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.

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.

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 value of ¹8F-FDG PET/CT in the management of malignant peripheral nerve sheath tumors.

PURPOSE: Our objective was to determine how positron emission tomography (PET)/CT had been used in the clinical treatment of malignant peripheral nerve sheath tumor (MPNST) patients at The University of Texas MD Anderson Cancer Center. METHODS: We reviewed a database of MPNST patients referred to MD Anderson Cancer Center during 1995-2011. We enrolled 47 patients who underwent PET/CT imaging. Disease stage was based on conventional imaging and PET/CT findings using National Comprehensive Cancer Network (NCCN) guidelines. Treatment strategies based on PET/CT and conventional imaging were determined by chart review. The maximum and mean standardized uptake values (SUVmax, SUVmean), metabolic tumor volume (MTV), total lesion glycolysis (TLG), change in SUVmax, change in MTV, and change in TLG were calculated from the PET/CT studies before and after treatment. Response prediction was based on imaging studies performed before and after therapy and categorized as positive or negative for residual tumor. Clinical outcome was determined from chart review. RESULTS: PET/CT was performed for staging in 16 patients, for restaging in 29 patients, and for surveillance in 2 patients. Of the patients, 88 % were correctly staged with PET/CT, whereas 75 % were correctly staged with conventional imaging. The sensitivity to detect local recurrence and distant metastasis at restaging was 100 and 100 % for PET/CT compared to 86 and 83 % for conventional imaging, respectively. PET/CT findings resulted in treatment changes in 31 % (5/16) and 14 % (4/29) of patients at staging and restaging, respectively. Recurrence, MTV, and TLG were prognostic factors for survival, whereas SUVmax and SUVmean were not predictive. For 21 patients who had imaging studies performed both before and after treatment, PET/CT was better at predicting outcome (overall survival, progression-free survival) than conventional imaging. A decreasing SUVmax ≥ 30 % and decrease in TLG and MTV were significant predictors for overall and progression-free survival. CONCLUSION: PET/CT is valuable in MPNST management because of its high accuracy in staging and high sensitivity and accuracy in restaging as well as improvements in treatment planning. MTV from baseline staging studies is predictive of survival. Additionally, change in SUVmax, TLG, and MTV accurately predicted outcomes after treatment.

Growth inhibitory and anti-tumour activities of OSU-03012, a novel PDK-1 inhibitor, on vestibular schwannoma and malignant schwannoma cells.

BACKGROUND: Vestibular schwannomas (VS) frequently express high levels of activated AKT. Small-molecule inhibitors of AKT signalling may have therapeutic potential in suppressing the growth of benign VS and malignant schwannomas. METHOD: Primary VS and Schwann cells, human malignant schwannoma HMS-97 cells and mouse Nf2(-/-) Schwann cells and schwannoma cells were prepared to investigate the growth inhibitory and anti-tumour activities of OSU-03012, a celecoxib-derived small-molecule inhibitor of phosphoinositide-dependent kinase-1. Cell proliferation assays, apoptosis, Western blot, in vivo xenograft analysis using SCID mice and immunohistochemistry were performed. RESULTS: OSU-03012 inhibited cell proliferation more effectively in both VS and HMS-97 cells than in normal human Schwann cells. The IC5) of OSU-03012 at 48h was approximately 3.1 microM for VS cells and 2.6 microM for HMS-97 cells, compared with the IC(50) of greater than 12 microM for human Schwann cells. Similarly, mouse Nf2(-/-) schwannoma and Nf2(-/-) Schwann cells were more sensitive to growth inhibition by OSU-03012 than wild-type mouse Schwann cells and mouse schwannoma cells established from transgenic mice carrying the NF2 promoter-driven SV40 T-antigen gene. Like VS cells, malignant schwannoma HMS-97 cells expressed high levels of activated AKT. OSU-03012 induced apoptosis in both VS and HMS-97 cells and caused a marked reduction of AKT phosphorylation at both the Ser-308 and Thr-473 sites in a dose-dependent manner. In vivo xenograft analysis showed that OSU-03012 was well tolerated and inhibited the growth of HMS-97 schwannoma xenografts by 55% after 9 weeks of oral treatment. The anti-tumour activity correlated with reduced AKT phosphorylation. CONCLUSION: OSU-03012 is a potential chemotherapeutic agent for VS and malignant schwannomas.

