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ABSTRACT: Gastric neuroendocrine neoplasms differ considerably in histology, clinicopathologic background, stage, and patient outcome, implying a wide spectrum of therapeutic options, hence the need for improved diagnostic and prognostic criteria to select appropriate therapy. Here, we tested the European NeuroEndocrine Tumor Society and the novel World Health Organization 2010 grade and stage classifications together with additional clinicopathologic and histologic parameters in a series of 209 gastric neuroendocrine neoplasms with a median follow-up of 89 months. Fifty-one grade 3 neuroendocrine carcinomas and 15 mixed endocrine-exocrine carcinomas of poor outcome were separated from 143 neuroendocrine tumors, including 132 G1 or G2 enterochromaffin-like (ECL) cell neoplasms and 11 G1 gastrin-cell, somatostatin-cell, or serotonin-cell tumors. Most G1 cases had excellent prognosis, even when metastatic, whereas G2 and G3 neoplasms had worse or very severe prognosis, respectively. The European NeuroEndocrine Tumor Society-World Health Organization 2010 proliferative grading system well correlated with patient survival. Structural histologic parameters were equally predictive and when combined with the European NeuroEndocrine Tumor Society-World Health Organization 2010 grading system in a "global grade" improved tumor prognostic stratification. The European NeuroEndocrine Tumor Society-World Health Organization 2010 staging system proved effective. Introduction of novel T (T(1a) and T(1b) or deep submucosal) and N categories (N(1), <3 nodes metastases; N(2), ≥3) allowed a simplified, equally informative 3-stage TNM system. Such improved diagnostic and prognostic criteria for gastric neuroendocrine neoplasms are proposed and discussed.
Human pathology 04/2011; 42(10):1373-84. · 3.03 Impact Factor
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ABSTRACT: Nonneoplastic and neoplastic proliferative lesions of endocrine cells of the gastrointestinal tract are detailed. A multistep continuum from hyperplasia, dysplasia to neoplasia is identified for histamine-producing enterochromaffin-like (ECL) cells of the gastric corpus. Most gastric neuroendocrine tumors (NETs) are silent and composed by ECL cells, the second most frequent neuroendocrine neoplasms being the high-grade neuroendocrine carcinoma (NEC). In the duodenum, preneoplastic lesions are similarly described for gastrin (G) and somatostatin (D) cells. G-cell NETs are the most frequent neuroendocrine tumors of the duodenum, either functioning or nonfunctioning, followed by D-cell NETs and gangliocytic paraganglioma (GCP). No systematic definition of nonneoplastic lesions exists for endocrine cells of the ileum, appendix, and colon-rectum. The most frequent ileal NETs are serotonin-producing enterochromaffin (EC)-cell NETs (classic carcinoid), associating with functional syndrome only in presence of liver metastases. Neoplasms are usually larger in the colon as compared with the small lesions observed in the rectum. High-grade NECs are observed in the colon and rectum-sigmoid, often associate with nonendocrine neoplastic components, and fare an aggressive course with poor outcome and short survival.
Endocrinology and metabolism clinics of North America 12/2010; 39(4):713-27. · 3.56 Impact Factor
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ABSTRACT: By qPCR we found that EDG3 and SHC3 were amplified in 60% of ependymomas but none in choroid plexus papillomas. In ependymomas EDG3 and SHC3 amplification increased Shc3 protein levels while EDG3 was less affected. Both proteins were co-immunoprecipitated from ependymoma and Shc3 was tyrosine phosphorylated thus presumably active. We showed by digestion with N-glycosidase-F that EDG3 was glycosylated indicating that EDG3 protein was not retained in the endoplasmic reticulum. The co-immunoprecipitation of Shc3 and EDG3 proteins from ependymomas with amplification of SHC3 and EDG3 genes suggests that the two proteins co-operate and are important for ependymomas in vivo.
Cancer letters 09/2009; 290(1):36-42. · 4.86 Impact Factor
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Marco Lucioni,
Giampiero Beluffi,
Laura Bandiera,
Marco Zecca, Frediano Inzani,
Giacomo Fiandrino,
Alessandra Viglio,
Mauro Stronati,
Vittorio Necchi,
Roberta Riboni,
Franco Locatelli,
Marco Paulli
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ABSTRACT: In children < 2 years of age, cutaneous involvement is the most frequent presentation of Langerhans cell histiocytosis (LCH). Cutaneous LCH can be localized or associated with dissemination and organ dysfunction. The clinical course is variable, ranging from spontaneous regression to a fatal outcome. We describe a female newborn presenting with congenital cutaneous lesions who rapidly developed pulmonary infiltrates and multiple osteolytic lesions. Skin biopsy showed a dermal infiltrate of medium to large cells morphologically and phenotypically consistent with LCH. The clinical course was rapidly fatal in spite of chemotherapy. No strict correlation between morphology and prognosis has been documented in LCH, but, in our case, distinct morphological and immunohistochemical features (CD56 expression and no E-Cadherin expression) may have contributed to an aggressive clinical course.
Pediatric Blood & Cancer 08/2009; 53(6):1107-10. · 1.89 Impact Factor
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ABSTRACT: Paragangliomas may have composite forms in which they combine features of a typical paraganglioma with those of a neural component consisting of either neuroblastoma, ganglioneuroblastoma, or ganglioneuroma. These variants are rare and generally located in the adrenal. Herein, we describe a retroperitoneal, extra-adrenal composite paraganglioma-ganglioneuroma of a 57-year-old woman. Radiological evaluation revealed a nodular mass with apparent pancreatic location, with findings suggestive of an endocrine tumor, yielding the diagnosis of a pancreatic endocrine tumor. At histology the neoplasm, strictly adhering to the external surface of the pancreatic gland but well demarcated, displayed a main central region with typical paraganglioma features and cells arranged in cords and in a nesting "zellballen" pattern, positive for neuroendocrine markers, and a distinct peripheral area consisting of dense bundles of wavy spindled Schwann cells, with scattered ganglionic cells.
Endocrine Pathology 02/2009; 20(3):191-5. · 1.36 Impact Factor
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International journal of cardiology 02/2007; 114(1):114-7. · 7.08 Impact Factor
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The Journal of thoracic and cardiovascular surgery 11/2006; 132(4):972-4. · 3.41 Impact Factor
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Acta Neuropathologica 04/2006; 111(3):286-8. · 9.32 Impact Factor
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The Journal of heart and lung transplantation: the official publication of the International Society for Heart Transplantation 02/2006; 25(1):148-50. · 3.54 Impact Factor
