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*

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.
Diagnosis ; Ganglioglioma ; Ganglion Cysts ; Ganglioneuroma* ; Humans ; Lung* ; Male ; Neck ; Neoplasm Metastasis ; Neuroendocrine Tumors* ; Neurofibromatosis 1 ; Scoliosis ; Young Adult

OBJECTIVETo summarize the clinical manifestation and diagnosis of ganglioneuroma in spine and investigate the clinical effect of surgical treatment.

METHODSThe clinical data of 6 patients underwent a surgery for ganglioneuroma in spine from January 2008 to January 2015 were retrospectively analyzed. There were 4 males and 2 females, aged from 2 to 63 years old with an average of 34.6 years. The courses of disease were from 3 days to 17 years. Five patients complicated with superficial hypesthesia in correlative level of tumor, and the muscle strength under tumor plane had decreased at different levels, with the strength of grade II-IV. Two cases complicated with hypermyotonia and positive bilateral Hoffmann's and Babinski sign. Five cases were sporadic lesion in correlative spinal canal and one case complicated with the giant occupying lesion in thoracic cavity.

RESULTSSix operations had been performed including 5 en bloc and 1 subtotal resection. Postoperative pathological results showed tumor cells scattered or fasciculated inserted into Schwann cells in the stroma. In 2 patients complicated with radiculalgia before operation, 1 case was relieved and 1 was invariant after operation. All 4 patients with preoperative dyscinesia in the limbs obtained improvement after operation. All the patients were followed up from 0.3 to 6.8 years with an average of 2.5 years. At the final follow-up, according to ASIA grade, 5 cases were good and 1 case was invariant. During the follow-up, only 1 patient experienced chemoradiation because of merging ganglioneuroblastoma and receiving subtotal resection. No recurrence in other 5 cases.

CONCLUSIONGanglioneuroma is a benign and rare tumors in spine. Clinically, radicular pain and sensory-motor disorders are the main manifestations. Its diagnosis depends on pathological examination. Prognosis of surgical treatment is good.

Adolescent ; Adult ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Ganglioneuroma ; diagnosis ; surgery ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Spinal Neoplasms ; diagnosis ; surgery

Colonic diffuse ganglioneuromatosis is a benign neoplastic condition characterized by disseminated, intramural, or transmural proliferation of neural elements involving the enteric plexuses, sometimes associated with von Recklinghausen's disease and other multiple tumor syndromes. Colonic diffuse ganglioneuromatosis is usually large, ranging from 1 to 17 cm, and thus can distort the surrounding tissue architecture as well as infiltrate the adjacent bowel wall. However, colonic diffuse ganglioneuromatosis is an exceptional finding in adults and only individual cases are reported in the literature. Herein, we report two unusual cases of adult patients with colonic diffuse transmural ganglioneuromatosis presenting as a large subepithelial tumor.
Adult ; Aged ; Colon/metabolism/*pathology ; Colonoscopy ; Ganglioneuroma/*diagnosis/metabolism/pathology ; Humans ; Immunohistochemistry ; Male ; S100 Proteins/metabolism ; Tomography, X-Ray Computed

PURPOSE: Benign adrenal tumors other than hyper-functioning tumor and non-functioning cortical adenoma are extremely rare. The purpose of this study is to review the clinical features of these rare benign adrenal tumors and to analyze their prevalence and the proper surgical approach for them. METHODS: Among patients who underwent adrenalectomy in Samsung Medical Center, between 1997 and 2013, patients with benign adrenal tumor except non-functioning cortical adenoma, malignancy, and hyper-functioning tumor such as pheochromocytoma, aldosteronoma, and cortisol producing tumor were included. Clinical details, radiologic findings and pathologic findings as well as data associated with the surgical procedure were analyzed retrospectively. RESULTS: The percentage of these tumors among 703 surgically removed adrenal tumors was 10.7% (75 cases). They included 30 adrenal cysts, 18 ganglioneuromas, 11 myelolipomas, seven schwannomas, and the other six were rare adrenal tumors. Approximately 40% of the patients were operated under a diagnosis different from original tumors. Operation time and hospital stay of patients who underwent laparoscopic adrenalectomy were shorter than those of patients who underwent open adrenalectomy. CONCLUSION: These benign adrenal tumors are very rare and difficult to diagnose preoperatively. For the surgical treatment of these tumors, laparoscopic adrenalectomy is more preferable to conventional open adrenalectomy.
Adenoma ; Adrenalectomy ; Diagnosis ; Ganglioneuroma ; Humans ; Hydrocortisone ; Length of Stay ; Myelolipoma ; Neurilemmoma ; Pheochromocytoma ; Prevalence ; Retrospective Studies

We report an extremely rare case with bilateral and symmetric dumbbell ganglioneuromas of the cervical spine in an elderly patient. A 72-year-old man came by ambulance to our hospital due to progressive incomplete paraplegia. Magnetic resonance imaging demonstrated bilateral symmetric dumbbell tumors at the C1/2 level. We performed total resection of the intracanalar tumor, aiming at complete decompression of the spinal cord, and partial and subtotal resection of foraminal outside portions. Histopathological examination of the surgical specimen indicated the tumor cells to be spindle cells with the presence of ganglion cells and no cellular pleomorphism, suggesting a diagnosis of ganglioneuroma. Although the surgery was not curative, the postoperative course was uneventful and provided a satisfactory outcome. This is the fourth known case of cervical ganglioneuromas of the bilateral symmetric dumbbell type.
Aged ; Ambulances ; Decompression ; Diagnosis ; Ganglion Cysts ; Ganglioneuroma* ; Humans ; Magnetic Resonance Imaging ; Paraplegia ; Paresis* ; Spinal Cord ; Spine

Ganglioneuroma is an uncommon benign tumor and arise from neural crest cells or sympathetic and peripheral nerves. A 13-year-old boy was referred to our institute and presented with three-day history of right upper extremity weakness associated with neck pain for nearly one month. Magnetic resonance imaging showed right side dumbbell shaped extramedullary lesion at the C2-3 level extending outside through neural foramen. Paraspinal and posterior neck mass, cafe au lait spot was identified. We performed surgical decompression of the spinal cord by resection of intradural portion mass. Histopathological diagnosis was ganglioneuroma.
Adolescent ; Decompression, Surgical ; Diagnosis ; Ganglioneuroma* ; Humans ; Magnetic Resonance Imaging ; Male ; Neck ; Neck Pain ; Neural Crest ; Neurofibromatoses ; Peripheral Nerves ; Spinal Cord Compression* ; Spinal Cord* ; Upper Extremity

A case of intracranial ganglioneuroma arising from the trigeminal nerve in the pontine and cerebellopontine angle cistern, in a 44-year-old female, is presented with an emphasis on diffusion-weighted imaging findings. We will discuss on how the tumor in the very unusual location should be differentiated particularly focused on diffusion-weighted imaging findings.
Adult ; Contrast Media/diagnostic use ; Diagnosis, Differential ; Diffusion Magnetic Resonance Imaging/*methods ; Female ; Ganglioneuroma/*pathology/surgery ; Humans ; Trigeminal Nerve/*pathology/surgery

Chromogranin A ; metabolism ; Diagnosis, Differential ; Duodenal Neoplasms ; metabolism ; pathology ; surgery ; Ganglioneuroma ; metabolism ; pathology ; Gastrointestinal Stromal Tumors ; metabolism ; pathology ; Humans ; Male ; Middle Aged ; Neurofibroma ; metabolism ; pathology ; Paraganglioma ; metabolism ; pathology ; surgery ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism