Multidisciplinary Therapeutic Approach Research Articles

P3.26: Complications of the aortic conduit after intestinal transplantation with liver containing grafts - A single center experience

Introduction: Complications originating from the aortic conduit after intestinal transplantation (ITx) are currently not well described in the literature. The aim of this study was therefore to report a single center experience with diagnosis and management of complications affecting the donor aortic conduit after ITx with liver containing grafts. Methods: From 1998 to 2018, 35 ITx were performed in Gothenburg, Sweden. 29 of 35 grafts were liver containing intestinal allografts supplied by an aortic conduit (figure 1). The recipients were 24 adults (median age 38 years, range 16 – 66 years) and 5 children (median age 8 years, range 3– 10 years). The aortic conduit was anastomosed to either the aorta (n=20) or the iliac artery (n=9). The most common underlying conditions were intestinal failure (n=18) and non-resectable neuroendocrine pancreatic malignancies with liver metastases (n=6). Immunosuppression consisted primarily of ATG induction, tacrolimus and steroid bolus and tapering. Median donor age was 23 years (range 2–56 years) and median cold ischemic time was 443 min (range 90 - 873 min). Results: Four patients (4/29, 14%) presented with some type of complication originating from the aortic conduit requiring intervention (figure 2). The complications were: stenosis at the origin of the superior mesenteric artery (SMA) branch (n=1), acute bleeding caused by bacterial and fungal arteritis of the conduit (n=1), stenosis of the conduit caused by kinking of the conduit close to the aortic anastomosis (n=1), inflammatory aneurysm of the donor conduit, affecting the origins of the coeliac trunk and the SMA (n=1). The complications were diagnosed from months up to several years after ITx. Discussion In our experience, complications affecting the donor aortic conduit after ITx may present in the early or late post-transplantation period. The insidious clinical presentation and the relatively high prevalence (14%) may warrant specific post-transplantation surveillance of the donor aortic conduit. A timely multidisciplinary therapeutic approach involving interventional radiology was crucial. Figure 1. Donor aortic conduit anastomosed to the recipient aorta Figure 2. Complications affecting the donor conduit of 29 ITx with liver containing grafts

Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form.

Antiphospholipid syndrome (APS) is a multisystem autoimmune disease most commonly associated with recurrent arterial and venous thromboembolism and recurrent fetal loss. Other possible antiphospholipid antibody (aPL)-related clinical manifestations include cardiac involvement. The heart can be involved through immune mediated and /or thrombotic mechanisms. Mortality due to cardiovascular problems is elevated in APS. However, the cardiovascular risk in patients with primary APS (PAPS) compared with lupus-related APS is yet to be established. Cardiac symptoms of APS include valve abnormalities (thickening and vegetations), coronary artery disease (CAD), myocardial dysfunction, pulmonary hypertension, and intracardiac thrombi. Heart valve lesions are the most common cardiac manifestation, observed in approximately one third of PAPS patients and usually do not cause hemodynamic significance. Deposits of immunoglobulins including anticardiolipin (aCL), and of complement components, are commonly observed in affected heart valves from these patients. This suggests that an inflammatory process is initiated by aPL deposition, eventually resulting in the formation of valvular lesion. aPL may have a direct role in the atherosclerotic process via induction of endothelial activation. Multiple traditional and autoimmune-inflammatory risk factors are involved in triggering an expedited atherosclerotic arterial disease evident in APS. It is imperative to increase the efforts in early diagnosis, control of risk factors and close follow-up, in the attempt to minimize cardiovascular risk in APS. Clinicians should bear in mind that a multidisciplinary therapeutic approach is of paramount importance in these patients. This article reviews the cardiac detriments of APS, including treatment recommendations for each cardiac complication.

Current and Emerging Therapeutic Options for the Management of Rare Skeletal Diseases.

