Rare case of large Catecholamine Secreting Ganglioneuromain an Asymptomatic elderly male / Journal of the ASEAN Federation of Endocrine Societies

@#Ganglioneuromas (GNs) are benign tumors that originate from neural crest cells, composed mainly of mature ganglion cells. These tumors, which are usually hormonally silent, tend to be discovered incidentally on imaging tests and occur along the paravertebral sympathetic chain, from the neck to the pelvis and occasionally in the adrenal medulla. Rarely, GNs secrete catecholamines.1 Adrenal GNs occur most frequently in the fourth and fifth decades of life, whereas GNs of the retroperitoneum and posterior mediastinum are usually encountered in younger adults.2 Adrenal GNs are commonly hormonally silent and asymptomatic; even when the lesion is of substantial size.3We report an incidentally detected asymptomatic case of an adrenal ganglioneuroma with mildly elevated urinary catecholamine levels in an elderly male. After preoperative alpha blockade, the patient underwent open right adrenalectomy. Upon microscopic examination, the right adrenal mass proved to be a ganglioneuroma, maturing type and the immunohistochemistry examination showed immunoreactivity to synaptophysin, chromogranin, and CD 56, while S100 was strongly positive at the Schwannian stroma. Following resection, catecholamine levels normalized, confirming the resected right adrenal ganglioneuroma as the source of the catecholamine excess. This case represents a rare presentation of catecholamine-secreting adrenal ganglioneuroma in the elderly.

Cerebellar Dysplastic Gangliocytoma: Case Report

Lhermitte-Duclos disease (LDD), or cerebellar dysplastic gangliocytoma, is a rare type of cerebellar tumor, from unknown origin. Patients can be asymptomatic for several years, but there are usually imprecise neurological signs for long periods.

A Rare WHO Grade I Lesion of the Posterior Fossa with Recurrence Biological Behavior ­ Dysplastic Gangliocytoma of the Cerebellum: Case Report

Dysplastic gangliocytoma of the cerebellum (DGC) or Lhermitte-Duclos Disease is a rare lesion (World Health Organization [WHO] grade I) characterized by thickened folia and replacement of the internal granular layer by abnormal ganglion cells. More commonly, the compromised patients are young adults presenting ataxia, seizures, obstructive hydrocephalus, and increased intracranial pressure. Dysplastic gangliocytoma of the cerebellum is intimately associated with Cowden syndrome, a hereditary disorder caused by a germline mutation in the PTEN tumor suppressor gene on chromosome 10q23. Large neurons of DCG show vesicular nuclei with prominent nucleoli. Expansion of the internal granular layer determines vacuolization of the molecular layer and white matter, which can be related to the bright stripes identified on T2-weighted magnetic resonance imaging. Herein, the authors report a female patient who developed long- time recurrence of DGC and discuss pathological findings and differential diagnosis of this rare cerebellar lesion.

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.

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.

Lhermitte-Duclos Disease and Cowden Syndrome: A Case Report and Literature Review

Lhermitte-Duclos disease (LDD), also known as dysplastic gangliocytoma of the cerebellum, is a rare, usually benign, slow-growing tumor, that commonly affects patients aged 30 to 50 years-old. The manifestations of dysplastic cerebellar gangliocytoma are nonspecific and are related both to the mass effect produced by its growth and to the location of the lesion. Cerebellar symptoms such as ataxia are often present. In 40% of cases, the tumor is associated with Cowden syndrome, which is part of a group ofgenetic disorders called polypoid hamartoma complex. In this case report, the patient presented expansive lesion in the posterior fossa, compatible with LDD, associated with macrocephaly. These findings are consideredmajor criteria for Cowden syndrome. When together, they confirm the diagnoses. To our knowledge, this is the first report of the association of LDD and Cowden syndrome in Brazil.

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.

Robotic excision of complex adrenal mass with retrocaval extension and encasement of renal hilum with renal preservation

ABSTRACT Objective: The purpose of this video is to present robotic excision of a complex adrenal mass with retrocaval extension and encasement of renal hilum in a 16 year old boy. Biochemical screening was negative for metabolically active component. Computerized tomographic scan with contrast revealed a homogenous mass of approximately 10.8 cm x 6.2 cm x 4.2 cm in the suprarenal area on right side that was extend-ing behind inferior vena cava and encasing renal hilar vessels. Imaging findings were that of a classical ganglioneuroma. Material and methods: Robot assisted laparoscopic adrenalectomy with sparing of renal hilar vasculature was performed. With patient in lateral position, five ports were used, including one for liver retraction. Da Vinci® system with four arms was docked from over the right shoulder. The displaced renal hilar structures were identified by opening Gerota's fascia. Mass was dissected completely and removed through Pfan-nensteil incision. Results: Duration of procedure was 345 minutes and console time was 290 minutes. Blood loss was 250 mL. Post-operative renal doppler showed normal blood flow. He was discharged on post-operative day three. Histopathologic examination of specimen revealed ganglioneuroma arising from adrenal gland. Conclusion: Ganglioneuroma is a rare adrenal tumor with good prognosis on surgical removal. The advent of robotic surgery has made complex surgical procedures involving vital structures like inferior vena cava be performed using minimally invasive techniques without compromising oncologic principles.

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.

Neuroglioma intestinal: relato de caso / Intestinal neuroglioma: case report

MO Introdução: 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. Palavras-chave: Ganglioneuroma, ganglioneuroma intestinal, relato de caso.

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.

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.

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.

A Rare Case of Concomitant Intramedullary Gangliocytoma at the Cervicomedullary Junction in Patient with Neuroendocrine Tumor of Lung

Ganglion cell tumors (GCT) are divided into two subtypes : gangliocytoma and ganglioglioma. Intramedullary gangliocytomas are extremely rare. A 20-year-old male patient with pain of neck, who also had a previously known neuroendocrine tumor of lung, was operated for mass found in the cervicomedullary junction with a presumptive diagnosis of metastases. Only partial resection could be performed. Pathological diagnosis had been reported as gangliocytoma. Only ten cases of intramedullary gangliocytoma have been reported in the literature. Although association with scoliosis and Von Recklinghausen's disease were previously reported in the literature, no gangliocytoma case concomitant with endocrine tumor of lung have been published. Pathological study is the most important diagnostic method for gangliocytomas. Surgical excision is the primary treatment, but difficulty in total surgical tumor resection is the most important problem.

The polypoid ganglioneuroma associated with hyperplastic polyposis

No abstract available.

Ganglioneuroma retroperitoneal abdomino-pélvico / Abdomino-pelvicretroperitoneal ganglioneuroma

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)

A Malignant Transformation of a Spinal Epidural Mass from Ganglioneuroblastoma to Neuroblastoma

Ganglioneuromas are benign tumors. Surgical excision is the treatment of choice with very good prognosis. However, neuroblastomatous malignant transformation of ganglioneuromas was previously reported. We report a patient with spinal neuroblastoma recurrent from a ganglioneuroblastoma after disease free survival of 13 years. This is one of the rare examples of spinal neuroblastoma and to our knowledge the second case report with malignant transformation from a ganglioneuroblastoma or a ganglioneuroma. The present case is the only report in the literature with further genetic investigations.