Malignant peripheral nerve sheath tumor of tongue: a case report / 华西口腔医学杂志

Malignant peripheral nerve sheath tumor (MPNST) is a rare neurogenic malignant tumor. MPNST has aty-pical clinical symptoms and imaging presentations, difficult diagnosis, a high degree of malignancy, and poor prognosis. It usually occurs in the trunk, approximately 20% in the head and neck, and rarely in the mouth. This paper reports a case of MPNST of the tongue. A summary of the clinical features, diagnosis, and treatment of MPNST is presented in combination with a literature review to provide a reference for the diagnosis and treatment of this disease.

Tumor de nervio periférico: una localización poco habitual / Peripheral nerve tumor: an unusual location

Benign tumors of peripheral nerves called Schwannomas or neurilemomas, correspond to a rare pathology, represent 5% of all tumors of the upper extremity, and affects, mainly, the ulnar nerve. The incidence of Schwannoma in the literature for the radial nerve is not clearly established given the infrequency of its presentation, there are only reports of isolated cases The following publication presents the case of a male patient with a radial nerve schwannoma. Clinically, presents increased painful volume on palpation, well delimited, of soft consistency in the distal third of the right arm of 3 years of evolution, without history of previous trauma, without irradiation, or paresthesia, with preservation of motor and sensory function of radial, median and ulnar nerve. Considering that the involvement of the radial nerve is very low frequency, a review is carried out in PubMed, in the last 10 years, there are only 9 studies, grouped in case reports and imaging studies for diagnosis.

Tumor benigno de la vaina neural en rodilla. Reporte de caso y revisión bibliográfica / Knee neural sheath benign tumor. Case report and bibliographic revision

El schwannoma es el tumor benigno de nervio periférico más frecuente. Su presencia en los nervios de miembros inferiores es excepcional, donde representan el 1% de todos los schwannomas. Presentamos el caso de una mujer de treinta y un años que consulta por dolor en la cara anterior de la rodilla derecha, donde se palpa una masa blanda, dolorosa, de 1 cm aproximadamente y dolor en interlínea externa con signo de McMurray positivo. La RM evidenció una estructura ovoidea de señal quística, superficial al retináculo medial en su tercio proximal, de 10 × 8 × 8 mm y lesión del menisco externo en su tercio medio. Se realizó tratamiento artroscópico de la lesión meniscal externa y por vía abierta la exéresis marginal quirúrgica del tumor de partes blandas, con diagnóstico histopatológico de schwannoma. Los schwannomas de nervio periférico, aunque sean una entidad poco frecuente, deben considerarse en el diagnóstico diferencial de las masas dolorosas de la rodilla. Su tratamiento es la exéresis quirúrgica

Schwannoma is the most common benign peripheral nerve tumor, its presence being exceptional in the nerves of the lower limbs, where it represents 1% of all schwannomas. We present the case of a thirty-one-year-old woman who consulted for anterior knee pain, where a soft, painful mass of approximately 1cm and pain on the lateral joint line was assessed. McMurray's sign was positive. MRI showed an ovoid structure with a cystic signal, superficial and proximal to the medial retinaculum, measuring 10 × 8 × 8 mm and a tear in the body and posterior horn of the lateral meniscus. Arthroscopic treatment for the lateral meniscus tear and open surgical marginal excision of the soft tissue tumor were performed, with pathological diagnosis of schwannoma. Peripheral nerve schwannomas, although a rare entity, should be considered in the differential diagnosis of painful knee masses, their treatment being surgical excision

Schwannoma Presenting as a Scalp Mass: A Case Report with Magnetic Resonance Imaging Findings

Neurofibromatose tipo 1 com acometimento do nervo infraorbital: relato de caso / Neurofibromatosis type 1 with infraorbital nerve involvement: a case report

A neurofibromatose tipo 1 é uma doença autossômica dominante rara, com manifestações clínicas diversas. Sua apresentação mais marcante é a presença de neurofibromas (tumores da bainha neural) cutâneos ou internos, que também podem ocorrer de forma esporádica, associados a outras manifestações sistêmicas, como manchas café com leite e lesões oculares. Por serem tumores da bainha de mielina, os neurofibromas podem acometer diversos nervos periféricos, incluindo nervos da face. Apresentamos o caso de um paciente de 1 ano, portador de neurofibromatose tipo 1, com neurofibroma em nervo infraorbital direito, com o acesso proposto para tratamento cirúrgico que fornecesse ampla visualização e acesso a lesão, sem comprometimento estético importante, permitindo preservação de partes moles e adequado crescimento facial.