Increasing knowledge in the field of rare diseases has led to new therapeutic approaches in the last decade. Treatment strategies have been developed after elucidation of the underlying genetic alterations and pathophysiology of certain diseases (e.g., in osteogenesis imperfecta, achondroplasia, hypophosphatemic rickets, hypophosphatasia and fibrodysplasia ossificans progressiva). Most of the drugs developed are specifically designed agents interacting with the disease-specific cascade of enzymes and proteins involved. While some are approved (asfotase alfa, burosumab), others are currently being investigated in phase III trials (denosumab, vosoritide, palovarotene). To offer a multi-disciplinary therapeutic approach, it is recommended that patients with rare skeletal disorders are treated and monitored in highly specialized centers. This guarantees the greatest safety for the individual patient and offers the possibility of collecting data to further improve treatment strategies for these rare conditions. Additionally, new therapeutic options could be achieved through increased awareness, not only in the field of pediatrics but also in prenatal and obstetric specialties. Presenting new therapeutic options might influence families in their decision of whether or not to terminate a pregnancy with a child with a skeletal disease.

Pschychosocial support for distressed parents of Pediatric cancer patients

Survival rates for childhood cancer diagnosed before the age of 18 years have improved due to aggressive multidisciplinary therapeutic approaches. Parents have been found to be psychologically affected by their child’s diagnosis, treatment, side-effects of the treatment and child’s health status. Though parental emotional difficulties, such as depression and anxiety, have been reported to decrease after cancer treatment, parents continue to experience chronic post-traumatic stress (PTS) for a long time after their child’s treatment. Parents may also report persistent feelings of loss, uncertainty, and anxiety about the recurrence of the disease or the emergence of late effects in their child. Such illness-specific distress of the parents should be assessed in order to fully understand their psychosocial functioning. Sensitizing health care professionals to the identification of this factor and its associated difficulties is essential for the implementation of efficient intervention strategies. General health of parents plays an important role in continuation of care for the sick child and the treatment follow up. Parental distress and PTS are associated with the emotional and behavioral functioning of children with cancer. There is a genuine need to offer programs of psychological attention to parents of children with cancer in order to reduce their emotional and cognitive psychopathology and to help them adopt adequate coping strategies.

Competence in pulmonary endoscopy emergencies.

In clinical practice, interventional pulmonologists face several situations which can lead to dramatic consequences especially regarding ventilation and require immediate intervention. We describe the main pathological conditions where an urgent bronchoscopy is crucial because they act through mechanisms such as airway obstructions or alteration of the anatomic integrity of the tracheobronchial tree. We point out the problems resulting from inhalation of foreign bodies, one of the most dramatic respiratory emergencies typical in childhood which needs not only the appropriate endoscopic equipment suitable for the age, but also great experience in the management of the possible related complications. Massive hemoptysis is then discussed in order to help to choose the right endoscope and to clarify the steps requested to face this dramatic event. Lastly, iatrogenic tracheal injuries are described, in spite of their low occurrence. The correct endoscopic assessment of the lesions enables to select the proper multidisciplinary therapeutic approach together with surgeons and anesthetists. Due to their peculiarities, emergencies do not allow classic training so it is difficult to estimate the procedure volume necessary to achieve an adequate endoscopic experience. We think, in this field, it is advisable to refer to numbers proposed for elections endoscopic procedures. For these reasons, we consider desirable the use of simulators and clinic case discussions during interventional pulmonologist's training.

