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BACKGROUND: Ganglioneuromas are histologically benign neoplasms derived from the sympathetic nervous system, whose occurrence in the gastrointestinal tract is rare and often syndromic. According to the injury pattern and extension, lesions are divided into polypoid ganglioneuroma, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. This work aimed to present the incidental post mortem finding of diffuse ganglioneuromatosis of the gastrointestinal tract in a patient without syndromic involvement. CASE REPORT: We describe the case of a two-year-old female patient with surgically corrected type III tracheoesophageal atresia and fistulous recanalization, multiple episodes of aspiration pneumonia, and septic shock. During the last admission, she developed massive pulmonary hemorrhage and multi-organ failure. Post mortem histopathological study identified hypertrophy of the pylorus and enlarged enteric nerve trunks and plexuses with intermingled mature ganglion cells. We identified ganglioneuromatosis affecting all gastrointestinal tract segments with the predominance of the myenteric plexuses. CONCLUSIONS: Intestinal ganglioneuromatosis is a rare disease with a spectrum of lesions ranging from isolated to syndromic with high morbidity and mortality. Therefore, it is necessary to know the condition, investigate systematically when it is suspected, and rely on genetic studies to confirm or rule out any syndromic association.

INTRODUCCIÓN: Los ganglioneuromas son neoplasias histológicamente benignas derivadas del sistema nervioso simpático, cuya ocurrencia en el tubo digestivo es rara y comúnmente sindromática. De acuerdo con el patrón de la lesión y la extensión se dividen en ganglioneuroma polipoide, poliposis ganglioneuromatosa y ganglioneuromatosis difusa. El objetivo de este trabajo es presentar el hallazgo incidental post mortem de ganglioneuromatosis difusa del tubo digestivo en una paciente sin afectación sindromática. CASO CLÍNICO: Se describe el caso de un paciente de sexo femenino de 2 años con atresia traqueoesofágica tipo III corregida quirúrgicamente que cursó con recanalización fistulosa, múltiples episodios de neumonía por aspiración y choque séptico. Durante el último ingreso cursó con hemorragia pulmonar masiva y falla multiorgánica. En el estudio post mortem se identificó hipertrofia del píloro y de los troncos y plexos nerviosos entéricos con células ganglionares maduras entremezcladas. Se identificó ganglioneuromatosis que afectaba todos los segmentos del tubo digestivo, con predominio de los plexos mientéricos. CONCLUSIONES: La ganglioneuromatosis intestinal es una rara enfermedad que presenta un espectro de lesiones desde una forma aislada hasta sindromática con morbimortalidad elevada. Por ello, es necesario conocer la enfermedad, indagar sistemáticamente cuando se sospeche y apoyarse de estudios genéticos que confirmen o descarten alguna asociación sindromática.

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Resumen Introducción: Los ganglioneuromas son neoplasias histológicamente benignas derivadas del sistema nervioso simpático, cuya ocurrencia en el tubo digestivo es rara y comúnmente sindromática. De acuerdo con el patrón de la lesión y la extensión se dividen en ganglioneuroma polipoide, poliposis ganglioneuromatosa y ganglioneuromatosis difusa. El objetivo de este trabajo es presentar el hallazgo incidental post mortem de ganglioneuromatosis difusa del tubo digestivo en una paciente sin afectación sindromática. Caso clínico: Se describe el caso de un paciente de sexo femenino de 2 años con atresia traqueoesofágica tipo III corregida quirúrgicamente que cursó con recanalización fistulosa, múltiples episodios de neumonía por aspiración y choque séptico. Durante el último ingreso cursó con hemorragia pulmonar masiva y falla multiorgánica. En el estudio post mortem se identificó hipertrofia del píloro y de los troncos y plexos nerviosos entéricos con células ganglionares maduras entremezcladas. Se identificó ganglioneuromatosis que afectaba todos los segmentos del tubo digestivo, con predominio de los plexos mientéricos. Conclusiones: La ganglioneuromatosis intestinal es una rara enfermedad que presenta un espectro de lesiones desde una forma aislada hasta sindromática con morbimortalidad elevada. Por ello, es necesario conocer la enfermedad, indagar sistemáticamente cuando se sospeche y apoyarse de estudios genéticos que confirmen o descarten alguna asociación sindromática.

