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.

Hiperqueratosis plantar / Plantar hyperkeratosis

RESUMEN La hiperqueratosis es un trastorno caracterizado por el engrosamiento de la capa externa de la piel, que está compuesta de queratina, una fuerte proteína protectora. Puede ser causada por fricción, conllevando la aparición de callosidades, inflamación crónica, eccema o trastornos genéticos, como la ictiosis ligada al cromosoma X. Se presentó el caso de un paciente de 47 años, que acudió al Servicio de Ortopedia por lesión escamosa a nivel de ambas regiones plantares con dificultad para la marcha. El tratamiento quirúrgico fue el empleado en este paciente para la obtención de la biopsia exerética. La evolución fue favorable en el postoperatorio mediato e inmediato, y el paciente se incorporó de forma rápida a su vida normal. Con este caso se identificaron las características clínicas de la hiperqueratosis plantar, así como el uso de la biopsia exerética como estándar de oro para el diagnóstico positivo en los tumores periféricos. Un diagnóstico adecuado por el médico inicial, la interrelación del Servicio de Ortopedia con la consulta de tumores periféricos, y el tratamiento quirúrgico seleccionado, constituyen factores determinantes en la evolución favorable de los pacientes con este diagnóstico (AU).

ABSTRACT Hyperkeratosis is a disorder characterized by thickening of the outer layer of the skin, which is composed of keratin, a strong protective protein. It can be caused by friction, leading to callosities, chronic inflammation, eczema or genetic disorders such as X chromosome-linked ichthyosis. We presented the case of a patient, aged 47 years, who attended the Orthopedic Service for scaly lesion at the level of both plantar regions with difficulties to walk. Surgical treatment was used in this patient to obtain an exeretic biopsy. The evolution was favorable in both the mediate and immediate postoperative period, and the patient quickly returned to his normal life. This case identified the clinical characteristics of plantar hyperkeratosis and the use of exeretic biopsy as a gold standard for positive diagnosis in the peripheral tumors. An adequate diagnosis by the initial physician, the interrelation of the orthopedic service with the consultation of peripheral tumors, and the chosen surgical treatment are determining factors in the favorable evolution of patients with this diagnosis (AU).

Retrospective Analysis of 20 Patients Affected by Schwannomas in the Upper and Lower Limbs / Análise retrospectiva de 20 pacientes acometidos por schwannoma nos membros superior e inferior

Abstract Objective To analyze the epidemiological profile and evolution of 20 patients diagnosed with upper- and lower-limb schwannomas. Methods A group of patients was defined for a retrospective evaluation comprising the period between February 2002 and June 2018, in which we studied and evaluated 20 medical records of patients undergoing surgery due to schwannoma; the diagnosis was confirmed by an anatomopathological examination. Results Male and female patients were equally affected. The average age was 50.85 years, ranging from 12 to 77 years. There was a predominance of the upper limb and of the flexor face. The most affected nerve was the ulnar nerve. In total, 6 (30%) patients had transient postoperative complications. No cases of tumor recurrence were identified. Conclusion Schwannoma is a rare and difficult-to-diagnose lesion. It should always be considered as a hypothesis when facing a soft-tissue tumor affecting the limbs. The Tinel sign should be regarded, given its higher correlation with complications. The patients should be informed of the possible postoperative complications, which are frequent but usually transient.

Resumo Objetivo Analisar o perfil epidemiológico e a evolução de 20 pacientes diagnosticados com schwannoma nos membros superiores e inferiores. Métodos Definiu-se um grupo de pacientes para avaliação retrospectiva, compreendendo o período entre fevereiro de 2002 e junho de 2018, no qual foram estudados e avaliados 20 prontuários de pacientes submetidos a procedimento cirúrgico devido a schwannoma; a confirmação diagnóstica foi feita pelo exame anatomopatológico. Resultados Tanto os pacientes do sexo masculino quanto do feminino foram igualmente acometidos, e a média de idade foi de 50,85 anos, variando de 12 a 77 anos. Houve predomínio do membro superior e da face flexora. O nervo mais acometido foi o ulnar, e 6 (30%) pacientes apresentaram complicações pós-operatórias transitórias. Não foi identificado nenhum caso de recidiva tumoral. Conclusão O schwannoma é uma lesão rara e de difícil diagnóstico. Deve sempre ser considerada como hipótese quando se estiver diante de um tumor de partes moles acometendo os membros. O sinal de Tinel deve ser levado em consideração por conta de sua maior correlação com as complicações. Os pacientes devem ser informados quanto às possíveis complicações pós-operatórias, que são frequentes, mas, geralmente, transitórias.