Multidisciplinary Treatment of Pediatric Low-Grade Glioma: Experience of Children Cancer Hospital of Egypt; 2007-2012

Abstract Background: Pediatric gliomas comprise a clinically, histologically, and molecularly heterogeneous group of central nervous system tumors. The survival of children with gliomas influenced by histologic subtype, age, and extent of resection. Tumor grade emerged as the most determinant of survival except in the young age groups. The aim of this study was to evaluate the role of multidisciplinary therapeutic approach including surgery and chemotherapy, and their impact on the outcome in pediatric patients with low-grade glioma (LGG). Procedure: Study patients were prospectively enrolled onto the study. All patients were below 18-year-old, diagnosed as LGG between July 2007 and June 2012. Upfront surgical resection was attempted in all tumors other than optic pathway sites. Systemic chemotherapy was given according to CCG-A9952 protocol. Results: Total/near-total resection in 105/227 (46.3%) without adjuvant treatment, while 49/227 patients (21.5%) underwent subtotal tumor resection followed by chemotherapy for big residual (n = 26). Follow-up only was indicated for asymptomatic/small residual (n = 23). The radiological diagnosis was set in 18/227 (7.9%) patients; 13/18 had optic pathway glioma. The 3-year overall survival (OS) was 87.3% versus 65.5% event free survival (EFS) for the whole study patients with a follow-up period of 1–5 years. The OS and EFS for patients who did surgery with no adjuvant treatment (n = 128) were, respectively, 95.2% and 77.3% versus 87.4% and 65.1% for adjuvant chemotherapy group (n = 99); (P = 0.015 and P = 0.016 for OS and EFS, respectively). Conclusion: Pediatric LGGs comprise a wide spectrum of pathological and anatomical entities that carry a high rate of prolonged survival among children and adolescents. Surgical resection is the mainstay of treatment in most of tumors. Combined chemotherapy can be an acceptable alternative when surgery is not safely feasible.

Improved survival in AYAs with NRSTS treated at specialized cancer centers in California.

e23551 Background: Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) are a heterogeneous group of soft tissue tumors whose management benefits from a multidisciplinary therapeutic approach, especially for patients with metastatic or unresectable disease. Published data suggest that cancer treatment at a specialized cancer center (SCC) can mitigate poor outcomes for other cancers. Therefore, we examined the impact of location of treatment, sociodemographic and clinical factors on survival in adolescents and young adults (AYAs) with NRSTS. Methods: We performed a retrospective, population-based analysis of patients (ages 15-39) hospitalized within one year of diagnosis with first primary NRSTS between 2000–2014 using the California Cancer Registry linked with state hospitalization data. Patients were divided into two groups based on receiving all inpatient treatment at a SCC (Children’s Oncology Group and/or National Cancer Institute-designated center) versus part/none. Multivariable Cox proportional hazards regression identified factors associated with overall and sarcoma-specific survival. Kaplan Meier survival analysis was stratified by location of treatment. Results are presented as adjusted hazard ratios (HR) and 95% confidence intervals (CI). Results: Of the 1532 AYA patients with NRSTS, 40.4% percent received all their inpatient care at a SCC. Kaplan Meier analysis demonstrated a higher 5-year survival in patients who received all (vs part/none) care at a SCC (60.0% vs 51.8%; p = 0.0002). Multivariable analysis found that having all treatment at a SCC was associated with significantly better overall (HR 0.81, CI 0.68-0.96) and sarcoma-specific (HR 0.73, CI 0.58-0.92) survival. In addition, inferior survival was associated with African American race/ethnicity, nerve sheath tumor, tumor size > 5 cm, non-localized disease, comorbidities and body wall/visceral tumor location (vs extremity). Conclusions: Our findings demonstrate that treatment for NRSTS at a SCC independently predicts better survival in AYAs and confirms other known predictors of outcomes in this patient population. Eliminating barriers to treatment of AYAs with NRSTS at SCCs could improve survival.