Neurofibromatosis type 1 (NF1) is a rare autosomal dominant disease with multiple clinical manifestations. Its most significant presentation is cutaneous or subcutaneous neurofibromas (myelin sheath tumors), which may be associated with other systemic manifestations such as caféau- lait spots and eye involvement. Neurofibromas can affect several peripheral nerves, including the facial nerves. This report presents a case of a 1-year-old patient with NF1 with right infraorbital nerve neurofibroma in which the proposed access for surgical treatment allowed adequate visualization of the tumor with good aesthetic results, preservation of the soft tissues, and normal facial growth.

Histopathology and immunohistochemistry of peripheral neural sheath tumor and perivascular wall tumor in dog / Histopatologia e imuno-histoquímica de tumor de bainha de nervo periférico e de tumor de parede perivascular em cães

Soft tissue sarcomas (STS) comprise a heterogeneous group of malignancies derived from extra-skeletal mesenchymal tissues that may show similar histopathological changes. Histopathologic patterns suggestive of perivascular wall tumors (PWT) and peripheral nerve sheath tumors (PNST) have been described. This study investigated the histogenesis in a series of 71 cases of canine STS that showed morphological compatibility with what is described for PWT and PNST. Immunohistochemistry analysis were done to CD56, S100, SMA, Desmin, Von Willebrand Factor, NSE and GFAP. Twenty-one cases (29.6%) showed histopathologic features compatible with PWT, 23 cases (32.4%) with PNST and 27 cases (38.0%) shared both histopathological features. By immunohistochemistry, 59 (83.1%) cases showed positivity only for neural markers and 12 (16.9%) had simultaneous positivity for both neural and muscle markers. PNST was the most prevalent neoplasm and none of the cases were positive for muscle markers only. The histopathologic features were not useful to define the diagnosis of PWT, since most tumors were negative for muscle markers but positive for neural markers. Due to this immunoreactivity and the morphologic features, future studies may propose guidelines for the classification of these neoplasms.(AU)

Sarcoma de tecidos moles (STM) compreende um grupo heterogêneo de neoplasias malignas, derivadas de tecidos extraesqueléticos, que podem apresentar alterações histopatológicas similares. Os padrões histopatológicos sugestivos de tumor de parede perivascular (TPP) e de tumor de bainha de nervo periférico (TBNP) têm sido descritos. Este estudo investigou a histogênese de uma série de 71 STM caninos, que apresentavam compatibilidade morfológica com o que é descrito para TPP e TBNP. A análise imuno-histoquímica foi feita para CD56, S100, SMA, Desmina, Fator Von Willebrand, NSE e GFAP. Vinte e um casos (29,6%) apresentaram características histopatológicas compatíveis com TPP, 23 casos (32,4%) com TBNP e 27 casos (38,0%) apresentaram características histopatológicas de ambos. Na imuno-histoquímica, 59 (83,1%) casos apresentaram positividade somente para marcadores neurais e 12 (16,9%) tiveram positividade simultânea tanto para marcadores neurais como para marcadores musculares. TBNP foi a neoplasia mais prevalente e nenhum dos casos foi positivo para somente para marcadores musculares. As características histopatológicas não foram úteis para definir o diagnóstico de TPP, uma vez que a maioria foi negativa para marcadores musculares, mas positiva para marcadores neurais. Devido a essa imunorreatividade e às características morfológicas, pesquisas futuras poderão propor orientações para a classificação dessas neoplasias.(AU)

Schwannoma of the Tongue Base with Imaging Features and Differential Diagnosis: a Rare Case Report and Literature Review

Schwannoma or neurilemmoma is a benign peripheral nerve sheath tumor that arises from Schwann cells. Approximately 25–45% of all schwannomas occur in the head and neck regions, and the intraoral presentation of these is only 1%. We report a rare case of a patient presenting tongue base schwannoma with characteristic imaging features on computed tomography and magnetic resonance imaging.