Peripheral nerve tumor, a differential diagnosis with primary neural leprosy: five case reports / Tumor de nervo periférico, diagnóstico diferencial com hanseníase neural primária: relato de cinco casos

From December 1999 to May 2015, five patients with nerve tumors were sent to Lauro de Souza Lima Institute. It was suspected that they suffered from primary neural leprosy towards the tuberculoid clinical form, a prevalence of 4.5:10000 among the new patients assessed during the study period. All of the patients had similar clinical conditions characterized by mononeuropathy with nerve tumor associated with pain, absence of skin lesions and positive Mitsuda reaction. The authors report the main clinical characteristics and complementary tests: immunologic investigation of Mitsuda's reaction and the antigen Phenolic GlicoLipid-1 test (PGL-1), bacilloscopic index, neurophysiologic study and image procedures. All patients were submitted to tumor resection and anatomopathological study. Four out of the five patients were diagnosed with peripheral nerve tumor (one of them with malignant schwannoma, two of them with benign schwannomas and the other with neural fibrolipoma), whereas the fifth patient was diagnosed with tuberculoid leprosy.

No período de dezembro de 1999 a maio de 2015, foram encaminhados ao Instituto Lauro de Souza Lima (ILSL) cinco pacientes com tumoração em nervos suspeitos de hanseníase neural primária (HPN) da forma clinica tuberculoide, uma prevalência de 4,5/10000 entre os casos novos atendidos nesse período. Todos os pacientes apresentavam quadro clínico semelhante caracterizado por mononeuropatia com tumoração do nervo associada à dor, ausência de lesões de pele e reação de Mitsuda positiva. Os autores relatam as principais características clinicas e os exames complementares: investigação imunológica da reação de Mitsuda e o teste do antigeno Glicolipídeo-Fenólico-1, índice baciloscópico, avaliação neurofisiológica e estudos de imagem. Todos os pacientes foram submetidos a ressecção cirúrgica do tumor e estudo anatomopatológico. Dentre os cinco pacientes, quatro foram diagnosticados como tumor de nervo periférico (um Schwannoma maligno, dois Schwannomas benignos e um fibrolipoma neural) e um como hanseníase tuberculoide.

Ancient schwannoma of vagus nerve mimicking hamartoma.

Schwannomas arising from vagus nerve sheath are rare mediastinal neurogenic tumours. Schwannomas usually arise from left hemithorax. Unlike a hamartoma, radiologically, calcification is rarely seen in schwannomas. We present the rare case of an ancient schwannoma arising from vagus nerve sheath from the right hemithorax presenting with gross calcification.

Management of desmoid-type fibromatosis involving peripheral nerves / Tratamento da fibromatose tipo desmoide envolvendo nervos periféricos

Desmoid-type fibromatosis is an uncommon and aggressive neoplasia, associated with a high rate of recurrence. It is characterized by an infiltrative but benign fibroblastic proliferation occurring within the deep soft tissues. There is no consensus about the treatment of those tumors. We present a surgical series of four cases, involving the brachial plexus (two cases), the median nerve and the medial brachial cutaneous nerve. Except for the last case, they were submitted to multiple surgical procedures and showed repeated recurrences. The diagnosis, the different ways of treatment and the prognosis of these tumoral lesions are discussed. Our results support the indication of radical surgery followed by radiotherapy as probably one of the best ways to treat those controversial lesions.

