Surgical outcome of spinal schwannoma and neurofibroma

SUMMARY OBJECTIVE: The aim of this study was to evaluate the outcome and surgical complications in patients with spinal schwannoma or neurofibroma surgically treated at the Hospital das Clínicas of the State University of Campinas. METHODS: This was a retrospective cohort study, using medical records of patients operated between 2011 and 2021. The sample distribution was verified using the Kolmogorov-Smirnov test. The dynamics between qualitative variables were assessed using Fisher's exact test. We used means analysis to assess patient improvement based on Frankel scores. RESULTS: A total of 16 patients were evaluated, of whom 56.25% (9) were men and 43.75% (7) were women. There were 13 (81.25%) patients with schwannomas and 3 (18.75%) with neurofibromas. Patients with deficits had neurological improvement, such as walking or with at least Frankel D or E after surgery. Laminectomy, performed in 8 patients (50%), and laminoplasty, used in 9 patients (56.25%), were the main techniques. CONCLUSION: The surgical approach was proved to be an effective and safe alternative to the treatment of these tumors, with neurological improvement and minor surgical complications.

Neurofibromatosis laríngea pediátrica: reporte de localización atípica / Pediatric neurofibromatosis of the larynx: report of atypical location

Los neurofibromas laríngeos (NFL) son tumores benignos poco frecuentes de localización principalmente supraglótica. Se manifiestan con síntomas obstructivos de la vía aérea. El tratamiento es la resección completa del tumor mediante abordaje endoscópico; se reserva la cirugía abierta para tumores de gran extensión. Se presenta el caso de un paciente pediátrico con localización atípica de NFL asociado a neurofibromatosis tipo 1 (NF1). Se realizó resección endoscópica del tumor y la anatomía patológica informó neurofibroma plexiforme. Es importante sospechar de esta patología en todo niño con estridor inspiratorio atípico progresivo. Se sugiere seguimiento a largo plazo por la alta probabilidad de recidiva.

Laryngeal neurofibromas (LNFs) are rare benign tumors mainly located in the supraglottis. LNFs occur with airway obstruction symptoms. The treatment is complete resection via an endoscopic technique; the open approach is reserved for large tumors. Here we describe the case of a pediatric patient with LNF of atypical location associated with neurofibromatosis type 1 (NF-1). The tumor was resected with an endoscopic technique, and the pathological study reported a plexiform neurofibroma. It is important to suspect this condition in any child with atypical, progressive inspiratory stridor. Long-term follow-up is recommended due to the high rate of recurrence

Neurofibromatosis in the gluteus and posterior region of the thigh: case report / Neurofibromatose em glúteo e região posterior da coxa: relato de caso

Introduction: Neurofibromatosis is an autosomal dominant disorder, and type 1 is associated with an increased risk of tumor formation with neurocutaneous involvement. The variable evolution, often with limiting tumors, in addition to the significant incidence of cases requiring treatment, makes it fundamental to discuss procedures already performed in medical practice for early, careful, and individualized recognition of the diagnosis and treatment of the patient. The report aims to present a surgical case of neurofibromatosis, calling attention to the surgical technique, the characteristics of the disease, and the importance of the procedure in the quality of life of patients limited by the condition. Case Report: A 23-year-old male patient with a large mass neurofibroma in the gluteus and posterior surface of the right leg, in addition to café au lait stains in the distal third of the legs. He was treated with surgery to remove the tumor and a flap and graft in the affected region. The procedures were performed by a multidisciplinary team, allowing the total removal of the tumor mass, with subsequent skin grafting in the hip and thigh lesion on the right side and the fasciocutaneous flap in VY in the area. There were no significant complications in the immediate postoperative period. Conclusion: Neurofibromas can become limiting and impair patients' quality of life with neurofibromatosis type 1; therefore, early management and diagnosis are essential. Although the condition does not present a cure, there is a need for research into less invasive and preventive treatments for injuries.

