Pediatric extraspinal sacrococcygeal ependymoma (ESE): an Italian AIEOP experience of six cases and literature review

Background: Primary pediatric extraspinal sacrococcygeal ependymoma (ESE) is a very rare disease, poorly described in literature, whose diagnostic, therapeutic, and follow-up approach is still controversial. Methods: We describe six cases of pediatric ESE treated at Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) centers in Italy since 1983, with a review of the literature. Results: All six patients had primary sacrococcygeal disease (two presacral and four subcutaneous) with median age of 10 years. Three patients were males, and two of them are metastatic at diagnosis; 3/6 had myxopapillary ependymoma grade I and 3/6 had classic ependymoma grade II. Five patients underwent surgical resection with complete removal only in one case with coccygectomy. Adjuvant chemoradiotherapy was administered to one metastatic patient obtaining a complete remission. Two patients relapsed at 3 and 8 years from diagnosis: they were treated with salvage chemotherapy (high-dose sequential chemotherapy with myeloablative regimen in one case), surgery, and radiotherapy achieving complete remission (CR). All six patients are in complete continuous remission (CCR) at a median follow-up of 12.8 years. Conclusions: Pediatric patients with this peculiar disease need to be referred to specialized pediatric cancer centers that can provide multidisciplinary treatment after a centralized pathology review. Our experience highlights the role of chemotherapy and radiotherapy in adjuvant and relapse setting. The final prognosis is relatively optimistic, but with a careful follow-up due to the high risk of recurrence

Outcome of relapsed/refractory acute promyelocytic leukaemia in children, adolescents and young adult patients — a 25‐year Italian experience

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Multilateral comparison of total factor productivity and convergence in Italian agriculture (1951-2002)

This article uses the index number approach to estimate the regional differences of agricultural output, input and total factor productivity (TFP) in Italy. Data cover the period 1951 to 2002 and are taken from Agrefit, which is a new database of Italian agriculture at regional level. Comparisons across space and time are constructed in two steps: first, bilateral Fisher indexes, which are not transitive, are spatially chained to obtain transitivity, using the EKS method, then, multilateral indexes are linked chronologically over time by means of the so-called TFGG method, which satisfies temporal fixity. The second focus of the paper is to test for convergence in agricultural productivity. We consider the problems of heterogeneity and stability of cross-section growth regressions using a hierarchical Bayesian method based on panel data.Index number approach, Agricultural TFP, Multilateral comparisons, Panel data, Regional convergence.

Divari di produttivita' del lavoro nell'economia italiana: 1951-97

Research Project cofinanced by MIUR 'Long-term structural changes in the regions of the European Union', 2001Consiglio Nazionale delle Ricerche - Biblioteca Centrale - P.le Aldo Moro, 7, Rome / CNR - Consiglio Nazionale delle RichercheSIGLEITItal

Familial haemophagocytic lymphohistiocytosis-related plasma cell neoplasm: a case report

none8nononePizzi M; Sabattini E; Goteri G; Pierani P; Bacci F; Sagramoso C; Righi S; Pileri SA.Pizzi M; Sabattini E; Goteri G; Pierani P; Bacci F; Sagramoso C; Righi S; Pileri SA

Cutaneous Melanoma in Children and Adolescents: The Italian Rare Tumors in Pediatric Age Project Experience

none11OBJECTIVE: To describe a series of cutaneous melanoma in children collected by the Italian Rare Tumors in Pediatric Age project. STUDY DESIGN: From 2000 to 2012, 54 patients younger than 18 years of age were prospectively registered and treated at 12 Italian pediatric centers on the basis of the same diagnostic/therapeutic recommendations and with the same forms to record clinical data. RESULTS: Considering the estimated annual incidence in Italy, the registered cases accounted for 30% of those expected in children and 10% of adolescents. Clinically, 47% of the tumors were amelanotic and 81% were raised, 39% of cases had tumor thickness >2 mm, and 36% had lymph node involvement. For the whole series, 5-year event-free survival and overall survival rates were 75.2% and 84.6%, respectively. Patient survival correlated with tumor stage and ulceration. No relapses were recorded for T1-2 (thickness <2 mm), N0, and stage 0-I-II cases. CONCLUSION: We suggest that the variables influencing survival in children with melanoma are the same as for adults, the clinical approach used in adults is feasible in children, and pediatric cases are more likely to have advanced disease at diagnosis but similar survival. New effective drugs are needed for advanced disease, and biological studies and international cooperative schemes are warranted.noneAndrea Ferrari;Gianni Bisogno;Giovanni Cecchetto;Mario Santinami;Andrea Maurichi;Aldo Bono;Marco Vajna De Pava;Paolo Pierani;Patrizia Bertolini;Carlo Riccardo Rossi;Gian Luca De SalvoAndrea, Ferrari; Bisogno, Gianni; Cecchetto, Giovanni; Mario, Santinami; Andrea, Maurichi; Aldo, Bono; Marco Vajna De, Pava; Paolo, Pierani; Patrizia, Bertolini; Rossi, CARLO RICCARDO; Gian Luca De, Salv