Relationship between Tumor Diameters and Antoni Classification for Schwannoma of the Lower Lip.

Schwannoma is an uncommon benign tumor in the oral and maxillofacial region, and development of schwannoma in the lower lip is rare. Herein, we present the case of a 68-year-old woman who visited Nihon University Itabashi Hospital complaining of a painless mass in the lower lip. The lesion was surgically resected under local anesthesia. On histopathological examination, the resected specimen was a mixture of Antoni types A and B schwannoma. No recurrence has been seen over a postoperative follow-up period of 58 months. In the schwannoma of the lower lip, the mean tumor volume was compared for type A and the mixed type, which tended to be larger in the mixed type. No previous reports have described the relationship between the size of schwannoma in the lower lip and Antoni classification. Therefore, this report discusses the possibility of a relationship between tumor size and Antoni classification for schwannomas in the lower lip.

Effect of Antibiotic Treatment on Attenuated Salmonella typhimurium VNP20009 Mediated Schwannoma Growth Control.

BACKGROUND/AIM: This study evaluated the effect of enrofloxacin antibiotic treatment on the ability of an attenuated Salmonella typhimurium (S. typhimurium) strain VNP20009 to control schwannoma growth in a preclinical mouse schwannoma tumor model. MATERIALS AND METHODS: The antitumor efficacy of VNP20009 intratumoral (i.t.) injection was assessed in a syngeneic mouse-NF2 schwannoma model, with and without subcutaneous (s.c.) injection of enrofloxacin beginning on day-1 or day-8 post-VNP20009 injection. S. typhimurium colonization was assessed in excised tumor samples following antibiotic treatment. RESULTS: I.t. injection of the VNP20009 significantly decreased the growth of schwannoma tumors in mice compared to PBS-treated controls. Treatment of mice with enrofloxacin on day-1 post-VNP20009 injection resulted in abrogation of VNP20009-mediated tumor growth control. In contrast, tumor growth in i.t. VNP20009-injected mice infused with enrofloxacin beginning on day 8 was significantly decreased compared to i.t. PBS-injected controls. Enrofloxacin significantly reduced the number of viable VNP20009 bacteria in excised tumor samples within one day of antibiotic infusion. Viable bacteria were either few or essentially eliminated at the end of the experiment in antibiotic-treated animals compared to VNP20009-only. CONCLUSION: Viable VNP20009 can persist for as long as 2.5 weeks following intratumoral injection of schwannoma, during which time tumor growth is retarded. Antibiotic treatment starting 1-day following i.t. VNP20009 abrogated bacterial tumor growth control, whereas initiation of antibiotics 8-days following i.t. VNP20009 was associated with control of tumor growth, albeit less than seen in animals unexposed to antibiotics.

Thoracic Schwannoma as an Unusual Cause of Sciatic Pain in the Chiropractic Office: A Case Report.

BACKGROUND Sciatica, which is pain radiating along the sciatic nerve distribution, is most often caused by degenerative conditions of the lower back and rarely results from lesions of the spinal cord. This case report illustrates a patient with suspected lumbar radiculopathy, ultimately diagnosed with a thoracic schwannoma after visiting a chiropractor. CASE REPORT A 61-year-old woman presented to a chiropractor with progressively worsening lower back pain radiating to the right lower extremity and unsteady gait. Her symptoms began 10 years previously and she had been diagnosed by an orthopedist with lumbar disc herniation causing radiculopathy via magnetic resonance imaging (MRI) and was treated with physical therapy and diclofenac. Upon examination by the chiropractor, the patient had bilateral lower-extremity neurologic deficits, hyperreflexia, and upgoing Babinski responses. The chiropractor ordered thoracic MRI, but the patient preferred to initiate a trial of care, which was unsuccessful. After a delay imposed by cardiac bypass surgery, the patient returned to the chiropractor with worsened symptoms and underwent thoracic MRI, revealing an intraspinal mass at T9. The patient underwent laminectomy and tumor removal, with histopathology confirming a diagnosis of schwannoma. The patient gradually improved after surgery with the help of rehabilitative exercises. CONCLUSIONS Clinicians should suspect a spinal cord lesion as a cause of sciatic pain when patients fail to improve with treatment, have a broad sensorimotor distribution unexplained by lumbar imaging findings, hyperreflexia/pathological reflexes, or other atypical findings (eg, negative neural tension tests). In such cases, MRI of the cervical and thoracic spine may be indicated.

