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Zangari A.,Azienda Ospedaliera San Camillo Forlanini | Zaini J.,University of Tuscia | Caterina G.,University of Rome La Sapienza
Current Genomics

Bladder masses are represented by either benign or malignant entities. Malignant bladder tumors are frequent causes of disease and death in western countries. However, in children they are less common. Additionally, different features are found in childhood, in which non epithelial tumors are more common than epithelial ones. Rhabdomyosarcoma is the most common pediatric bladder tumor, but many other types of lesions may be found, such as malignant rhabdoid tumor (MRT), inflammatory myofibroblastic tumor and neuroblastoma. Other rarer tumors described in literature include urothelial carcinoma and other epithelial neoplasms. Rhabdomyosarcoma is associated to a variety of genetic syndromes and many genes are involved in tumor development. PAX3-FKHR and PAX7-FKHR (P-F) fusion state has important implications in the pathogenesis and biology of RMS, and different genes alterations are involved in the pathogenesis of P-F negative and embryonal RMS, which are the subsets of tumors most frequently affecting the bladder. These genes include p53, MEF2, MYOG, Ptch1, Gli1, Gli3, Myf5, MyoD1, NF1, NRAS, KRAS, HRAS, FGFR4, PIK3CA, CTNNB1, FBXW7, IGF1R, PDGFRA, ERBB2/4, MET, BCOR. Malignant rhabdoid tumor (MRT) usually shows SMARCB1/INI1 alterations. Anaplastic lymphoma kinase (ALK) gene translocations are the most frequently associated alterations in inflammatory myofibroblastic tumor (IMT). Few genes alterations in urothelial neoplasms have been reported in the paediatric population, which are mainly related to deletion of p16/lnk4, overexpression of CK20 and overexpression of p53. Here, we reviewed available literature to identify genes associated to bladder malignancies in children and discussed their possible relationships with these tumors. © 2016 Bentham Science Publishers. Source

Strigari L.,Regina Elena Cancer Institute | Sciuto R.,Regina Elena Cancer Institute | Rea S.,Regina Elena Cancer Institute | Carpanese L.,Regina Elena Cancer Institute | And 6 more authors.
Journal of Nuclear Medicine

Radioactive 90Y-selective internal radiation (SIR) sphere therapy is increasingly used for the treatment of nonresectable hepatocellular carcinoma (HCC). However, the maximum delivered dose is limited by severe injury to the nontarget tissue, including liver parenchyma. Our study aimed to implement radiobiologic models for both tumor control probability (TCP) and normal-tissue complication probability (NTCP) to describe more effectively local response and the liver toxicity rate, respectively. Methods: Patients with documented HCC, adequate bone marrow parameters, and regular hepatic and pulmonary function were eligible for the study. Patients who had pulmonary shunt greater than 20% of 99mTc-labeled macroaggregated albumin or any uncorrectable delivery to the gastrointestinal tract, reverse blood flow out of the liver, or complete portal vein thrombosis were excluded. Patients received a planned activity of the 90Y-SIR spheres, determined using the empiric body surface area method. The dose distribution was determined using posttreatment (3-dimensional) activity distribution and Monte Carlo dose voxel kernel calculations, and the mean doses to healthy liver and tumor were calculated for each patient. Response was defined according to Response Evaluation Criteria in Solid Tumors (RECIST) and recommendations of the European Association for the Study of the Liver (EASL). Criteria were used to assess possible liver toxicities. The parameters of TCP and NTCP models were established by direct maximization of the likelihood. Results: Seventy-three patients were treated. With an average dose of 110 Gy to the tumor, complete or partial response was observed in 74% and 55% of patients according to the EASL guideline and RECIST, respectively, and the predicted TCPs were 73% and 55%, respectively. With a median liver dose of 36 Gy (range, 6-78 Gy), the ≥grade 2 (G2), ≥grade 3 (G3), and ≥grade 4 (G4) liver toxicities were observed in 32% (23/73), 21% (15/73), and 11% (8/73) of patients, respectively. The parameters describing the ≥G2 liver toxicity data using the NTCP model were a tolerance dose of the whole organ leading to a 50% complication probability of 52 Gy (95% confidence interval, 44-61 Gy) and a slope of NTCP versus dose of 0.28 (95% confidence interval, 0.18-0.60), assuming n = 1. Conclusion: The radiobiologic approach, based on patient-specific dosimetry, could improve the 90Y-microsphere therapeutic approach of HCC, maintaining an acceptable liver toxicity. Copyright © 2010 by the Society of Nuclear Medicine, Inc. Source

Minisola G.,Azienda Ospedaliera San Camillo Forlanini
Giornale Italiano di Medicina del Lavoro ed Ergonomia

Musculoskeletal diseases are the most frequent cause of pain in the working population. Rheumatic diseases are chronic illnesses, cause of functional impairment, relevant working disability and absence from work; however, affected patients maintain a significant functional ability. In this context, the "Fit for work" project, operating in Italy since 2012, promotes the management of chronic musculoskeletal conditions through the realization, also in our country, of a rheumatic medical assistance network in behalf of workers affected by rheumatic diseases and other musculoskeletal disabiliting conditions. Source

Basile A.,Azienda Ospedaliera San Camillo Forlanini
Journal of Foot and Ankle Surgery

We present a retrospective study investigating the results of the subjective assessment of displaced intra-articular calcaneal fractures in a selected cohort of 42 patients treated operatively, with a follow-up duration of at least 3 years. The adjusted American Orthopaedic Foot and Ankle Society questionnaire, Foot Function Index, and visual analog scale were used to quantify the subjective evaluations. Our hypothesis was that good subjective results could be predicted and obtained in patients with specific characteristics if anatomic reduction of the fracture was achieved. The results of the study confirmed our hypothesis. A number of specific subgroup analyses were undertaken. The study confirmed that Böhler angle restoration and the quality of reduction of the subtalar joint facet are important prognostic factors related to the outcome. In contrast, gender and Sanders type had less influence at the intermediate-term follow-up results. The main weaknesses of the present study included its retrospective nature, the lack of a control group managed nonoperatively for comparison, and the small sample size. Moreover, the operating surgeon performed the radiographic measurement and categorized the quality of the surgical reconstruction. © 2012 American College of Foot and Ankle Surgeons. Source

Filippetti R.,Azienda Ospedaliera San Camillo Forlanini | Pitocco R.,Azienda Ospedaliera San Camillo Forlanini
American Journal of Dermatopathology

A 14-year-old girl stated that she has vulvar pruritus, pain, and bleeding and underwent a complete gynecological evaluation during which a suspicious 2-cm achromic lesion on her vulva was observed. Initially, it was suspected to be a pyogenic granuloma, in relation to the patient's age. A biopsy was taken, and a histopathological diagnosis of amelanotic mucosal melanoma was made. Pyogenic granuloma is a reactive hyperproliferation vascular response to trauma or other stimuli. It predominantly occurs in the second decade of life in young females, in relation to the vascular effects of female hormones. Primary mucosal melanoma is a rare and aggressive neoplasm, characterized by a higher aggressiveness and a worse prognosis than her cutaneous counterpart. The female genital tract is the second most common site of onset of mucosal melanoma; it represents the 3% of melanomas diagnosed in women. © 2015 Wolters Kluwer Health, Inc. Source

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