Multiple non-ossifying fibromas as a cause of pathological femoral fracture in Jaffe-Campanacci syndrome.

BACKGROUND: Jaffe-Campanacci is a rare syndrome characterised by the association of café-au-lait spots, axillary freckles, multiple non-ossifying fibromas of the long bones and jaw, as well as some features of type 1 neurofibromatosis. There are less than 30 reported cases, and a genetic profile has not yet been determined. Furthermore, it has not been clarified whether it is a subtype of type 1 neurofibromatosis or a separate syndrome. The risk of pathological fracture is over 50%, due to substantial cortical thinning of the weight-bearing bones. CASE PRESENTATION: A 17-year-old female patient, known for type 1 neurofibromatosis, presented with a low-energy distal femoral fracture due to disseminated large non-ossifying fibromas. Investigations revealed all of the distinctive signs of Jaffe-Campanacci syndrome. Both her distal femurs and proximal tibias exhibited multiple non-ossifying fibromas. The fracture was treated by open reduction and internal plate fixation. Some of the bony lesions were biopsied to confirm the diagnosis. The fracture healed eventless, as did the lesions biopsied or involved in the fracture. The other ones healed after curettage and bone grafting performed at the time of plate removal. CONCLUSION: Jaffe-Campanacci is a rare syndrome having unclear interactions with type 1 neurofibromatosis, which still needs to be characterised genetically. It is associated with a high risk of pathological fracture, due to the presence of multiple large non-ossifying fibromas of the long bones, with an expected normal healing time. Curettage and bone grafting promote healing of the lesions and should be considered to prevent pathological fracture. We agree with other authors that all patients with newly-diagnosed type 1 neurofibromatosis should undergo an osseous screening to detect disseminated non-ossifying fibromas, and evaluate the inherent risk of pathological fracture

The unresolved case of sacral chordoma: from misdiagnosis to challenging surgery and medical therapy resistance.

PURPOSE: A sacral chordoma is a rare, slow-growing, primary bone tumor, arising from embryonic notochordal remnants. Radical surgery is the only hope for cure. The aim of our present study is to analyse our experience with the challenging treatment of this rare tumor, to review current treatment modalities and to assess the outcome based on R status. METHODS: Eight patients were treated in our institution between 2001 and 2011. All patients were discussed by a multidisciplinary tumor board, and an en bloc surgical resection by posterior perineal access only or by combined anterior/posterior accesses was planned based on tumor extension. RESULTS: Seven patients underwent radical surgery, and one was treated by using local cryotherapy alone due to low performance status. Three misdiagnosed patients had primary surgery at another hospital with R1 margins. Reresection margins in our institution were R1 in two and R0 in one, and all three recurred. Four patients were primarily operated on at our institution and had en bloc surgery with R0 resection margins. One had local recurrence after 18 months. The overall morbidity rate was 86% (6/7 patients) and was mostly related to the perineal wound. Overall, 3 out of 7 resected patients were disease-free at a median follow-up of 2.9 years (range, 1.6-8.0 years). CONCLUSION: Our experience confirms the importance of early correct diagnosis and of an R0 resection for a sacral chordoma invading pelvic structures. It is a rare disease that requires a challenging multidisciplinary treatment, which should ideally be performed in a tertiary referral center

Patients' satisfaction with community treatment: a pilot cross-sectional survey adopting multiple perspectives.

ACCESSIBLE SUMMARY: Patients' satisfaction is scarcely studied within the context of community treatment for adolescents. Thus, this study adopts a multiple perspective on patients' satisfaction (including service users as well as staff members). The results highlighted that all informants (patients, foster carers in foster homes and professional caregivers from community treatment teams) perceived the patients to be satisfied, with foster carers reporting the highest patient satisfaction rate. Considering the patient satisfaction rate from multiple perspectives provides complementary understandings. Clinical outcomes and, specifically, a reduction in emotional difficulties were related to patient's satisfaction, but only from the patients' perspective. ABSTRACT: Community treatment (CT) teams in Switzerland provide care to patients who are unable to use regular child and adolescent mental health services (i.e. inpatient and outpatients facilities). No study has considered patients' self-rated satisfaction alongside with staff members' perspectives on patient satisfaction. Thus, adopting a cross-sectional survey design, we collected patients' satisfaction using the Client Satisfaction Questionnaire (CSQ-8), rated by multiple informants (patients, foster carers in foster homes and professional caregivers from CT teams). Professional caregivers assessed clinical outcomes using the Health of the Nation Outcome Scale for Children and Adolescents. The results indicated that all informants were satisfied with the community treatment teams. The satisfaction scores were not correlated across informants; however, the alleviation of emotional symptoms was correlated with patients' satisfaction. This study indicated that the use of a combined approach including the views of service users and professionals gives important complementary information. Finally, in our sample, lower emotional symptoms were linked to enhanced patient satisfaction. This study demonstrated the importance of considering multiple perspectives to obtain the most accurate picture of patients' satisfaction. Second, focusing on the reduction of emotional symptoms might lead to a higher degree of patients' satisfaction

On twisted Fourier analysis and convergence of Fourier series on discrete groups

We study norm convergence and summability of Fourier series in the setting of reduced twisted group $C^*$-algebras of discrete groups. For amenable groups, F{\o}lner nets give the key to Fej\'er summation. We show that Abel-Poisson summation holds for a large class of groups, including e.g. all Coxeter groups and all Gromov hyperbolic groups. As a tool in our presentation, we introduce notions of polynomial and subexponential H-growth for countable groups w.r.t. proper scale functions, usually chosen as length functions. These coincide with the classical notions of growth in the case of amenable groups.Comment: 35 pages; abridged, revised and update