Abstract Background: Ganglioneuromas are histologically benign neoplasms derived from the sympathetic nervous system, whose occurrence in the gastrointestinal tract is rare and often syndromic. According to the injury pattern and extension, lesions are divided into polypoid ganglioneuroma, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. This work aimed to present the incidental post mortem finding of diffuse ganglioneuromatosis of the gastrointestinal tract in a patient without syndromic involvement. Case report: We describe the case of a two-year-old female patient with surgically corrected type III tracheoesophageal atresia and fistulous recanalization, multiple episodes of aspiration pneumonia, and septic shock. During the last admission, she developed massive pulmonary hemorrhage and multi-organ failure. Post mortem histopathological study identified hypertrophy of the pylorus and enlarged enteric nerve trunks and plexuses with intermingled mature ganglion cells. We identified ganglioneuromatosis affecting all gastrointestinal tract segments with the predominance of the myenteric plexuses. Conclusions: Intestinal ganglioneuromatosis is a rare disease with a spectrum of lesions ranging from isolated to syndromic with high morbidity and mortality. Therefore, it is necessary to know the condition, investigate systematically when it is suspected, and rely on genetic studies to confirm or rule out any syndromic association.

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Composite pheochromocytoma (CP) is a very rare tumor originating from neural crest cells, predominantly composed of pheochromocytoma (PCC), a chromaffin cell tumor arising in adrenal medulla, and ganglioneuroma, a tumor derived from autonomic ganglion cells of the nervous system. Moreover, CP may be present in the hereditary syndromes of which pheochromocytoma is part. Literature offers scarce data on this subject, and particularly about its biological behavior, clinical evolution, and molecular profile. We report the phenotype and outcome of three cases of CP (PCC and ganglioneuroma components), followed up at the Endocrine Service of the Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro, UFRJ, Rio de Janeiro, Brazil. Two nonsyndromic patients (cases 1 and 2) were negative to germline mutations in genes VHL, SDHB, SDHC, SDHD, SDHAF2, TMEM127, and MAX, while the third case (case 3) had clinical diagnosis of neurofibromatosis syndrome. Cases 1, 2, and 3 were diagnosed at 29, 39, and 47 years old, respectively, and were followed up for 3, 17, and 9 years without no CP recurrence. All cases had apparent symptoms of catecholaminergic excess secreted by PCC. Ganglioneuroma, the neurogenic component present in all three cases, had a percentage representation ranging from 5% to 15%. Tumors were unilateral and large, measuring 7.0 cm × 6.0 cm × 6.0 cm, 6.0 cm × 4.0 cm × 3.2 cm, and 7.5 cm × 6.0 cm × 4.5 cm, respectively. All cases underwent adrenalectomy with no recurrence, metastasis, or development of contralateral tumor during follow-up. Genetic testing has been scarcely offered to CP cases. However, a similar frequency of genetic background is found when compared with classic PCC, mainly by the overrepresentation of NF1 cases in the CP subset. By literature review, we identified a notorious increase in cases reported with CP in the last decade, especially in the last 3 years, indicating a recent improvement in the diagnosis of this rare disorder in clinical practice.

