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

Mesenchymal stromal cells from Shwachman-Diamond syndrome patients fail to recreate a bone marrow niche in\ua0vivo and exhibit impaired angiogenesis

Shwachman-Diamond syndrome (SDS) is a rare multi-organ recessive disease mainly characterised by pancreatic insufficiency, skeletal defects, short stature and bone marrow failure (BMF). As in many other BMF syndromes, SDS patients are predisposed to develop a number of haematopoietic malignancies, particularly myelodysplastic syndrome and acute myeloid leukaemia. However, the mechanism of cancer predisposition in SDS patients is only partially understood. In light of the emerging role of mesenchymal stromal cells (MSCs) in the regulation of bone marrow homeostasis, we assessed the ability of MSCs derived from SDS patients (SDS-MSCs) to recreate a functional bone marrow niche, taking advantage of a murine heterotopic MSC transplant model. We show that the ability of semi-cartilaginous pellets (SCPs) derived from SDS-MSCs to generate complete heterotopic ossicles in\ua0vivo is severely impaired in comparison with HD-MSC-derived SCPs. Specifically, after in\ua0vitro angiogenic stimuli, SDS-MSCs showed a defective ability to form correct networks, capillary tubes and vessels and displayed a marked decrease in VEGFA expression. Altogether, these findings unveil a novel mechanism of SDS-mediated haematopoietic dysfunction based on hampered ability of SDS-MSCs to support angiogenesis. Overall, MSCs could represent a new appealing therapeutic target to treat dysfunctional haematopoiesis in paediatric SDS patients