Experimental and numerical studies of inclined cables: free and parametrically-forced vibrations

Because of few experimental studies in the inclined cable literature, this paper is aimed at experimental modelling and investigating the linear free and nonlinear forced vibrations of sagged inclined cables, by discussing the relevant outcomes in the background of theoretical and numerical achievements. Attention is paid to the identification of cable hybrid modes due to system asymmetry, which gives rise to an avoidance phenomenon in the natural frequency spectrum, and to the investigation of some typical 3-D nonlinear dynamics involving the simultaneous parametric/external excitation due to a harmonically time-varying support movement. Large-amplitude out-of-plane/in-plane multi-modal interactions due to non-planar/planar internal resonances are experimentally observed and complemented by space-time numerical simulation of the associated, geometrically nonlinear, partial-differential equations of parametrically-forced cable motion. Overall, the experimental and numerical results highlight the fundamental linear/nonlinear dynamic characteristics of inclined cables, and the crucial role played by the asymmetry induced by cable inclination, in addition to the significant effects of cable sag and dynamic extensibility

Experimental and Analytical Investigation into the Effect of Ballasted Track on the Dynamic Response of Railway Bridges under Moving Loads

Ballasted tracks are among the most widespread railway track typologies. The ballast possesses multiple functions. Among them, it significantly affects the dynamic interaction between a rail bridge and a moving load in terms of damping and load distribution. These effects entail accurate modeling of the track-ballast-bridge interaction. The paper presents a finite-difference formulation of the governing equations of the track and the bridge, modeled as Euler-Bernoulli (EB) beams, and coupled by a distributed layer of springs representing the ballast. The two equations are solved under a moving load excitation using a Runge-Kutta family algorithm and the finite-difference method for the temporal and spatial discretization, respectively. The authors validated the mathematical model against the displacement response of a rail bridge with a ballasted substructure. In a first step, the modal parameters of the bridge, obtained from ambient vibration measurements, are used to estimate the bending stiffness of an equivalent EB beam representative of the tested bridge. In a second step, the authors estimated the coupling effect of the ballast by assessing the model sensitivity to the modeling parameters and optimizing the agreement with the experimental data. Comparing the bridge's experimental displacement responses highlights the ballast's significant effect on the load distribution and damping. The considerable difference between the damping estimated from output-only identification and that determined from the displacement response under moving load proves the dominant role of the ballast in adsorbing the vibrations transmitted to the bridge under the train passage and the different damping sources under high-amplitude excitation. The authors discuss the tradeoff between model accuracy and computational effort for a reliable estimation of ballasted tracks response under moving loads

Fibrous hamartoma of Infancy: An Italian multi-istitutional experience.

Fibrous hamartoma of infancy: an Italian multi-institutional experience. Carretto E, Dall'Igna P, Alaggio R, Siracusa F, Granata C, Ferrari A, Cecchetto G. Source Division of Paediatric Surgery, Department of Paediatrics, University of Padua, Padua, Italy. Abstract BACKGROUND: Fibrous hamartoma (FH) of infancy is a benign mesenchymal tumor, occurring as a superficial mass. Complete excision is curative. OBJECTIVE AND METHODS: The clinical features and treatment results of 18 children with FH are described. RESULTS: Local excision was the most common procedure. Surgery was radical in 10 patients, with microscopic residual disease in 6; all of them are alive with no evidence of disease 2 to 49 months after diagnosis. One patient, treated with a local reexcision for macroscopic residual disease (and chemotherapy for a synchronous desmoid fibromatosis) is well 83 months after diagnosis; the last patient, with a lesion of the labia majora, only underwent biopsy and is doing well, awaiting plastic surgery. LIMITATIONS: The results did not reach statistical significance due to difficulties in collecting cases. CONCLUSIONS: FH should be treated by complete excision; in our experience a nonradical excision was also able to achieve the cure. An aggressive approach should be avoided, as the overall prognosis is excellent. PMID: 16635660 [PubMed - indexed for MEDLINE