Introdução: A neurofibromatose é um distúrbio autossômico dominante e o tipo 1 está associado a um aumento do risco de formação de tumores com acometimento neurocutâneo. A evolução variável, muitas vezes com tumorações limitantes, além da incidência significativa de casos que necessitam de tratamento, torna fundamental a discussão de condutas já realizadas na prática médica para um reconhecimento precoce, cuidadoso e individualizado do diagnóstico e do tratamento do enfermo. O relato objetiva apresentar um caso cirúrgico de neurofibromatose, chamando atenção para a técnica cirúrgica, as características da doença e a importância do procedimento na qualidade de vida de pacientes limitados pela afecção. Relato de Caso: Paciente de 23 anos, sexo masculino, com neurofibroma de grande massa em glúteo e face posterior da perna direita, além de manchas café com leite em terço distal de pernas. Foi tratado com uma cirurgia de retirada do tumor, além de retalho e enxerto na região acometida. Os procedimentos foram realizados por equipe multidisciplinar, possibilitando a retirada total da massa tumoral, com posterior realização de enxerto de pele na lesão do quadril e coxa em lado direito, e o retalho fasciocutâneo em V-Y na área. Não houve complicações significativas nos pós-operatórios imediatos.

Neurofibroma solitario en maxilar superior: reporte de caso inusual / Solitary neurofibroma in upper maxillary: an unusual case report

Los neurofibromas solitarios han sido reportados en la literatura, pero son casos muy raros; por definición se presentan en pacientes que no tienen enfermedad de Von Recklinghausen, que se hereda en forma autosómica dominante, y se presenta con mayor frecuencia en hombres. Es un tumor benigno que puede presentarse solitario o múltiple; su aparición en la cavidad oral suele ser muy rara, pero su sitio de predilección es la lengua, seguido de la mucosa alveolar, paladar y encía gingival. Aparecen con más frecuencia durante la tercera década de vida, aunque se describen casos desde los 10 meses hasta los 70 años de edad. En este reporte se expondrá el caso clínico de un paciente de sexo femenino, de 17 años de edad, que acudió a consulta a la clínica dental centenario por presentar una lesión superior que cubría parte de las coronas anatómicas en piezas dentales del maxilar superior izquierdo, además, en el reporte de estudio inmunoquimicohistológico se diagnosticó neurofibroma, con expresión de S-100. Consideramos de suma importancia el reconocimiento de estos crecimientos intraorales para lograr establecer un diagnóstico definitivo cierto y veraz de la situación (AU)

Solitary neurofibromas have been reported in the literature, but they are very rare cases. By definition they occur in patients who do not have Von Recklinghausen disease, which is inherited in an autosomal dominant manner; It occurs more frequently in men. It is a benign tumor that can appear solitary or multiple. Its appearance in the oral cavity is usually very rare, but its site of preference is the tongue, followed by the alveolar mucosa, palate, and gingival gingiva. They appear more frequently during the third decade of life, although cases from 10 months to 70 years of age are described. This report will present the clinical case of a 17-year-old female patient who came to the Centennial Dental Clinic for consultation presenting an elevated lesion covering part of the anatomical crowns in dental pieces in the left upper jaw and in the report. Neurofibroma was diagnosed from the Immunochemicalhistological study, with S-100 expression. We consider the recognition of these intraoral growths of utmost importance in order to establish a true and truthful definitive diagnosis of the situation (AU)

Pigmented neurofibroma with hypertrichosis / Neurofibroma pigmentado con hipertricosis

Abstract Background: Pigmented (or melanocytic) neurofibroma (PN) constitutes only 1% of cases and is considered a rare variant of neurofibroma containing melanin-producing cells. In addition, the association of PN with hypertrichosis is infrequent. Case report: We describe the case of an 8-year-old male with a neurofibromatosis type 1 (NF1) diagnosis, who presented a light brown hyperpigmented plaque, smooth and well-demarcated, and hypertrichosis on the left thigh. The skin biopsy showed characteristics of neurofibroma; however, in the deep portion of the lesion, melanin deposits positive for S100, Melan-A, and HMB45 were observed, thus establishing the diagnosis of pigmented neurofibroma. Conclusions: Although PN is a rare subtype of neurofibroma, it is considered a chronically progressive benign tumor containing melanin-producing cells. These lesions can appear alone or in association with neurofibromatosis. Since this is a tumor that can be confused with other skin lesions, biopsy analysis is essential to differentiate it from other pigmented skin tumors, such as melanocytic schwannoma, dermatofibrosarcoma protuberans, neurocristic hamartoma, or neuronevus. Surveillance is part of the treatment, and surgical resection is sometimes performed.