Property (T) and rigidity for actions on Banach spaces

We study property (T) and the fixed point property for actions on $L^p$ and other Banach spaces. We show that property (T) holds when $L^2$ is replaced by $L^p$ (and even a subspace/quotient of $L^p$), and that in fact it is independent of $1\leq p<\infty$. We show that the fixed point property for $L^p$ follows from property (T) when 1. For simple Lie groups and their lattices, we prove that the fixed point property for $L^p$ holds for any $1< p<\infty$ if and only if the rank is at least two. Finally, we obtain a superrigidity result for actions of irreducible lattices in products of general groups on superreflexive Banach spaces.Comment: Many minor improvement

Towards ensemble asteroseismology of the young open clusters Chi Persei and NGC 6910

As a result of the variability survey in Chi Persei and NGC6910, the number of Beta Cep stars that are members of these two open clusters is increased to twenty stars, nine in NGC6910 and eleven in Chi Persei. We compare pulsational properties, in particular the frequency spectra, of Beta Cep stars in both clusters and explain the differences in terms of the global parameters of the clusters. We also indicate that the more complicated pattern of the variability among B type stars in Chi Persei is very likely caused by higher rotational velocities of stars in this cluster. We conclude that the sample of pulsating stars in the two open clusters constitutes a very good starting point for the ensemble asteroseismology of Beta Cep-type stars and maybe also for other B-type pulsators.Comment: 4 pages, Astronomische Nachrichten, HELAS IV Conference, Arecife, Lanzarote, Feb 2010, submitte

Nonlinear spectral calculus and super-expanders

Nonlinear spectral gaps with respect to uniformly convex normed spaces are shown to satisfy a spectral calculus inequality that establishes their decay along Cesaro averages. Nonlinear spectral gaps of graphs are also shown to behave sub-multiplicatively under zigzag products. These results yield a combinatorial construction of super-expanders, i.e., a sequence of 3-regular graphs that does not admit a coarse embedding into any uniformly convex normed space.Comment: Typos fixed based on referee comments. Some of the results of this paper were announced in arXiv:0910.2041. The corresponding parts of arXiv:0910.2041 are subsumed by the current pape

Abscopal effect induced by cryoablation in a 55-year-old patient with metastatic dedifferentiated liposarcoma: a case report.

Metastatic dedifferentiated liposarcoma (DDLPS) is primarily managed with chemotherapy, yet with poor response rate. Locoregional therapies, such as radiotherapy and percutaneous cryoablation, can provide palliation for inoperable metastatic sarcomas. In rare instances, those ablative therapies can elicit an immune-mediated regression of untreated metastases in a process named the abscopal effect. With the growing use of immunotherapy, reports on the abscopal effect have become more frequent during the last decade. A 55-year-old patient with no prior medical history was diagnosed with a stage IV DDLPS. The patient was first treated with induction chemotherapy followed by en bloc resection and adjuvant radiotherapy. After two local relapses treated with chemotherapy, the patient developed a systemic disease progression. While progressing on immunochemotherapy, the patient underwent palliative percutaneous cryoablation. Three months after the procedure, the &lt;sup&gt;18&lt;/sup&gt; fluorodeoxyglucose positron emission tomography/computed tomography ( &lt;sup&gt;18&lt;/sup&gt; FDG PET/CT) showed regression of the distant metastasis alongside the regression of the cryoablated tumor, suggesting an abscopal effect. The occurrence of the abscopal effect after progressive disease suggests that cryoablation triggered a systemic immune response, highlighting the potential of this treatment combination. However, it remains a rare phenomenon, and further research and clinical trials are required to determine optimal treatment sequencing

Occurrence and prognosis of lymph node metastases in patients selected for isolated limb perfusion with soft tissue sarcoma.

&lt;b&gt; &lt;i&gt;Background and Objectives:&lt;/i&gt; &lt;/b&gt; Extensive surgery is often required for advanced soft tissue sarcoma (STS) of the limb. In the 1980s, a new approach was developed: isolated limb perfusion (ILP). This study aimed to assess incidence and impact on patient survival based on lymph node metastasis with systematic radical lymphadenectomy during ILP. &lt;b&gt; &lt;i&gt;Methods:&lt;/i&gt; &lt;/b&gt; Retrospective study of 57 consecutive patients treated by ILP for limb STS with simultaneous radical lymph node dissection in our tertiary referral center between 1992 and 2015. &lt;b&gt; &lt;i&gt;Results:&lt;/i&gt; &lt;/b&gt; Median age was 62 years (19-87) and 30 patients were male (53%). Lymph node involvement was observed in 13 patients (N1, 23%), regarded as metastatic spreading in 4 angiosarcomas, 3 epithelioid sarcomas, 2 leiomyosarcomas, 2 undifferentiated sarcomas and 2 synovial sarcomas. For the N0 patient group, median survival was 73.9 months (CI 95% 41.9-105.9) compared to 15.1 months (CI 95% 7.4-22.6) in case of metastatic lymph node ( &lt;i&gt;p&lt;/i&gt; =0.002). The median disease-free survival was 33,0 months (CI 95% 12,5-53.5) in N0 group and 8.0 months (CI 95% 4.0-11.9) in N1 ( &lt;i&gt;p&lt;/i&gt; =0.006). &lt;b&gt; &lt;i&gt;Conclusions:&lt;/i&gt; &lt;/b&gt; Lymph node metastases of STS patients selected for ILP seemed to have a negative impact on both overall and disease-free survival. Radical lymph node dissection should be included in ILP procedure