OBJECTIVE: The Transatlantic Australasian Retroperitoneal Sarcoma Working Group conducted a retrospective study on the disease course and clinical management of ganglioneuromas. BACKGROUND: Ganglioneuromas are rare tumors derived from neural crest cells. Data on these tumors remain limited to case reports and single-institution case series. METHODS: Patients of all ages with pathologically confirmed primary retroperitoneal, intra-abdominal, and pelvic ganglioneuromas between January 1, 2000, and January 1, 2020, were included. We examined demographic, clinicopathologic, and radiologic characteristics, as well as clinical management. RESULTS: Overall, 328 patients from 29 institutions were included. The median age at diagnosis was 37 years with 59.1% of patients being female. Symptomatic presentation comprised 40.9% of cases, and tumors were often located in the extra-adrenal retroperitoneum (67.1%). At baseline, the median maximum tumor diameter was 7.2 cm. One hundred sixteen (35.4%) patients underwent active surveillance, whereas 212 (64.6%) patients underwent resection with 74.5% of operative cases achieving an R0/R1 resection. Serial tumor evaluations showed that malignant transformation to neuroblastoma was rare (0.9%, N=3). Tumors undergoing surveillance had a median follow-up of 1.9 years, with 92.2% of ganglioneuromas stable in size. With a median follow-up of 3.0 years for resected tumors, 84.4% of patients were disease free after resections, whereas recurrences were observed in 4 (1.9%) patients. CONCLUSIONS: Most ganglioneuromas have indolent disease courses and rarely transform to neuroblastoma. Thus, active surveillance may be appropriate for benign and asymptomatic tumors particularly when the risks of surgery outweigh the benefits. For symptomatic or growing tumors, resection may be curative.
- MeSH
- dospělí MeSH
- ganglioneurom * chirurgie MeSH
- lidé MeSH
- nádory měkkých tkání * MeSH
- neuroblastom * MeSH
- progrese nemoci MeSH
- retroperitoneální nádory * chirurgie MeSH
- retrospektivní studie MeSH
- sarkom * chirurgie patologie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
- Research Support, N.I.H., Extramural MeSH
A large tumorous mass completely surrounding and compressing the coeliac trunk was identified on computed tomography in a young woman with a six-month history of progressive abdominal pain. The tumor was excised along with the coeliac trunk and the proximal parts of its branches. The hepatic artery was reconstructed with an aorto-hepatic autogenous bypass. Postoperatively the patient had neurogenic diarrhea, which subsided on medical treatment. Seven months after surgery the patient is in a good state of health and living a normal life.
- Klíčová slova
- aorto-hepatic bypass, coeliac trunk, ganglioneuroma, oncovascular surgery,
- MeSH
- arteria coeliaca diagnostické zobrazování patologie chirurgie MeSH
- arteria hepatica diagnostické zobrazování patologie chirurgie MeSH
- disekce MeSH
- dospělí MeSH
- ganglioneurom diagnostické zobrazování patologie chirurgie MeSH
- lidé MeSH
- ligace MeSH
- vena saphena transplantace MeSH
- výsledek terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
UNLABELLED: The case of a 15-year-old girl with a large ganglioneuroma in the mediastinum and spinal canal is presented. The tumour initially manifested as scoliosis. Its diagnosis was made on the basis of CT scanning and magnetic resonance imaging, and confirmed by thoracoscopic mediastinal biopsy. Radical tumour excision was indicated. The first stage involved removal of the tumour from the spinal canal through a posterior approach, and transpedicular fixation of the spinal column with correction of the curve. At the second-stage procedure, the tumour was removed from the pleural cavity and mediastinum through thoracotomy. At two years after surgery, the spondylodesis was completed with autologous bone grafts that healed within 6 months. At 3-year follow-up the patient was with neither clinical findings, nor subjective complains and imaging methods showed no signs of tumour recurrence. KEY WORDS: ganglioneuroma, scoliosis, correction, thoracotomy, spinal canal, mediastinum.
- MeSH
- ganglioneurom diagnostické zobrazování terapie MeSH
- lidé MeSH
- magnetická rezonanční tomografie metody MeSH
- mladiství MeSH
- nádory mediastina diagnostické zobrazování terapie MeSH
- nádory páteře diagnostické zobrazování terapie MeSH
- počítačová rentgenová tomografie metody MeSH
- skolióza etiologie terapie MeSH
- torakoskopie metody MeSH
- transplantace kostí metody MeSH
- výsledek terapie MeSH
- Check Tag
- lidé MeSH
- mladiství MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
We present the case of a 26-year-old female patient with coincidental ultrasound finding of a neck tumour located below the left thyroid lobe. The tumour slightly increased in size over a year. Fine needle aspiration indicated the neuroectodermal origin of the tumour and surgical removal was performed. Although the tumour mass was carefully and successfully removed, post-operational Horners syndrome appeared. The tumour tissue was diagnosed as ganglioneuroma by histopathology.
