RAGE signaling deficiency in rhabdomyosarcoma cells causes upregulation of PAX7 and uncontrolled proliferation.

Embryonal rhabdomyosarcomas (ERMSs) show elevated levels of PAX7, a transcription factor that marks quiescent adult muscle stem (satellite) cells and is important for proliferation and survival of activated satellite cells and whose timely repression is required for myogenic differentiation. However, the mechanism of PAX7 accumulation in ERMSs and whether high PAX7 causes uncontrolled proliferation in ERMS remains to be elucidated. The receptor for advanced glycation end-products (RAGE, encoded by AGER) transduces a myogenic and anti-proliferative signal in myoblasts, and stable transfection of the ERMS cell line TE671, which does not express RAGE, with AGER results in reduced proliferation and formation of tumor masses in vivo, and enhanced apoptosis and myogenic differentiation.

Helicobacter pylori and gastroesophageal reflux disease: a cross sectional study.

Unlabelled: BACKGROUND\

Aims: Helicobacter Pylori (H. Pylori) is a key pathogenetic factor in gastritis, peptic ulcer disease, gastric carcinoma and lymphoma but its relationship with gastroesophageal reflux disease (GERD) is controversial. The aim of the study is to estimate the possible association between the presence of H.

Undifferentiated sarcoma: does it exist? A clinicopathologic study of 7 pediatric cases and review of literature.

Undifferentiated sarcomas are primitive mesenchymal tumors that cannot be classified among standardized histopathologic entities. Whether they represent a homogeneous group with common histogenesis and clinical behavior or comprise a variety of tumors able to differentiate along specific maturative lineages is still debated. To identify prognostic and histogenetic markers, we analyzed 7 undifferentiated sarcomas (4 in the trunk and 3 in the extremities).

Morphologic Overlap between Infantile Myofibromatosis and Infantile Fibrosarcoma: A Pitfall in Diagnosis.

Infantile myofibromatosis (IM) is a distinctive mesenchymal disorder with different clinical forms, including solitary, multicentric, and generalized with visceral involvement. A wide morphologic spectrum is encountered, with the extremes resembling congenital infantile fibrosarcoma (CIFS) and infantile hemangiopericytoma. We report a series of lesions with mixed features of CIFS and IM and compare them in order to further define their clinicopathologic features and the significance of the so-called composite fibromatosis.

Polypoid angiomyofibroblastoma-like tumor of the oral cavity: a hitherto unreported soft tissue tumor mimicking embryonal rhabdomyosarcoma.

We report on a previously unrecognized fibro-myofibroblastic tumor in the oral cavity of a 15-year-old girl. Morphologically, the tumor mimicked a rhabdomyosarcoma, botryoid variant. It was composed of mitotically active small- to medium-sized, vimentin+/desmin+, round- to oval- to epithelioid-shaped cells embedded in an alternating fibrous to myxoid/edematous stroma.

Malignant glomus tumour: a case report and review of the literature.

Purpose: Glomus tumours are characteristically benign solitary tumours. At our knowledge, about 23 reports are present in literature regarding the malignant counterpart, but only a minority developed metastases. We describe a locally aggressive glomus tumour with lymphnode metastasis.

Expression of maspin in papillary Ta/T1 bladder neoplasms.

Background: The aim of the present study was to evaluate maspin expression in bladder urothelial papillary neoplasms and test the results for correlation with clinicopathological parameters.

Patients And Methods: A total of 111 urothelial neoplasms from 66 patients were evaluated: pathological examination of primary tumours disclosed 48 pTa and 18pT1. Fourteen additional biopsies, negative for neoplasm, were collected as control biopsies.

Composite hemangioendothelioma of the oral cavity: case report and review of the literature.

Background: Composite hemangioendothelioma is a rare histopathologic feature, which is part of the hemangioendothelioma family. This is a heterogeneous group of vascular neoplasia with a high tendency to local relapse but a rare predisposition to metastatic spread. Composite hemangioendothelioma mainly affects adults and is usually localized in the distal extremity of the limbs.

Bisphosphonate-associated jawbone osteonecrosis: a correlation between imaging techniques and histopathology.

Objectives: Recently, jawbone osteonecrosis has been reported as a potential adverse effect of bisphosphonates administration. This paper considers and highlights histopathologic and radiologic features of this condition.

Study Design: Eleven patients, owing to unresponsiveness to conservative treatment and uncontrollable pain, underwent surgical resection of diseased jawbone after extensive hyperbaric oxygen therapy.

Maspin expression in adenocarcinoma of the ampulla of Vater: relation with clinicopathological parameters and apoptosis.

Aim: Maspin is a unique serine proteinase inhibitor which has tumor suppressor activity. The aim of the present study was to investigate the role of maspin in ampullary adenocarcinomas, its correlation with apoptosis and its value as a prognostic marker.

Patients And Methods: Twenty-three cases of ampulla of Vater adenocarcinoma were collected from archival material.

Spindle cell tumor with EWS-WT1 transcript and a favorable clinical course: a variant of DSCT, a variant of leiomyosarcoma, or a new entity? Report of 2 pediatric cases.

