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Introduction: The humerus is the second most common long bone site of metastatic disease from renal cell carcinomas (RCC) after femur. Surgery has an important role in the treatment of these lesions due to renal cell tumor's resistance to chemotherapy and radiotherapy. The aim of this study is to determinate if prosthetic replacement is an effective and safe solution in treatment of renal cancer. Hypothesis: Prosthetic replacement is an effective and safe solution in treatment of renal humeral metastasis. Material and methods: Fifty-six patients affected by RCC bone metastases of the humerus that underwent a surgical reconstruction were reviewed. Thirty-five lesions were localized on proximal third, 12 on the shaft, 9 on distal third. Among proximal 29 were treated with resection and endoprosthetic replacement and 6 with plate and cement. Six diaphyseal lesions were stabilized with intramedullary nailing, 5 with plate and cement and 1 with an intercalary prosthesis. Regarding distal lesions, 7 elbow prostheses and 2 plates and cement were used. Results: The average age was 63 years. Metastasis was single in 55% of cases, and in 45% metachronous. A pathologic fracture (PF) occurred in 64% of cases. Only 9% of patients had a mechanical complication, 7% an infection and 5% neurological deficit. A local recurrence occurred in 14% of patients. An implant failure has been observed in 10 patients, 5 for mechanical complications, 2 for infections and 3 for local recurrence; of these 7 were treated with a prosthesis and 3 with plate and cement. The mean value of MSTS score was 64%, 63% and 59% respectively in patients with proximal, diaphyseal and distal humerus metastases. Discussion: Solitary and metachronous bone metastases have a longer survival. Disease-free interval > 2 years is another important prognostic factor. Reconstruction with a modular prosthesis is recommended in proximal and distal third. Instead in diaphyseal lesions a closed reduction and fixation with intramedullary locked nailing are preferred. When surgical indications are correctly followed, good oncologic and functional outcomes are obtained, leading to markedly improvement of patients’ quality of life. Retrospective study: Level of evidence IV.

Extra-axial chordoma is an exceedingly rare tumor, with only 28 cases reported in the literature to date. Axial and extra-axial chordoma exhibits complete morphologic and immunophenotypic (expression of brachyury) overlap. However, in consideration of the non-canonical presentation, extra-axial chordoma is under-recognized and often misdiagnosed, most often as extraskeletal myxoid chondrosarcoma or myoepithelioma. To increase our understanding of the clinicopathologic features of extra-axial chordoma, six cases have been retrieved from the files of the Istituto Ortopedico Rizzoli and of the General Hospital of Treviso. The clinicoradiologic, morphologic, and molecular features have been analyzed, and the follow-up was updated. Our series included four female and two male patients; their ages ranged from 20 to 67 years (mean 45.8 years). All patients presented with a single mass localized in four cases in the soft tissue (posterior arm, left leg, dorsal aspect of the foot, and popliteal fossa), and in two cases in the bone (radius and second metacarpal bone). Grossly, the neoplasm was lobulated, with a fleshy cut surface and a diameter ranging between 0.8 and 8 cm (mean 3.4 cm). Morphologically, all six cases showed an epithelioid cell proliferation organized in nests and cords demarcated by fibrous septa and set in an abundant extracellular myxoid matrix. Neoplastic cells featured hyperchromatic nuclei and abundant vacuolated cytoplasm. Immunohistochemically, all six cases were strongly positive for EMA, cytokeratin AE1/AE3, S100, and brachyury. INI1 nuclear expression was retained. Smooth muscle actin, calponin, p63, and GFAP were all negative. Fluorescent in situ hybridization (FISH) analysis did not reveal rearrangements involving NR4A3, FUS, and EWSR1 genes. At follow-up (mean 55 months), all patients were alive without disease after local surgical treatment. One patient underwent thigh amputation following multiple local recurrences and inguinal node metastases treated with marginal resection. In conclusion, primary extra-axial chordoma is an extremely rare neoplasm with distinct morphological and immunohistochemical features. Immunomorphology and molecular analysis allow distinction from both extraskeletal myxoid chondrosarcoma and myoepithelioma. Complete surgical resection appears to be curative.

