Renal cell carcinoma in children: A clinicopathologic study

Purpose: To identify the prognostic factors, treatment, and outcome of children affected by renal cell carcinoma (RCC). Patients and Methods: The series included 41 patients (18 males and 23 females) with a median age of 124 months observed at the 11 Italian Association for Pediatric Hematology and Oncology centers from January 1973 to January 2001. Clinical data, surgical notes, pathologic findings, and summaries of therapy were taken from the charts. Results: Seven (17%) of the 41 patients had a papillary histology, and 34 (82.4%) had nonpapillary histology. Eighteen patients (43.9%) had stage I, one patient (2.4%) had stage II, two patients (4.8%) had stage IIIA, 10 patients (24.3%) had stage IIIB, and nine patients (21.9%) had stage IV disease. One patient had a bilateral involvement at diagnosis. Seven patients experienced disease recurrence. Lung and liver were the most common distant lesions and usually were fatal. In this study, the major factor influencing the prognosis was the stage. Event-free survival at 20 years was 53.5% for all patients. Overall survival at 20 years was 54.9% for all patients. Conclusion: RCC is a rare disease in children and adolescents. This neoplasm has a different clinical presentation in children compared with adults but the same outcome. In our experience, patients with localized disease could be cured by nephrectomy alone. Prospective studies in a larger number of patients are needed to confirm radiation therapy and biologic response modifiers as effective adjunct therapy in RCC stage III. The alternative therapy seems warranted in patients with advanced disease. © 2003 by American Society of Clinical Oncology

Mature and Immature Teratoma: A Report From the Second Italian Pediatric Study

Background. Teratomas demonstrate a benign clinical behavior, however they may recur with malignant components or as teratoma, and in a small group of patients prognosis could be fatal. After the ®rst Italian study, we collected cases of teratoma, alongside the protocol for malignant germ cell tumors. Procedure. Patients with teratoma were collected from 2004 to 2014. Teratomas were classi®ed according to the WHO classi®cations, as mature and immature. Patients with pathological aFP and/or bHCG, and those with a malignant germ cell component were not included. Results. The study enrolled 219 patients (150 mature, 69 immature teratomas) with a median age at diagnosis of 42 months. The primary sites involved were: 118 gonadal and 101 extragonadal teratomas. Two females with ovarian teratoma had a positive family history. Complete and incomplete surgeries were performed in 85% and 9% of cases. Seventeen events occurred: six females had a second metachronous tumor (5 contralateral ovarian teratoma, 1 adrenal neuroblastoma) and 11 teratomas relapsed/progressed (3 mature, 8 immature teratomas). Two patients died, one of progressive immature teratoma and one of surgical complications. At a median follow up of 68 months, the event-free, relapse-free, and overall survival rates were 90.6%, 94.3%, 98.6%, respectively. Conclusions. Teratomas show a good prognosis, especially the mature ones: surgery and follow-up remain the standard approach. Incomplete surgery in immature teratoma is the group at greatest risk of relapse. Bilateral ovarian tumors are a possibility, and the rare family predisposition to ovarian mature teratoma warrants further analyses

Mature and immature teratoma: A report from the second Italian pediatric study

none16noBACKGROUND: Teratomas demonstrate a benign clinical behavior, however they may recur with malignant components or as teratoma, and in a small group of patients prognosis could be fatal. After the first Italian study, we collected cases of teratoma, alongside the protocol for malignant germ cell tumors. PROCEDURE: Patients with teratoma were collected from 2004 to 2014. Teratomas were classified according to the WHO classifications, as mature and immature. Patients with pathological aFP and/or bHCG, and those with a malignant germ cell component were not included. RESULTS: The study enrolled 219 patients (150 mature, 69 immature teratomas) with a median age at diagnosis of 42 months. The primary sites involved were: 118 gonadal and 101 extragonadal teratomas. Two females with ovarian teratoma had a positive family history. Complete and incomplete surgeries were performed in 85% and 9% of cases. Seventeen events occurred: six females had a second metachronous tumor (5 contralateral ovarian teratoma, 1 adrenal neuroblastoma) and 11 teratomas relapsed/progressed (3 mature, 8 immature teratomas). Two patients died, one of progressive immature teratoma and one of surgical complications. At a median follow up of 68 months, the event-free, relapse-free, and overall survival rates were 90.6%, 94.3%, 98.6%, respectively. CONCLUSIONS: Teratomas show a good prognosis, especially the mature ones: surgery and follow-up remain the standard approach. Incomplete surgery in immature teratoma is the group at greatest risk of relapse. Bilateral ovarian tumors are a possibility, and the rare family predisposition to ovarian mature teratoma warrants further analyses. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.noneTerenziani M;D'Angelo P;Inserra A;Boldrini R;Bisogno G;Babbo GL;Conte M;Dall' Igna P;De Pasquale MD;Indolfi P;Piva L;Riccipetitoni G;Siracusa F;Spreafico F;Tamaro P;Cecchetto GTerenziani, M; D'Angelo, P; Inserra, A; Bisogno, Gianni; Bisogno, G; Babbo, Gl; Conte, M; Dall' Igna, P; De Pasquale, Md; Indolfi, P; Piva, L; Riccipetitoni, G; Siracusa, F; Spreafico, F; Tamaro, P; Cecchetto, Giovann

Risks of second primary cancer among patients with major histological types of lung cancers in both men and women

10.1038/sj.bjc.6605616British Journal of Cancer10271190-1195BJCA

Earthquake rupture forecasts for the mps19 seismic hazard model of Italy

In recent years, new approaches for developing earthquake rupture forecasts (ERFs) have been proposed to be used as an input for probabilistic seismic hazard assessment (PSHA). Zone-based approaches with seismicity rates derived from earthquake catalogs are commonly used in many countries as the standard for national seismic hazard models. In Italy, a single zone-based ERF is currently the basis for the official seismic hazard model. In this contribution, we present eleven new ERFs, including five zone-based, two smoothed seismicity-based, two fault-based, and two geodetic-based, used for a new PSH model in Italy. The ERFs were tested against observed seismicity and were subject to an elicitation procedure by a panel of PSHA experts to verify the scientific robustness and consistency of the forecasts with respect to the observations. Tests and elicitation were finalized to weight the ERFs. The results show a good response to the new inputs to observed seismicity in the last few centuries. The entire approach was a first attempt to build a community-based set of ERFs for an Italian PSHA model. The project involved a large number of seismic hazard practitioners, with their knowledge and experience, and the development of different models to capture and explore a large range of epistemic uncertainties in building ERFs, and represents an important step forward for the new national seismic hazard model

Methotrexate therapy and liver fibrosis: are human prolyl hydroxylase and type IV collagen reliable and sensitive markers?

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