Giant Common Peroneal Nerve Schwannoma Mimicking Synovial Sarcoma: An Unusual Case Report.

Schwannoma, also known as neurilemmoma or Schwann cell tumor, is one of the most common neoplasms of the nerve sheath which usually appears at the head, neck, or upper extremity. Schwannoma occurrence in the lower extremity originating from the common peroneal nerve is rarely reported according to literary findings. We report a case of a 32-year-old man who presented with a 6-month history of a growing lump in the left knee. MRT revealed a well-defined 9.6 cm × 7.8 cm × 6.5 cm multilobular mass of heterogeneous consistency with areas of necroses with a likely diagnosis of synovial sarcoma. After surgery, a final histopathological assessment of the tumor demonstrated Antoni A and B patterns with nuclear palisading, hallmarks of a schwannoma. Postoperatively the patient suffered a neurological complication-impaired dorsiflexion of the left foot. The patient started immediate physiotherapy in the Department of Rehabilitation. Three weeks after the operation, gradual improvement in neurological function was observed. To date, complete tumor excision combined with microscopic analysis and immunohistochemical staining remains the gold standard in diagnosing and treating a peripheral nerve schwannoma. Moreover, the use of additional nerve monitoring tools during surgery could help to prevent complications.

Primary intradural Extraosseous Ewing's sarcoma of the cauda equina: A case report and literature review.

Intradural Extraosseous Ewing sarcoma (IEES) is an infrequent occurrence. We report a case of a 66-year-old male who presented with a 2-month history of low back pain and bilateral S1 sciatica, with acute sphincter dysfunction. Imaging studies revealed an intradural extramedullary lesion in the cauda equina spanning from level L4 to S1. The patient underwent partial removal of the intradural lesion. Histopathological examination showed the presence of small round cells, which were consistent with Ewing Sarcoma. The patient was then treated with targeted radiation therapy and chemotherapy. The rarity of IEES in this specific location underscores the significance of evaluating and managing patients with intradural spinal tumors with careful consideration of this diagnosis. To further investigate this condition, we conducted a thorough review of the literature on IEES involving the lumbar spine and cauda equina. Our analysis revealed that patients with this condition frequently exhibit rapidly progressive neurological symptoms likely attributed to hemorrhagic transformation. This characteristic may serve as a distinguishing factor from other lesion types, particularly benign ones. Our study provides a comprehensive summary that can offer direction for clinical management in comparable uncommon and novel cases.

[Interdisciplinary Treatment Of Tumorous And Tumour-Like Lesions Of Peripheral Nerves]. / Interdisziplinäre Behandlung von Raumforderungen in Assoziation zu peripheren Nerven: Tumore und tumorähnliche Läsionen.

Tumorous or tumour-like lesions of peripheral nerves are generally rare, heterogeneous and challenging to diagnose and treat. They may become apparent by a palpable swelling (lump) near nerves, sensory and/or motor deficits, pain to touch or neuropathic pain. In 91% of cases, tumours are benign. The differentiation of entities and their characteristics as well as a function-preserving resection strategy are highly relevant. Misdiagnosis and inadequate treatment can lead to severe deficits and pain syndromes. Benign tumours include schwannomas and neurofibromas, which can occur sporadically but can also be associated with neurogenetic tumour disposition syndromes if they occur more frequently. Rarer benign nerve tumours include perineuriomas, lipomas, aggressive fibrosis (desmoid tumours), paragangliomas and haemangiomas. Ganglion cysts are described as tumour-like lesions. The association of nerve tumours with neurogenetic syndromes and the correct classification of potentially malignant lesions such as MPNST (malignant peripheral nerve sheath tumour) or intermediate stages such as ANNUBPs (atypical neurofibromatous neoplasms with unknown biological potential) pose particular challenges. Interdisciplinarity is highly relevant for clinical treatment and a correct diagnosis. The aim of our work is to provide an overview of the relevant entities, diagnostic evaluation and contemporary treatment strategies based on the current data situation and taking into account the recently published interdisciplinary AWMF S2k guideline "Diagnosis and Treatment of Peripheral Nerve Tumours".