Background and purpose Only a few studies have considered the role of comorbidities in the prognosis of amyotrophic lateral sclerosis (ALS) and have provided conflicting results. Methods Our multicentre, retrospective study included patients diagnosed from 1 January 2009 to 31 December 2013 in 13 referral centres for ALS located in 10 Italian regions. Neurologists at these centres collected a detailed phenotypic profile and follow-up data until death in an electronic database. Comorbidities at diagnosis were recorded by main categories and single medical diagnosis, with the aim of investigating their role in ALS prognosis. Results A total of 2354 incident cases were collected, with a median survival time from onset to death/tracheostomy of 43 months. According to univariate analysis, together with well-known clinical prognostic factors (age at onset, diagnostic delay, site of onset, phenotype, Revised El Escorial Criteria and body mass index at diagnosis), the presence of dementia, hypertension, heart disease, chronic obstructive pulmonary disease, haematological and psychiatric diseases was associated with worse survival. In multivariate analysis, age at onset, diagnostic delay, phenotypes, body mass index at diagnosis, Revised El Escorial Criteria, dementia, hypertension, heart diseases (atrial fibrillation and heart failure) and haematological diseases (disorders of thrombosis and haemostasis) were independent prognostic factors of survival in ALS. Conclusions Our large, multicentre study demonstrated that, together with the known clinical factors that are known to be prognostic for ALS survival, hypertension and heart diseases (i.e. atrial fibrillation and heart failure) as well as haematological diseases are independently associated with a shorter survival. Our findings suggest some mechanisms that are possibly involved in disease progression, giving new interesting clues that may be of value for clinical practice and ALS comorbidity management.

BACKGROUND: Surgical treatment of pelvic tumors with or without acetabular involvement is challenging. Primary goals of surgery include local control and maintenance of good quality of life, but the procedures are marked by significant perioperative morbidity and complications. QUESTIONS/PURPOSES: We wished to (1) evaluate the frequency of infection after limb salvage surgical resection for bone tumors in the pelvis; (2) determine whether infection after these resections is associated with particular risk factors, including pelvic reconstruction, radiotherapy or chemotherapy, type of resection, and age; and (3) analyze treatment of these infections, particularly with respect to the need of additional surgery or hemipelvectomy. METHODS: From 1975 to 2010, 270 patients with pelvic bone tumors (149 with chondrosarcoma, 40 with Ewing's sarcoma, 27 with osteosarcoma, 18 with other primary malignant tumors, 11 with metastatic tumors, and 25 with primary benign tumors) were treated by surgical resection. Minimum followup was 1.1 years (mean, 8 years; range, 1-33 years). The resection involved the periacetabular area in 166 patients. In 137 patients reconstruction was performed; in 133 there was no reconstruction. Chart review ascertained the frequency of deep infections, how they were treated, and the frequency of resection arthroplasty or hemipelvectomies that occurred thereafter. RESULTS: A total of 55 patients (20%) had a deep infection develop at a mean followup of 8 months. There were 20 infections in 133 patients without reconstruction (15%) and 35 infections in 137 patients with reconstruction (26 %). Survivorship rates of the index procedures using infection as the end point were 87%, 83%, and 80% at 1 month, 1 year, and 5 years, respectively. Infection was more common in patients who underwent pelvic reconstruction after resection (univariate analysis, p = 0.0326; multivariate analysis, p = 0.0418; odds ratio, 1.7718; 95% CI, 1.0243-3.0650); no other risk factors we evaluated were associated with an increased likelihood of infection. Despite surgical débridements and antibiotics, 16 patients (46%) had the implant removed and five (9%) underwent external hemipelvectomy (four owing to infection and one as a result of persistent infection and local recurrence). CONCLUSIONS: Infection is a common complication of pelvic resection for bone tumors. Reconstruction after resection is associated with an increased risk of infection compared with resection alone, without significant difference in percentage between allograft and metallic prosthesis. When infection occurs, it requires removal of the implant in nearly half of the patients who have this complication develop, and external hemipelvectomy sometimes is needed to eradicate the infection. LEVEL OF EVIDENCE: Level IV, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.