Solitary olfactory groove schwannoma.

Intracranial schwannomas that do not involve major cranial nerves in the posterior fossa are uncommon, especially if they are not associated with neurofibromatosis type II (NF-2). Subfrontal olfactory groove schwannomas are extremely rare. We report a cystic schwannoma arising from the olfactory groove in a 38-year-old Caucasian male who presented with headache, vomiting and visual impairment. We briefly review the pertinent literature, stress the distinctive clinical and neuroradiological findings and discuss the surgical planning and theories about the pathogenesis of schwannomas in this unusual location.

Ileal schwannoma developing into ileocolic intussusception.

Intussusception is rare in adults. We describe a 47-year-old man with ileal schwannoma that led to ileocolic intussusception. Abdominal ultrasonography, abdominal CT scan and barium enema confirmed an ileal tumor. Colonoscopy revealed a peduncular submucosal tumor (SMT) 75 mm long with an ulcerated apex at the ascending colon. The provisional diagnosis was a gastrointestinal stromal tumor of the terminal ileum. Ileocecal resection was carried out and the tumor was histologically diagnosed as schwannoma. Abdominal pain resolved postoperatively. This case reminds us that ileal schwannoma should be included in the differential diagnosis of intussusception caused by an SMT in the intestine.

Spinal arteriovenous fistulae with a concomitant cauda equina schwannoma.

BACKGROUND: Perimedullary spinal arteriovenous malformations or direct spinal arteriovenous fistulaes (AVFs) may be associated with other vascular abnormalities, such as arteriovenous malformations, venous ectasis, and aneurysms, but rarely have been reported with intraspinal intradural tumors. PURPOSE: The authors present an interesting case of type IV-A spinal AVF concomitant with a cauda equina schwannoma. STUDY DESIGN: The diagnostic procedures and surgical outcome were described. METHODS: The patient underwent surgery, the vessel feeding the AVFs was identified and cauterized, and the spinal tumor was removed. The fistula was small and located inside the tumor. The pathology revealed AVF and schwannoma, respectively. RESULTS: After surgery, the patient's symptoms began to improve and subside. Two years after surgery, follow-up magnetic resonance imaging showed no vascular lesion and tumor in the spinal canal. CONCLUSIONS: The association of spinal AVFs and cauda equina schwannoma has not been reported previously in any literature. The patient presents the symptoms of myelopathy associated with a spinal vascular lesion; it has to be noted that a concomitant and related intradural spinal tumor may exist.

Intracerebral schwannoma clinically and radiologically mimicking meningioma.

A case of intracerebral schwannoma (ICS) occurring in a 33-year-old woman is presented. The patient's history of headache, numbness, tingling and the recent development of weakness of the right upper extremity with right facial droop began during pregnancy. Magnetic resonance imaging (MRI) showed a 4 x 2 x 2 cm heterogeneous, gadolinium-enhanced mass at the left frontoparietal junction, with peritumoral edema and a dural-based attachment. During her pregnancy, the mass increased in size. The surgically resected specimen consisted of lobulated, somewhat gelatinous soft tissue. Microscopically, the tumor demonstrated classic biphasic Antoni type A and B patterns, admixed with degenerative changes. Immunohistochemically, the neoplastic cells were positive for S-100 protein (diffuse and strong), CD34 (primarily in Antoni B areas), glial fibrillary acidic protein (GFAP; weak and diffuse) and calretinin (mainly in Antoni A areas), while none was positive for CD31, estrogen and progesterone receptors, bcl-2, or epithelial membrane antigen (EMA). Ultrastructurally, basal laminae and Luse bodies were identified. The differential diagnosis includes fibrous meningioma, solitary fibrous tumor, and ICS. Twenty-seven cases of ICS were reviewed in which the histological diagnosis was confirmed immunohistochemically or ultrastructually, and the cases were summarized (including the present case). A combined use of immunostains (S-100 protein, EMA, CD34, and maybe calretinin) is of great help in distinguishing ICS from its histological mimickers.

