Extra-adrenal peripheral neuroblastic tumors: A clinicopathological study of 18 cases

Background: Peripheral neuroblastic tumors arise from the sympathoadrenal lineage of the neural crest. They have been classified according to the International Neuroblastoma Pathology Committee (INPC) into Four categories according to International Neuroblastoma Pathology Committee (INPC): a) Neuroblastoma (NB) b) Ganglioneuroblastoma (GNB), nodular c) Ganglioneuroblastoma, intermixed, and d) Ganglioneuroma (GN). Because of the rarity of extra-adrenal peripheral neuroblastic tumors, limited information is available regarding the chemotherapy of NB and GNB. A few case reports or case series with a small number of patients have been documented in the literature. Aim: To describe the clinicopathological characteristics of extra-adrenal peripheral neuroblastic tumors. Materials and Methods: Clinical, histopathological, and immunohistochemistry (IHC) findings of 18 cases were retrieved. Immunohistochemistry at the time of diagnosis was performed using Ventana Benchmark XT. The mean value was calculated using the Microsoft Office Excel 2019 software. Results: The posterior mediastinum was the most commonly affected extra-adrenal site in our study. Neuroblastoma consisted of eight cases (six in children, two in adults), of which four cases were poorly differentiated and the other four cases were differentiating. Two cases had favorable histology. The bone marrow and cervical lymph node metastasis were documented. Of the four GNB cases, one patient developed bone metastasis. All patients of NB and GNB received combination chemotherapy. One out of six GN patients presented with a large retroperitoneal mass encasing the aorta and renal vessels, mimicking a sarcoma. Conclusion: Extra-adrenal peripheral neuroblastic tumors do not pose any diagnostic issue in adequate tissue sampling. In limited material, immunohistochemistry is needed. The chemotherapy regimen has not been standardized due to rarity. Further molecular testing and targeted therapy may be of help in the future.

Renal Ganglioneuroma.

Background: Renal ganglioneuroma is an extremely rare benign tumour that affects neural crest cells. Here we report a case of renal ganglioneuroma with nodal metastasis managed by surgical resection. Case report: A 38-year-old female presented with anorexia and early satiety. On examination, she had a palpable mass per abdomen. CT scan revealed a large heterogenous mass in retroperitoneum in right suprarenal region with enlarged retrocrural lymph nodes. She underwent right radical nephrectomy as the mass was inseparable from the right kidney. Postoperative period was uneventful. HPE revealed renal ganglioneuroma. Conclusion: Primary renal ganglioneuroma is difficrult to differentiate from other etroperitoneal lesions. Surgical resection is the most effective treatment.

Ganglioneuromatosis intestinal difusa a lo largo del tubo digestivo / Diffuse intestinal ganglioneuromatosis along the gastrointestinal tract

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.

Ganglioneuroma de mediastino posterior: a propósito de un caso

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.

Ganglioneuroma cervical. Presentación de un caso en edad pediátrica y revisión de la literatura / Cervical Ganglioneuroma. A Pediatric Case Presentation and Review of the Literature

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.

Ganglioneuroma suprarrenal gigante: Reporte de Un caso

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.

Paraspinal Ganglioneuromain an Adolescent with Painless Scoliosis: Case Report and Literature Review

Adolescent scoliosis secondary to ganglioneuroma is a rare entity with a clinical presentation mimicking adolescent idiopathic scoliosis that can lead to delayed or misdiagnosis. We present this case with a literature review to illustrate the importance of careful evaluation of the clinical and radiological findings in reaching a proper, timely mannered diagnosis. Clinical and radiological evaluation with a keen eye for signs of the atypical curve and radiological abnormalities on plain films and getting more detailed imaging with CT and MRI would lead to earlier diagnosis of secondary causes of scoliosis and direct proper treatment plan.

Ganglioneuroma suprarrenal. Reporte de un caso / Adrenal ganglioneuroma. A case report / Ganglioneuroma suprarrenal. Relato de caso

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.