CD74 regulates complexity of tumor cell HLA class II peptidome in brain metastasis and is a positive prognostic marker for patient survival

Despite multidisciplinary local and systemic therapeutic approaches, the prognosis for most patients with brain metastases is still dismal. The role of adaptive and innate anti-tumor response including the Human Leukocyte Antigen (HLA) machinery of antigen presentation is still unclear. We present data on the HLA class II-chaperone molecule CD74 in brain metastases and its impact on the HLA peptidome complexity.We analyzed CD74 and HLA class II expression on tumor cells in a subset of 236 human brain metastases, primary tumors and peripheral metastases of different entities in association with clinical data including overall survival. Additionally, we assessed whole DNA methylome profiles including CD74 promoter methylation and differential methylation in 21 brain metastases. We analyzed the effects of a siRNA mediated CD74 knockdown on HLA-expression and HLA peptidome composition in a brain metastatic melanoma cell line.We observed that CD74 expression on tumor cells is a strong positive prognostic marker in brain metastasis patients and positively associated with tumor-infiltrating T-lymphocytes (TILs). Whole DNA methylome analysis suggested that CD74 tumor cell expression might be regulated epigenetically via CD74 promoter methylation. CD74high and TILhigh tumors displayed a differential DNA methylation pattern with highest enrichment scores for antigen processing and presentation. Furthermore, CD74 knockdown in vitro lead to a reduction of HLA class II peptidome complexity, while HLA class I peptidome remained unaffected.In summary, our results demonstrate that a functional HLA class II processing machinery in brain metastatic tumor cells, reflected by a high expression of CD74 and a complex tumor cell HLA peptidome, seems to be crucial for better patient prognosis.

Food allergy: Children's symptom levels are associated with mothers' psycho-socio-economic variables

BackgroundAllergies affect children's health as well as their quality of life, stress levels, and family budget. The available literature suggests that family, social and psychological factors are affected by allergic pathologies such as rhinitis, asthma and atopic dermatitis. However, few studies have focused on quantifying such association in child food allergy. This study aims to enhance the understanding of the associations between caregiver variables and children's Food Allergy (FA). MethodsThe study involved 206 participants: 103 mothers plus 103 children with IgE mediated FA. The analyses excluded two outliers comprising 101 subjects. For statistical analyses, each dyad –mother/child- was considered to be one subject unity. A between-subjects one-way ANOVA determined the association of children's cutaneous, gastric and respiratory symptoms with anxiety, depression, perceived social support and socioeconomic factors in the mothers. ResultsThere are significant associations between children's allergic symptoms (gastric and cutaneous) and mothers' psychological state (anxiety and depression); family budget; social interactions (with friends, family and partner); understanding of health care required by their child; and sleep disorders. Respiratory symptoms did not show any significant associations with the dependent variables. ConclusionFA is a process in which children's symptoms are significantly associated with socioeconomic and psychological variables of the mothers. The presence or absence of some specific symptoms is directly associated with specific impacts on the mothers. An understanding of such dynamics supports the consideration of a comprehensive and multidisciplinary therapeutic approach to offer more ecological healthcare for “families living with FA.”

Cáncer de próstata y cáncer de testículo

Testicular cancerThis is the most common tumour between the ages of 15 and 35 years. There is a high cure rate; therefore, a prompt, multidisciplinary therapeutic approach is essential. In patients with advanced disease, treatment is based on established prognostic factors that determine life expectancy and first-line therapy. However, standard treatment has not been defined for patients in whom the disease reappears or progresses after first-line treatment. New lines of research have been opened thanks to knowledge of this tumour's molecular biology. Prostate cancerThis is the most common tumour in males. The great majority are diagnosed at local stage, in part due to PSA screening, making it possible to establish radical treatment with curative intent. However, this is a very heterogeneous disease which forces us to stratify patients into prognostic groups and thus orient treatment in the most appropriate manner. In recent years major developments have taken place to combat advanced disease, in that several drugs have been approved with different therapeutic strategies to tackle the disease.

Bone marrow response as a potential biomarker of outcomes in glioblastoma patients.