Congenital pancreatoblastoma: a case report

The literature describes 15 cases of congenital pancreatoblastoma (PB): 5 had prenatal diagnosis, none had metastases at diagnosis, 7 were associated with BeckwitheWiedemann syndrome (BWS). In 13 cases resection was radical, while in 2 there were macroscopic residues. Only one patient underwent chemotherapy after distant recurrence. All children are alive except one who died because of problems related to BWS. Our goal is to describe the approach adopted in an infant with congenital PB treated in our center. After a prenatal third semester diagnosis of abdominal anechoic lesion, the radiological investigations (ultrasound, MRI) performed at birth described a cystic lesion of unclear nature. We proceeded to laparoscopic exploration, transformed into open approach after the detection of a lesion located in the body of the pancreas; this lesion was resected, preserving the head and tail of pancreas. The histological diagnosis showed a completely excised PB. After excluding metastatic lesions, we decided to perform only careful follow-up without chemotherapy. The follow-up at 12 months is negative. Although PB is a malignant tumor that requires a multidisciplinary treatment, the congenital cases seem to have a less aggressive biological behavior. The treatment, therefore, in case of complete resection, could be only surgical, followed by a careful follow-up. These forms are often associated with congenital BWS, but in our case the patient did not have the typical characteristics of the syndrome

SURGERY IN MALIGNANT GERM CELL TUMORS OF CHILDHOOD. RESULTS OF THE SECOND ITALIAN COOPERATIVE STUDY TCG 98

Analysis of treatment and results of the patientsenrolled in the Italian TCG-98 Study, still open and comparison of data with those of the previous Studt TCG-9

NTRK3 overexpression in undifferentiated sarcomas with YWHAE and BCOR genetic alterations

The BCOR family of tumors includes a number of undifferentiated sarcomas, occurring in various age groups and anatomic sites, characterized by a spindle and round cell phenotype and diffuse immunoreactivity for BCOR. Prior RNA sequencing data revealed that NTRK3 was a top-upregulated gene in BCOR-CCNB3 sarcomas. In this study, we investigate a large cohort of tumors harboring BCOR/YWHAE genetic alterations for NTRK3 upregulation at both the mRNA and protein levels, compared with other sarcoma types. Pan-Trk immunohistochemistry was assessed for intensity and extent. A correlation between NTRK3 expression and the type of BCOR alteration and BCOR immunoreactivity was also performed. Most soft tissue undifferentiated round cell sarcomas with YWHAE or BCOR rearrangements or BCOR internal tandem duplications (ITD) showed NTRK3, but not NTRK1 or NTRK2, upregulation by RNA sequencing data analysis. Cytoplasmic pan-Trk immunoreactivity was also observed in most soft tissue round cell sarcomas with YWHAE rearrangements (100%), BCOR ITD (80%), and BCOR-CCNB3 fusions (67%), as well as clear cell sarcomas of kidney (75%), another BCOR family tumor, and ossifying fibromyxoid tumors with ZC3H7B-BCOR fusion (100%), with variable staining intensity and extent. Pan-Trk staining was also seen in solitary fibrous tumors (100%) and less frequently in synovial sarcoma and Ewing sarcoma, but rarely in other sarcomas tested. Tumors harboring rare fusion variants of BCOR, such as BCOR-CHD9, a novel fusion identified by targeted RNA sequencing, and KMT2D-BCOR, were also positive for pan-Trk staining and NTRK3 overexpression. In conclusion, NTRK3 upregulation resulting in pan-Trk overexpression is common in the BCOR family of tumors as well as in subsets of BCOR-expressing sarcomas through alternative mechanisms. The therapeutic implication of this finding awaits further investigation

PLEUROPULMONARY BLASTOMA: A DIFFERENTIAL DIAGNOSIS OF CHRONIC COUGH. LONG TERM SURVIVALAFTER MULTIMODAL AGGRESSIVE THERAPY