Resumen Introducción: El neurofibroma pigmentado (NP) o melanocítico constituye solamente el 1% de los casos y se considera como una variante rara del neurofibroma que contiene células productoras de melanina. Además, la asociación de NP con hipertricosis es muy rara. Caso clínico: Se describe el caso de un paciente de sexo masculino de 8 años 2 meses de edad con diagnóstico de neurofibromatosis tipo 1 (NF1), quien presentaba en la cara anterior del muslo izquierdo una placa hiperpigmentada de color café claro, bien delimitada y de consistencia suave, e hipertricosis. La biopsia de piel presentó cambios característicos de neurofibroma; sin embargo, en la porción profunda de la lesión se observaron depósitos de melanina positivos para S100, Melan-A y HMB45, con lo que se estableció el diagnóstico de neurofibroma pigmentado. Conclusiones: Aunque el NP es un subtipo raro del neurofibroma, se considera que es un tumor benigno de evolución crónica de células productoras de melanina. Estas lesiones aparecen en solitario o asociadas con neurofibromatosis. Dado que es un tumor que puede confundirse con otras lesiones cutáneas, es fundamental el análisis de la biopsia para diferenciarlo de otros tumores cutáneos pigmentados, como el schwanoma melanocítico, dermatofibrosarcoma protuberans, hamartoma neurocrístico o neuronevus. La vigilancia es parte del tratamiento y, en ocasiones, se lleva a cabo la resección quirúrgica.

Neurofibromatosis Type 1 in a Child with Plexiform Neurofibroma Pressing the Urinary System / 罕见病研究

A 3-year-old male patient was diagnosed with neurofibromatosis type 1(NF1) for two years. The patient has multiple neurofibromas in retroperitoneum, lumbococcygeal paravertebral, lumbosacral spinal canal, and foramina. Due to retroperitoneal mass compression, the child suffered from urological complications such as hydronephrosis, ureterdilation, neurogenic bladder, etc., which seriously affected the urination function and resulted in multiple surgical treatments. Currently, the patient has been treated with mitogen activates extracelluar signal-regulated kinases(MEK) inhibitor selumetinib targeted therapy, and has voluntarily urinated, and his general state is better than before medication. The diagnosis and treatment of this case reflects the importance of multidisciplinary collaboration in the diagnosis and treatment of rare diseases.

Histopathological Spectrum of Myxoid Soft‑Tissue Neoplasms in a Tertiary Care Center with a Special Focus on Vascular Patterns: A 13‑Year Compilation

Introduction: Myxoid soft‑tissue tumors are a diverse group of tumors which have similar histomorphology but have varied geneticsequence and clinical outcome, hence differentiating and diagnosing them is a challenge for any pathologist. This study describes the varioushistomorphological spectrum and vascular pattern of various myxoid soft‑tissue tumors. Materials and Methods: This was a retrospective and prospective observational study of myxoid soft‑tissue tumors over a period of 13 years. A total of 224 cases with myxoid morphology were included and were examined morphologically with a special focus on the vascular pattern. SPSS v 24 was used for statistical analysis. Results: The predominant lesions were benign in 164 (73.21%) cases, followed by malignant lesions in 43 (19.19%) cases and intermediate lesions 17 (7.58%) cases. Both benign and malignant lesions showed a male preponderance and were seen to arise predominantly from the extremities. The most common benign myxoid lesions in this study were of neural origin with myxoid neurofibroma constituting 65 (29. 01%) cases, followed by schwannoma 38 (16.9%) cases. Myxoid dermatofibrosarcoma protuberans was the most common intermediatelesion. Tumors with adipocytic differentiation were the predominant lesions among the malignant group, i.e myxoid liposarcoma seen in 17 (7.5%) cases. Conclusions: Vascular pattern in the myxoid lesions are subtle yet crucial in arriving at a histo‑morphological diagnosis. Further studies correlating the vascular pattern with the genetic profile of these tumours can help arriving at a histo‑morphological diagnosisof myxoid lesions.