Schwannoma de nervio facial con reanimación facial con Neurotizacion Hipoglosofacial / SCHWANNOMA OF THE FACIAL NERVE WITH FACIAL RESUSCITATION WITH FACIAL HYPOGLOSSAL NEUROTIZATION

El schwannoma es una patología rara del nervio facial. Su diagnóstico preoperatorio es dificultoso dado que no tiene síntomas ni signos patognomónico de la enfermedad. La disección del nervio facial en su tronco y sus ramas con electroestimulacion es la forma de quirúrgica de sospecharlo intraoperatoriamente. La descompresión parcial o exeresis completa deberá ser considerado de acuerdo a la experiencia del equipo quirúrgico en reconstrucción nerviosa. La reparación del nervio facial como primera opción debe el injerto inmediato o sutura termino terminal. La neurotización es un procedimiento quirúrgico que le provoca al paciente simetría facial con manejo de oclusión ocular y manejo de comisura bucal, debe ser realizado antes del año de la injuria nerviosa. La rehabilitación del nervio facial necesita de un equipo multidisciplinario y la colaboración permanente del paciente para conseguir los objetivos propuestos.

Schwannoma is a rare pathology of the facial nerve. Its preoperative diagnosis is difficult since it has no symptoms or pathognomonic signs of the disease. The dissection of the facial nerve in its trunk and its branches with electrostimulation is the surgical way to suspect it intraoperatively. Partial decompression or complete exeresis should be considered according to the experience of the surgical team in nerve reconstruction. The repair of the facial nerve as a first option should be the immediate graft or end-to-end suture. Neurotization is a surgical procedure that causes the patient facial symmetry with management of ocular occlusion and management of the corner of the mouth, it must be performed within a year of the nerve injury. The rehabilitation of the facial nerve requires a multidisciplinary team and the permanent collaboration of the patient to achieve the proposed objectives.

Uvulopalatopharyngoplasty for nasal blockage 21 years after total rhinectomy.

BACKGROUND: Total rhinectomy is an invasive procedure that significantly impairs the intranasal turbulence, humidification and heating of inspired air. The use of uvulopalatopharyngoplasty for the treatment of sleep-disordered breathing disorders such as primary snoring and obstructive sleep apnoea has diminished over the past years because of the emergence of less invasive procedures and alternative therapeutic options. This clinical record presents the treatment of a long-term side effect of total rhinectomy using uvulopalatopharyngoplasty. CASE REPORT: In 1997, a 62-year-old male underwent total rhinectomy for a nasal schwannoma, followed by rehabilitation with a nasal prosthesis. Twenty-one years later, he presented with severe complaints of nasal blockage and breathing difficulties during both daytime and night-time. Clinical examination revealed no major anomalies besides significant velopharyngeal narrowing. Thus, in 2019, uvulopalatopharyngoplasty was performed to re-establish velopharyngeal patency. Hereafter, the symptoms of nasal blockage disappeared, resulting in an improved quality of life. CONCLUSION: Uvulopalatopharyngoplasty may prove useful to treat selected patients with daytime breathing difficulties due to velopharyngeal narrowing.

Impact of mind-body treatment interventions on quality of life in neurofibromatosis patients: A systematic review and meta-analysis.