ITAL J PEDIATR 2006;32:122-125 CASE REPORT CASO CLINICO Pleuropulmonary blastoma: a differential diagnosis of chronic cough. Long-term survival after multimodal aggressive therapy Blastoma pleuro-polmonare: rara diagnosi differenziale da tosse cronica. Remissione a lungo termine dopo aggressiva terapia multimodale P. D''ANGELO, V. MANZO*, S. VENEZIA*, R. ALAGGIO", E SIRACUSA*, M. LO CURTO* Unit\ue0 Operativa di Oncoematologia Pediatrica, Ospedale dei Bambini " G . Di Cristina", Palermo; * Dipartimento Materno Infantile, Universit\ue0 di Palermo; ** Istituto di Anatomia Patologica, Universit\ue0 di Padova Summary Pleuropulmonary blastoma (PPB) in childhood is a rare clinicopathologic entity distinct from adult pneumoblastoma. This tumour may originate from the lung, the pleura, or the mediastinum; it can metastasize and is usually associated with a poor outcome. We report the case of a 5-year-old boy who developed PPB manifesting with respiratory distress. At the standard x-ray and magnetic resonance imaging of the chest there was opacity covering the entire right lung. The histological and immunohistological tests led to the diagnosis of blastematous, malignant mesenchymatous PPB with pluridirectional differentiation. Treatment consisted of preoperative chemotherapy to reduce tumour volume, complete surgical resection of the residuai tumour mass, and post-surgical chemotherapy. Following this approach, the child is alive in continuous complete remission 9 years after diagnosis. Riassunto II Blastoma Pleuro-Polmonare (BPP) infantile \ue8 un ''entit\ue0 clinico patologica ben distinta dal Pneumoblastoma dell''adulto. Questo tumore pu\uf2 prendere origine dal polmone, dalla pleura o dal mediastino; pu\uf2 metastatizzare e ha spesso una prognosi infausta. Riportiamo il caso di un bambino di 5 anni, in cui il BPP si manifest\uf2 con un distress respiratorio; la radiografia del torace e la Risonanza Magnetica hanno evidenziato una grossa massa che occupava l''emitorace destro. L''esame istopatologico ha permesso di porre diagnosi di BPP. Il paziente \ue8 stato trattato con chemioterapia, che ha ridotto il volume della massa, con asportazione del tumore e chemioterapia post-operatoria; tale trattamento ha consentito i''eradicazione della malattia; il paziente \ue8 in remissione completa continua a 9 anni dalla diagnosi. Introduction Pleuropulmonary blastoma (PPB) is an extremely rare and aggressive malignancy of childhood. It was originally described as a distinct entity by Manivel et al. ''. Prior to its identification it was reported in the early literature by Spencer as pulmonary blastoma or embryonic sarcoma 2. It is characterized by primitive mesenchymal tissue and epithelial tubular structures resembling the foetal lung. The eponymous PPB defines the paediatric variety of pulmonary blastoma. In PPB, the dysembrionic neoplasm shows blastematous and sarcomatous components and a lack of carcinomatous components (which are instead present in adult pulmonary blastoma), sometimes on previous dysplastic pulmonary conditions 3 . PPB is classified in 3 subtypes: type I (cystic), type II (mixed solid and cystic) and type III (solid)4. Key words Pleuropulmonary blastoma \u2022 Childhood lung cancer \u2022 Adjuvant chemotherapy Parole chiave Blastoma pleuropolmonare \u2022 Tumore polmonare infantile \u2022 Chemioterapia post-operatoria Submitted: March 16, 2005 Accepted: July 19, 2005 Correspondence: Prof. Margherita Lo Curto Dipartimento di Pediatria Universit\ue0 di Palermo Istituto Materno Infantile via Cardinale Rampolla 1 Palermo, Italy Tel. +39 091 6555476 E-mail: [email protected]. 122 PLEUROPULMONARY BLASTOMA IN A CHILD The predominant clinical features are cough, tachypnea, fever, respiratory distress; secondary pneumothorax 5 and chest pain have also been reported 6. Since these features are not specific, an infectious disease is often erroneously diagnosed; hence, when eventually detected, the neoplasm is often very large, may even involve an entire hemithorax, and present metastases. Despite the different therapeutic procedures - surgery, chemotherapy and radiotherapy - prognosis is often poor: Indolfi et al. 7 report 42% and Priest et al. 6 45% of event free survival (EFS) at 2 years. Poor prognostic factors are histological subtype II or III6 , a maximum diameter greater than 5 cm 7 , failure to completely remove the mass, extrapulmonary effusion such as pleura or pericardium, metastases 8. We report the case of a five-year-old child who, despite the large tumour size at