Histopathological Approach To The Diagnosis Of Benign Peripheral Nerve Sheath Tumors At Uncommon Location

PARIPEX - INDIAN JOURNAL F RESEARCH |O December - 202Volume - 11 | Issue - 12 | 2 | PRINT ISSN No. 2250 - 1991 | DOI : 10.36106/paripex ABSTRACT Peripheral nerve sheath tumors are relatively common lesions that exhibit a wide morphological and biological spectrum. In the presence of classical morphological and immunohistochemical features, the histological diagnosis is usually straightforward, but they may represent diagnostic challenges. Schwannoma is a slow growing encapsulated tumor of neuroectodermal derivation that originates from the Schwann cells of the neural sheath. Most commonly presents as cerebellopontine angle mass but extracranial sites are known and needs to be considered in the differential diagnosis. Intraparotid and penile schwannomas are extremely rare. Their diagnosis could be missed on cytology due to cystic changes. Isolated plexiform neurofibroma of the tongue is again a rare tumor. Plexiform pattern recognition is important for the pathologist as these varients might show malignant transformation. Overall Fine needle aspiration cytology though inconclusive at times, help a lot initially to delineate between benign Vs malignant lesions. Moreover its minimally invasive so can be performed easily at the uncommon locations. Histopathology remains the gold standard. Clinicopathological and imaging correlation is must for definite diagnosis and treatment.

Neurofibromatosis tipo 1 y manifestación en el Territorio Maxilofacial: Reporte de un caso y Revisión de la Literatura

Introducción: El neurofibroma corresponde a un tumor benigno que compromete la vaina neural del tejido nervioso, asociándose íntimamente a la neurofibromatosis. Debido al compromiso de nervios periféricos y/o centrales, su expresión clínica es muy variada producto de la compresión y/o desplazamiento de estructuras vecinas dificultando así su diagnóstico. Objetivo: El objetivo de este artículo, es el de realizar una revisión de la literatura en relación con la neurofibromatosis y sus manifestaciones en el territorio máxilofacial en conjunto con la presentación de un caso de hipercondilismo mandibular asociado a un neurofibroma en la región de la articulación temporomandibular en un paciente con antecedentes de neurofibromatosis. Métodos: Se presenta el caso de un paciente con laterognasia y antecedentes de Neurofibromatosis tipo I (NF1). Por medio de estudio imagenológico, se confirma Hipercondilismo derecho y presencia de una zona radiolúcida relacionada con el cuello del cóndilo comprometido, cuyo resultado histopatológico confirmo el diagnóstico de neurofibroma. Conclusiones: Existe una muy variada clínica en pacientes con NF1, presentando una predisposición a la formación de neurofibromas y alteraciones oseas que pudiesen comprometer el territorio máxilofacial y causar asimetrías faciales. Debido a esto, resulta imprescindible tener conocimiento y consideración de ambas patologías para una correcta planificación del tratamiento de los pacientes.

Introduction: Neurofibroma is a benign tumor that compromises the neural sheath of nerve tissue, intimately associated with Neurofibromatosis. Due to the involvement of peripheral and/or central nerves, its clinical expression is wide as a result of compression and/or displacement of neighboring structures, making its diagnosis difficult. Objective: The aim of this article is to review the literature on Neurofibromatosis and its manifestations in the maxillofacial territory, along with the presentation of a case of mandibular Hyperchondylism associated with a neurofibroma in the temporomandibular joint region in a patient with a history of Neurofibromatosis. Methods: We present the case of a patient with laterogenesis and a history of Neurofibromatosis type I (NF1). By imaging study, a right Hyperchondylism is detected along with the presence of a radiolucent area related to the neck of the compromised condyle, whose histopathological result confirmed the diagnosis of neurofibroma. Conclusions: There is a diverse clinical picture in patients with NF1, presenting a pre-disposition to neurofibromas development and bone abnormalities, leading to the compromise of the maxillofacial territory and causing facial asymmetries. Because of this, it is essential to have knowledge and consideration of both pathologies for the right planning of patient treatment.

PENILE NEUROFIBROMA IN A 10-YEAR - OLD CHILD: A RARE CASE STUDY

Neurofibromas of the penis occur uncommonly and their solitary occurrence is even rarer. We report a case of a ten year old child presenting with a solitary neurofibroma located on penis. A provisional clinical diagnosis of penile retention cyst was made and histopathological examination releaved a benign spindle cell lesion and few close differential diagnosis were made, which on application of immunohistochemistry was diagnosed as neurofibroma of penis. Surgical excision of the lesion was done and there was no recurrence after a year of follow up.