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RESUMEN Los tumores de origen neuroectodérmico son la principal causa de tumores de mediastino. De ellos el ganglioneuroma es el de mayor benignidad y el menos frecuente, con una incidencia de 1 en 100,000 niños. Ocurre predominantemente en la infancia con una media de presentación a los siete años. La mayor parte de estos tumores cursan asintomáticos y son diagnosticados de manera incidental. El 43 % de ellos tienen manifestación intratorácica y están localizados en los ganglios simpáticos del mediastino posterior. Los medios de diagnóstico por imágenes aportan ventajas para mostrar la localización, tamaño y extensión del tumor. Estos exámenes resultan de gran beneficio para el cirujano. El pronóstico es generalmente favorable, lo que constituye signo de buen pronóstico, la aparición posterior a los cinco años de edad y las localizaciones en el mediastino posterior. Se presenta el caso de una paciente a la que se le realizó toracoscopia con resección total del tumor, con evolución favorable durante su seguimiento. A esta paciente se le diagnosticó un ganglioneuroma en mediastino posterior. Se realizó una revisión bibliográfica a propósito de este caso. Se presenta este reporte porque el ganglioneuroma es un tumor benigno poco frecuente en edad pediátrica.

ABSTRACT Neuroectodermal tumors are the main cause of mediastinal tumors. Of these, the ganglioneuroma is the most benign and the least frequent, with an incidence of 1 in 100,000 children. It occurs predominantly in childhood with a mean presentation at age seven. Most of these tumors are asymptomatic and diagnosed incidentally. The 43 % of them showing intrathoracic manifestation and located in the sympathetic ganglia of the posterior mediastinum. Imaging studies provide advantages for the location, size and extent of the tumor. These tests are of great benefit to the surgeon. The prognosis is generally favorable, which is a sign of good prognosis when it appears after five years of age and when it is located in the posterior mediastinum. The case of a patient who underwent thoracoscopy with total resection of the tumor, with a favorable evolution during her follow-up is presented. This patient was diagnosed with a ganglioneuroma in the posterior mediastinum. A literature review was carried out regarding this case. This report is presented because ganglioneuroma is a rare benign tumor in children.

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Ganglioneuromas are slow growing tumours arising from tissues of the neural crest, mainly in autonomic nervous system. They are frequently localized in the posterior mediastinum being the intraspinal involvement very uncommon. We present the case of a female patient with bilateral cervical ganglioneuroma, together with a review of the cases published to date, emphasizing in the main characteristics of these tumours and including them as part of neurofibromatosis type 1 spectrum.

Los ganglioneuromas son tumores de lento crecimiento que se originan en tejidos derivados de la cresta neural, principalmente en el sistema nervioso autónomo. Se localizan sobre todo en el mediastino posterior, siendo la afectación intraespinal muy poco frecuente. Presentamos el caso de una mujer intervenida de ganglioneuromas localizados en la columna cervical y agrupamos los casos descritos previamente en la literatura inglesa revisando las características principales de estas neoplasias e incluyéndolas en el espectro de manifestaciones de la neurofibromatosis tipo 1.

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BACKGROUND: Ganglioneuroma (GN) is ranked by the International Neuroblastoma Pathology Classification as a benign tumor. It can occur anywhere along the sympathetic nerve chain and surgical excision is the treatment of choice. CASE PRESENTATION: An 18-year-old female patient sought medical assistance after 6 months of constant dorsal and back pain radiating from the thoracic region to the right abdominal flank. Magnetic resonance imaging revealed a solid nodular lesion with heterogeneous post-contrast enhancement and lobulated contours, centered on the right foramina of D12-L1, with a projection to the intracanal space, which compressed and laterally displaced the dural sac and had a right paravertebral extension between the vertebral bodies of D11 and superior aspect of L2. Ganglioneuroma was diagnosed using immunohistochemical analysis. It was decided to use a surgical approach in two stages: robot assisted for the anterior/retroperitoneal mass and a posterior hemilaminectomy/microsurgical approach to attempt total resection, avoiding the traditional anterior thoracoabdominal surgical incision and optimizing the patient's postoperative outcomes. No postoperative adverse events were noted, and the patient was discharged on postoperative day 5. CONCLUSION: This retroperitoneal GN presentation was peculiar because it originated at the D12 nerve root, which extended to the retroperitoneal space and inside the spinal canal. We hope that our case report can assist future decisions in similar circumstances.