diagnosis and histological subtype II, after a treatment with chemotherapy before and after surgery, is in continuous complete remission (CCR) nine years after the diagnosis. Case report A 5-year-old boy was admitted to our ward for hyporexia, cough, shortness of breath, progressive thinning and pallor in the previous 2 months. The physical examination showed poor clinical conditions, tachydyspnea (R.F. 45/min.), hypophonesis and reduction of the physiologic vescicular murmure of the middle and lower regions of the right lung, meteoric abdomen with the liver margin 5 cm below the right costai margin. The results of the laboratory investigations were Hb 8.5 g/dl, white blood cells 18.800/ul (N 68%, L 22%, M 6%, E 4%), platelets 611.000/u.l, VES (K.I.) 65, CRP 2.4 mg/dl; serum levels of copper 168 ixg/dl, ferritin 292 ng/dl, LDH 1.261 u/1, oc-FP 6.3 u/1. Chest radiographs showed a bulky mass in the right hemithorax displacing the mediastinum leftward and the liver downward (Fig. 1). The thoracic-abdominal ultrasound scan showed a poorly confined voluminous mass, having diameters of 120 x 86 mm, with echogenic-hyperechogenic structure and some hypo-anechogenic areas, arising in right hemithorax and displacing the liver and right kidney downwards. A magnetic resonance imaging (MRI) of the thorax showed a mass involving entirely the right hemitorax, with a centrai hemorrhagic component that displaced the mediastinum and the heart to the left. The patient underwent surgical thoracotomy, which revealed an unencapsulated mass with smooth surface and tense-elastic consistency, entirely covered by pleura, not adherent to the thoracic wall; since the conspicuous extension of the mass did not allow resection, only a biopsy was performed. Microscopically, the biopsy specimen showed a predominantly solid neoplasm with focal cysts. The tumour contained mesenchymal elongated cells arranged in sheets, and more primitive blastematous foci. There Fig. 1. Posterior-anterior chest radiograph at presentation, showing a bulky mass, displacing the mediastinum. was no evidence of typical rhabdomyoblasts or cartilage. Cysts exhibited an epithelial lining, with flattened to columnar cells and an underlying layer of primitive mesenchymal cells. The morphologic appearance was consistent with a diagnosis of PPB type II. Immunostains emphasized the doubl\ue9 component with a positive staining for cytokeratin (MNF116, pancytokeratin) in the epithelial component and a positive vimentin staining in mesenchymal component. Occasionai spindle cells were positive for desmin; oc-fetoprotein, S-100 protein, CD99, NB84A were negative in both the epithelial and the stremai component. In order to stage the disease the patient underwent total body bone scan with "Tc-MDP, brain and abdominal CT scan, and bone marrow aspirate; no metastatic spread was detected, and a stage III was defined. The child underwent chemotherapy with carboplatinum (CBP) 400 mg/m2 + etoposide (VP16) 150 mg/m2 days 1, 2; vincristine (VCR) 1.5 mg/m2 + actinomycin-D (ACT-D) 1.5 mg/m2 day 21 + ifosfamide (IFO) 1500 mg/m2 days 21-23, for overall 3 cycles; thereafter, 2 cycles were scheduled, including VCR 1.5 mg/m2 + ACTD 1.5 mg/m2 day 1, doxorubicin 40 mg/m2 days 1-2 and IFO 1500 mg/m2 days 1-3. The number of cycles were established according to the features of imaging studies. A chest x-ray survey showed a very good response (Fig. 2) to chemotherapy. Six months after the diagnosis complete resection of the tumour was performed through a right posterior-lateral thoracotomy by the fifth intercostal space. The tumour was capsulated and located between the upper and middle lobe of the right lung, displacing caudally the middle and lower pulmonary lobes. The centrai zone of the mass was composed of hyalinized fibrous stroma nodules and very small fragments of blastomatosous tumoral tissue, at about 2 cm from the resection borders. The neoplasm was almost entirely necrotic. 