Background Prostheses can be used in elbow reconstruction in both primary and metastatic lesions. Several authors have reported their experience with different types of implant, but not with modular prostheses. Hypothesis Limb salvage using an elbow prosthesis is effective in obtaining good functional results and reliable local tumor control. Material and methods Forty-seven patients treated at the Rizzoli Institute for elbow neoplasm from 1990 to 2012 were evaluated. There were 30 primary tumors (64%), 24 bone tumors and 6 soft tissue sarcomas, and 17 bone metastases. Elbow reconstruction used a modular prosthesis in 25 patients and a standard prosthesis in 22. Reconstruction was primary in 30 patients and secondary in 17. Results At last control, 15 (32%) were dead of disease (DOD) at a mean follow-up of 35 months, 12 (25%) were alive with disease (AWD) at a mean follow-up of 29 months, 19 (40%) showed no evidence of disease (NED) at a mean follow-up of 80 months. Early complications were related to unexpected neurological damage, observed in 12 patients (25%): in 5 cases the deficit resolved in a mean 6 months; in the others, no or only partial recovery was observed. Two implants (4%) developed infection: 1 was treated with antibiotic therapy, and the other required implant revision. One implant showing cement extrusion was revised. In 3 patients (6%) radiography showed a radiolucent halo around the stem (2 humeral, 1 ulnar); no measures were taken, as the patients were completely asymptomatic at every follow-up. In 3 patients (6%) partial resorption of the allograft was observed on X-ray, but remained unchanged at last follow-up, without pain or functional impairment. Seven local recurrences (15%) were observed, at a mean of 16 months after surgery; 5 were treated by resection and/or radiotherapy, and 2 by amputation. Mean functional scores on MEPS and MSTS were respectively 84% and 22/30 (73%). Conclusions Elbow prostheses provided better function in primary than in metastatic tumor. Elbow prosthesis reconstruction after tumor resection is a viable option both for primary and secondary bone neoplasms. Type of study Therapeutic. Level of evidence IV, retrospective study.

BACKGROUND: Infection of megaprostheses after bone tumor resection is a major concern; management is challenging. This study evaluated the survivability from such infections, the microbial isolates, treatment tactics, and outcome of megaprosthesis reconstructions. MATERIALS AND METHODS: We studied 1,161 patients retrospectively who underwent megaprosthesis reconstruction for limb salvage after a sarcoma from 1983 to 2010. The mean followup was 9 y (range 3-20 y). We evaluated the overall survival of the megaprosthesis reconstructions in patients with infection and the survival with respect to the type of megaprosthesis, site of reconstruction, cemented or cementless fixation, type of tumor, adjuvant treatments, microbial isolate(s), treatment tactics, and outcome. RESULTS: The incidence of infection was 8.6%. The most common microbial isolate was Staphylococcus epidermidis (47%). Overall survival with definitive management of infection was 88% at 10 y and 84% at 20 y. Survival was higher for cementless reconstructions and not different with respect to the type of megaprosthesis, site of reconstruction, or adjuvant therapy. Infections resolved completely with one- or two-stage surgery in 75% of patients. The rate of amputation because of infection was 21%. CONCLUSIONS: Megaprosthesis reconstructions may be infected in 8.6% of cases. Infections more commonly occur late, caused usually by S. epidermidis. The survival rate is higher with cementless megaprosthesis reconstructions and no different with respect to the type of tumor or megaprosthesis or the adjuvant treatments. One-stage revision is effective for acute post-operative infections; however, two-stage revision surgery is necessary for early and late infections. The rate of amputation because of occurrence or persistence of megaprosthesis infection is 21%.

BACKGROUND Incidence and risk factors for delirium during clozapine treatment require further clarification. METHODS We used computerized pharmacy records to identify all adult psychiatric inpatients treated with clozapine (1995-96), reviewed their medical records to score incidence and severity of delirium, and tested associations with potential risk factors. RESULTS Subjects (n = 139) were 72 women and 67 men, aged 40.8 +/- 12.1 years, hospitalized for 24.9 +/- 23.3 days, and given clozapine, gradually increased to an average daily dose of 282 +/- 203 mg (3.45 +/- 2.45 mg/kg) for 18.9 +/- 16.4 days. Delirium was diagnosed in 14 (10.1 % incidence, or 1.48 cases/person-years of exposure); 71.4 % of cases were moderate or severe. Associated factors were co-treatment with other centrally antimuscarinic agents, poor clinical outcome, older age, and longer hospitalization (by 17.5 days, increasing cost); sex, diagnosis or medical co-morbidity, and daily clozapine dose, which fell with age, were unrelated. CONCLUSIONS Delirium was found in 10 % of clozapine-treated inpatients, particularly in older patients exposed to other central anticholinergics. Delirium was inconsistently recognized clinically in milder cases and was associated with increased length-of-stay and higher costs, and inferior clinical outcome.