Neurofibromatosis laríngea: a propósito de un caso / Laryngeal neurofibromatosis: a case report

Resumen El neurofibroma laríngeo es poco frecuente, representa menos del 0,1% de las neoplasias benignas de la laringe. Puede presentarse aislado, o más comúnmente asociado a neurofibromatosis tipo I. Se presenta el caso de un paciente varón de 40 años, ya diagnosticado de neurofibromatosis tipo I, que presenta masa supraglótica submucosa asintomática, diagnosticada como hallazgo casual en una intubación por una cirugía previa programada.

Abstract Laryngeal neurofibroma is rare, representing less than 0.1% of benign tumors of the larynx. It can occur in isolation or more commonly associated with type I neurofibromatosis. The case of a 40-year-old male patient, already diagnosed with type I neurofibromatosis, is presented with an asymptomatic submucosal supraglottic mass, diagnosed as a chance finding in intubation due to a previous scheduled surgery.

Reconstrução do antebraço com matriz dérmica acelular após excisão de neurofibroma plexiforme gigante: relato de casos / Forearm reconstruction with acellular dermal matrix after giant plexiform neurofibroma excision: case reports

Introdução: O neurinoma plexiforme gigante é um neuroectoderma e uma doença hereditária. É um tumor cutâneo incomum associado à NF1, caracterizado como um tumor benigno da bainha do nervo periférico envolvendo múltiplos fascículos nervosos. Os objetivos da reconstrução da cobertura do antebraço são proteger as estruturas que vão até o punho e a mão e evitar cicatrizes que levem à perda de movimento. Tanto o antebraço quanto a mão desempenham papéis funcionais e sociais. O manejo bem-sucedido de feridas complexas é necessário para a reabilitação funcional geral desses pacientes. Relato do Caso: Paciente do sexo feminino, 31 anos, apresentou-se na divisão de cirurgia plástica com neurofibroma plexiforme gigante no antebraço direito. Após ressecção cuidadosa, todos os tendões anteriores do antebraço foram expostos. O defeito foi coberto com Pelnac T enxertável (espessura de 3mm e tamanho 12 X 24cm2), fixados com pontos monocryl 4-0. Após 10 dias, a matriz dérmica acelular foi removida e um enxerto de malha de pele de espessura parcial foi colocado. No dia 7, a matriz dérmica acelular apresentou bons sinais de ingestão. No dia 17, observamos uma sobrevida do enxerto de 95%. No seguimento de 3 meses, a reconstrução estava estável, sem defeitos de contorno, a mão apresentava amplitude de movimento completa e o paciente não apresentava problemas nas atividades diárias. Conclusões: A matriz dérmica acelular parece ser uma opção útil na cobertura de defeitos complexos no antebraço, permitindo menor morbidade e rápida recuperação funcional.

Introduction: Giant plexiform neurinoma is a neuroectoderm and inherited disease. It is an uncommon skin tumor associated with NF1, characterized as a benign peripheral nerve sheath tumor surrounding multiple nervous fascicles. The goals of forearm coverage reconstruction are to protect the structures running to the wrist and hand and prevent scarring that leads to movement loss. Both forearm and hand play functional and social roles. Successful management of complex wounds is necessary for the overall functional rehabilitation of these patients. Case Report: A 31-year-old woman presented at the plastic surgery division with a giant plexiform neurofibroma in the right forearm. After careful resection, all anterior forearm tendons were exposed. The defect was covered with graftable Pelnac T (thickness of 3mm and sizing 12 X 24cm2), fixed with 4-0 monocryl sutures. After 10 days, the acellular dermal matrix silicone layer was removed, and a split-thickness skin meshed graft was placed. On day 7, the acellular dermal matrix showed good signs of intake. On day 17, we observed a 95% graft survival. At the 3-month follow-up, reconstruction was stable without contouring defects, the hand had full range of motion, and the patient had no problems in daily activities. Conclusions: Acellular dermal matrix appears to be a useful option in covering complex defects in the forearm, allowing for less morbidity and rapid functional recovery.