A fibromatose do tipo desmoide é uma lesão tumoral agressiva e rara, associada a alto índice de recorrência. É caracterizada pela fibroblástica infiltrativa, porém benigna, que ocorre no interior de tecidos moles profundos. Não existe consenso com relação ao tratamento desses tumores. Apresentamos uma série cirúrgica de quatro casos comprometendo o plexo braquial (dois casos), o nervo mediano e o nervo cutâneo medial do braço. Com exceção do último caso, todos foram submetidos a múltiplos procedimentos cirúrgicos e apresentaram recorrências repetidas. São discutidos o diagnóstico, as diferentes formas de tratamento e o prognóstico dessas lesões tumorais. Nossos resultados apoiam o conceito de que cirurgia radical seguida por radioterapia é uma das melhores formas de se tratar essas controvertidas lesões.

Charcot-Marie-Tooth 1A Concurrent with Schwannomas of the Spinal Cord and Median Nerve

We identified Charcot-Marie-Tooth disease type 1A (CMT1A) in a family with schwannomas in the spinal cord and median nerve. The CMT1A in this family showed an autosomal dominant pattern, like other CMT patients with PMP22 duplication, and the family also indicated a possible genetic predisposition to schwannomas by 'mother-to-son' transmission. CMT1A is mainly caused by duplication of chromosome 17p11.2-p12 (PMP22 gene duplication). A schwannoma is a benign encapsulated tumor originating from a Schwann cell. A case of hereditary neuropathy with liability to pressure palsies (HNPP) concurrent with schwannoma has been previously reported. Although it seems that the co-occurrence of CMT1A and schwannomas in a family would be the result of independent events, we could not completely ignore the possibility that the coincidence of two diseases might be due to a shared genetic background.

Aspectos histoquímicos e imunoistoquímicos nos neoplasmas do sistema nervoso periférico / Histochemical and immunohistochemical features of peripheral nerve neoplasms

Doze casos de tumores do sistema nervoso periférico, sete schwannomas e cinco neurofibromas foram diagnosticados entre 1964 e 2004, em caninos e bovinos. Histologicamente, as células predominantes nos schwannomas eram fusiformes organizavam-se em paliçada ou em feixes aleatórios. Nos neurofibromas as células neoplásicas eram fusiformes e se originavam da periferia dos nervos formando ninhos e feixes. O tecido conjuntivo era mais abundante nos neurofibromas e os colágenos dos tipos I e III eram os principais constituintes desses neoplasmas. Os neurofibromas foram caracterizados por uma concentração alta e difusa de mastócitos, provavelmente devido à origem das células neoplásicas do perineuro e epineuro. A coloração de AgNOR não se mostrou eficiente como indicador de prognóstico nos neoplasmas analisados. Imunoistoquímicamente houve forte marcação para vimentina (100 por cento) e S100 (100 por cento) em ambos os tipos de tumor. A maioria dos schwannomas (75 por cento) foi positiva para a proteína ácida fibrilar glial; os neurofibromas só apresentaram marcação nas células de Schwann dos fascículos nervosos

A retrospective study of peripheral nerve tumors was made from 1964 to 2004. The tumors summed up 12, being seven schwannomas and five neurofibromas. Schwannomas were composed of spindle-shaped cells either in a palisading pattern or random bundles loosely textured and neurofibromas by spindle cells. The connective tissue components were more prevalent in neurofibromas with a characteristic deployment of collagens type I and III. In AgNOR techniques, both benign and malignant schwannomas and neurofibromas did not show differences. Mast cells stained by toluidine blue were more prevalent in neurofibromas which are rich in reactive endoneurium. Schwannomas (100 percent) and neurofibromas (100 percent) were positive for vimentin and S100 protein, so they prove to be reliable for the diagnosis of peripheral nerve tumors. GFAP marked cells were found in three schwannomas and in Schwann cells within neurofibromas

Abnormal dip phenomenon: a characteristic electrophysiological marker in interdigital neuropathy of the foot