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RESUMEN El ganglioneuroma constituye un tumor neuroectodérmico primitivo benigno, que deriva de los ganglios del sistema simpático y está compuesto por células de Schwann maduras, células ganglionares y fibras nerviosas. Se presenta el caso de un raro tumor de cuello en una paciente de sexo femenino en edad pediátrica, con antecedentes de masa tumoral cervical desde hacía dos años. En los estudios por imagen realizados se diagnosticó una lesión sólida heterogénea con calcificaciones puntiformes, que comprimía estructuras óseas y vasculares adyacentes. Para obtener histología se realizó biopsia con aguja gruesa ecodirigida, informándose por anatomía patológica el diagnóstico de ganglioneuroma cervical. Debido a la baja incidencia del ganglioneuroma como variedad tumoral, y a su vez, la rara localización cervical en un escaso porciento de los pacientes, la presente investigación se propone enriquecer la literatura científica nacional e internacional, aportando un nuevo caso que sirva de base para futuras investigaciones, respetando los principios éticos de la investigación en salud.

ABSTRACT The ganglioneuroma is a benign primitive neuroectodermal tumor, which derives from the ganglia of the sympathetic system and is composed of mature Schwann cells, ganglion cells, and nerve fibers. A rare neck tumor, in a female pediatric patient with a history of cervical tumor mass for two years is presented. In the imaging studies performed, a heterogeneous solid lesion with punctate calcifications was diagnosed, compressing adjacent bone and vascular structures. To obtain histology, an ultrasound-guided core needle biopsy was performed, and the diagnosis of cervical ganglioneuroma was informed by pathological anatomy. Due to the low incidence of ganglioneuroma as a tumor variety, and in turn, the rare cervical location in a small percentage of patients, the present research aims to enrich the national and international scientific literature, providing a new case that serves as the basis for future research, respecting the ethical principles of health research.

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Resumen El ganglioneuroma (Gn) es una neoplasia benigna, rara, derivada de la cresta neural, compuesta de células ganglionares maduras y células de Schwann. Solo una pequeña proporción de Gn se origina en la médula suprarrenal, siendo esta localización más frecuente en niños y adultos jóvenes. Hemos encontrado menos de 30 publicaciones reportadas en la literatura. Presentamos el caso de una paciente femenina de 37 años de edad, con dolor intermitente en fosa renal izquierda y flanco ipsilateral de 5 meses de evolución, sin otros síntomas acompañantes. Se realizó ecografía abdominal, tomografía axial computada (TAC) y resonancia magnética nuclear (RMI) abdomino pélvica, en las cuales se identificó formación expansiva solida, de 85 x 58 x 74 mm, de contornos definidos, adyacente a riñón izquierdo. Las características por imagen no permitieron descartar lesión benigna. Se realizó perfil hormonal completo para despistaje de funcionalidad. Dadas las características imagenológicas y el tamaño de la lesión, se decide tratamiento quirúrgico, considerando a feocromocitoma silente como principal diagnóstico presuntivo pre quirúrgico. La evolución postoperatoria fue excelente. Al examen microscópico, se observa proliferación nodular, de bordes delimitados, formado por células fusadas, con núcleos bipolares y citoplasmas elongados con aisladas células ganglionares. En la inmunomarcación presenta aisladas células ganglionares positivas para Cromogranina A y Sinaptofisina, proteína S100 positivo difuso, hallazgos consistentes con ganglioneuroma suprarrenal. El hallazgo de una masa suprarrenal en paciente joven, sin síntomas específicos, con características no sugestivas de adenoma, debe considerar el diagnostico de ganglioneuroma como parte de las lesiones incidentales adrenales y destacamos la importancia del diagnostico diferencial con feocromocitoma y carcinoma adrenal ya que comparten similares características imagenológicas. El tratamiento de elección es la resección quirúrgica completa, con pronóstico excelente en la mayoría de los casos.

