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.
Adrenal Glands ; Catecholamines ; Ganglioneuroma

Adult ; Colon ; Ganglioneuroma/surgery* ; Humans ; Intestinal Neoplasms/surgery*

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.
Cough ; etiology ; Ganglioneuroma ; complications ; Humans ; Magnetic Resonance Imaging ; Neurons ; Staining and Labeling

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.
Catecholamines ; Ganglioneuroma ; Mediastinum ; Neck ; Neural Crest ; Pelvis

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.
Adrenal Glands ; Aged ; Aged, 80 and over ; Female ; Ganglia ; Ganglioneuroma ; Humans ; Mediastinum ; Neck ; Rare Diseases ; Sympathetic Nervous System

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.
Colon ; Duodenum ; Endosonography ; Enteric Nervous System ; Female ; Ganglion Cysts ; Ganglioneuroma ; Gastrointestinal Tract ; Humans ; Lymph Nodes ; Middle Aged ; Multiple Endocrine Neoplasia ; Neurofibromatoses ; Neurons ; Rectum

Cranial Fossa, Posterior ; Cranial Nerve Neoplasms ; diagnosis ; pathology ; Ganglioneuroma ; diagnosis ; pathology ; Humans ; Male ; Middle Aged ; Skull Base Neoplasms ; diagnosis ; pathology ; Trigeminal Ganglion

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.
Abdomen ; Child ; Diagnosis ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Ganglioneuroma* ; Humans ; Neural Crest ; Spine*

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.
Autonomic Nervous System ; Child ; Flushing ; Ganglioneuroma* ; Humans ; Sweat ; Sweating ; Sympathetic Nervous System ; Thoracic Surgery

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.
Chemotherapy, Adjuvant ; Female ; Ganglioneuroma* ; Humans ; Meningioma ; Middle Aged ; Neurilemmoma ; Neurofibroma ; Recurrence ; Sacrum*