123 P. D''ANGELO ET AL. Fig. 2. Posterior-anterior chest radiograph, after chemotherapy, before surgical excision. After surgery the patient underwent 2 more cycles of chemotherapy with CBP 400 mg/m2 + VP 16 150 mg/m2/day x 2 days. There was clinical and imaging evidence of a progressive normalisation of lung morphology and function. The patient was monitored with clinical and radiological investigations according to the following schedule: chest radiograms every 3 months the first year, every 6 months the second and third year, every 12 months for the 4t h, 5l h and 6* year; MRI at 1 and 3 years after withdrawal of therapy. Nine years after the diagnosis, the child is in continuous complete remission. Discussion PPB in childhood is very rare. Our patient, as most of those reported in the literature 5 8 , presented unspecific respiratory symptoms; the x-ray revealed a large intrathoracic mass, suggesting the need for further imaging studies. It is important to emphasize the role of an early imaging examination (x-ray, ultrasound scan, CT or MRI) to detect as soon as possible the mass, in order to proceed to more specific investigations to elucidate the nature and staging of this malignant tumour. Radiographic findings of pleuropulmonary blastoma are not specific, especially when most of the neoplasm is cystic, resembling the radiographic features of teratoma. In this respect we note that PPB may initially manifest with clinical and radiologie signs and symptoms of pneumothorax 5 and may arise from other dysplastic conditions; as a matter of fact, cystic pulmonary adenomatoid malformation (CPAM) can be associated with PPB, which is also described in association with some congenital dysembriogenic abnormalities as cystic nephroma 3 . The clinical and radiological presentation in our patient showed mediastinal involvement; the mass was not excisable at the first surgical look because the neoplasm involved the pleura and was very large. The histopathologic diagnosis was consistent with type II PPB. The features described usually correlate with a poor prognosis 6 8 . The patient was submitted to intensive multiagent neoadiuvant chemotherapy, which reduced the tumour mass, making the complete surgical resection feasible, and allowing eradication of the malignancy. Such intensive multiagent chemotherapy is in most cases necessary for the reduction and complete excision of the tumor, which represents the most favourable factor for long term survival. In a recent report describing 11 patients 7 , two underwent total excision of the tumour at diagnosis, and were both alive without disease at 23 and 132 months respectively, with no adjuvant chemotherapy administered in the latter; another 3 patients remained disease free, two after macroscopic total resection and polychemotherapy and one after polychemotherapy and delayed complete surgery. The effectiveness of chemotherapy has also been reported by other Authors 8 1\ub0. The choice of the antiblastic agents used in our patients was due to their known effectiveness on mesenchymal and epithelial tumors n. Our patient was not treated with radiotherapy, which has proven to be effective in few patients 1. In conclusion, this case suggests that PPB may be taken in consideration for the differential diagnosis in respiratory distress. According to our experience and to other literature reports, total remission of this condition may be achieved with complete surgical excision (primary or delayed) and intensive chemotherapy. References 1 Manivel JC, Priest JR, Watterson J, Steiner M, Woods WG, Wick MR. Pleuropulmonary blastoma: the so called pulmonary blastoma of childhood. Cancer 1988;62:1516-26. 2 Spencer H. Pulmonary blastoma. J Pathol Bacteriol 1961 ;82:161-5. 3 Priest JR, Watterson J, Woods WG, Brid RI. Pleuropulmonary blastoma: a marker forfamilial disease. J Pediatr 1996;128:220-4. 4 Dehner LP. Watterson J, Priest J. Pleuropulmonaiy blastoma. A unique intrathorac\uecc-pulmonary neoplasm of childhood. In: Askin FB, Langston C, Rosemberg HS, eds. Pulmonary disease: perspectives in pediatrie pathology. Basel: Karger 1995, p. 214-26. 5 Guler E, Kutluk MT, Yalcin B, Cila A, Kale G, Buyukpamukcu M. Pleuropulmonary blastoma in a child presenting with pneumothorax. Tumori 2001;87:340-2. 6 Priest JR, McDermott MB, Bathia S, Watterson J, Manivel JC, Dehner LP. Pleuropulmonary blastoma. A clinic-pathologic study ofSOcases. Cancer 1997;80:146-61. 7 Indolfi P, Casale F, Carli M, Bisogno G, Ninfo V, Cecchetto G, et al. Pleuropulmonary blastoma: management and prognosis of 11 cases. Cancer 2000;89:1396-401. 8 Romeo C, Impellizzeri P, Grosso M, Vitarelli E, Gentile C. Pleu- 124 PLEUROPULMONARY BLASTOMA IN A CHILD ropulmonary blastoma: long-term survival and literature review. Med Pediatr Oncol 1999;33:372-6. Parsons SK, Fishman SJ, Hoorntje LE, Jaramillo D, Marcus KC, Perez-Atayde AR, et al. Aggressive multimodal treatment of pleuropulmonary blastoma. Ann Thorac Surg 2001;72:939-42. Ozkajnak MF, Ortega JA, Laug W, Gilsanz V, Isaacs H Jr. Role of chemotherapy in pediatrie pulmonary blastoma. Med Pediatr Oncol 1990;18:53-6. 12