Thoracic Intraspinal Ganglioneuroma with Cough as Its Main Symptom:Report of One Case / 中国医学科学院学报

The ganglioneuroma is a benign tumor originating from sympathetic ganglion cells.It often locates in the posterior mediastinum,retroperitoneum,and adrenal medulla.The intraspinal ganglioneuromas is relatively rare in clinical practice,which mainly locates in the cervical and thoracolumbar segments.A patient with main symptom of cough was examined by magnetic resonance imaging before operation in our center.Intraspinal ganglioneuromas was confirmed in the left intervertebral cavity area.Total resection of the tumor via the posterior median approach was performed.HE staining showed the mature ganglion cells were scattered.The patient was followed up for three months and no tumor recurrence occured.

A Case of Incidental Retroperitoneal Ganglioneuroma

Ganglioneuroma is a rare benign tumor originating from the neural crest cells. It occurs most commonly in the retroperitoneum and posterior mediastinum and is often found in the neck or pelvis. It may be detected incidentally or detected by pressure effects on the adjacent structures due to its slow growth. However, some functional tumors may secrete catecholamines and present with some clinical symptoms. Complete surgical excision is the treatment of choice. We describe here a case of a retroperitoneal ganglioneuroma which was removed completely by surgery. We review the literature and discuss the clinical features of a ganglioneuroma.

A Case of Multiple Posterior Cervical Ganglioneuromas in Elderly Patient / 대한두경부종양학회지

Ganglioneuroma (GN) is benign neurogenic tumor arising from ganglia of the sympathetic nervous system. They are mostly found at posterior mediastinum, retroperitoneum, and adrenal gland, whereas only 1–5% occurred in the cervical region. GN usually present as a single, painless and slow-growing mass, but multiple cervical occurrences are extremely rare. An 80-year-old woman came to our clinic complained of posterior neck mass for three years. We performed surgical excision, and it was finally diagnosed as GN. We report the unique and rare disease entity with a brief literature review.

A radiological and histological comparison of two unusual adrenal incidentalomas

@#Introduction: The increasing use of radiological imaging studies has given rise to ‘incidentalomas’. Case Report: We describe two unusual and diverse incidental adrenal gland lesions, an adenomatoid nodule and a mature ganglioneuroma. Both are deemed ‘indeterminate’ on radiological assessment. On histology, an adenomatoid nodule is composed of variably-dilated thin-walled cysts lined by bland flattened cells and solid areas of tubules lined by eosinophilic cells with plump nuclei and prominent nucleoli. The lining cells are immunoreactive for calretinin and WT1 while negative for CK5/6, ERG and CD31. Mature ganglioneuroma features fascicles of bland spindle cells with intermixed mature ganglion cells disposed within a background myxoid stroma with no immature neuroblastic component. These spindled Schwann cells are S100 positive. Discussion: Both adenomatoid nodule and mature ganglioneuroma are rare benign adrenal tumours that need to be differentiated from other, more common adrenal lesions. The management of adrenal incidentalomas is challenging. Surgical excision is indicated if an adrenal incidentaloma is more than 4 cm in size, shows malignant features on imaging or evidence of hormone excess. Keywords:

Retroperitoneoscopic resection of retroperitoneal nonadrenal ganglioneuromas: our technique and clinical outcomes

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.

A Case of Duodenal Ganglioneuroma Manifesting as a Subepithelial Tumor

Ganglioneuroma of the gastrointestinal tract is a rare tumor that consists of ganglion cells, nerve fibers, and supporting cells of the enteric nervous system. Ganglioneuromas are usually associated with genetic disorders such as the multiple endocrine neoplasia syndrome or neurofibromatosis. Ganglioneuromas of the gastrointestinal tract predominantly involve the colon and rectum, and reports about duodenal ganglioneuromas are few. Herein, we report a case of duodenal ganglioneuroma treated with endoscopic resection. A 56-year-old female patient visited our hospital because of a subepithelial tumor in the second portion of the duodenum. She had no remarkable medical or family history and revealed no history of genetic disorders. Endoscopic ultrasonography and abdominal computed tomography revealed a tumor located mainly in the submucosal layer, without any regional lymph node involvement. Endoscopic resection of the lesion was performed, and the pathological examination confirmed a duodenal ganglioneuroma.