OBJECTIVE: The nerve conduction findings in interdigital neuropathy of the foot (IDN; Morton's neuroma) have rarely been reported. We analyzed the nerve conduction data in 23 patients with suspected IDN studied between 1982 and 2002. METHOD: Diagnosis of IDN was made on the basis of clinical features. All patients underwent routine nerve conduction studies and a near-nerve needle sensory nerve conduction study of the interdigital nerves by Oh's method in the symptomatic foot. RESULTS:Of the 23 patients, the diagnosis of definite IDN was made in 13 cases and of possible NDN in the others cases. Nineteen were females. Twenty two patients had only one nerve affected. One patient had two nerves affected. The III-IV interdigital nerve was affected in 17 cases and the II-III interdigital nerve in 7 cases. The near-nerve needle nerve conduction showed abnormality in the affected interdigital nerves in all definite IDN cases and confirmed the diagnosis of IDN in 10 cases by the abnormal dip phenomenon (a selective decrease of 50 percent or more in the sensory CNAP amplitude of the affected nerve compared with that of the preceding interdigital nerve). In 11 possible IDN cases, IDN was identified by the abnormal dip phenomenon. CONCLUSION: The near-nerve needle sensory nerve conduction of the interdigital nerves is a highly sensitive diagnostic test, and abnormal dip phenomenon is the most characteristic electrophysiological marker for the diagnosis of IDN.

OBJETIVO: Os achados da condução nervosa na neuropatia interdigital do pé (NIP) têm sido raramente descritos. Nós analisamos os dados da condução nervosa de 23 pacientes com suspeita de NIP entre 1982 e 2002. MÉTODO: O diagnóstico de NIP foi clínico. Todos os pacientes foram submetidos a estudos de condução nervosa de rotina e ao estudo de condução sensitiva dos nervos interdigitais com agulha justa-neural pelo método de Oh. RESULTADOS: Dos 23 pacientes, o diagnóstico de NIP foi definitivo em 13 casos é possível nos demais. Dezenove pacientes eram mulheres e 22 tinham somente um nervo afetado. Apenas um paciente teve dois nervos comprometidos. O nervo interdigital III-IV foi afetado em 17 casos e o nervo interdigital II-III em 7 casos. A condução nervosa justa-neural foi anormal nos nervos interdigitais acometidos em todos os casos definitivos e confirmou o diagnóstico de neuropatia interdigital em 10 casos pelo fenômeno da diminuição de amplitude anormal (uma diminuição seletiva de 50 por cento ou mais da amplitude do PANS do nervo afetado quando comparado com o nervo anterior. Em 11 casos possíveis, a neuropatia interdigital foi identificada pelo fenômeno da diminuição de amplitude anormal. CONCLUSÃO: A condução nervosa sensitiva justa-neural dos nervos interdigitais do pé é um teste diagnóstico altamente sensível e o fenômeno da diminuição da amplitude anormal é o marcador eletrofisiológico mais característico para o diagnóstico de neuroma de Morton.

Traumatic Neuroma around the Celiac Trunk after Gastrectomy Mimicking a Nodal Metastasis: A Case Report

Traumatic neuroma is a well-known disorder that occurs after trauma or surgery involving the peripheral nerve and develops from a nonneoplastic proliferation of the proximal end of a severed, partially transected, or injured nerve. We present a case of traumatic neuroma around the celiac trunk after gastrectomy in a 56-year-old man, which was confirmed by pathology. CT demonstrated the presence of a lobulated, homogeneous, hypoattenuating mass around the celiac trunk, mimicking a nodal metastasis.

Schwannomatosis Involving Peripheral Nerves: A Case Report

Schwannomatosis or neurilemmomatosis has been used to describe patients with multiple nonvestibular schwannomas with no other stigmata of neurofibromatosis type-2 (NF-2). In our case, schwannomatosis, multiple schwannomas were present in a 21-yr-old woman with no stigmata or family history of NF-1 or NF-2. She had no evidence of vestibular schwannoma or other intracranial tumors. Multiple peripheral tumors were found in the carotid space of the neck, and soft tissue of posterior shoulder, lower back, ankle and middle mediastinum. All of those tumors were completely limited to the right side of the body. All surgically removed tumor specimens in this patient proved to be schwannomas.

Intrasternomastoid spinal accessory nerve schwannoma: clinical and radiological correlation.

Accessory nerve schwannoma are extremely rare. The preoperative consideration of this diagnosis will lead to appropriate surgical management with preservation of motor nerve function. In this article we review the literature and report a case treated successfully based on preoperative MRI findings.