Differences in incidence and survival in osteosarcoma reports are considerable worldwide.This study describes the incidence and survival of patients with osteosarcoma in Argentina with data from the National Pediatric Cancer Registry (ROHA), and the impact of age, gender, stage, regional, and socioeconomic indicators on outcome.Pediatric patients with osteosarcoma reported to ROHA from 2000 through 2013 were analyzed, the annual age-standardized incidence rate (ASR) was calculated using the National Vital Statistics, and survival was estimated. The extended human development index (EHDI) for each reporting region was used as an indicator of socioeconomic status.There were 515 cases of osteosarcoma identified, yielding an ASR of 3.2/1,000,000 children (0-14 years old). The ASR did not vary significantly by year of diagnosis but ranged from 4.0/1,000,000 in the Cuyo/Western Central region to 2.7/1,000,000 in the northeast region (P0.000). The estimated 5-year survival rate was 45% (95% confidence interval [CI] 44-51%), with no difference by sex, diagnosis year, region, or EHDI score (P0.1 in all cases). Survival rate for localized disease was 52% (95% CI 45-57%) and for metastatic 22% (95% CI 15-30%).In Argentina, ASR of osteosarcoma is similar to that in high-income countries, but survival is lower in all regions. Future work will focus on identification and reduction of causes of preventable treatment failure.

Aims Serum C3 is a powerful indicator of the risk of myocardial infarction, which correlates with body mass index, serum lipids and blood pressure. This study was performed to ascertain whether such correlations may be explained by an association of C3 with fasting insulin, and to assess comparatively the relationships of C3 and traditional risk factors to previous myocardial infarction. Methods and Results The fasting levels of C3, insulin, and the main risk factors were evaluated in 1090 unselected men aged 55-64 years, including 129 cases of previous ischaemic events (51 myocardial infarctions). In multivariate analysis C3 was associated with insulin (r=0.27, P

Hibernomas are rare benign adipose tumors composed of brown fat cells with granular, multivacuolated cytoplasm admixed with white adipose tissue. They account for 1.6% of benign lipomatous tumors and approximately 1.1% of all adipocytic tumors. They are more common in the third and fourth decades of life. The most common location is the thigh, followed by the shoulder, back, and head and neck. Four histological types have been reported; abundant vascularity is characteristic, and atypias are rare. The treatment of choice for hibernomas is complete surgical excision. Metastases or malignant transformation have not been reported. This article presents a series of 17 patients with hibernomas diagnosed and treated at our institution from January 1986 to December 2009. Six men and 11 women (M:F, 1:2) had a mean age of 38 years (range, 10 months to 64 years). All patients underwent surgical treatment; 14 patients had marginal and 3 had wide excision. Adjuvants such as radiation therapy, chemotherapy, or embolization were not administered for any patient. The most common symptom was a painless palpable mass, followed by a tender or painful mass; in 2 patients, the tumor was an incidental finding. The duration of symptoms ranged from 1 month to 10 years (mean, 27 months). The most common location was the thigh, followed by the buttock, scapula, and neck. The most common histological variant was the typical variant followed by the lipoma-like variant. At a mean follow-up of 5 years (range, 1–9 years), local recurrences were not observed.

17beta-Estradiol (E2) exerts neurotrophic and neuroprotective effects in the retina as well as in other CNS structures, independently of sex. Retinal effects, however, have not been supported by evidence on local synthesis, and whether CNS 17beta-estradiol is formed in a neurosteroidogenic pathway starting from cholesterol conversion into pregnenolone is a question still left unanswered. In the adult male rat retina, we have previously showed localization and activity of the P450 side chain cleavage (P450scc) enzyme, which is involved in pregnenolone synthesis. Here, we demonstrate both the mRNA and protein expression of 3beta-hydroxysteroid dehydrogenase (3beta-HSD), P450aromatase and also of P450scc, but only the protein expression of P450 17alpha-hydroxylase/lyase (P450c17). Using radiolabeled pregnenolone and testosterone as precursors, in the isolated and intact retina of adult male rats, E2 is produced in a large amount by each precursor within 1-4h, suggesting a highly active metabolic pathway towards its formation. The immunolocalization pattern shows enzymes and estrogen receptor subtypes (ERalpha, ERbeta) scattered in the retina with different intensities throughout the layers. The results point to the adult male rat retina as a neurosteroidogenic structure where E2 synthesis via a progesterone pathway and the presence of estrogen receptors provide important clues for understanding the neurotrophic and neuroprotective effects of the steroid hormone.