OBJECTIVE Glioblastoma (GBM) is a highly aggressive malignancy that requires a multidisciplinary therapeutic approach of surgery, chemotherapy, and radiation therapy, but therapy is frequently limited by side effects. The most common adverse effect of chemotherapy with temozolomide (TMZ) is myelosuppression. It remains unclear whether the degree of bone-marrow suppression might serve as a biomarker for treatment outcome. The aim of the current study was to investigate whether the degree of bone-marrow toxicity in patients treated with TMZ correlates with overall survival (OS) and MRI-based time to progression (progression-free survival [PFS]). METHODS Complete blood counts and clinical and imaging information were collected retrospectively from 86 cases involving GBM patients who had completed both radiation therapy and at least 6 monthly cycles of chemotherapy with TMZ. RESULTS Using a multivariate Cox proportional hazard model, it was observed that MGMT promoter methylation, wild-type EGFR, younger patient age at diagnosis, and treatment-induced decreases in white blood cell counts were associated with improved OS. The 2-year survival rate was 25% and 58% for patients with increases and decreases, respectively, in white blood cell counts from baseline over 6 months of TMZ treatment. Consistent with the literature, IDH mutation and MGMT promoter methylation were associated with better PFS and OS. IDH mutation and MGMT promoter methylation were not correlated with changes in peripheral red blood cell or white blood cell counts. CONCLUSIONS Decreases in white blood cell counts might serve as a potential biomarker for OS and PFS in malignant glioma patients treated with radiation therapy and TMZ. It remains unclear whether treatment-induced changes in white blood cell counts correlate with drug-induced antitumor activity or represent an independent factor of the altered local and systemic tumor environment. Additional studies will be needed to determine dose dependence for chemotherapy based upon peripheral blood counts.

Central nervous system involvement in breast cancer patients: Is the therapeutic landscape changing too slowly?

Central nervous system (CNS) involvement from breast cancer (BC) has been historically considered a relatively rare event. However, the development of new therapeutic strategies with a better control of extra-cranial disease and a longer overall survival (OS) has determined an increased incidence of brain metastases. Patients with HER2-positive or triple negative BC have higher occurrence of CNS involvement than patients with luminal-like disease. Moreover, after development of brain metastases, the prognosis is highly influenced by biological subtype. In patients with multiple brain metastases who experience important neurological symptoms, palliative treatment, with or without whole brain radiation therapy (WBRT), needs to be considered the first step of a multidisciplinary therapeutic approach. Patients with a good performance status and 1-3 brain lesions should be considered for radical surgery; patients technically inoperable with 4-5 metastases smaller than 3cm may undergo stereotactic radiosurgery. The role of systemic therapy in the management of patients with brain metastases is controversial. Preliminary data suggest that systemic therapy after WBRT may improve survival in BC patients with brain lesions. In patients with HER2-positive disease, several retrospective or post hoc analyses showed a longer brain progression-free survival with trastuzumab in combination with or followed by other anti-HER2 drugs (such as pertuzumab, lapatinib, and T-DM1). Until now, no new strategies or drugs are available for triple-negative and luminal-like BC.

Recurrent parotid swelling secondary to masseter muscle hypertrophy: a multidisciplinary diagnostic and therapeutic approach

Purpose: To present a patient with an atypical recurrent parotid swelling due to masseter muscle hypertrophy and the diagnostic/therapeutic assessment to treat this condition.Case Report: A patient referring recurrent right facial swelling underwent a complete multidisciplinary assessment of the parotid region that revealed masseter muscle hypertrophy, confirmed by means of clinical (otolaryngological and gnathological evaluation), radiological (utrasonography, dynamic magnetic resonance imaging, and cone beam computed tomography), instrumental (electromyography to evaluate the right masseter muscle function and kinesiography to evaluate maximum right deflection – MaxRDefl and maximum opening – MaxMO) and sialendoscopy assessment where T0 indicates the pre-treatment values. All electromyographic and kinesiographic parameters were evaluated six months after the orthodontic application of a neuromuscular orthosis at T1. At T1, the effectiveness of the orthodontic therapy was demonstrated by the complete resolution of symptoms, and instrumental results documented more efficient muscle activity at rest and during clenching and a better mandibular position. At EMG T1, the resting and post-TENS values were, respectively, 1.2 and 1.8 microV. At kinesiography, MaxRDefl increased from 10.2 (T0) to 10.5 mm (T1); maxMO increased from 41.2 (T0) to 48 mm (T1).Conclusion: The proposed multidisciplinary assessment based on otolaryngological, gnathological, and radiological evaluation may be useful in the case of recurrent parotid swelling secondary to masseter muscle hypertrophy to plan an appropriate management with a removable neuromuscular orthosis.