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Background: Ganglioneuroma is a rare tumor derived from the neural crest that can occur in any sympathetic tissue. It corresponds to 0.3% to 2% of incidental adrenal tumors and <250 have been reported in the literature so far. Case Presentation: We present a case of a 30-year-old Caucasian woman presented with a large bilobed adrenal tumor found on a CT scan during the investigation of acute abdominal pain. The image also showed a rare anatomic variation of a left-sided inferior vena cava. Biochemical work-up for adrenal incidentaloma showed normal markers. Since we could not rule out malignancy, the patient was subjected to laparoscopic adrenalectomy and the pathology report showed an adrenal ganglioneuroma, a rare nonfunctioning tumor of the adrenal. Conclusion: Ganglioneuroma can present as a large bilobed adrenal tumor. The laparoscopic approach is feasible and safe. Preoperative planning is needed and vascular variations can be challenging during the procedure.

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Resumen: Introducción: el ganglioneuroma suprarrenal es un tumor benigno excepcional originado en las células nerviosas derivadas de la cresta neural. El objetivo de este trabajo es comunicar un caso clínico de un ganglioneuroma suprarrenal derecho sintomático. Caso clínico: paciente de 37 años, sexo femenino, que consultó por dolor abdominal inespecífico que luego de la valoración imagenológica y estudio funcional hormonal, se diagnosticó una tumoración suprarrenal derecha no funcionante. Con dicho diagnóstico se realizó la adrenalectomía por abordaje convencional retroperitoneal y el estudio anatomopatológico de la pieza diagnosticó un ganglioneuroma. Discusión: los ganglioneuromas habitualmente son asintomáticos y no funcionantes, por lo que su diagnóstico es incidental en un alto porcentaje de casos. Otros se presentan con una sintomatología inespecífica. Su diagnóstico etiológico preoperatorio raramente es realizado y su confirmación es anatomopatológica. Tienen indicación quirúrgica los sintomáticos, los que superan los 6 cm, o persisten dudas diagnósticas de malignidad. El pronóstico es bueno, siendo excepcional la recidiva.

Summary: Introduction: adrenal ganglioneuroma is an exceptional benign tumour which originates from neural crest cells. The study aims to inform about a clinical case of asymptomatic right adrenal ganglioneuroma. Clinical case: 37-year old patient, female, who consulted for non-specific abdominal pain which, after imaging assessment and functional and hormone test was diagnosed with non-functioning tumour of the right adrenal gland. Upon this diagnosis, adrenalectomy using the conventional retroperitoneal approach was performed, and the pathology study of the piece confirmed the diagnosis of ganglioneuroma. Discussion: ganglioneuromas are usually asymptomatic and non-functioning, so diagnosis is incidental in a large percentage of cases. Others present non-specific symptoms. Preoperative etiological diagnosis is rare, and confirmation is usually after pathology study. Symptomatic tumours over 6cm long have an indication of surgery, as well as those suspicious of malignancy. Prognosis is good, relapses being exceptional.

Resumo: Introdução: o ganglioneuroma suprarrenal é um tumor benigno excepcional originado nas células nervosas derivadas da crista neural. O objetivo deste trabalho é descrever o caso clínico de um ganglioneuroma suprarrenal direito sintomático. Caso clínico: paciente de 37 anos, sexo feminino que consultou por dor abdominal inespecífica que foi diagnosticado como uma tumoração suprarrenal direita não funcionante depois da avaliação de estudos de imagem e funcional hormonal. Com este diagnóstico realizou-se adrenalectomia por abordagem convencional retroperitoneal e o laudo anatomopatológico diagnosticou um ganglioneuroma. Discussão: os ganglioneuromas geralmente são assintomáticos e não funcionantes por isso seu diagnóstico é acidental em uma alta proporção dos casos. Outros se apresentam com sintomatologia inespecífica. O diagnóstico etiológico pré-operatório raramente é realizado e sua confirmação é feita pela anatomia patológica. Quando são sintomáticos, têm mais de 6 cm ou se persistem dúvidas sobre sua malignidade têm indicação cirúrgica. O prognóstico é bom, e a recidiva é excepcional.