Background/aim. Transthoracic electrical bioimpedance is a non-invasive technique for the evaluation of systemic haemodynamics. Compared to Doppler ultrasound, it has the advantage of being operator-independent, providing continuous monitoring and being less influenced by postural changes. Until now, transthoracic electrical bioimpedance has been applied to a very limited extent in liver cirrhosis. We, therefore, aimed to compare transthoracic electrical bioimpedance and echocardiography in the assessment of haemodynamic status in cirrhotic patients. Patients/methods. Thirteen patients with compensated cirrhosis, 10 patients with cirrhosis and ascites and 12 controls were enrolled. Haemodynamic parameters (stroke volume, cardiac output, heart rate, mean arterial pressure and vascular peripheral resistance) were assessed simultaneously by transthoracic electrical bioimpedance monitoring with BioZ.com™ for at least 10 min and Doppler ultrasound. Results. The absolute mean values of haemodynamic parameters obtained by the two techniques were quite similar in all groups; furthermore, a good agreement between transthoracic electrical bioimpedance and echocardiography measurements was found for all the parameters. Finally, transthoracic electrical bioimpedance proved easy to employ and provided continuous real-time monitoring of cardio-circulatory variations. Conclusions. The present study showed a significant correlation between transthoracic electrical bioimpedance and echocardiography in the assessment of systemic haemodynamics in patients with cirrhosis, supporting the employment of transthoracic electrical bioimpedance in pathophysiological studies requiring real-time continuous monitoring of haemodynamic parameters.

We have investigated recurrence of stroke in a consecutive series of young adults, aged 16 to 45 years, after a first cerebral infarction.From January 1, 1988 to December 31, 1996 we submitted those patients to a diagnostic protocol including angiographic, cardiological, and haematological investigations. The patients were followed at 6 month intervals up to December 31, 1998.We have evaluated and followed-up 135 patients, 71 men and 64 women, who were 3.99% of all the admitted stroke patients. At 12 months after stroke, 83 patients had returned to work, 40 patients were mildly to moderately handicapped, 4 were using a wheel-chair, and 8 had died. Follow-up was 26 to 123 months (mean 68.8). Recurrence of stroke, always of ischaemic nature, was seen in 15 patients (11.1%), 3 to 76 months after the first stroke (mean 27.4), for an annual incidence of 2.26%. Recurrence was significantly associated with Partial Anterior Circulation Syndrome and Haematological subtype of first stroke (respectively, P = 0.0209 and P = 0.0135, chi2 test), but not with age (or = or35 years) or risk factors. Repetition of stroke was never fatal, but it caused heavy disability in 13 patients, 8 of whom had completely or nearly completely recovered after the first event.Our data suggest that recurrence of stroke is a major clinical problem also for the patients aged less than 45 years and that it might be more frequent with specific clinical syndromes and etiologic subtypes of first stroke.

Introduction - We have undertaken a prospective study to measure anticardiolipin antibodies of IgG isotype within the first few hours of an acute non-hemorrhagic stroke. Material and methods - We have collected blood samples at entry from one hundred patients (53 men and 47 women), mean age 67.4 years, referred within 6 h of a first-ever non-hemorrhagic stroke, and from an equal number of age- and gender-matched control patients. Results - IgG anticardiolipin antibodies were ≥10 GPL in 26 patients and in 5 controls (p