BCOR Overexpression Is a Highly Sensitive Marker in Round Cell Sarcomas With BCOR Genetic Abnormalities

open10With the advent of next-generation sequencing, an increasing number of novel gene fusions and other abnormalities have emerged recently in the spectrum of EWSR1-negative small blue round cell tumors (SBRCTs). In this regard, a subset of SBRCTs harboring either BCOR gene fusions (BCOR-CCNB3, BCOR-MAML3), BCOR internal tandem duplications (ITD), or YWHAE-NUTM2B share a transcriptional signature including high BCOR mRNA expression, as well as similar histologic features. Furthermore, other tumors such as clear cell sarcoma of kidney (CCSK) and primitive myxoid mesenchymal tumor of infancy also demonstrate BCOR ITDs and high BCOR gene expression. The molecular diagnosis of these various BCOR genetic alterations requires an elaborate methodology including custom BAC fluorescence in situ hybridization (FISH) probes and reverse transcription polymerase chain reaction assays. As these tumors show high level of BCOR overexpression regardless of the genetic mechanism involved, either conventional gene fusion or ITD, we sought to investigate the performance of an anti-BCOR monoclonal antibody clone C-10 (sc-514576) as an immunohistochemical marker for sarcomas with BCOR gene abnormalities. Thus we assessed the BCOR expression in a pathologically and genetically well-characterized cohort of 25 SBRCTs, spanning various BCOR-related fusions and ITDs and YWHAE-NUTM2B fusion. In addition, we included related pathologic entities such as 8 CCSKs and other sarcomas with BCOR gene fusions. As a control group we included 20 SBRCTs with various (non-BCOR) genetic abnormalities, 10 fusion-negative SBRCTs, 74 synovial sarcomas, 29 rhabdomyosarcomas, and other sarcoma types. In addition, we evaluated the same study group for SATB2 immunoreactivity, as these tumors also showed SATB2 mRNA upregulation. All SBRCTs with BCOR-MAML3 and BCOR-CCNB3 fusions, as well as most with BCOR ITD (93%), and all CCSKs showed strong and diffuse nuclear BCOR immunoreactivity. Furthermore, all SBRCTs with YWHAE-NUTM2B also were positive. SATB2 stain was also positive in tumors with YWHAE-NUTM2B, BCOR-MAML3, BCOR ITD (75%), BCOR-CCNB3 (71%), and a subset of CCSKs (33%). In conclusion, BCOR immunohistochemical stain is a highly sensitive marker for SBRCTs and CCSKs with BCOR abnormalities and YWHAE-rearrangements and can be used as a useful diagnostic marker in these various molecular subsets. SATB2 immunoreactivity is also present in the majority of this group of tumors.openKao, Yc; Sung, Ys; Zhang, L; Jungbluth, Aa; Huang, Sc; Argani, P; Agaram, Np; Zin, A; Alaggio, R; Antonescu, CrKao, Yc; Sung, Ys; Zhang, L; Jungbluth, Aa; Huang, Sc; Argani, P; Agaram, Np; Zin, A; Alaggio, Rita; Antonescu, C