nervous system tumours.

Child↗
PubMed2002

Primary intracranial fibrosarcoma.

INTRODUCTION: Primary fibrosarcomas of the brain are very rare tumors, so that information regarding the treatment is scarce. We report the contributions that different therapeutic options made to the treatment of a child with one of these aggressive tumors. CASE REPORT: A 13-year-old boy underwent a complete resection of a left temporo-parietal mass that had been diagnosed as a fibrosarcoma by two independent pathologists. Adjuvant chemotherapy with vincristine, actinomycin-D, ifosfamide and Adriamycin was started, but after 3 months tumor relapse was evident. The boy subsequently received radiation therapy during which there was evidence of progressive tumor shrinkage. A second surgery was performed 6 months after radiotherapy and a small enhancing lesion, revealed to be gliosis, was resected. The child remains alive and well 44 months after diagnosis. CONCLUSION: Our experience supports the importance of total resection followed by radiation therapy, and radiotherapy should be started as soon as possible after surgical resection, rather than administering chemotherapy first.

Adolescent↗
PubMed2002

Surgical view of the treatment of patients with hepatoblastoma: results from the first prospective trial of the International Society of Pediatric Oncology Liver Tumor Study Group.

BACKGROUND: Surgical resection is the cornerstone of treatment for patients with hepatoblastoma (HB). The Society of Pediatric Oncology Liver Tumor Study Group launched its first prospective trial (SIOPEL-1) with the intention to treat all patients with preoperative chemotherapy and delayed surgical resection. The objective of this article was to assess the assumed surgical advantages of primary chemotherapy. METHODS: Between 1990 and 1994, 154 patients age < 16 years with HB were registered on SIOPEL-1. The pretreatment extent of disease was assessed, and, after undergoing biopsy, patients were treated with cisplatin 80 mg/m(2) intravenously over 24 hours and doxorubicin 60 mg/m(2) intravenously over 48 hours by continuous infusion (PLADO). Generally, tumors were resected after four of a total of six courses of PLADO. RESULTS: One hundred twenty eight patients underwent surgical resection (13 patients underwent primary surgery, and 115 patients underwent delayed surgery after PLADO). A pretreatment surgical biopsy was performed in 96 of 128 patients (75%). Biopsy complications occurred in 7 of 96 patients (7%). Twenty-two patients showed pulmonary metastases at the time of diagnosis, and 7 patients underwent thoracotomy. Operative morbidity and mortality were 18% and 5%, respectively. Complete macroscopic surgical resection was achieved in 106 patients (92%), including 6 patients who underwent orthotopic liver transplantation. The actuarial 5-year event free survival (EFS) rate for all 154 patients in the study was 66%, and the overall survival (OS) rate was 75%. For the 115 patients who were included in the surgical analysis that followed the exact protocol, the EFS and OS rates were 75% and 85%, respectively. CONCLUSIONS: Biopsy is a safe procedure and should be performed routinely. Preoperative chemotherapy seems to make tumor resection easier. Reresection of a positive resection margin does not necessarily have to be performed, because postoperative chemotherapy showed good results. Resection of lung metastases can be curative if there is local control of the primary tumor; however, results showed that the patient's prognosis was worse. Surgical morbidity or mortality rates were not necessarily higher in large multicenter studies. More importantly, countries of lesser economic status also can contribute effectively to these trials.

Adolescent↗
PubMed2002

Undifferentiated sarcoma of the liver in childhood: a curable disease.

BACKGROUND: Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare childhood hepatic tumor, and it is generally considered an aggressive neoplasm with an unfavorable prognosis. METHODS: The Soft Tissue Sarcoma Italian and German Cooperative Groups enrolled 17 children with UESL in studies conducted between 1979 and 1995. They were treated using the same multimodal approach as for patients with sarcomas including conservative surgery at diagnosis, multiagent chemotherapy, and second-look operation in cases of residual disease. Radiotherapy was occasionally used (2 of 17 patients). RESULTS: Twelve patients are alive with follow-up ranging from 2.4 to 20 years. Eight underwent complete tumor resection either at diagnosis or after preoperative chemotherapy, and all are currently alive. After initial chemotherapy tumor reduction was evident in six of nine evaluable cases. Overall three patients died of disease and one of a surgical complication. One child died in second complete remission for a non-disease-related cause. CONCLUSIONS: The current prognosis of UESL no longer should be regarded as poor. Modern multimodal treatment and supportive therapy have improved survival.

Adolescent↗
PubMed2002

Primary peripheral PNET/Ewing's sarcoma of the dura: a clinicopathologic entity distinct from central PNET.

We describe two cases of peripheral primitive neuroectodermal tumor-Ewing's sarcoma (PNET-ES) arising intracranially in the leptomeninges. Both tumors exhibited a primitive undifferentiated round-cell morphology. Immunohistochemical stains revealed strong membrane expression of CD99 in both cases. A t(11;22)(q24;q12) could be demonstrated with reverse transcriptase-polymerase chain reaction in one case, whereas fluorescence in situ hybridization analysis performed in the second case showed a rearrangement of the EWS gene. The occurrence of PNET-ES at this site is very unusual. Immunophenotypical as well as genetic analysis play a key role in the diagnosis and the distinction from central PNET.

12E7 Antigen↗