We report 2 intra-abdominal tumors originally diagnosed as leiomyosarcomas, occurring in adolescents, one as a second malignancy after a Hodgkin lymphoma. Both tumors exhibited unusual morphologic features characterized by spindle cells arranged in sheets or in fascicles, devoid of the typical desmoplastic stroma. Cytokeratins and mesenchymal markers, including smooth muscle actin, desmin, and muscle specific actin, were coexpressed in the tumor cells, whereas EMA was negative.

An unusual case of malignant pilocytic astrocytoma occurring in the eye.

Pilocytic astrocytoma is a central nervous system neoplasia that arises during pediatric age. Only few cases have been documented in patients older than 50 years old. It is a low-grade lesion that can rarely undergo malignant changes presenting the histologic features of a high-grade glioma.

Three cases of pancreatic serous cystadenoma and endocrine tumour.

Aims: To report three cases of serous cystadenoma and endocrine tumour in the same pancreas, to review the literature and to evaluate the clinicopathological features of the tumours.

Cases: Three women (71, 57 and 31 years old) were admitted to hospital, two for diseases unrelated to the pancreas and the third for increasing obstructive jaundice in von Hippel-Lindau disease. Preoperative examination showed two distinct lesions in the first patient and only cystic lesions in the other two.

Primitive myxoid mesenchymal tumor of infancy: a clinicopathologic report of 6 cases.

Soft tissue sarcomas in the first year of life are rare, and the most common sarcomas in infancy are embryonal rhabdomyosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, congenital infantile fibrosarcoma, and primitive sarcomas such as undifferentiated sarcoma. In this study, we report 6 cases of a primitive myxoid mesenchymal tumor of infancy (PMMTI), which previously may have been included under the diagnostic categories of congenital-infantile fibrosarcoma or infantile fibromatosis. PMMTI occurred in 6 infants, 3 of whom had a congenital presentation of a soft tissue mass.

Expression of survivin, p53, and caspase 3 in Barrett's esophagus carcinogenesis.

The regulation of apoptosis, as a distinctive form of programmed cell death, in multistep Barrett's esophagus (BE) carcinogenesis is poorly understood. The aim of this study was to investigate, in the intestinal metaplasia-dysplasia-carcinoma sequence, the role of survivin, an inhibitor of apoptosis; the p53 protein, a tumor suppressor gene involved in cell cycle control; and caspase 3, a protease-inducing apoptosis and inhibited by survivin. Immunohistochemical expression was tested in 40 cases of BE, including 11 low-grade and 19 high-grade dysplasias (HGD), and samples were obtained from 40 surgical specimens of esophagectomy performed for HGD or Barrett's adenocarcinoma.

Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody.

FLI-1 nuclear transcription factor has been proposed as a useful tool in the differential diagnosis of small round cell sarcomas. Recently, FLI-1 has been reported as the first nuclear marker of endothelial differentiation. However, its clinical use has been hampered by major interpretation problems, due to the presence of background staining as well as staining variation between different lots of the same antiserum.

Pediatric-type sarcomas in adult patients.

Sarcomas included in the broad group of small round-cell tumors (SRCT) and some non-SRCT lesions that typically are seen in pediatric-age patients can rarely occur in adults. However, there are differences in the anatomic sites that are involved and the prognosis in these two patient groups. The diagnosis of pediatric-type sarcomas in adults is often challenging because of the unusual contextual clinical setting and morphologic features.

Aggressive angiomyxoma: a second case of metastasis with patient's death.

Aggressive angiomyxoma is a rare tumor that predominates in the female genital tract. Multiple relapses may occur in adjacent organs and tissues, but metastases have not been reported. We present a case of aggressive angiomyxoma in a young woman with multiple local recurrences that metastasized to the lungs, killing the patient.

Leiomyosarcoma at the site of an ileal neobladder: a heretofore unreported occurrence.

We report a case of leiomyosarcoma arising from the intestinal side of an orthotopic ileal neobladder in a male patient 2 years after surgery for papillary urothelial carcinoma of the bladder.

Rhabdomyosarcoma in infants younger than one year old: a report from the Italian Cooperative Group.

Background: The management of rhabdomyosarcoma (RMS) in patients age < 1 year is particularly problematic and requires a tailored therapeutic approach. We report on the Italian Cooperative Group's 20-year study of 50 children with RMS who were age < 1 year at diagnosis.

Methods: Patients were treated using multimodality therapeutic approaches that were based on three consecutive protocols.

MYCN expression in human rhabdomyosarcoma cell lines and tumour samples.

The MYCN oncogene encodes a phosphoprotein that acts as a transcription factor and is involved in the regulation of cell proliferation and differentiation in normal as well as in cancer cells.MYCN amplification and expression have been reported in various tumours, including neuroblastoma and lung cancer, but little is known about its expression in human rhabdomyosarcoma. MYCN expression and amplification were studied in five alveolar and five embryonal rhabdomyosarcoma cell lines and in 19 tumour biopsies.

Undifferentiated sarcoma of the liver in childhood: a curable disease.

Background: Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare childhood hepatic tumor, and it is generally considered an aggressive neoplasm with an unfavorable prognosis.

Methods: The Soft Tissue Sarcoma Italian and German Cooperative Groups enrolled 17 children with UESL in studies conducted between 1979 and 1995. They were treated using the same multimodal approach as for patients with sarcomas including conservative surgery at diagnosis, multiagent chemotherapy, and second-look operation in cases of residual disease.