Multiple strategies to avoid life-threatening blood loss during intralesional resection of a giant Plexiform Neurofibroma: A case report

@#Giant plexiform neurofibroma (PNs) are benign peripheral nerve sheath tumors known to contain multiple fascicles of nerve and numerous friable vascular components. Most consult due to significant disfigurement and functional deficit. Though surgery is the current standard of therapy, there is high reservation in pushing through with resection in most cases. The reservation stems from the recognized difficulty in controlling intraoperative life-threatening hemorrhage. A 25-year-old female came in our institution due to multiple debilitating giant PNs on her scalp, back, neck, shoulder, and chest. She opted for debulking surgery despite possible complications and recurrence. Multiple modalities used to prevent massive bleeding in this case included preoperative arterial embolization, energy sealing device, cutting linear stapler, and interlocking retention sutures. The aim of this case report was to discuss the utility of each of these techniques, the advantages and disadvantages of each approach based on our experience.

Deep Vein Thrombosis in the Setting of Neurofibromatosis Type 1: Case Report

Neurofibromatosis type 1 (NF1) is a genetic syndrome which typically presents with neurological manifestations. Some of the patients may also present with vasculopathies, among which arterial aneurysms and stenosis are the most common. Deep vein thrombosis (DVT) has rarely been described, and, to the best of our knowledge, the present is the first report of DVT due to venous compression by a neurofibroma in the setting of NF1. This is the case of a 23-year-old male with NF1 who experienced DVT due to compression of the left posterior tibial veins by a large tumor arising from the tibial nerve. The DVT was acutely treated with enoxaparin and then with rivaroxaban. Two months after the diagnosis, Doppler ultrasonography showed partial recanalization and persistence of the DVT. The patient was then referred to neurosurgery for surgical resection of the tumor. There were no complications during the procedure, and the patient did not present postoperative neurological deficits. The final histopathological diagnosis was of a benign neurofibroma. After one year of follow-up with vascular surgery, the patient presented no more episodes of DVT. In case there is a tumor compressing the deep vessels of the leg and promoting DVT, surgical resection with microsurgical techniques may be curative.

Neurofibroma plexiforme gigante del nervio ciático en una adulta burkinesa / Giant plexiform neurofibroma of the sciatic nerve in a Burkinabé adult

Se presenta el caso de una paciente de 36 años de edad con antecedente patológico de enfermedad de Von Reklinghausen, quien fue asistida en el Servicio de Cirugía General del Centro Hospitalario de Kossodô en Burkina Faso por presentar un tumor gigante en la región posterior del muslo derecho. Los exámenes complementarios confirmaron el presunto diagnóstico de neurofibroma plexiforme gigante del nervio ciático. Durante el procedimiento quirúrgico se extirpó un tumor infrecuente cuyo peso excedió los 22,5 kg. Con el tratamiento rehabilitador posoperatorio del miembro operado la paciente evolucionó satisfactoriamente y se le dio el alta hospitalaria 7 días después.

The case report of a 36 years patient with pathological history of Von Reklinghausen disease is presented. She was assisted in the General Surgery Service of the Hospital Center from Kossodô in Burkina Faso presenting a giant tumor in the back region of the right thigh. The complementary exams confirmed the presumed diagnosis of giant plexiform neurofibroma of the sciatic nerve. During the surgical procedure an uncommon tumor was removed which weight exceeded the 22.5 kg. With the postoperative rehabilitative treatment of the operated member the patient had a favorable clinical course and she was discharged from the hospital 7 days later.

Neurofibroma de la vía biliar: una causa rara de ictericia colestásica que imita el tumor de Klatskin

El neurofibroma de la vía biliar es una enfermedad extremadamente rara y generalmente asintomática, sin embargo, puede ocasionar ictericia obstructiva y simular un tumor de Klatskin conduciendo a un tratamiento quirúrgico radical y mayor morbilidad del paciente. Presentamos el caso de una mujer de 62 años colecistectomizada hace 10 años, con ictericia y dolor en hipocondrio derecho, así como exámenes auxiliares compatibles con colestasis. Se observó dilatación de la vía biliar y presencia de un tumor en el conducto hepático izquierdo mediante colangioresonancia magnética. El diagnóstico clínico preoperatorio fue Colangiocarcinoma hiliar, pero el estudio histopatológico reveló una neoplasia compuesta por células fusocelulares sin atipia ni actividad mitótica, cuya estirpe neurogénica se sustentó por su positividad a proteína S100 en inmunohistoquímica. Reportamos el caso dada su poca frecuencia en la literatura y su relevancia, al ser una entidad benigna, como diagnóstico diferencial de cáncer.