Schwannoma antiguo: variante rara de schwannoma / Old schwannomaa. a rare type of schwannoma

OBJETIVO: A propósito de un caso deschwannoma retroperitoneal con características histológicaspoco frecuentes, se evalúa su significación clínica eindicaciones de manejo terapeutico.MÉTODOS: Presentación de un caso de schwannomaretroperitoneal benigno, como hallazgo incidental, alpracticar una Resonancia Magnética, en una paciente de70 años cuya clínica era de una lumbociática de seismeses de evolución. Revisión bibliográfica.RESULTADOS: Tras la exéresis quirúrgica se llegó al diagnósticode schwannoma retroperitoneal antiguo. No seadministra ningún tratamiento complementario. No se haevidenciado recidiva durante seguimiento. Los cambioshistológicos que se ven en los casos de schawannomaantiguo se han interpretado como degenerativos, indicandouna larga evolución, que ha motivado su denominaciónde “antiguos”. Algunos de los casos fueron diagnosticadoscomo malignos.CONCLUSIONES: El schwannoma antiguo es una variantepoco habitual del schwannoma común benigno.Microscópicamente muestra áreas de tejido hipocelular ynúcleos pleomórficos sugestivos de malignidad. Su manejoterapéutico es similar al de otros procesos benginosretroperitoneales, mediante exéresis quirúrgica y seguimiento

OBJECTIVES: We report one case ofretroperitoneal schwannoma with unfrequent histologicalcharacteristics evaluating its clinical significance andtherapeutic options.METHODS: We report the case of a benign retroperitonealschwannoma as an incidental MRI finding in a 70-yearoldfemale patient with symptoms of lumbosciatic painfor 6 months. We perform a bibliographic review.RESULTS: After surgical excision the diagnosis wasretroperitoneal old schwannoma. No additionaltreatment was given. There is no evidence of recurrenceon follow-up. The histological changes apparent in thecases of old schwannoma have been interpreted asdegenerative, indicating a long evolution, which hasmotivated its denomination as old. Some cases havebeen diagnosed as malignant.CONCLUSIONS: The old schwannoma is a rarevariety of the benign common schwannoma.Microscopically its shows areas of hypocellular tissueand pleomorphic nuclei suggestive of malignancy.Therapeutic management is similar to other benignretroperitoneal processes, surgical excision and followup

[Thalamic intracerebral schwannoma: case report]. / Schwanoma intracerebral talâmico: relato de caso.

The intracranial schwannomas cover about 8% of all the brain tumors, although, those localized inside the encephalic parenchyma are rare only 55 cases being reported in the literature. The histopathologic diagnosis is based on the cellular type and arrangement common to the nervous sheath tumors, the immunohistochemical findings (GFAP, S-100 protein, EMA) and electronmicroscopic findings as well. The clinical presentation related to the intraparenchimatous schwannoma is variable, depending on its localization, the image diagnosis does not show any typical aspect. The surgical treatment is curative once a complete extirpation is obtained. These tumors are benign but one case reported presented malignant signs. We present a patient that had a thalamic schwannoma with histological confirmation. We found no case with this localization in the literature.

Tumores neurogénicos benignos de cuello / Benign neurogenic tumors of neck

Los tumores neurogénicos de cuello son poco habituales. Esta escasa frecuencia explica el motivo por el cual, usualmente, no se postula su diagnóstico. En una masa cervical ubicada en la región de los nervios craneales, plexo cervical, cadena simpática cervical o nervios periféricos mayores, debe considerarse un origen neurogénico. Una resección no planificada puede ocasionar secuelas neurológicas que en algunas ocasiones podrían evitarse. Presentamos 3 casos tratados en el Servicio de Cirugía del Hospital de Valdivia, describiendo las principales características clínicas e histológicas de cada uno de ellos, su tratamiento y evolución. Además, se entrega una revisión actualizada en relación a esta rara e infrecuente patología

Schwannoma in the sigmoid colon: report of a case.

