Malignant TFE3-rearranged perivascular epithelioid cell neoplasm (PEComa) presenting as a subcutaneous mass.

Perivascular epithelioid cell neoplasms (PEComas) are a group of mesenchymal tumours with concurrent melanocytic and myogenic differentiation. Although many cases are sporadic, PEComas can be associated with tuberous sclerosis. A distinct subset of deep-seated PEComas has been shown to carry TFE3 fusions. To our knowledge, this is the first reported case of primary subcutaneous malignant PEComa with molecular confirmation of TFE3 gene rearrangement.

Therapist Effects and the Impact of Early Therapeutic Alliance on Symptomatic Outcome in Chronic Fatigue Syndrome.

UNLABELLED: Few studies have examined therapist effects and therapeutic alliance (TA) in treatments for chronic fatigue syndrome (CFS). Therapist effects are the differences in outcomes achieved by different therapists. TA is the quality of the bond and level of agreement regarding the goals and tasks of therapy. Prior research suffers the methodological problem that the allocation of therapist was not randomized, meaning therapist effects may be confounded with selection effects. We used data from a randomized controlled treatment trial of 296 people with CFS. The trial compared pragmatic rehabilitation (PR), a nurse led, home based self-help treatment, a counselling-based treatment called supportive listening (SL), with general practitioner treatment as usual. Therapist allocation was randomized. Primary outcome measures, fatigue and physical functioning were assessed blind to treatment allocation. TA was measured in the PR and SL arms. Regression models allowing for interactions were used to examine relationships between (i) therapist and therapeutic alliance, and (ii) therapist and average treatment effect (the difference in mean outcomes between different treatment conditions). We found no therapist effects. We found no relationship between TA and the average treatment effect of a therapist. One therapist formed stronger alliances when delivering PR compared to when delivering SL (effect size 0.76, SE 0.33, 95% CI 0.11 to 1.41). In these therapies for CFS, TA does not influence symptomatic outcome. The lack of significant therapist effects on outcome may result from the trial's rigorous quality control, or random therapist allocation, eliminating selection effects. Further research is needed. TRIAL REGISTRATION: ISRCTN74156610.

Pulp-dentin Regeneration: Current State and Future Prospects.

The goal of regenerative endodontics is to reinstate normal pulp function in necrotic and infected teeth that would result in reestablishment of protective functions, including innate pulp immunity, pulp repair through mineralization, and pulp sensibility. In the unique microenvironment of the dental pulp, the triad of tissue engineering would require infection control, biomaterials, and stem cells. Although revascularization is successful in resolving apical periodontitis, multiple studies suggest that it alone does not support pulp-dentin regeneration. More recently, cell-based approaches in endodontic regeneration based on pulpal mesenchymal stem cells (MSCs) have demonstrated promising results in terms of pulp-dentin regeneration in vivo through autologous transplantation. Although pulpal regeneration requires the cell-based approach, several challenges in clinical translation must be overcome-including aging-associated phenotypic changes in pulpal MSCs, availability of tissue sources, and safety and regulation involved with expansion of MSCs in laboratories. Allotransplantation of MSCs may alleviate some of these obstacles, although the long-term stability of MSCs and efficacy in pulp-dentin regeneration demand further investigation. For an alternative source of MSCs, our laboratory developed induced MSCs (iMSCs) from primary human keratinocytes through epithelial-mesenchymal transition by modulating the epithelial plasticity genes. Initially, we showed that overexpression of ΔNp63α, a major isoform of the p63 gene, led to epithelial-mesenchymal transition and acquisition of stem characteristics. More recently, iMSCs were generated by transient knockdown of all p63 isoforms through siRNA, further simplifying the protocol and resolving the potential safety issues of viral vectors. These cells may be useful for patients who lack tissue sources for endogenous MSCs. Further research will elucidate the level of potency of these iMSCs and assess their transdifferentiation capacities into functional odontoblasts when transplanted into the root canal microenvironment.

Cannabis use and outcome of recent onset psychosis.