Individuals with neurofibromatosis (NF) experience poorer quality of life (QoL), in part contributed by the clinical manifestations of NF, such as functional disability, chronic pain, and altered physical appearance. Mind-body therapies (MBTs) tailored to NF have been developed, and have demonstrated promising potential to improve QoL in this population. We sought to systematically review current evidence on the effectiveness of MBTs in addressing QoL deficits in NF patients. Databases were reviewed between the date of inception and June 2020, using search terms: neurofibromatosis, schwannomatosis, psychotherapy, mind-body, mindfulness, meditation, resiliency, and behavioral therapy. Quality appraisal was assessed using the Cochrane Risk of Bias Tools and National Institutes of Health Study Quality Assessment Tools. We conducted a meta-analysis of mean differences and reported aggregate effect estimates with 95% confidence intervals. A total of 10 articles, including randomized-controlled trials and pre-post studies, were identified. Meta-analytic results of randomized-controlled trial data from six citations demonstrated MBTs were associated with improved physical (MD = 13.63, 95%CI 6.95-20.30, P < .0001, I2 = 24%), psychological (MD = 14.11, 95%CI 6.44-21.78, P = .0003, I2 = 38%), social (MD = 9.63, 95%CI 2.93-16.33, P = .005, I2 = 0%), and environmental QoL (MD = 14.14, 95%CI 8.28-20.00, P < .00001. I2 = 0%) in NF patients. These associations were maintained at 6-months follow-up for physical, psychological, and environmental QoL (P < .05). Our findings suggest that NF-adapted MBT strategies are associated with improving QoL in diverse NF populations, including NF2 patients experiencing deafness and youth NF patients. Providers and caregivers for NF should be aware of the potential benefits of MBT in chronic NF management.

[A Case of Trochlear Nerve Neurinoma Presenting with Pathological Laughter].

Trochlear nerve neurinomas are rare and solitary tumors without neurofibromatosis are extremely rare. We report a case of trochlear nerve neurinoma presenting with pathological laughter and diplopia. A 40-year-old male patient presented with diplopia and pathological laughter 2 months before admission. MRI showed a multicystic enhanced mass in the left tentorial incisura compressing the midbrain and the upper pons. The tumor was excised using the left trans-Sylvian approach with partial uncal resection. After excision of the tumor, the left trochlear nerve was identified on the surface of the lateral midbrain. The nerve was connected to the tumor. Pathological laughter completely resolved after the operation. This is the second reported case of trochlear nerve neurinoma presenting with pathological laughter. The lesion responsible for pathological laughter could be the midbrain, upper pons, diencephalon, or all of these.

Physiotherapeutic management of a patient with spinal Schwannoma: A case report.

INTRODUCTION: Tumors are generally considered as red flags to manual therapy. The purpose of this report is to describe the clinical course of a patient diagnosed with spinal Schwannoma at L2-L3 level, who was referred to physiotherapy (PT) for the treatment of low back pain radiating to the left lower limb. CASE DESCRIPTION: A 30-year old man previously diagnosed with L2-L3 Schwannoma was referred for physiotherapy for the treatment of radiating pain. The patient had not responded favorably to symptomatic management. As the patient's history and physical examination were consistent with a mechanical dysfunction, it was decided to manage the patient along similar lines. The patient was treated with McKenzie extension and central postero-anterior mobilization over T4-T6 vertebrae. This intervention was followed up with strengthening and aerobic exercises. RESULTS: The outcomes were measured by the numerical rating scale (NRS), Oswestry Disability Index (ODI) and the Global rate of Change (GRC) scale. The patient responded well to McKenzie extension exercises and Thoracic mobilization. The NRS (7 at rest; 10 on activity) at the time of referral reduced to (2 on activity) at the time of discharge. The outcomes were maintained after 7 months. CONCLUSION: This case suggests that there is a need to undertake a detailed musculoskeletal examination and mobilization may be safely performed in patients diagnosed with spinal schwannomas. An individualized tailored approach may be beneficial in these patients.

Repetitive nerve block for neuropathic pain management: a case report.