Schwannoma benigno do nervo intercostal simulando neoplastia de pulmão: relato de caso / Benign intercostal nerve schwannoma simulating pulmonary neoplasm: case report

Homem de 37 anos, branco, tabagista, foi internado com tosse seca e dor no hemitórax esquerdo (HE). Ao exame, macicez e murmúrio vesicular diminuído no terço médio do HE. A tomografia axial computadorizada de tórax revelou lesão expansiva nodular do 7º arco costal, projetando-se para o interior do HE, com densidade de parte mole e discreto realce pelo contraste. A histologia e a imuno-histoquímica foram compatíveis com schwannoma benigno. Consideramos que o diagnóstico de schwannoma benigno intercostal deve ser aventado ante toda massa intratorácica posterior ou lateral que curse com escassa sintomatologia e ausência de manifestações clínicas de malignidade.

Cavernous angioma of the cauda equina: case report

Relatamos um caso de angioma cavernoso de cauda equina em mulher de 44 anos de idade com sintomas de lombociatalgia associada a fraqueza de membros inferiores e disfunção esfincteriana vesical e anal. Exame de ressonância magnética evidenciou lesão expansiva intradural heterogênea e hiperintensa na cauda eqüina. Indicado tratamento cirúrgico com remoção completa através de laminectomia L3 e L4. O exame anatomopatológico foi compatível com angioma cavernoso. Os onze casos encontrados na literatura são revisados correlacionando a apresentação clínica, tratamento proposto e prognóstico.

Leiomyosarcoma in peripheral nerve: the first case report.

A neoplasm of the peripheral nerves can be obscured, especially during the early phase. The authors report a patient with sciatic nerve leiomyosarcoma. The patient's presentation and initial management are unique. A 51-year-old man with clinical manifestations of von Recklinghausen's disease reported numbness and weakness of the left leg for one and a half years. The symptoms gradually progressed. The symptoms were consistent with peripheral neuritis. The patient developed foot drop one month before coming to our service. Two episodes of biopsy confirmed leiomyosarcoma. A long, large sciatic nerve leiomyosarcoma was found intra-operatively, positioned from the upper thigh to the point where the tibial nerve passes beneath the upper margin of the soleus muscle. Surgical resection was done and confirmed the diagnosis. Decreased sensation was still intact after resection.

Tumores neurogénicos de nervios periféricos: estudio por imágenes / Neurogenic tumors of peripheral nerves: study by images

Se revisa la manifestación en imágenes de los tumores benignos y malignos que afectan los nervios periféricos. Se destacan sus características en diferentes modalidades en especial ultrasonografía y resonancia magnética.

Anatomía + imágenes + táctica: fórmula para abordar con éxito el espacio parafaríngeo / Anatomy + preoperative images + surgical approach selection for parapharyngeal sucessfull exeresis

Antecedentes: La bibliografía confunde la denominación del espacio parafaríngeo, originando series numéricamente grandes cuyas patología y ubicación no se ajustan a la verdadera anatomía. Objetivo: Analizar el valor de las imágenes en la selección del abordaje quirúrgico seguro y suficiente. Lugar de aplicación: Centros privado y público referenciales de cabeza y cuello. Diseño: Retrospectivo. Población: 43 enfermos con predominio masculino en relación 1:1. La edad media fue 46,2 años (17-83). El 88,3 por ciento sin tratamientos previos. Método: El 37,2 por ciento eran asintomáticos. Se utilizaron como métodos diagnósticos tomografía computarizada en el 65,1 por ciento, en menor proporción resonancia magnética, angiografía, ultrasonido, punción citológica, sialografía, tomografía lineal y radiología simple. Se utilizó abordaje cervicoparotídeo en el 48,9 por ciento, seguidos del cervical, cefalocervicoparotídeo y craneofacial. Resultados: Se efectuó parotidectomía total en el 60,4 por ciento y resección del tumor en el 39,6 por ciento. El 62,8 por ciento fueron tumores de glándulas salivales. Se produjeron 2 óbitos perioperatorios, 1 recidiva y 7 secuelas quirúrgicas. El seguimiento medio fue 76,8 meses (1-240). Conclusiones: Se requiere un perfecto conocimiento anatómico para la mejor interpretación de las imágenes y un seguro abordaje que permita la completa exéresis de éstos tumores. Debe comenzarse por una tomografía computarizada o resonancia magnética según disponibilidad, hacer angiografía en los vascularizados o punción en los sólidos