An algorithmic approach to the management of peripheral nerve tumours.

This article reviews the pathology and management of peripheral nerve tumours, including a framework for investigation and decision-making. Most tumours are benign, including schwannomas and neurofibromas, but malignant peripheral nerve sheath tumours can occur. The risk of malignant change is remote for schwannomas but higher for neurofibromas, particularly in neurofibromatosis type 1. Magnetic resonance imaging is useful for defining the relationship of a swelling with adjacent nerves but is not definitive for tissue diagnosis. Increasing size, pain and neurological deficit suggest malignant change and TruCut needle biopsy is indicated, although there is a risk of sampling error. Excision biopsy preserving nerve function may be carried out for benign tumours to relieve symptoms. Malignant tumours require a multidisciplinary approach. Complete surgical excision with clear margins is the only curative treatment and may be supplemented with radiotherapy and chemotherapy. However, prognosis remains poor, particularly for patients with neurofibromatosis.

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.

Tumores de los nervios periféricos: estudio retrospectivo de 66 casos / Peripheral nerve tumours: A 66-case retrospective study

ANTECEDENTES/OBJETIVOS: Los tumores de los nervios periféricos son lesiones poco frecuentes que se categorizan en general dentro de los tumores de partes blandas, por lo que suelen ser manejados por diferentes disciplinas quirúrgicas, como cirugía plástica, cirugía ortopédica y traumatológica, dermatología, cirugía general y neurocirugía. Un conocimiento apropiado en cuanto a la indicación terapéutica y el manejo quirúrgico es fundamental de cara a evitar un déficit neurológico en el paciente. MATERIALES Y MÉTODOS: Estudio restrospectivo donde se presenta la experiencia conjunta de 2 equipos quirúrgicos especializados en la cirugía de los nervios periféricos, 66 lesiones tumorales, en un total de 64 pacientes. RESULTADOS: La localización más frecuente fue el miembro inferior y el miembro superior con 48 lesiones tumorales. El diagnóstico anatomopatológico más frecuente fue el de schwannoma, con un 51,5%. La resección tumoral completa se logró en el 81,8% de las lesiones y siempre con estimulación o monitorización neurofisiológica. La complicación posquirúrgica más frecuente fue el dolor neuropático o un trastorno sensitivo transitorio en el 15% de las cirugías, apareciendo solo un déficit motor persistente en 2 casos que requirieron reconstrucción con injerto nervioso. CONCLUSIONES: Si bien el manejo terapéutico de los tumores benignos como los schwannomas puede considerarse relativamente sencillo aplicando los conceptos básicos en microcirugía de los nervios periféricos, los tumores malignos, si bien son muy infrecuentes, requieren de un manejo multidisciplinar. La opción de la biopsia prequirúrgica a día de hoy sigue siendo un tema controvertido y sin acuerdo entre los diferentes autores; en nuestra opinión, no es necesaria su realización de forma percutánea en la mayoría de los casos

BACKGROUND/OBJECTIVES: Peripheral nerve tumours (PNT) are rare lesions that are generally categorised as soft tissue tumours, so they are usually managed by a range of surgical disciplines such as plastic, orthopaedic and trauma surgery, dermatology, general surgery and neurosurgery. Appropriate knowledge of the therapeutic indication and surgical management are essential in order to avoid neurological deficit. MATERIALS AND METHODS: This is a retrospective study of 66 tumour lesions in 64 patients, acquired from the joint experience of 2 surgical teams specialised in peripheral nerve surgery. RESULTS: The most common location was the lower limb and upper limb with 48 tumour lesions. The most common pathological diagnosis was Schwannoma, accounting for 51.5%. Complete tumour resection was achieved in 81.8% of the lesions and always with neurophysiological stimulation and/or monitoring. The most common postsurgical complication was neuropathic pain and/or a transient sensory disorder in 15% of surgeries, with only one persistent motor deficit appearing in 2cases that required nerve graft reconstruction. CONCLUSIONS: Although the therapeutic management of benign tumours such as Schwannomas can be considered to be relatively simple thanks to the application of the basic concepts of peripheral nerve microsurgery, malignant tumors, despite being very rare, require multidisciplinary management. The option of pre-surgical biopsy remains a controversial issue and no consensus has been reached among the different authors. In our opinion, percutaneous biopsy is not necessary in most cases

Schwannoma del plexo cervical en una mujer de 76 años / Schwannoma of the cervical plexus in a 76-year-old woman

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Schwannoma palatino / Schwannoma palatine

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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.