One of the most common complication in haemodialysis patients is thrombosis of the arteriovenous fistula (AVF). Thirty-five patients with a total of 42 thromboses of the angioaccess were infused via a small needle: (i) into the feeding artery (50% of the cases); (ii) into a AVF venous segment of the arteriovenous fistula (42.8%); (iii) directly into the thrombus (7.1%), by rt-PA. After an initial pulse of 5-10 mg, according to body weight, the drug was continuously infused by a pump with the speed automatically programmed in 30 Brescia-Cimino autologous AV fistulae and 12 polytetrafluoroethylene (PTFE) grafts. A complete thrombolysis with return of bruit and thrill was obtained in 71.4% of the cases using a mean drug dose of 21 mg and an infusion time of 3.8 h. All the successful cases underwent haemodialysis via AVF on the same day. No bleeding occurred at remote sites. Local bleeding occurred in 16% of the cases; in no case was it so severe as to require the suspension of the therapy or blood transfusions. The median cumulative duration of patency after thrombolysis was 32.4 months. Respectively 21, 12 and two patients had a functioning angioaccess after 3.6, 32.4 and 36 months from the lytic approach. Failure of the treatment was not related to the patients' gender or age, AVF age, route of administration of the drug, type of vessel (natural or artificial), or delay between the discovery of the fistula occlusion and the start of the therapy. In unsuccessful cases an organic lesion of the vessels was documented by angiography or echo colour Doppler. In summary, rt-PA local infusion provides a useful means of preservation of AV fistulae and may be used as the therapy of first choice in dialysis patients without active bleeding or high bleeding risk.

In order to define the validity of a screening program for hypertension in schools, aiming to prevent an hypertensive status in adult age, we screened 861 children from schools on the area of our Medical District (U.S.L. 22--Vittoria--Sicily). The object of our study is to search for risk factors of hypertension, detectable at school age and, if existing, which age is more suitable for a screening program. We randomly sampled 861 children from different schools, aging 6, 10 and 13 years. The first step was to define the prevalence of hypertension and obesity in the sample, and the correlation between the two factors (transversal study). We found an overall prevalence of obesity at 6, 10, and 13 years of 6.69%, 18.22% and 17.7% respectively. The prevalence of hypertension was 4.01%, 11.4%, and 6.98% at 6, 10 and 13 years respectively. The second step is to create a personal record of each child, which will be followed up to the age of 18 years, containing familial and nutritional informations, periodic evaluations of height, weight, blood pressure and biologic sampling (longitudinal study). Our preliminary results confirmed the correlation between obesity and hypertension, although they do not help to clarify why the prevalence of hypertension decreases from 10 to 13 years whereas obesity maintains the same prevalence at the mentioned ages.

An essential introduction for a campaign of health education, to take preventive measures against caries, is to have knowledge of the prevailing situation of the pathology. The research analysed a sample consisting of 1371 pupils attending the "scuole elementari" and the "scuole medie" in the municipal districts of Vittoria, Acate and Scoglitti. The methodological statement consist of an analysis regarding experimental epidemiology. The emerged data, proposed in diagrams and tables, show that caries frequency increases in the pupils' ages, going from a value of 57.7% at the age of 6 to a value of 75.2% at the age of 13; besides, it is statistically meaningful the caries difference with regard to the sex at the age of 13. So the DMF and dmf values, although not alarming point out the necessity of a preventive intervention.

BACKGROUND: Studies for patients with pelvic chondrosarcomas are limited. This study determines the outcome of patients with pelvic chondrosarcomas, and whether there is any association with tumors' grade, type, stage, margins and pelvic location. MATERIALS AND METHODS: We retrospectively studied 215 patients with pelvic chondrosarcomas. All patients had biopsy and histological diagnosis of their tumors followed by limb salvage or amputation. We staged patients using the Musculoskeletal Tumor Society system. We performed a univariate and multivariate analysis of the survival to death, local recurrence and metastasis with respect to grade, type (central vs. peripheral), stage, margins, and pelvic location, and the survival to death of patients with and without local recurrence. RESULTS: Grade was the most important univariate and multivariate predictor of the survival of the patients. Dedifferentiation was associated with significantly lower overall survival. Peripheral chondrosarcomas predicted survival only in the univariate analysis. Surgical margins predicted local recurrence only in the multivariate analysis. Periacetabular location was associated with lower survival to death and local recurrence. The occurrence of local recurrence compromises the overall survival. CONCLUSION: Grade was the most important predictor of the overall survival of patients with chondrosarcomas of the pelvis.