Superficial malignant peripheral nerve sheath tumor from recurrent neurofibroma in the abdominal wall of a patient without neurofibromatosis type 1 / 영남의대학술지

Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5-10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST occurs more often at younger ages than sporadic MPNST, but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.

Schwannoma of the uterine cervix: a rare case report

Schwannomas are neurogenic tumors, which are among the most varied tumors with respect to morphology, clinical associations, and presentations; they occur in a wide variety of sites. The uterine cervix is a rare site of occurrence and only 15 cases of schwannomas of the uterine cervix, including 5 benign and 10 malignant cases, have been reported to date. Thus, schwannomas of the uterine cervix may pose diagnostic difficulty. Here, we report a case of benign schwannoma of the uterine cervix in a 37-year-old female, who presented with vaginal spotting.

Immunohistochemical Markers for Schwannomas, Neurofibromas and Malignant Peripheral Nerve Sheath Tumors­What Can the Recent Literature Tell Us?

Introduction Schwannomas and neurofibromas are the two most common benign neoplasms of the peripheral nerve sheath, and although they are generally easy to distinguish, in some cases, they can closely resemble one another. Furthermore, malignant peripheral nerve sheath tumors (MPNSTs), another example of peripheral nerve sheath neoplasm, may likewise constitute, due to their morphology and lack of specific immunohistochemical markers, a challenging diagnostic. Objective To bring attention to new and promising biomarkers for schwannomas, neurofibromas and MPNSTs and to outline, based on the recent literature, a immunohistochemical profile for each neoplasm at hand, as well as to emphasize the need for further studies that could help us understand their diagnostic potential and disrupt our dependence of limited and nonspecific biomarkers. Methods An overview of the recent literature published in English on both the classical promising immunohistochemical markers of schwannomas, neurofibromasand MPNSTs was performed. We discarded case reports. Conclusions There is still a lack of specific biomarkers for peripheral nerve tumors. However, plenty of new immunohistochemical markers have been coming to light with presumed higher specificity and more diverse helpful uses than the classical ones. For example, Sox10 is a good biomarker for differentiating schwannomas and neurofibromas from sarcomas, calretinin schwannomas from neurofibromas, TLE1 and HMGA2 MPNSTs from sarcomas, and nestin, EGFR, p16 and Ki-67 MPNSTs from different types of schwannomas and neurofibromas. There is still need for further studies; however, the potential of some of these promising markers, among others, should not be disregarded.