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ABSTRACT Objective: To report our experience of retroperitoneoscopic technique in semi-lateral decubitus position for the retroperitoneal nonadrenal ganglioneuromas in 18 patients, and to evaluate its clinical outcomes. Materials and Methods: From January 2012 to May 2016, 18 patients with retroperitoneal nonadrenal ganglioneuromas underwent retroperitoneoscopic resection. With the patients in semi-lateral decubitus position, a 4-port retroperitoneal approach was used. Data were collected on the tumor size, tumor location, perioperative outcomes, pathology, and last-known disease status. We reviewed the operative videos to identify surgical tips and tricks. Results: All procedures were carried out successfully without converting to open surgery. The tumors had an average size of 5.2cm. The mean operative time was 86.5 min, with a mean estimated blood loss of 85.4mL. There were three patients suffering from intraoperative complications. Postoperatively, all patients achieved an uneventful recovery; the mean postoperative hospital stay was 5.5 days. The postoperative pathology revealed to be retroperitoneal ganglioneuromas. With a mean follow-up of 39.5 months, all patients were recurrence free. The review of the operative videos revealed several tips and tricks, including keeping peritoneum and posterior Gerota fascia intact to provide a favorable operative exposure of tumors, and placing the harmonic scalpel through different ports during tumor dissection. Conclusions: With the patient in semi-lateral decubitus position and a 4-port retroperitoneal approach, retroperitoneoscopic resection of retroperitoneal nonadrenal ganglioneuroma is a feasible, effective, and safe procedure. This approach has distinct advantages including direct access to the tumor, optimal exposure of tumor and less intraperitoneal interference.

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Magnetic resonance neurography is a technique for identifying anatomy and pathologic lesions of nerves, and has emerged as a helpful technique for localizing lesions and elucidating the underlying etiology. Ganglioneuromas are highly differentiated benign tumors. This lesion is rare and exhibits undetermined symptoms, the features of using the magnetic resonance neurography are a great ally to determine its diagnosis. The authors illustrate a case of retroperitoneal ganglioneuroma emphasizing its image characteristics using magnetic resonance neurography with the diagnosis confirmed by histopathological examination.

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OBJECTIVE: To report our experience of retroperitoneoscopic technique in semi-lateral decubitus position for the retroperitoneal nonadrenal ganglioneuromas in 18 patients, and to evaluate its clinical outcomes. MATERIALS AND METHODS: From January 2012 to May 2016, 18 patients with retroperitoneal nonadrenal ganglioneuromas underwent retroperitoneoscopic resection. With the patients in semi-lateral decubitus position, a 4-port retroperitoneal approach was used. Data were collected on the tumor size, tumor location, perioperative outcomes, pathology, and lastknown disease status. We reviewed the operative videos to identify surgical tips and tricks. RESULTS: All procedures were carried out successfully without converting to open surgery. The tumors had an average size of 5.2cm. The mean operative time was 86.5 min, with a mean estimated blood loss of 85.4mL. There were three patients suffering from intraoperative complications. Postoperatively, all patients achieved an uneventful recovery; the mean postoperative hospital stay was 5.5 days. The postoperative pathology revealed to be retroperitoneal ganglioneuromas. With a mean follow-up of 39.5 months, all patients were recurrence free. The review of the operative videos revealed several tips and tricks, including keeping peritoneum and posterior Gerota fascia intact to provide a favorable operative exposure of tumors, and placing the harmonic scalpel through different ports during tumor dissection. CONCLUSIONS: With the patient in semi-lateral decubitus position and a 4-port retroperitoneal approach, retroperitoneoscopic resection of retroperitoneal nonadrenal ganglioneuroma is a feasible, effective, and safe procedure. This approach has distinct advantages including direct access to the tumor, optimal exposure of tumor and less intraperitoneal interference.