Abstract P2-12-04: Is there any benefit to perform extensive nodal dissection in primary or recurrent aggressive form of breast cancer?

Abstract Background: Breast oncologic surgery and especially nodal surgery has become ever more minimally invasive. However, some aggressive breast cancers exhibit at their primary or recurrent presentation extensive nodal invasion at the axillary, retropectoralis and sometimes supraclavicular and cervical levels. Surgical treatment of these tumors is not standardized. Material and methods: Between January 2012 and April 2015, 7 primary breast cancer patients (group I) and 7 recurrent breast cancer patients (group II) were included in a prospective, non randomized study approved by our local ethics committee. All the patients had cytologically proven retropectoralis and infraclavicular lymph node invasion and 7 of them had cytologically proven cervical lymph node invasion (5 in the group of primary tumors and 2 in the group of recurrences). Four of the 7 primary tumors were triple negative and 3 were HER2 positive tumors, while 4 out of the 7 recurrent tumors were triple negative and 3were HER2 positive. All the patients underwent PET/CT and breast MRI at baseline. Visceral metastases were absent in all cases. In the group of primary tumors, all the patients were treated with neoadjuvant chemotherapy (plus trastuzumab for the 3 HER2 positive tumors); in the group of recurrent tumors, neoadjuvant chemotherapy associated with trastuzumab was administered in 3 patients, while the 4 other patients underwent complementary mastectomy plus extensive nodal surgery followed by chemotherapy. Radiotherapy was administered in all primary breast cancer patients and cervical radiotherapy was administered in 3 of the 7 recurrent diseases. The following parameters were assessed: disease-free survival, overall survival and adverse events of surgical treatment. Results: All the patients are still alive after a relatively short mean duration of follow-up [24 months in group I (6 to 40 months) and 29 months in group II (3 to 39 months)]. Six of the 7 patients in group I have no signs of recurrence, one has metastatic evolution (bilateral cervical and mediastinal node evolution) and is currently receiving chemotherapy in combination with a PARP inhibitor. In group II, 6 of the 7 patients have no signs of recurrence and one has metastatic evolution (inguinal nodes and bone metastases), treated with chemotherapy and HER2-targeted therapy. The major adverse event is arm lymphedema, affecting 4 out of 14 patients (28%). No persistent pain nor motor troubles are noted. Discussion: Patients with nodal metastases outside the axilla seem to benefit from extensive surgery integrated in a multidisciplinary therapeutic approach. Some studies have demonstrated survival benefits for patients undergoing surgical resection of these nodes. Conclusion: In aggressive breast tumors (HER2 positive or triple negative tumors) presenting with extensive nodal invasion, surgical excision of these nodal metastases must be integrated in the multidisciplinary treatment and patients need to be followed prospectively for a long time to confirm survival benefits. Citation Format: Berliere M, Duhoux F, Nardai P, Schmitz S, Taburiaux L, Galant C, Leconte I, Piette P, Lengele B. Is there any benefit to perform extensive nodal dissection in primary or recurrent aggressive form of breast cancer?. [abstract]. In: Proceedings of the Thirty-Eighth Annual CTRC-AACR San Antonio Breast Cancer Symposium: 2015 Dec 8-12; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2016;76(4 Suppl):Abstract nr P2-12-04.