Schwannoma is a common neoplasm in the peripheral and central nervous systems. Sciatic nerve schwanommas are rare. We report the case of a 50-year-old woman who was referred for treatment of persistent neuropathic pain in the left lower limb after resection of a schwannoma on the left S1 nerve root. The patient's history goes back when she was 27 years old and started to have electric-like pain in her lower left limb upon intercourse. Examination revealed a left ovarian cyst which was surgically removed. Her pain persisted despite taking nonsteroidal anti inflammatory drugs (NSAIDs). Several years later a schwannoma on the left S1 nerve root was detected. The patient had surgical excision of the left S1 nerve root at the plexus along with the schwannoma. Following the surgery, she experienced pain upon sitting and touch, and had a limp in her left leg. She was prescribed NSAIDs, antidepressant and pregabalin. Despite the pharmacological treatment, the patient had persistent mild pain. Upon physical examination, the incision from her previous surgery was 4 cm away from the sacral midline and parallel to S1 and S2. The length of the incision was 3 cm. The patient had severe allodynia upon palpation at the area between S1 and L5 and the visual analog scale (VAS) score increased from 3 to 10. She had severe pain at rest and movement. Her neurologic exam revealed that the left lower extremity motor power showed mild weakness in the leg abduction, foot eversion, plantar and toes flexion, and in the hip extension. The sensory exam showed severe reduction in pinprick and temperature sensation in the lateral aspect of foot, lower leg and dorsolateral thigh and buttocks. Nerve stimulator guided injection was performed at the pain trigger point being 1 cm above the midline of the incision. Upon nerve stimulation the contraction of the gluteal muscle was observed. Then, 20 mL of the anesthetic mixture were injected. The patient had immediate pain relief after the block (VAS 1/10). She remained pain free for 15 days after which pain reappeared but with less severity (3/10). Repetitive sciatic nerve block was performed in a progressive manner and was shown to be effective in managing neuropathic pain.

2016 Children's Tumor Foundation conference on neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis.

Organized and hosted by the Children's Tumor Foundation (CTF), the Neurofibromatosis (NF) conference is the premier annual gathering for clinicians and researchers interested in neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN). The 2016 edition constituted a blend of clinical and basic aspects of NF research that helped in clarifying different advances in the field. The incorporation of next generation sequencing is changing the way genetic diagnostics is performed for NF and related disorders, providing solutions to problems like genetic heterogeneity, overlapping clinical manifestations, or the presence of mosaicism. The transformation from plexiform neurofibroma (PNF) to malignant peripheral nerve sheath tumor (MPNST) is being clarified, along with new management and treatments for benign and premalignant tumors. Promising new cellular and in vivo models for understanding the musculoskeletal abnormalities in NF1, the development of NF2 or SWN associated schwannomas, and clarifying the cells that give rise to NF1-associated optic pathway glioma were presented. The interaction of neurofibromin and SPRED1 was described comprehensively, providing functional insight that will help in the interpretation of pathogenicity of certain missense variants identified in NF1 and Legius syndrome patients. Novel promising imaging techniques are being developed, as well as new integrative and holistic management models for patients that take into account psychological, social, and biological factors. Importantly, new therapeutic approaches for schwannomas, meningiomas, ependymomas, PNF, and MPNST are being pursued. This report highlights the major advances that were presented at the 2016 CTF NF conference.

Towards an optimal paradigm for intraoperative auditory nerve monitoring with auditory steady state responses.