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Ganglioneuromas são neoplasias neuroectodérmicas, bem diferenciadas, de crescimento lento e na maioria das vezes assintomáticos. São derivados do desenvolvimento de células neuronais do sistema nervoso simpático. Podem ser divididos em três subgrupos: 1) ganglioneuromas polipoides solitários, 2) ganglioneuromatose poliposa e 3) ganglioneuromatose difusa. O diagnóstico é essencialmente morfológico. O principal tratamento é a ressecção cirúrgica, sendo a quimioterapia e radioterapia utilizada quando não for possível nova abordagem cirúrgica. Metodologia: Realizado análise retrospectiva do caso de paciente atendida no ambulatório do serviço Coloproctologia do Hospital do Servidor Público Municipal. Relato de Caso: Descrevemos uma paciente, de 44 anos, com história familiar de câncer de reto em mãe e câncer de cólon em avó materna, sem queixas gastrointestinais, que realizou colonoscopia para rastreio de câncer colorretal sendo evidenciado pólipo séssil avermelhado de 0,5cm em flexura esplênica tendo sido realizado polipectomia com anatomopatológico evidenciando ganglioneuroma intestinal.

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Los ganglioneuromas son los tumores benignos, formados por células maduras de la cresta neural, más frecuentes del sistema nervioso simpático en adultos jóvenes. Se presenta el caso de un varón de 25 años con una lesión retroperitoneal extensa y exéresis parcial, con buena evolución durante 6 meses(AU)

Ganglioneuromas are the benign tumors from mature cells of the neural crest more often seen from the sympathetic system on young adults. We present a young male with and extent retroperitoneal tumor with partial removal with a good evolution for 6 month(AU)

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Se presenta el caso clínico de un paciente, sexo femenino, 52 años de edad, que al examen clínico presentaba un aumento de volumen a nivel del ángulo mandibular derecho. Los estudios imagenológicos mostraron una extensa masa cervical paravertebral, encapsulada, de límites netos, sin evidencias de calcificaciones en el cuerpo de la lesión como tampoco compromiso del tejido óseo vecino, que se extiende desde la base de cráneo hasta la región de la cara interna del ángulo mandibular derecho. Se determinó la cirugía, como tratamiento, la cual presentó como complicaciones post operatorias, trastornos en la deglución y síndrome de Horner. Por último la biopsia post operatoria arrojó ganglioneuroma y linfonodos sin lesiones histológicas.

A case report of a patient, female, 52 years old, clinical examination showed a volume increase at the right mandibular angle. Imaging studies showed a large cervical paravertebral mass, encapsulated, net limits without evidence of calcification in the body of the lesion nor involvement of neighboring bone tissue, extending from the base of the skull to the region of the inner right mandibular angle. Surgery was determined as treatment, presenting, impaired swallowing and Horner syndrome as post-operative complications. Finally, the postoperative biopsy showed, ganglioneuroma and lymph nodes without histological lesions.

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O ganglioneuroma é um tumor do sistema nervoso simpático, podendo estar associado à hipersecreção de substâncias vasoativas responsáveis por sintomas e sinais variados, como a hipertensão arterial. Os autores apresentam um caso de ganglioneuroma e uma revisão da literatura, focando os aspectos mais importantes do diagnóstico e da terapêutica. A paciente apresentava crises hipertensivas sintomáticas recorrentes, tendo realizado estudo imaginológico que detectou uma imagem nodular na adrenal direita. Sendo os tumores neuroblásticos indistinguíveis radiologicamente, procedeu-se à excisão, confirmando-se o diagnóstico pela análise anatomopatológica. Este caso demonstra a variabilidade clínica dos ganglioneuromas, a utilidade da imaginologia e a importância do estudo histológico.

The ganglioneuroma is a tumor of the sympathetic nervous system and may be associated with hypersecretion of vasoactive substances responsible for various symptoms and signs such as hypertension. The authors report a case of ganglioneuroma and a literature review, focusing on the most important aspects of diagnosis and therapy. The patient had recurrent symptomatic hypertensive crises, having performed an imaging study that found a nodule in the right adrenal gland. As neuroblastic tumors are radiologically undistinguishable, the patient underwent excision, confirming the diagnosis by pathology analysis. This case demonstrates the clinical variability of ganglioneuroma, the usefulness of imaging and the importance of histological study.