Introdução Schwannomas e neurofibromas são as duas neoplasias benignas mais comuns a acometer o tecido nervoso periférico, e apesar de geralmente serem facilmente distinguíveis, em alguns casos, elas podem ser muito semelhantes. Além disso, os tumores malignos da bainha dos nervos periféricos (TMBNPs), outro exemplo de neoplasia da bainha do nervo periférico, podem da mesma forma constituir, pela sua morfologia e falta de marcadores imuno-histoquímicos específicos, um diagnóstico desafiador. Objetivo Chamar a atenção para novos e promissores biomarcadores para schwannomas, neurofibromas e TMBNPs e delinear, a partir da literatura atual, um perfil imuno-histoquímico para cada neoplasia em questão, além de enfatizar a necessidade de futuros estudos que possam elucidar-nos acerca de seu potencial diagnóstico e, por ventura, romper nossa dependência de biomarcadores inespecíficos e limitados. Método Foi feita uma revisão da literatura recente incluindo artigos em língua inglesa sobre os marcadores imunohistoquímicos clássicos e os promissores para schwannomas, neurofibromas e TMBNPs. Descartamos relatos de caso. Conclusão Ainda há uma falta de biomarcadores específicos para as neoplasias acima. Contudo, vários novos marcadores imuno-histoquímicos têm surgido, e com futuros estudos poderemos talvez definir biomarcadores específicos e indispensáveis para os casos desafiadores de neurofibromas, schwannomas e TMBNPs. Por exemplo, o Sox10 é um bom biomarcador para diferenciar schwannomas e neurofibromas de sarcomas; a calretinina é um bom marcador para diferenciar schwannomas de neurofibromas; os biomarcadores TLE1 e HMGA2 podem ajudar a diferenciar TMBNPs de sarcomas, e a nestina, o receptor do fator de crescimento epidérmico (EGFR), o gene p16 e a proteína Ki-67 podem diferenciar TMBNPs de diferentes tipos de schwannomas e neurofibromas. Ainda há necessidade de novos estudos; contudo, o potencial de alguns desses marcadores, dentre outros, não deveria ser negligenciado.

Analysis According to Characteristics of 18 Cases of Brachial Plexus Tumors : A Review of Surgical Treatment Experience

OBJECTIVE: Because the anatomical structure of the brachial plexus is very complex, surgical treatment of tumors in this region is challenging. Therefore, a lot of clinical and surgical experience is required for successful treatment; however, many neurosurgeons have difficulty accumulating this experience owing to the rarity of brachial plexus tumors. The purpose of this report is to share our surgical experience with brachial plexus tumor with other neurosurgeons.METHODS: The records of 18 consecutive patients with brachial plexus tumors who underwent surgical treatment between January 2010 and December 2017 in a single institution were retrospectively reviewed. The surgical approach was determined according to the tumor location and size, and intraoperative neurophysiological monitoring (IONM) was used in most of cases to prevent iatrogenic nerve injury during surgery. In addition, to evaluate the differences in tumor characteristics according to pathologic diagnosis, the tumors were divided twice into two groups, based on two separate classifications, and statistical analysis was performed.RESULTS: The 18 brachial plexus tumors comprised 15 (83.3%) benign peripheral nerve sheath tumors including schwannoma and neurofibroma, one (5.6%) malignant peripheral nerve sheath tumor, one (5.6%) benign tumor of non-neural sheath origin (neurogenic cyst), and one (5.6%) metastatic tumor (papillary carcinoma). The authors analyzed relationship between tumor size/location and tumor characteristic parameters such as age, size, right-left, and pathology. There were no statistically significant differences except a tendency of bigger tumor size in young age.CONCLUSION: For a successful surgical outcome, an appropriate surgical approach is essential, and the appropriate surgical approach is determined by the location and size of the tumor. Furthermore, applying IONM may prevent postoperative complications and it is favorable option for brachial plexus tumors surgery.

Analysis According to Characteristics of 18 Cases of Brachial Plexus Tumors : A Review of Surgical Treatment Experience

OBJECTIVE: Because the anatomical structure of the brachial plexus is very complex, surgical treatment of tumors in this region is challenging. Therefore, a lot of clinical and surgical experience is required for successful treatment; however, many neurosurgeons have difficulty accumulating this experience owing to the rarity of brachial plexus tumors. The purpose of this report is to share our surgical experience with brachial plexus tumor with other neurosurgeons. METHODS: The records of 18 consecutive patients with brachial plexus tumors who underwent surgical treatment between January 2010 and December 2017 in a single institution were retrospectively reviewed. The surgical approach was determined according to the tumor location and size, and intraoperative neurophysiological monitoring (IONM) was used in most of cases to prevent iatrogenic nerve injury during surgery. In addition, to evaluate the differences in tumor characteristics according to pathologic diagnosis, the tumors were divided twice into two groups, based on two separate classifications, and statistical analysis was performed. RESULTS: The 18 brachial plexus tumors comprised 15 (83.3%) benign peripheral nerve sheath tumors including schwannoma and neurofibroma, one (5.6%) malignant peripheral nerve sheath tumor, one (5.6%) benign tumor of non-neural sheath origin (neurogenic cyst), and one (5.6%) metastatic tumor (papillary carcinoma). The authors analyzed relationship between tumor size/location and tumor characteristic parameters such as age, size, right-left, and pathology. There were no statistically significant differences except a tendency of bigger tumor size in young age. CONCLUSION: For a successful surgical outcome, an appropriate surgical approach is essential, and the appropriate surgical approach is determined by the location and size of the tumor. Furthermore, applying IONM may prevent postoperative complications and it is favorable option for brachial plexus tumors surgery.