Schwann cell tumor occurring in the intestines is rare. A 68-year-old female came to our hospital because of hematemesis. Barium enema and colonoscopic examination revealed submucosal tumor in the sigmoid colon. Laboratory data showed mild anemia. No other abnormal finding was found in the blood chemistry. Tumor marker levels of carcinoembryonic antigen (CEA), CA19-9, alpha feto protein (AFP) and neuron specific enolase (NSE) were within normal limits. The exploratory laparotomy confirmed a large sigmoid colon tumor. She received sigmoid colectomy. The resected specimen was a submucosal tumor with central depression, measuring 4.7 x 3.5 x 3.0 cm in size. The cut surface of the tumor was yellowish hue with necrosis. Histological examination showed spindle-shaped tumor cells with palisading comma-shaped nuclei and the nuclear pleomorphism. Immunohistochemical examination revealed that the tumor was positive for S-100 protein staining, and negative for Actin and for H.H.F. staining. These findings showed that this tumor was of Schwann cell origin. We report here the case in detail of a schwannoma in the sigmoid colon.

A schwannoma of the cecum: case report and review of Japanese schwannomas in the large intestine.

A 66-year-old Japanese man had a positive fecal occult blood test at a regular check-up, and a large polypoid mass was detected in the cecum by barium enema study. Colonoscopy showed a submucosal tumor with ulcer protruding into the cecal lumen. A large-forceps biopsy specimen was taken from the bottom of the ulcer. With the tentative diagnosis of neurogenic tumor, ileocecal resection was performed. The tumor showed spindle-cell proliferation in a concentric or fascicular pattern. Immunohistochemically, the tumor cells were diffusely positive for S-100 protein, and they had intracytoplasmic periodic acid Schiff (PAS)-positive crystalloids. The mitosis count was low (about 1 per 20 high-power fields). The pathological diagnosis of this tumor was benign gastrointestinal schwannoma. A large number of schwannoma cases have been reported since 1910 when Verocay reported it as a true tumor that stemmed from Schwann cells and did not contain neuroganglion cells. However, gastrointestinal schwannomas are rare, and schwannomas of the large intestine are extremely rare. We reviewed 40 cases already reported in Japan and this present case in order to clarify the clinicopathological features of this tumor.

[Osteomalacia cured by resection of cutaneous neurinoma]. / Ostéomalacie guérie par résection d'un neurinome cutané.

INTRODUCTION: Regression of osteomalacia after exeresis of a skin tumor is unusual. CASE REPORT: A 62-year-old man had suffered from bone and joint symptoms for several years due to osteomalacia which was confirmed both biologically and histologically. The patient also had a plantar neurinoma. After exeresis of the tumor the biological results returned to normal levels within one week followed by regression of the clinical signs of osteomalacia. DISCUSSION: The neurinoma in this patient was apparently the cause of osteomalacia, since signs of the disease disappeared after exeresis. To date, three cases of neurinoma associated with osteomalacia have been published, including a single case with skin localization. The tumor would secrete a substance which inhibits the synthesis of vitamin D and enhances phosphorus excretion.

Preoperative shunts in suprasellar tumours.

A series of 22 cases of large suprasellar tumours was studied retrospectively. These patients had undergone a preoperative shunt to facilitate surgical exposure of the tumour and to reduce intracranial pressure in critically ill patients. Our observations on the clinical progress of these patients following the shunt is analysed. Nine patients worsened to a varying degree following insertion of the shunt. Seven patients became drowsy, one developed worsening of vision and a hemiparesis, and one became an akinetic mute. The worsening was probably related to alteration of intracranial pressure dynamics and resultant compression in the hypothalamic region by subtle superior migration of the tumour. Operative difficulties were encountered during the dissection of the tumour from the neural structures in these cases. We conclude that with the availability of modern decongestive measures and basal surgical exposures, preoperative shunting is rarely indicated and can be occasionally dangerous. In the absence of actual proof, it is postulated that the secondary ventricular enlargement appears to be a protective mechanism of the brain helping to reduce tumour pressure on the basal forebrain structures.

Benign nerve sheath tumor of the sigmoid colon.

A rare case of a nerve sheath tumor of the sigmoid colon is presented. The radiological appearance of this tumor is described. The diagnostic roles of ultrasound sonography (US) and the barium enema in differentiating submucosal colon tumors are emphasized.

Malignant schwannoma.

This paper reports a patient suffering from a malignant peripheral nerve sheath tumor, also known as a Schwannoma or neurilemoma. The patient demonstrated an increase in left thigh circumference, without apparent trauma. The report establishes the relationship of the lack of trauma and relatively mild symptomatology with malignant conditions, as well as the need for chiropractors to do thorough history, physical and radiological examinations. A brief review of pathophysiology and incidence is given.