El ganglioneuroma es un tumor del sistema nervioso simpático, y puede estar asociado con la hipersecreción de sustancias vasoactivas responsables de diversos síntomas y signos, como la hipertensión arterial. Los autores presentan un caso de ganglioneuroma y una revisión de la literatura, centrándose en los aspectos más importantes del diagnóstico y la terapéutica. El paciente tenía crisis hipertensivas sintomáticas recurrentes, e hizo un estudio imaginológico que detectó una imagen nodular en la glándula adrenal derecha. Al ser los tumores neuroblásticos radiológicamente indistinguibles, se procedió a la extirpación, lo que confirma el diagnóstico mediante el análisis anatomopatológico. Este caso demuestra la variabilidad clínica de ganglioneuroma, la utilidad de la imagiologia y la importancia del estudio histológico.

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Objetivo: discutir a abordagem e o manejo laparoscópico de um raro caso de ganglioneuroma de adrenal em incidentaloma. Descrição do caso: um paciente masculino de 45 anos teve diagnóstico de incidentaloma de adrenal com 7,5 cm à ultrassonografia e padrão tomográfico incaracterístico. A suprarrenalectomia laparoscópica transperitoneal durou 63 minutos e utilizou quatro portais (um de 12, um de 11 e dois de 5 mm) para a abordagem da glândula, a qual foi removida por uma ampliação do portal de 12 mm até cerca de 3,5 cm, em saco coletor. O sangramento computado foi inferior a 50 ml. O exame anatomopatológico revelou tratar-se de ganglioneuroma. Houve excelente evolução pós-operatória, com alta hospitalar 72 horas apos o procedimento. Conclusões: a propedêutica dos incidentalomas de adrenal é motivo de controvérsias na literatura. Este relato busca chamar a atenção para a possibilidade do ganglioneuroma como causa de incidentaloma de adrenal, principalmente em pacientes jovens. A remoção cirúrgica desse tumor normalmente resulta em cura clínica e a abordagem laparoscópica é a opção preferencial.

Aims: To discuss the diagnostic and therapeutic options in a rare case of ganglioneuroma as cause of adrenal incidentaloma. Case description: A 45-year old male presented with a diagnosis of adrenal incidentaloma during routine clinical evaluation. The lesion measured 7.5 cm on ultrasound, and computerized tomography findings were non-characteristic. A transperitoneal laparoscopic adrenalectomy was performed in 63 minutes of surgery time, through four ports (12, 11, and two of 5 mm). The specimen was removed through an augmentation of the 12 mm port until roughly 3.5 cm, inside an endocatch bag. Intraoperative bleeding was less than 50 ml.Pathological examination revealed an adrenal ganglioneuroma.Postoperative course was uneventful, and the patient was discharged home 72 hours after the procedure. Conclusions: In the literature reviewed, the diagnostic and therapeutic approaches of adrenal incidentalomas are controversial. Our report points out to the possibility of ganglioneuroma as a cause of adrenal incidentalomas, especially among young patients. Its surgical removalgenerally results in cure, and the laparoscopic approach is currently the preferred option.

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RESUMO

Ganglioneuromas cutâneos são lesões raramente descritas na literatura médica e, deforma ainda mais especial, em associação com outras lesões. Relatamos o achado de umamassa cervical detectada por exame de ultra-som pré-natal, que regrediu no decorrer dagravidez. Após o nascimento, extensa área pigmentada foi observada na mesma localizaçãoda massa, além de um tumor sólido palpável no seu interior. O diagnóstico diferencialcom outros tumores derivados de células da crista neural é importante, assim como acorrelação entre o névus piloso e o ganglioneuroma cutâneo.

Cutaneous ganglioneuroma have been occasionally reported in the literature. We describea case in which a cervical mass firstly detected by routine prenatal ultrasound examinationregressed throughout the months until delivery. After birth, an extensive area ofpigmentation was found at the same location, with a solid mass was perceptible within.The differential diagnosis between other neural crest tumors should be established, aswell the correlation between congenital nevus and cutaneous ganglioneuroma.

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