Malignant Triton tumor: a rare cause of sciatic pain and foot drop / Tumor de Triton maligno: uma causa rara de dor ciática e pé pendente

ABSTRACT Malignant peripheral nerve sheath tumors (MPNST) are very rare and are frequently localized in the buttocks, thigh, arm, or paraspinal region; one variant is the malignant Triton tumor, with rhabdomyosarcomatous differentiation. The authors present a challenging differential diagnosis of a sciatic pain and foot drop in a woman with history of lumbar disk herniation, which was found to be caused by a Triton tumor of the sciatic nerve. She underwent surgical excision, followed by radiation and chemotherapy. Malignant Triton tumor cases have rarely been described and reported in the literature. The recommended treatment is radical excision followed by high-dose radiotherapy and chemotherapy. The prognosis, although poor, depends on the location, grade, and completeness of surgical margins.

RESUMO Os tumores malignos da bainha dos nervos periféricos (TMBNP) são muito raros e localizam-se mais frequentemente na região nadegueira, paraespinal, coxa ou braço; uma variante é o tumor de Triton maligno, com uma diferenciação rabdomiosarcomatosa. Apresentamos um diagnóstico diferencial desafiante de dor ciática e pé pendente em uma paciente com antecedentes de hérnia discal lombar, que se descobriu que era causada por um tumor de Triton do nervo ciático. A paciente foi submetida a excisão cirúrgica, seguida de radio e quimioterapia. Poucos casos de tumores de Triton malignos foram descritos e relatados na literatura. O tratamento recomendado é a excisão radical, seguida de radioterapia em alta dose e quimioterapia. O prognóstico, embora mau, depende da localização, do grau e das margens cirúrgicas da exérese.

Schwannoma lumbosacro gigante / Schwannome lombo-sacré géant / Giant lumbosacral schwannoma

Fundamento: el schwannoma espinal es un tumor de las vainas nerviosas y se comporta intradural y extramedular y provienen de las células embrionarias de la cresta neural de los nervios periféricos. Representan entre el 25 y el 30 por ciento de los tumores y afectan a adultos entre 40 y 60, son raros en niños y no hay predilección por el sexo. Objetivo: presentar un caso de schwannoma lumbosacro gigante que se diagnosticó en el Hospital Universitario Manuel Ascunce Domenech de Camagüey y recibió tratamiento quirúrgico en el Instituto Nacional de Neurología y Neurocirugía. Reporte de caso: paciente MB, de 38 años con antecedentes de salud con historia de dolor lumbosacro de dos años de evolución, intensificado en los últimos 8 meses que se irradiaba a miembros inferiores con parestesias y pérdida de la fuerza muscular. Además presentaba dificultad para la deambulación, constipación, incontinencia urinaria y eyaculación retardada. La tomografía axial computarizada informó un extenso proceso tumoral intrarraquídeo con crecimiento longitudinal anterior, posterior y lateral desde la cuarta vértebra lumbar hasta la tercera sacra (L4-S3), que producía lisis de la porción posterior de los cuerpos vertebrales, los pedículos y el borde anterior de S3 y la imagen de resonancia magnética reveló gran área ligeramente hipointensa en T1 e hiperintensa en T2, que ocupaba el canal vertebral desde L3 hasta la cuarta vertebra sacra (S4). La biopsia informó un tumor constituido por células de aspecto fusocelular dispuesto en fascículos compactos, otras áreas más laxas compuestas por células de citoplasma claro, abundantes vasos sanguíneos, compatible con un schwannoma, tumor benigno de las vainas nerviosas. Se remitió para tratamiento quirúrgico en el Instituto Nacional de Neurología y Neurocirugía con evolución favorable luego de la resección amplia e instrumentación. Conclusiones: el schwannoma lumbosacro es un reto para los cirujanos y la afectación sacra es rara. El diagnóstico anatomopatológico es fundamental y la cirugía es el tratamiento de elección, donde la resección completa del tumor es muy difícil en ocasiones debido a la posibilidad de lesionar estructuras nerviosas importantes. Se recomienda el seguimiento radiológico por el riesgo de recidivas(AU)