PURPOSE: To test the hypothesis that recent onset psychotic patients who use cannabis will have psychotic symptoms that are more severe and more persistent than those who do not use cannabis. SUBJECTS AND METHODS: We carried out a 4-year follow-up study of a cohort of 119 patients with recent onset of psychosis. The patients were divided into four groups according to duration of cannabis use, taking index admission and follow-up as reference points. RESULTS: Those subjects who persisted in the use of cannabis had more positive (but not negative) symptoms and a more continuous illness at follow-up. LIMITATIONS: The main limitations of the study were: the relatively small sample size, and that the excess of male subjects and the presence of cannabis induced psychosis could have a confusing impact on the interpretation of the results. CONCLUSION: It is possible that psychotic patients who use cannabis are at a greater risk of a more continuous illness with more positive symptoms than those who do not.

Endonasal transsphenoidal removal of tuberculum sellae meningiomas: technical note.

OBJECTIVE: Tuberculum sellae meningiomas traditionally have been removed through a transcranial approach. More recently, the sublabial transsphenoidal approach has been used to remove such tumors. Here, we describe use of the direct endonasal transsphenoidal approach for removal of suprasellar meningiomas. METHODS: Three women, aged 32, 34, and 55 years, each sought treatment for visual loss and headaches. In each patient, magnetic resonance imaging (MRI) showed a suprasellar mass causing optic chiasmal and optic nerve compression (average size, 2 x 2 cm). All three patients underwent tumor removal via an endonasal approach with the operating microscope. Suprasellar exposure was facilitated by removal of the posterior planum sphenoidale. Ultrasound was used to help define tumor location before dural opening. The extent of tumor removal was verified with angled endoscopes in all patients, and with intraoperative MRI in one patient. The surgical dural and bony defects were repaired in all patients with abdominal fat, titanium mesh, and 2 to 3 days of cerebrospinal fluid lumbar drainage. Nasal packing was not used. RESULTS: There were no postoperative cerebrospinal fluid leaks or meningitis. One patient required a reoperation 2 weeks after surgery to reduce the size of her fat graft, which was causing optic nerve compression; within 24 hours, her vision rapidly improved. At 3 months after surgery, all three patients had normal vision, no new endocrinopathy, and no residual tumor on MRI. At 10 months after surgery, one patient had a small asymptomatic tumor regrowth seen on MRI. CONCLUSION: The endonasal approach with the operating microscope appears to be an effective minimally invasive method for removing relatively small midline tuberculum sellae meningiomas. Intraoperative ultrasound, the micro-Doppler probe, and angled endoscopes are useful adjuncts for safely and completely removing such tumors. Longer follow-up is needed to monitor for tumor recurrence in these patients.

Cerebral hemispherectomy in pediatric patients with epilepsy: comparison of three techniques by pathological substrate in 115 patients.

OBJECT: Cerebral hemispherectomy for intractable seizures has evolved over the past 50 years, and current operations focus less on brain resection and more on disconnection. In addition, cases involving cortical dysplasia and Rasmussen encephalitis are being identified and surgically treated in younger individuals. Few studies have been conducted to compare whether there are perioperative differences based on hemispherectomy technique and/or pathological substrate in pediatric patients with epilepsy. METHODS: In this study the authors compared, stratified by disease, anatomical (37 cases) and Rasmussen functional hemispherectomy (32 cases) with a new modified lateral hemispherotomy (46 cases). Pathological processes included cortical dysplasia (55 cases), Rasmussen encephalitis (21 cases), infarction/ischemia (27 cases), and other/miscellaneous (12 cases). The authors found differences in perioperative clinical factors based on operative technique and/or pathological substrate. In terms of technique, the lateral hemispherotomy was associated with the least intraoperative blood loss, shortest intensive care unit stay, and lowest complication rate. The anatomical hemispherectomy was associated with the longest hospital stay, delayed oral food intake, highest postsurgery fevers, and the highest incidence of shunt requirement. The functional hemispherectomy was associated with the highest reoperation rate for recurrent seizures (25%). In terms of pathology, patients with cortical dysplasia were the youngest at surgery, suffered the greatest amount of blood loss, and required the longest operative/anesthesia times compared with the other pathologically defined groups. Postoperative seizure control (range 0.5-2 years) was not statistically different according to technique or disease process and was similar to that in cases of pediatric temporal lobe epilepsy. CONCLUSIONS: The authors found differences in perioperative risks and hospital course but not postsurgery seizure control, which vary by hemispherectomy technique and/or disease process. The modified lateral hemispherotomy approach offers various advantages related to operative blood loss and reoperation compared with anatomical and functional hemispherectomies that are especially relevant in younger patients with cortical dysplasia and Rasmussen encephalitis with small and/or malformed ventricles.