MRI features of intraspinal ganglioneuroma / 中国医学影像技术

Objective To analyze MRI features of intraspinal ganglioneuroma,in order to improve the ability of preoperative diagnosis.Methods MRI data of 9 patients of intraspinal ganglioneuroma confirmed pathologically were analyzed retrospectively.The location,morphology,size,signal intensity and degree of enhancement of these lesions were observed.Results Totally 9 lesions were enrolled.Four lesions occurred in the cervical spine,2 in the thoracic spine and 3 in the lumbosacral spine.Seven lesions were dumbbell shaped,involving both intra and extra-canalicular,and 2 lesions present as nodules in the foramen.All the lesions demonstrated homogeneous or heterogeneous high signal on T2WI,with only 1 lesion showed cystic change and necrosis.Contrast-enhanced scanning was performed in 8 lesions,and marked enhancement was shown in 3 lesions,mild-moderate enhancement in 5 lesions.Strand-shaped enhancement took place in 4 lesions.The intervertebral foramina of all the lesions were enlarged,but without bone destruction.Conclusion Intraspinal ganglioneuroma has some characteristics on MRI,which may be helpful to accurate preoperative diagnosis.

Giant Ganglioneuroma of Thoracic Spine: A Case Report and Review of Literature

Ganglioneuroma (GN) is a rare benign tumor of neural crest origin usually found in the abdomen, but may occasionally present at uncommon sites including the cervical, lumbar, or sacral spine. However, GNs of thoracic spine are extremely rare. In this report, we describe a 12-year-old girl with giant GN in the thoracic spine, who underwent successful resection (T1–4 level) of the tumor. Histopathological examination confirmed the diagnosis. GN should be considered in the differential diagnosis of any paraspinal mass. A high index of suspicion and correlation of clinico-radiological findings is necessary in differentiating a large benign tumor from a malignant growth. Complete surgical excision is the treatment of choice; however tumor size and location need to be considered for the surgical approach (one-step or multiple surgeries). Close follow-up after surgery is mandatory.

Composite pheochromocytoma-ganglioneuroma: a clinicopathologic and literature review / 临床与实验病理学杂志

Purpose To explore the clinicopathological features,immunophenotype,differential diagnosis and prognosis of the composite pheochromocytoma (CP)-ganglioneuroma.Methods 3 cases of CP-ganglioneuroma were stained by immunohistochemical SP method,and the related literatures were reviewed.Results 3 cases of CP-ganglioneuroma were one male and 2 females,the age were 37-64.Case 3 were of primary mediastinal.Microscopically,the tumor tissues were composed of two components:one type of tumor cells were arranged in nests with a predominant Zellballen pattern,round or oval nuclei,fine granular cytoplasm and rare mitotic,another part of the neoplasm showed scattered and aggregated distributed ganglion cells in the background of neurofibromatosis which aligned bundles and interwoven,the edge of the tumor was still residual adrenal tissue.Immunohistochemically,components of pheochromocytoma were positive for CD56,CgA,Syn,vimentin and negative for SMA,Melan-A,α-inhibin NF with low Ki-67 proliferation index.S-100 was positive in supporting cells,ganglioneuroma components were positive for NF,S-100 with low Ki-67 proliferation index.CgA and Syn were weakly positive or negative in the ganglion cells.Conclusion CP is a relatively rare tumor,which can not be distinguished from pheochromocytoma in clinical and radiological diagnosis.The corresponding clinical treatment and follow-up management should be taken according to the different ingredients (benign or malignant).

Ganglioneuroma of the Sacrum

Presacral ganglioneuromas are extremely rare benign tumors and fewer than 20 cases have been reported in the literature. Ganglioneuromas are difficult to be differentiated preoperatively from tumors such as schwannomas, meningiomas, and neurofibromas with imaging modalities. The retroperitoneal approach for resection of presacral ganglioneuroma was performed for gross total resection of the tumor. Recurrence and malignant transformation of these tumors is rare. Adjuvant chemotherapy or radiation therapy is not indicated because of their benign nature. We report a case of a 47-year-old woman with a presacral ganglioneuroma.

Harlequin Syndrome Following Resection of Mediastinal Ganglioneuroma

Harlequin syndrome is a rare disorder of the sympathetic nervous system characterized by unilateral facial flushing and sweating. Although its etiology is unknown, this syndrome appears to be a dysfunction of the autonomic nervous system. To the best of our knowledge, thus far, very few reports on perioperative Harlequin syndrome after thoracic surgery have been published in the thoracic surgical literature. Here, we present the case of a 6-year-old patient who developed this unusual syndrome following the resection of a posterior mediastinal mass.

The polypoid ganglioneuroma associated with hyperplastic polyposis

No abstract available.