Descending necrotizing mediastinitis: Systematic review on its treatment in the last 6 years, 75 years after its description

ABSTRACTBackgroundDescending necrotizing mediastinitis is a serious infection involving the neck and the chest, in which an odontogenic, pharyngeal, or cervical infection spreads rapidly to the thoracic cavity, with a high death rate by sepsis and organic failure if not treated quickly and properly.MethodsA systematic search in the electronic database PubMed was conducted using the keywords “mediastinitis” and “descending necrotizing mediastinitis” resulting in 2560 items, filters were activated (systematic review, meta‐analysis, and clinical trial) resulting in 60 articles, from which we selected relevant articles on the topic.ResultsThe best available evidence we could obtain was from 26 case series with evidence level III. The overall mortality in this period was 17.5%.ConclusionFor mediastinitis limited to the upper part of the mediastinum, transcervical drainage may be sufficient; cases that extended below the tracheal carina may require cervical and transthoracic drainage. A multidisciplinary therapeutic approach has allowed a reduction in its mortality. © 2016 Wiley Periodicals, Inc. Head Neck 38: E2275–E2283, 2016

Imaging Features, Differential Diagnosis and Management of Leiomyosarcomas: Case Series and Review of the Literature

Leiomyosarcoma (LMS) is a relatively rare malignant tumor showing smooth-muscle differentiation and accounting for 7-10% of all soft tissue tumors (STTs). LMS occurs most commonly in retroperitoneum and extremities but can potentially involve every site of the body. Diagnosis is finally provided by a histological examination; nevertheless multiplanar imaging can suggest a radiological diagnosis of soft tissue sarcoma prior to biopsy and allow a precise assessment of primary tumor extent and systemic spreading. Computerized tomography (CT) is often the first imaging modality assessment especially for abdominopelvic LMSs and also the cornerstone of staging. CT usually shows a large, heterogeneous and unspecific mass with central areas of hemorrhage or necrosis and peripheral contrast enhancement. Magnetic resonance imaging (MRI) findings are not specific and show a nonfatty mass iso-intense to skeletal muscle on T1-weighted images and high-signal in T2-weighted images with a decreasing rim-to-center pattern of enhancement after gadolinium administration. Imaging also helps in differential diagnosis that mainly concern other STT, gastrointestinal stromal tumors (GISTs), primitive neuroendocrine tumors (PNETs), and lymphomas. Prognosis of LMS is poor and patients should be referred to hospitals with extensive experience in managing sarcomas using multidisciplinary therapeutic approach including surgery, chemotherapy and radiotherapy. The aim of this review is to underline the most important radiological features that could indicate a diagnosis of LMS and in particular to draw the attention to LMSs of the limbs as one of the most frequent location even if often overlooked in literature.

Primer uspešnega timskega pristopa zdravljenja izzvane vulvodinije

AbstractBackground: Vulvodynia has been defined as vulvar discomfort, most often described as burning pain, occurring in the absence of relevant findings or a specific, clinically identifiable, neurologic disorder. Vulvodynia is a genital syndrome of multi-causal origin and requires a team approach. The purpose of the case report of provoked vulvodynia is to show the efficacy of individualized, multifaceted and multidisciplinary therapeutic team approach used by a gynaecologist with special knowledge of sexology and a physiotherapist specialising in treatment of pelvic floor dysfunction. Case report: A 35-year old patient presented with a complaint of genital itching and consequently burning pain which first occurred during sexual intercourse one year previously. Afterwards a gynaecologist with special knowledge of sexology performed a biopsychosocial assessment, broader gynaecological examination and psychoeducation. Diagnosed was provoked vulvodynia. Than she was assessed by the physiotherapist specialising in treatment of pelvic floor dysfunction and treated with TENS. To assess the effectiveness of treatment were used Female Sexual Function Index questionnaire and the visual analogue scale before and after the team approach treatment. Conclusions: In our case individualized, multifaceted and multidisciplinary therapeutic approach proved to be a good choice for treating genital syndrome of provoked vulvodynia of multi-causal origin.