Auditory steady state responses (ASSR) may offer an alternative to brainstem auditory evoked potentials for monitoring of the auditory nerve during surgical procedures. In the current study, we evaluated the influence of noise on ASSR characteristics in total intravenous anesthesia (TIVA). Simulated ASSR in real noise recorded during surgery under TIVA were constructed with known parameters. Influence of amplitude, modulation frequency, averaging sweeps and detection threshold on ASSR were evaluated. High amplitude, more sweeps and a liberal threshold facilitated detection. High amplitude ASSR (80 nV) were detected in up to 45 % with 16 s of data, in 80-90 % with 112 s. Near-threshold ASSR were detected in 0.8-25 %. False positives ranged between 0.3 and 10.3 %. Number of sweeps did not influence false positives. Amplitude errors varied between -61 and +39 % and improved with more averages but not with different thresholds. Modulation rate demonstrated the strongest influence on all parameters. 110 Hz yielded best, 90 Hz the worst results. Choice of parameters strongly influences detection and characteristics of ASSR. Optimal parameters enabled detection after 16 s in 45 %. Due to specific noise characteristics, modulation has a critical impact, which is currently not sufficiently recognized in ASSR studies.

A Case of Upper Eyelid Schwannoma

PURPOSE: To report a rare case of upper eyelid schwannoma presenting as a chalazion. CASE SUMMARY: A 54-year-old male presented to our clinic with a slowly growing, painless recurred mass located in the middle area of the right upper eyelid margin. Surgical incision had been performed on a similar mass two year previous, although no histological analysis had been performed. On examination, a 4 × 3-mm-sized, firm, nonpigmented mass was palpable in the right upper eyelid, and no signs of neurofibromatosis were present elsewhere. The lesion was initially thought to be an eyelid mass, so we performed an excisional biopsy under local anesthesia. The lesion was easily isolated from the surrounding tissue and was excised completely. Histopathologically, the excised mass showed a compact arrangement of spindle cells forming palisades with Verocay bodies (Antoni A patterns). Immunohistochemistry revealed diffuse and strong S-100 protein positivity. These findings resulted in the diagnosis of eyelid schwannoma. CONCLUSIONS: Because of its rarity and solitary feature, eyelid schwannoma can be confused with chalazion. Thus, ophthalmologists should consider schwannoma in the differential diagnosis of a slowly growing, painless recurred mass or a lesion with malignant transformation after incomplete excision.

Photothermal therapy improves the efficacy of a MEK inhibitor in neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors.

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive tumors with low survival rates and the leading cause of death in neurofibromatosis type 1 (NF1) patients under 40 years old. Surgical resection is the standard of care for MPNSTs, but is often incomplete and can generate loss of function, necessitating the development of novel treatment methods for this patient population. Here, we describe a novel combination therapy comprising MEK inhibition and nanoparticle-based photothermal therapy (PTT) for MPNSTs. MEK inhibitors block activity driven by Ras, an oncogene constitutively activated in NF1-associated MPNSTs, while PTT serves as a minimally invasive method to ablate cancer cells. Our rationale for combining these seemingly disparate techniques for MPNSTs is based on several reports demonstrating the efficacy of systemic chemotherapy with local PTT. We combine the MEK inhibitor, PD-0325901 (PD901), with Prussian blue nanoparticles (PBNPs) as PTT agents, to block MEK activity and simultaneously ablate MPNSTs. Our data demonstrate the synergistic effect of combining PD901 with PBNP-based PTT, which converge through the Ras pathway to generate apoptosis, necrosis, and decreased proliferation, thereby mitigating tumor growth and increasing survival of MPNST-bearing animals. Our results suggest the potential of this novel local-systemic combination "nanochemotherapy" for treating patients with MPNSTs.

Preclinical validation of anti-nuclear factor-kappa B therapy to inhibit human vestibular schwannoma growth.