- MeSH
- dospělí MeSH
- ganglioneurom diagnóza chirurgie MeSH
- lidé MeSH
- nádory hlavy a krku diagnóza chirurgie MeSH
- nádory měkkých tkání diagnóza chirurgie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Various types of neurogenous tumours occur in the retroperitoneum. Hormonally inactive tumours are often asymptomatic and their diagnosis is mostly accidental. Authors are presenting their recent experience with three cases. They discuss diagnostic options and the therapy of retroperitoneal schwannoma, ganglioneuroma and paraganglioma.
- MeSH
- ganglioneurom patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- neurilemom patologie MeSH
- paragangliom patologie MeSH
- retroperitoneální nádory patologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Eight cases of ganglioneuroma were examined for a presence of perineural cell differentiation, using the immunohistochemical markers epithelial membrane antigen (EMA), claudin-1 and GLUT-1. The mean age of the patients was 42.3 years (range 26-68 years), six patients were females and two were males. Five tumors were located in the adrenal gland and 3 tumors in the retroperitoneum. Morphology of the tumors was typical, i.e., they were composed of neuroid spindle cell population and scattered mature appearing ganglion cells. Spindle cells positive for perineural cell markers claudin-1 and GLUT-1 were found in all lesions, at least focally. EMA+ cells were seen in 2 of 8 tumors. These perineural-type cells were often arranged in organoid fashion around the schwannoid bundles or around the vessels. Our findings indicate that perineural cell differentiation is commonly present in ganglioneuromas.
- MeSH
- buněčná diferenciace MeSH
- claudin-1 analýza MeSH
- dospělí MeSH
- ganglioneurom metabolismus patologie MeSH
- imunohistochemie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mucin 1 analýza MeSH
- nádorové biomarkery analýza MeSH
- nádory nadledvin metabolismus patologie MeSH
- periferní nervy cytologie MeSH
- přenašeč glukosy typ 1 analýza MeSH
- retroperitoneální nádory metabolismus patologie MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- Názvy látek
- claudin-1 MeSH
- mucin 1 MeSH
- nádorové biomarkery MeSH
- přenašeč glukosy typ 1 MeSH
An unusual case of ganglioneuroma with perineural cell differentiation is presented. The tumor was removed from the mediastinum in a 34-year-old male patient. Histologically, it contained neuroid bundles of bland spindle cells, scattered ganglion cells, and some foci of adipocytic metaplasia. Immunohistochemically, the tumor showed expected expressions of S100 protein, neurofilament protein and calretinin. In addition, many spindle cells were positive for perineural cell markers EMA, claudin-1, and GLUT-1. These cells were often arranged in an organoid fashion around the schwannoid bundles. This case indicates that the cells of ganglioneuroma can mature simultaneously towards both Schwann cell and perineural cell phenotypes.
- MeSH
- feochromocytom patologie MeSH
- ganglioneurom patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory nadledvin patologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Three cases of pediatric primary parapharyngeal space tumors, namely a Burkitt's lymphoma, lipoblastoma and a ganglioneuroma are described here, along with a literature review. These disorders are very rare, accounting for less than 20% of all parapharyngeal neoplasms. In comparison to adults, there are differences in pathomorphologic spectrum of pediatric parapharyngeal tumors. The authors describe the prevalence of malignant tumors, mainly soft tissues sarcomas. The salivary gland tumors and paragangliomas which dominate in adult populations, are extremely rare in pediatric populations where the most common benign tumor to be encountered is that of neurogenic origin. It follows that these features modify the diagnostic and therapeutic approach.
- MeSH
- Burkittův lymfom diagnóza chirurgie MeSH
- ganglioneurom diagnóza chirurgie MeSH
- kojenec MeSH
- lidé MeSH
- lipom diagnóza chirurgie MeSH
- nádory hltanu diagnóza chirurgie MeSH
- předškolní dítě MeSH
- Check Tag
- kojenec MeSH
- lidé MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
We report a case of a pigmented composite paraganglioma-ganglioneuroma of the urinary bladder in a 70-year-old female. Grossly, the tumor measured 6.5 cm in diameter and had arisen from the base of the urinary bladder. Histologically, the tumor was composed of approximately equal components of paraganglioma and ganglioneuroma, which were partly separated and partly mixed, and intermingled with each other. There were foci of ample dark brown pigmentation in the cytoplasm of chromaffin paraganglioma cells. The pigment was Masson-Fontana-positive and had been bleached by hydrogen peroxide (H2O2). Electron microscopy showed large, abundant, pleomorphic electron-dense granules consistent with neuromelanin. In addition, there were numerous electron-dense neurosecretory-type granules. Neuromelanin, melanin or lipofuscin are occasionally observed in paragangliomas, although the occurrence of these pigments has never been described in a composite tumor originating from either adrenal medulla or extraadrenal paraganglia. To the best of our knowledge, our report represents the first case of pigmented composite paraganglioma-ganglioneuroma and expands the morphological spectrum of these unusual tumors.
- MeSH
- elektronová mikroskopie MeSH
- ganglioneurom metabolismus patologie MeSH
- imunohistochemie MeSH
- lidé MeSH
- nádory močového měchýře metabolismus patologie MeSH
- paragangliom metabolismus patologie MeSH
- senioři MeSH
- Check Tag
- lidé MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- práce podpořená grantem MeSH