Background: The spinal schwannoma is a tumor of the nerve sheaths that presents in intradural and extramedular areas. It comes from the embryonic cells of the neural crest of the peripheral nerves. They represent 25-30 percent of tumors and it affects adults aging 40 and 60. It is rare in children and there is no sex difference. Objective: Present a case of giant lumbosacral schwannoma that was diagnosed at Manuel Ascunce Domenech University Hospital in Camagüey. This patient received surgical treatment at the National Institute of Neurology and Neurosurgery. Case report: 38-year-old white male patient with a history of lumbosacral pain of two years of evolution came to consultation. His pain intensified in the last 8 months, radiating to lower limbs with paresthesias and loss of muscle strength. In addition, he had constipation, urinary incontinence, difficulty at walking, and delayed ejaculation. Computed tomography revealed an extensive intrarectal tumor process with anterior, posterior and lateral longitudinal growth from the fourth lumbar vertebra to the third sacrum (L4-S3), which produced lysis of the posterior portion of the vertebral bodies, pedicles, and S3 anterior border. Magnetic resonance imaging revealed a large area slightly hypointense in T1 and hyperintense in T2, which occupied the vertebral canal from L3 to the fourth sacral vertebra (S4). The biopsy reported a tumor-like fusocellular cells arranged in compact fascicles, other more lax areas of clear cytoplasm cells, abundant blood vessels, compatible with a schwannoma, benign tumor of the nerve sheaths. The patient was referred for surgical treatment at the National Institute of Neurology and Neurosurgery with favorable evolution after extensive resection and instrumentation. Conclusions: Lumbosacral schwannoma is a challenge for surgeons. Sacral involvement is rare. Histopathologic diagnosis is fundamental and surgery is the treatment of choice, in which complete resection of the tumor is very difficult at times due to the possibility of injuring important nerve structures. Radiological follow-up is recommended for the risk of relapses(AU)

Fondement: Le schwannome rachidien est une tumeur de comportement intradural et extramédullaire des gaines nerveuses, provenant des cellules embryonnaires de la crête neurale des nerfs périphériques. Il représente 25 à 30 pourcent des tumeurs, touchant généralement les adultes âgés de 40 à 60 ans. Chez l'enfant, il est rare. Il touche les deux sexes en égal. Objectif: Présenter un cas de schwannome lombo-sacré géant diagnostiqué à l'hôpital universitaire "Manuel Ascunce Domenech", à Camagüey, et traité chirurgicalement à l'Institut national de neurologie et neurochirurgie, à La Havane. Rapport de cas: Patient, blanc, âgé de 38 ans, ayant une histoire de douleur lombo-sacrée de deux ans d'évolution, intensifiée dans ces huit derniers mois et irradiée aux membres inférieurs avec paresthésie et perte de la force musculaire. Il avait aussi des difficultés pour la marche, et souffrait de constipation, incontinence urinaire et éjaculation retardée. La tomographie axiale calculée par ordinateur a montré un large processus tumoral intrarachidien, avec une croissance longitudinale antérieure, postérieure et latérale depuis la quatrième vertèbre lombaire jusqu'à la troisième sacrée (L4-S3), produisant la lyse de la portion postérieure des corps vertébraux, des pédicules et du bord antérieur de S3, et l'imagerie par résonance magnétique a révélé une vaste zone légèrement hypointense en T1 et hyperintense en T2, occupant le canal vertébral depuis L3 jusqu'à la quatrième vertèbre sacrée (S4). La biopsie a indiqué une tumeur constituée de cellules d'aspect fusocellulaire disposées en fascicules compacts, d'autres zones plus détendues composées de cellules à cytoplasme clair, beaucoup de vaisseaux sanguins, évoquant un schwannome, c.-à-d., une tumeur bénigne des gaines nerveuses. Le patient a été renvoyé vers l'Institut national de neurologie et neurochirurgie pour un traitement chirurgical. Après une grande résection et appareillage, son évolution a été favorable. Conclusions: Le schwannome lombo-sacré constitue un défi pour les chirurgiens parce que cette affection est très rare. Son diagnostic anatomopathologique est essentiel. L'intervention chirurgicale est le traitement de choix, mais la résection complète de la tumeur est parfois très difficile due à la proximité de structures nerveuses importantes. Il est conseillé un suivi radiologique de risque de récidive(AU)

Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 / Tumor maligno da bainha de nervo periférico com e sem Neurofibromatose tipo 1

ABSTRACT Objective In this study, we review the institution’s experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). Methods Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range, 3–84 years) and 55.4% were female; 41 patients (44.6%) had NF1-associated tumors. Results Mean tumor sizes were 15.8 ± 8.2 cm and 10.8 ± 6.3 cm for patients with and without NF1, respectively. Combined two- and five-year overall survival was 48.5% and 29%. Multivariate analysis confirmed the association of tumor size greater than 10 cm (hazard ratio (HR) 2.99; 95% confidence interval (CI) 1.14–7.85; p = 0.0258) and presence of NF1 (HR 3.41; 95%CI 1.88–6.19; p < 0.001) with a decreased overall survival. Conclusion Tumor size and NF1 status were the most important predictors of overall survival in our population.

RESUMO Objetivo Relatamos a experiência institucional no tratamento de tumores malignos da bainha de nervo periférico (TMBNP) e comparamos o prognóstico entre pacientes com e sem neurofibromatose tipo 1 (NF1). Métodos Foram incluídos neste estudo 92 pacientes num período de 20 anos. Foi realizada uma análise retrospectiva dos prontuários, das características do tumor e do tratamento. A idade mediana era 43,5 anos (variação 3–84 anos) e 55,4% dos pacientes eram mulheres; 41 pacientes (44,6%) tinham tumores associados à NF1. Resultados O diâmetro médio dos tumores era 15,8 ± 8,2cm e 10,8 ± 6,3cm para pacientes com e sem NF1, respectivamente. A sobrevida combinada em 2 e 5 anos foi de 48,5% e 29%. A análise multivariada confirmou que o tamanho do tumor acima de 10cm (hazard ratio (HR) 2.99; 95% intervalo de confiança (IC) 1.14–7.85; p = 0.0258) e a presença de NF1 (HR 3.41; 95%IC 1.88–6.19; p < 0.001) estão associados a uma pior sobrevida. Conclusões O tamanho do tumor e a associação com NF1 foram os preditores mais importantes de sobrevida na nossa população.

Case report and immunohistochemical study of a malignant peripheral nerve sheath tumor in a dog / Relato de caso e estudo imuno-histoquímico de tumor maligno da bainha do nervo periférico em um cão

Malignant peripheral nerve sheath tumor is rare and rarely reported in dogs. The term neurofibroma/sarcoma is classically used when the tumor is composed of Schawann and perineural cells. This work describes the clinical, histopathological, and immunohistochemical case of a subcutaneous malignant peripheral nerve sheath tumor, most likely a malignant neurofibroma located in the pelvic member of a Rottweiler dog. Histopathological features and immunohistochemical analysis corroborated the diagnosis, showing positivity for S-100 protein, vimentin, and CD57, and was useful to distinguish this type of neoplasm from other malignancies of similar morphologies.(AU)

