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Biomedical subjects

Alberto Pagani

Publications and source records attributed to Alberto Pagani.

4 recordsLinked to original sources

alpha-fetoprotein expression in a dedifferentiated liposarcoma.

Extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. We describe a unique case of relapsing retroperitoneal dedifferentiated liposarcoma with biochemical, immunohistochemical, and molecular evidence of alpha-fetoprotein (AFP) ectopic production. The lesion was associated to elevated AFP plasma levels that subsided after tumor removal. Immunohistochemical studies showed AFP production by a minority of tumor cells and reverse transcriptase polymerase chain reaction confirmed AFP mRNA expression. Finding of MDM2 and CDK4 iperexpression by immunohistochemistry confirmed the diagnosis of dedifferentiated liposarcoma.

Aged↗

Potential applications of molecular biology in neuroendocrine tumors.

The impact of molecular biology procedures on neuroendocrine (NE) tumor biology is gradually evolving from purely academic and research studies to clinical applications. This review deals with applications of molecular techniques in neuroendocrine tumors, with special reference to their potential for diagnostic, prognostic, or therapeutic impact. Since the cloning of the genes involved in inherited endocrine tumor syndromes, molecular analysis of the responsible genetic alterations has become a routine diagnostic tool to select affected patients and their relatives, and also an interesting approach to investigate the pathogenesis of neuroendocrine tumors. Assessment of the clonal composition of endocrine tumors could be useful to differentiate hyperplastic versus either adenomatous or carcinomatous conditions, as well as to better understand the clonal relationship between different neoplastic populations in mixed tumors. In addition, molecular approaches allow high sensitivity both in defining the neuroendocrine phenotype in poorly differentiated tumors and in searching for micrometastasis during the follow up of patients with endocrine tumors. Finally, the detection of peptide hormone receptors (e.g., oxytocin and somatostatin receptors) and the development of potent synthetic analogs of such peptides, are opening promising applications in the diagnosis and therapy of endocrine tumors.

Biomarkers, Tumor↗

Detection procedures for neuroblastoma cells metastatic to blood and bone marrow: blinded comparison of chromogranin A heminested reverse transcription polymerase chain reaction to tyrosine hydroxylase nested reverse transcription polymerase chain reaction and to anti-GD2 immunocytology.

Specific and sensitive tumor cell detection is becoming increasingly important for diagnosing and staging as well as for the therapeutic management of neuroblastoma patients. We propose a chromogranin A heminested reverse transcription polymerase chain reaction (CgA hn RT-PCR) procedure for the detection of neuroblastoma minimal residual disease in peripheral blood and bone marrow samples. The results were checked in comparison with the presently available procedures (i.e., with the tyrosine hydroxylase nested RT-PCR [TH n RT-PCR] and with the immunocytochemical approach using anti-GD2 antibodies). Controls from healthy patients or from people with unrelated disease (12 samples of bone marrow and 23 samples of peripheral blood) and serial dilution experiments using neuroblastoma cell lines (SKNLP, SKNFI, STA6, STA8) showed CgA hn RT-PCR full specificity and sensitivity ranging from 10(3) to 10(6) (depending on the cell line). The results compared favorably with those obtained using TH n RT-PCR. Preliminary data obtained analyzing bone marrow and peripheral blood specimens from stage IV neuroblastomas showed substantially overlapping results between CgA and TH n RT-PCR procedures. Our data support the potential usefulness of CgA heminested RT-PCR as a specific and sensitive procedure for minimal disease detection in neuroblastoma. A prospective evaluation of this tool in clinical studies might be warranted.

Adolescent↗

Primitive Neuroectodermal Tumor of the Meninges: An Histological, Immunohistochemical, Ultrastructural, and Cytogenetic Study.

We report a case of primitive neuroectodermal tumor (PNET) arising from the meninges in a 30-yr-old female patient whose clinical and radiological features were consistent with meningioma. The neoplasm was composed of primitive small, round cells, growing in sheets and nests in continuity with meningeal layers. Ultrastructurally, the neoplastic cells were characterized by large and regular nuclei, primitive cytoplasm with pools of glycogen, and lack of dense core granules. The neuroectodermal nature of the tumor was confirmed by positive immunostaining for vimentin, neurofilaments, neuron specific enolase (NSE), and 013 (an antibody raised against MIC2 antigen). Further support to the diagnosis was obtained by reverse transcriptase-polymerase chain reaction (RT-PCR) detection of Chromogranin A and Secretogranin II genes. t(1 1;22) translocation was also observed by RT-PCR, a finding that was not recorded in previously described intracranial PNET. The tumor followed a malignant course, recurring and spreading to chest wall and sacroiliac region over a 10-yr period. The meningeal location enlarges the topographic spectrum of intracranial PNET, and this tumor has to be considered in the differential diagnosis with meningioma.

Journal Article↗