Vestibular schwannomas (VSs), the most common tumors of the cerebellopontine angle, arise from Schwann cells lining the vestibular nerve. Pharmacotherapies against VS are almost non-existent. Although the therapeutic inhibition of inflammatory modulators has been established for other neoplasms, it has not been explored in VS. A bioinformatic network analysis of all genes reported to be differentially expressed in human VS revealed a pro-inflammatory transcription factor nuclear factor-kappa B (NF-κB) as a central molecule in VS pathobiology. Assessed at the transcriptional and translational level, canonical NF-κB complex was aberrantly activated in human VS and derived VS cultures in comparison to control nerves and Schwann cells, respectively. Cultured primary VS cells and VS-derived human cell line HEI-193 were treated with specific NF-κB siRNAs, experimental NF-κB inhibitor BAY11-7082 (BAY11) and clinically relevant NF-κB inhibitor curcumin. Healthy human control Schwann cells from the great auricular nerve were also treated with BAY11 and curcumin to assess toxicity. All three treatments significantly reduced proliferation in primary VS cultures and HEI-193 cells, with siRNA, 5 µM BAY11 and 50 µM curcumin reducing average proliferation (±standard error of mean) to 62.33% ± 10.59%, 14.3 ± 9.7%, and 23.0 ± 20.9% of control primary VS cells, respectively. These treatments also induced substantial cell death. Curcumin, unlike BAY11, also affected primary Schwann cells. This work highlights NF-κB as a key modulator in VS cell proliferation and survival and demonstrates therapeutic efficacy of directly targeting NF-κB in VS.

Potential use of acupuncture in the treatment of first bite syndrome.

OBJECTIVES: First bite syndrome (FBS) is an early postoperative pain syndrome characterized by the sudden onset of pain in the parotid region at the first bite of each meal. The etiology is not certain, hence a standardized therapy does not exist. METHODS: A 50-year old woman referred to us complaining of a swelling in the right parotid region. Fine-needle aspiration biopsy (FNAB) was diagnostic for pleomorphus adenoma of the deep lobe of the parotid gland. A 50-year old man presented with a mass in the right side of the neck, FNAB was diagnostic for parapharyngeal space neurinoma. The first patient was submitted to total parotidectomy with facial nerve preservation, the second to extracapsular dissection of the tumor. A week after surgery both patients developed FBS. A qualitative/quantitative description of pain was obtained by means of a self-coded questionnaire. The score ranges from 8 to 44, corresponding to the lowest and the highest discomfort possible, respectively. Acupuncture was used to treat these 2 patients. The treatment protocol comprised 6 sessions, one per week, lasting 30 minutes each. RESULTS: Our questionnaire was administered before and after treatment and the score dropped from 33 to 25 in the female, from 30 to 15 in the male patient. CONCLUSION: FBS is a complication of upper cervical surgery with a high morbidity rate. We describe the first two cases of FBS that were successfully treated with acupuncture in our ENT department. We believe that this procedure may represent a valid therapeutic alternative in the future.

Contralateral hearing loss after vestibular schwannoma excision: a rare complication of neurotologic surgery.

We report a rare case of contralateral hearing loss after vestibular schwannoma excision in a 48-year-old man who underwent surgery via a suboccipital approach for removal of a nearly 2-cm lesion involving the right cerebellopontine angle. Postoperatively, the patient awoke with bilateral deafness, confirmed by both audiometry and spontaneous otoacoustic emissions. The patient was treated aggressively with high-dose intravenous steroids, vitamins E and C, and oxygen. Over the next several months he had gradual recovery of most of the hearing in his left (unoperated) ear. Contralateral hearing loss may develop after vestibular schwannoma excision; multiple pathophysiologic mechanisms for this occurrence have been proposed.

Magnetic resonance imaging localization with cod liver oil capsules for the minimally invasive approach to small intradural extramedullary tumors of the thoracolumbar spine.

OBJECT: Accurate intraoperative localization of small intradural extramedullary thoracolumbar (T-1 to L-3 level) spinal cord tumors is vital when minimally invasive techniques, such as hemilaminectomy, are used to excise these lesions. In this study, the authors describe a simple and effective method of preoperative MRI localization of small intradural extramedullary tumors using cod liver oil capsules. METHODS: Thirty-five patients with intradural tumors underwent preoperative MRI localization the evening prior to surgery. Patients were positioned prone in the MRI gantry, mimicking the intraoperative position. Nine capsules were placed in 3 rows to cover the lesion. This localization was used to guide the level for a minimally invasive approach using a hemilaminectomy to excise these tumors. RESULTS: The mean patient age was 51.5 ± 14.3 years, and the mean body mass index was 24.1 ± 3.5 kg/m(2). Twenty-two tumors involved the thoracic spine, and 13 involved the upper lumbar spine from L-1 to L-3. The mean tumor size was 2.2 ± 1.0 cm. Localization was accurate in 34 patients (97.1%). CONCLUSIONS: Accurate localization with the described method is quick, safe, cost-effective, and noninvasive with no exposure to radiation. It also reduces operating time by eliminating the need for intraoperative fluoroscopy.