The neurofibroma of the bile duct is an extremely rare and generally asymptomatic disease, however, it maybe cause obstructive jaundice and mimic a Klatskin tumor, leading to radical surgical treatment and increased patient morbidity. We present the case of a 62-year-old woman who underwent cholecystectomy 10 years ago, with jaundice and pain in the right upper quadrant, as well as auxiliary tests compatible with cholestasis. Dilation of the bile duct and the presence of a tumor in the left hepatic duct were observed by magnetic cholangioresonance. The preoperative clinical diagnosis was hilar cholangiocarcinoma, but the histopathological study revealed a neoplasm composed of spindle cells without atypia or mitotic activity, whose neurogenic lineage supported by its positivity to protein S100 in immunohistochemistry. We report the case given its infrequency in the literature and its relevance, as it is a benign entity, as a differential diagnosis of cancer.

Atypical oral features of patient with neurofibromatosis type 1: case report / Características orais atípicas de paciente com neurofibromatose tipo 1: relato de caso

RESUMEN El propósito de este artículo es ayudar a los dentistas clínicos a identificar la neurofibromatosis tipo 1 y sus variantes. Presentamos un caso raro de un hombre de 68 años, afectado desde la infancia por la presencia de nódulos generalizados en todo el cuerpo, provocando deformaciones estéticas. Curiosamente, el impacto en la cavidad oral no fue significativo, con nódulos solo en la encía, un área de manifestación raro. La radiografía panorámica mostró un ligero agrandamiento del canal mandibular. Tras un año de seguimiento, el paciente falleció por complicaciones cardiovasculares provocadas por el síndrome.

Mucosal Schwann cell hamartoma of the gallbladder

Mucosal Schwann cell hamartoma (MSCH) is a rare benign neurogenic tumor characterized by pure S100p positive spindle cell proliferation. Most cases occur in the distal colon. Involvement of the gall bladder is exceedingly rare. There have been no reports of recurrence or a syndromic association with MSCH. Herein, we describe a case of MSCH of the gallbladder in a 55-year-old female patient with prior history of gastrointestinal neurofibromas who presented with abdominal pain. MR imaging revealed choledocholithiasis, gallbladder thickening, and marked biliary and pancreatic ductal dilation. The patient subsequently underwent cholecystectomy with choledochoduodenostomy. Histologic evaluation of the gallbladder showed diffuse expansion of the mucosa with S100p positive cells with spindly nuclei and indistinct cytoplasmic borders and diagnosis of MSCH of the gallbladder was rendered.

Clinical analysis of multi-disciplinary team management in the treatment of giant neurofibroma in maxillofacial and neck region / 口腔疾病防治

Objective @#To investigate the multi-disciplinary team (MDT) management in the treatment of giant neurofibroma in maxillofacial and neck region, to provide reference for clinical practice.@*Methods@#Retrospective analysis was conducted on the perioperative whole-process management process of 2 cases of giant neurofibroma in maxillofacial and neck region jointly formulated treatment plan by oral and maxillofacial surgery department with the assistance of the department of anesthesiology, ICU, vascular surgery, thoracic surgery, etc.@*Results@#MDT treatment process (anesthesia-embolization-collaborative surgery-ICU-post-operative management) of the two patients was smoothly conducted according to the pre-operative plan. There were no adverse events or accidents that were not predicted by the risk assessment from multiple teams during the operation, and no serious complications occurred after the operation. The post-operative pathological report of both cases was "neurofibroma". Wounds in both patients healed in stage I. The course of treatment was smooth, and the surgical treatment was completed without serious complications. @*Conclusion@# MDT management can play a positive role in the diagnosis and treatment of giant maxillofacial and neck neurofibroma so that patients can obtain safer and more effective diagnosis and treatment.

Solitary intratracheal neurofibroma: a rare tracheal tumor

Intratracheal neurofibromas are rare benign tumors with a tendency to transform into malignant form. Intratracheal neurofibroma remain asymptomatic and are incidentally found during routine radiography or may present with wheeze, cough and lower respiratory tract infection. We recently operated a 38 year old gentleman, who presented with progressive shortness of breath and chronic cough for two years. Examination was normal. CT identified a supracarinal, intratracheal mass. Rigid bronchoscopy revealed a hyper vascular mass, attached to membranous trachea. We performed resection anastomosis via right posterolateral thoracotomy. Histopathology revealed neurofibroma. Patient recovered well and was discharged after eight days.