O tumor maligno da bainha do nervo periférico é raro e pouco descrito em cães. Classicamente, o termo "neurofibroma/sarcoma" é empregado quando o tumor é composto por células de Schwann e células perineurais. Neste relato são descritos os achados clínicos, histopatológicos e imuno-histoquímicos de um caso de tumor maligno da bainha do nervo periférico, provavelmente neurofibrossarcoma, localizado no subcutâneo do membro pélvico de um cão da raça Rottweiler. Os achados histopatológicos associados às observações imuno-histoquímicas contribuíram para o diagnóstico, sendo observada positividade para proteína S-100, vimentina e CD57, permitindo a diferenciação da neoplasia em questão de outros tumores malignos com características morfológicas similares.(AU)

An Intraosseous Schwannoma Combined with a Subchondral Fracture of the Femoral Head: a Case Report and Literature Review

Schwannomas are benign nerve sheath tumors that are typically located in soft tissue. Occasionally, schwannomas involve osseous structures. These intraosseous schwannomas are generally benign neoplasms that account for less than 0.2% of primary bone tumors. Schwannomas are very rarely observed in long bones. We present a case of a schwannoma affecting the proximal femur with a coincident subchondral fracture of the femoral head. A 38-year-old-male presented with left hip pain without deteriorating locomotor function. Plain film radiographs displayed a lobulating contoured lesion within the intertrochanteric portion of the femur. The magnetic resonance imaging (MRI) scans showed a tumor occupying the intertrochanteric region. Diffuse bone marrow edema, especially in the subchondral and head portions of the femur that was possibly due to the subchondral insufficiency fracture was also noted. The lesion was surgically excised and bone grafting was performed. Histologically, there was diffuse infiltrative growth of the elongated, wavy, and tapered cells with collagen fibers, which are findings that are characteristic of intraosseous schwannoma. Although very rare, intraosseous schwannoma should be included in the differential diagnosis of radiographically benign-appearing, non-aggressive lesions arising in the femur. The concomitant subchondral fracture of the femoral head confounded the correct diagnosis of intraosseous schwannoma in this case.

An Updated Nationwide Epidemiology of Primary Brain Tumors in Republic of Korea, 2013

BACKGROUND: This report aims to provide accurate nationwide epidemiologic data on primary brain and central nervous system (CNS) tumors in the Republic of Korea. We updated the data by analyzing primary brain and CNS tumors diagnosed in 2013 using the data from the national cancer incidence database. METHODS: Data on primary brain and CNS tumors diagnosed in 2013 were collected from the Korean Central Cancer Registry. Crude and age-standardized rates were calculated in terms of gender, age, and histological type. RESULTS: A total of 11,827 patients were diagnosed with primary brain and CNS tumors in 2013. Brain and CNS tumors occurred in females more often than in males (female:male, 1.70:1). The most common tumor was meningioma (37.3%). Pituitary tumors (18.0%), gliomas (12.7%), and nerve sheath tumors (12.3%) followed in incidence. Glioblastomas accounted for 41.8% of all gliomas. In children (<19 years), sellar region tumors (pituitary and craniopharyngioma), embryonal/primitive/medulloblastoma, and germ cell tumors were the most common tumors. CONCLUSION: This study should provide valuable information regarding the primary brain tumor epidemiology in Republic of Korea.

A Rare Case of Aggressive Melanotic Schwannoma Occurred in Spinal Nerve of a 59-Year-Old Male

Melanotic schwannoma (MS) is a rare variant of nerve sheath neoplasm that shows ultrastructural and immunophenotypical features of Schwann cells but also has cytoplasmic melanosomes and is reactive for melanocytic markers as well. Unlike conventional schwannoma, which is totally benign, MS has an unpredictable prognosis and is thought to have low-malignant potential. Herein, we present a rare case of recurrent MS in lumbar spine of a 59-year-old male.