Combinatorial therapy with tamoxifen and trifluoperazine effectively inhibits malignant peripheral nerve sheath tumor growth by targeting complementary signaling cascades.

Chemotherapeutic agents effective against malignant peripheral nerve sheath tumors (MPNSTs) are urgently needed. We recently found that tamoxifen potently impedes xenograft growth. In vitro, tamoxifen inhibits MPNST proliferation and survival in an estrogen receptor-independent manner; these effects are phenocopied by the calmodulin inhibitor trifluoperazine. The present study was performed to establish the mechanism of action of tamoxifen in vivo and optimize its therapeutic effectiveness. To determine if tamoxifen has estrogen receptor-dependent effects in vivo, we grafted MPNST cells in castrated and ovariectomized mice; xenograft growth was unaffected by reductions in sex hormones. To establish whether tamoxifen and trifluoperazine additively or synergistically impede MPNST growth, mice xenografted with neurofibromatosis type 1-associated or sporadic MPNST cells were treated with tamoxifen, trifluoperazine, or both drugs for 30 days. Both monotherapies inhibited graft growth by 50%, whereas combinatorial treatment maximally reduced graft mass by 90% and enhanced decreases in proliferation and survival. Kinomic analyses showed that tamoxifen and trifluoperazine have both shared and distinct targets in MPNSTs. In addition, trifluoperazine prevented tamoxifen-induced increases in serum/glucocorticoid regulated kinase 1, a protein linked to tamoxifen resistance. These findings suggest that combinatorial therapy with tamoxifen and trifluoperazine is effective against MPNSTs because these agents target complementary pathways that are essential for MPNST pathogenesis.

The relaxation response resiliency program (3RP) in patients with neurofibromatosis 1, neurofibromatosis 2, and schwannomatosis: results from a pilot study.

NF1, NF2, and Schwannomatosis are incurable tumor suppressor syndromes associated with poor quality of life. The aim of this study was to determine the feasibility, acceptability, and preliminary efficacy of an NF adapted, 8-week group mind body skills based intervention, the relaxation response resiliency program (3RP) aimed at improving resiliency and increasing satisfaction with life. Patients seen at MGH's Neurofibromatosis Clinic were offered participation if they described difficulties coping to a treating physician. Participants completed measures of life satisfaction, resiliency, stress, mood, lifestyle, pain, post-traumatic growth and mindfulness at baseline and after completing the 3RP program. The intervention had relative feasible enrollment rate (48% rate, 32 out of 67 of patients signing the informed consent form). However, out of the 32 patients who signed the informed consent, only 20 started the study (62.5%) and only 16 completed it (50%), suggesting problems with feasibility. The main reason cited for non-participation was burden of travel to the clinic. The intervention was highly acceptable, as evidenced by an 80% completion rate (16/20). Paired t tests showed significant improvement in resiliency, satisfaction with life, depression, stress, anxiety, mindfulness and post traumatic growth, with effect sizes ranging from 0.73-1.33. There was a trend for significance for improvement in somatization and sleepiness (p = 0.06), with effect sizes of 0.54-0.92 respectively. Statistically nonsignificant improvement was observed in all other measures, with effect sizes small to medium. In sum, the 3RP was found to be relatively feasible, highly acceptable and preliminary efficacious in decreasing symptom burden in this population, supporting the need of a randomized controlled trial.