Therapeutic interventions in parkinsonism: Corticobasal degeneration

Corticobasal degeneration (CBD) is a progressive neurodegenerative disorder resulting from pathological accumulation of tau protein and is included in the spectrum of Atypical Parkinsonism. The typical clinical phenotype of CBD is characterized by the Corticobasal syndrome (CBS). In recent years it has become clear that the clinical picture of CBS may be caused by different pathological conditions, resulting in frequent misdiagnosis.CBD has high morbidity and poor prognosis with no effective therapies. In this review, we will discuss the symptomatic treatment, the palliative care and the disease modifying strategies currently in use. Symptomatic treatment in patients with CBD may sometimes be useful for improving motor (parkinsonism, dystonia and myoclonus) and non-motor (cognitive-behavioral) symptoms, but the effects are often unsatisfactory. In addition, non-pharmacological strategies and palliative care are useful integrating components of the multidisciplinary therapeutic approach for patients with CBD. Despite many efforts, a disease-modifying treatment is still unavailable for CBD.

Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options.

Duchenne muscular dystrophy (DMD) is an X-linked myopathy resulting in progressive weakness and wasting of all the striated muscles including the respiratory muscles. The consequences are loss of ambulation before teen ages, cardiac involvement and breathing difficulties, the main cause of death. A cure for DMD is not currently available. In the last decades the survival of patients with DMD has improved because the natural history of the disease can be changed thanks to a more comprehensive therapeutic approach. This comprises interventions targeted to the manifestations and complications of the disease, particularly in the respiratory care. These include: 1) pharmacological intervention, namely corticosteroids and idebenone that significantly reduce the decline of spirometric parameters; 2) rehabilitative intervention, namely lung volume recruitment techniques that help prevent atelectasis and slows the rate of decline of pulmonary function; 3) scoliosis treatment, namely steroid therapy that is used to reduce muscle inflammation/degeneration and prolong ambulation in order to delay the onset of scoliosis, being an additional contribution to the restrictive lung pattern; 4) cough assisted devices that improve airway clearance thus reducing the risk of pulmonary infections; and 5) non-invasive mechanical ventilation that is essential to treat nocturnal hypoventilation, sleep disordered breathing, and ultimately respiratory failure. Without any intervention death occurs within the first 2 decades, however, thanks to this multidisciplinary therapeutic approach life expectancy of a newborn with DMD nowadays can be significantly prolonged up to his fourth decade. This review is aimed at providing state-of-the-art methods and techniques for the assessment and management of respiratory function in DMD patients.

Corticobasal degeneration: clinical characteristics and multidisciplinary therapeutic approach in 26 patients.

Corticobasal syndrome (CBS) is a sporadic tauopathy that manifests by a various combination of motor and cognitive deficits, which makes its diagnosis challenging. Treatment of CBS is symptomatic and based on evidence from other similar disorders due to the lack of studies on CBS. The aim of the study was to investigate low-frequency repetitive transcranial magnetic stimulation (rTMS) as a therapeutic tool in CBS. Twenty-six patients with clinically evident CBS according to Cambridge criteria were followed for 12-18 months while receiving low-frequency rTMS combined with pharmacological, rehabilitation treatment and botulinum toxin injection. The majority of patients are manifested with akinetic-rigid syndrome and cognitive dysfunction. There was improvement of the UPDRS and quality of life after 3 months of therapeutic interventions (P < 0.001 and <0.05, respectively). No significant deterioration in cognitive functions was detected over the study period. There was a significant reduction of caregiver burden after 3 months of interventions (P < 0.01); this improvement was maintained up to 18 months. Cognitive dysfunction is a frequent manifestation of CBS. CBS patients can benefit from multidisciplinary therapeutic approach employing low-frequency rTMS.