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.

Problemas diagnósticos en tumores del nervio periférico (II) / Diagnostic problems of peripheral nerve tumours (II)

En esta segunda parte se revisan los criterios diferenciales entre mixomas de vainas neurales y neurotekeomas, neuromas mucosos y neuromas traumáticos y las lesiones neurales pigmentadas (neurofibroma pigmentado y schwannoma pigmentado). Asimismo, se describen los criterios de malignidad en las lesiones precursoras del tumor maligno de vaina neural periférica, los distintos tipos de lesiones de células granulares y los hallazgos diferenciales más significativos de las lesiones neurales de células fusiformes que asientan en piel y tubo digestivo(AU)

In the second part of this study, the criteria for the differentiation between neural sheath myxomas and neurothekeomas, mucous neuromas and traumatic neuromas and pigmented neural lesions (pigmented neurofibroma and pigmented schwannoma) are discussed. Furthermore, the criteria for malignancy in precursor lesions of malignant peripheral nerve sheath tumours, the various types of granular cell lesions and the most significant differential findings in neural lesions with fusiform cells occurring in the skin and digestive tract are examined(AU)

Quistes intraneurales del nervio ciático poplíteo externo en edad pediátrica: presentación de 2 casos y revisión de la literatura / Intraneural cysts of the peroneal nerve in childhood: report of 2 cases and literature review

Los quistes intraneurales son lesiones benignas de contenido mucinoso que se ubican dentro del epineuro de algunos nervios periféricos. El ciático poplíteo externo es el nervio más afectado por esta entidad, de incidencia muy baja y cuya etiopatogenia ha sido motivo de controversia. Spinner et al. en el año 2003 publican su teoría articular, que explica la formación de estas lesiones y la causa de su recidiva frecuente tras su evacuación quirúrgica. Esta teoría, a día de hoy, es la más aceptada, y desde su aplicación ha demostrado ser importante en la reducción de las recidivas y la mejora de los resultados del tratamiento de los quistes intra­neurales. La mayoría de las series de casos publicadas describen las características clínicas, radiológicas y el pronóstico de esta entidad en adultos. A través de la revisión de los casos pediátricos existentes en la litera­tura, y junto con 2 nuevos casos que presentamos, este trabajo revisa los aspectos clínicos de los quistes intra­neurales en su presentación pediátrica y las diferencias con su presentación en adultos. Es importante conocer esta entidad ya que es uno de los diagnósticos diferen­ciales que deben ser tenidos en cuenta al estudiar un paciente con un pie caído, y su tratamiento correcto en tiempo y forma redunda muchas veces en una remisión completa de la sintomatología (AU)

Intraneural cysts are benign lesions filled with muci­nous content and located inside the epineurum of the peripheral nerves. Peroneal nerve is the most affected nerve. The entity is rare and its ethiopathology still remains to be definitely elucidated. In 2003 Spinner et al published their articular theory, implicated in the formation and frequent recurrence of these lesions after surgical treatment. The practical application of this theory, nowadays almost universally accepted, gene­rated an important diminution in the recurrence rate after surgical evacuation of this lesions, previously very elevated. Most of the cases of this entity are described in adults. In the present paper we describe two pedia­tric cases and analyze the clinical aspects of these cysts in pediatric and adults patients. Peroneal intraneural cysts are one of the differential diagnoses in foot drop, and an adequate treatment concludes in a complete remission of the symptoms (AU)

Linfoma primario del nervio ciático / Primary Linfoma of the sciatic nerve

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