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INTRODUCTION: Ganglioneuroma is the most common tumor of the sympathetic nervous system in the adults. It occurs most commonly in the mediastinum and retroperitoneum and are almost always benign tumors in nature. CASE REPORT: Here, we report a case of the retroperitoneal ganglioneuroma mimicking an ovarian tumor in a 20 years old female patient.
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BACKGROUND: Ganglioneuroma (GN) is considered by most to be a benign tumor of neural crest origin. It may evolve from differentiating neuroblastoma or may be diagnosed as primary ganglioneuroma. The rarity of this tumor and the lack of understanding of its biology often lead to inaccurate diagnosis and treatment. METHODS: The authors analyzed clinical features and biologic behavior of primary ganglioneuroma in 49 patients who were registered with but were not part of the national neuroblastoma trials. Data included age and symptoms at diagnosis, gender, tumor localization and size, (123)I-metaiodobenzylguanidine (mIBG) scintigraphy, secretion of catecholamines, histology, treatment, and outcome, whenever available. RESULTS: Patients with primary ganglioneuroma were significantly older than patients with neuroblastoma. Median age at diagnosis was 79 months compared with 16 months (P < 0.0001). Ganglioneuroma were equally distributed between males and females (1.13:1). A preference of thoracic (41.5%) and abdominal, nonadrenal tumors (37.5%) was observed compared with adrenal GN (21%). At diagnosis, thoracic tumors appeared larger than nonthoracic ones. Local lymph node metastases occurred in two patients. One ganglioneuroma had metastasized to soft tissues. (123)I-mIBG scintigraphy detected mIBG uptake at tumor site in 57% of the GN tumors. Levels of catecholamines in plasma and/or urine were increased in 39%. Slight immaturity of ganglion cells was observed in 93% of all ganglioneuroma tumors. None of the 22 tumors analyzed exhibited MYCN gene alterations. Although 12 patients had macroscopic residuals, no tumor progression or recurrence was observed in a median follow-up of 25 months. CONCLUSIONS: Ganglioneuroma may present with metabolic activity such as increased secretion of catecholamines and/or mIBG uptake. There are no specific diagnostic signs or symptoms discriminating ganglioneuroma and neuroblastoma tumors. Therefore, ganglioneuroma requires tissue investigation for diagnosis. Prognosis after surgical resection without further therapy seems to be excellent.
PURPOSE: To determine the radiologic appearance of ganglioneuromas of the adrenal gland and extra-adrenal retroperitoneum. MATERIALS AND METHODS: A retrospective review was performed of imaging findings in nine patients with ganglioneuroma, two with ganglioneuroma-pheochromocytoma, and two with malignant peripheral nerve sheath tumor (PNST) that arose in a ganglioneuroma. Patients underwent computed tomography (CT) (n = 13), magnetic resonance (MR) imaging (n = 5), and ultrasonography (US) (n = 5). RESULTS: In nine patients with ganglioneuroma, the mass ranged in size from 2 x 4 x 4 cm to 21 x 21 x 22 cm. Imaging findings included attenuation entirely or predominantly less than that of muscle on contrast material-enhanced CT images (n = 9); heterogeneous, predominantly high signal intensity on T2-weighted MR images (n = 5); partial or complete surrounding of one or more major blood vessels with little or no luminal narrowing (n = 4) and calcifications (n = 4) on CT scans; and homogeneous, moderate echogenicity (n = 3) on US scans. CT findings in two patients with ganglioneuroma-pheochromocytoma included central necrosis (n = 1) and calcifications (n = 1). In the two patients with malignant PNST that arose in a ganglioneuroma, contrast-enhanced CT showed a large, markedly heterogeneous mass. CONCLUSION: Diagnosis of ganglioneuroma should be considered when typical CT or MR imaging findings are present. Findings in cases of ganglioneuroma-pheochromocytoma and malignant PNST arising in a ganglioneuroma appear to be nonspecific.
STUDY DESIGN: A case report of a lumbar dumbbell tumor that showed a sequential change in histology from ganglioneuroma into rhabdomyosarcoma after long-term dormancy. OBJECTIVE: To present a case of non-neurogenic malignancy arising from ganglioneuroma. SUMMARY OF BACKGROUND DATA: Ganglioneuromas rarely form the dumbbell variety. Whereas conversion of ganglioneuroma into neurogenic malignancies occurs occasionally, between ganglioneuroma and mesenchymal malignancies it has so far been limited to a case in which ganglioneuroma developed after chemo- and radiotherapy for orbital rhabdomyosarcoma. METHODS: A 61-year-old man was referred with a recurrent retroperitoneal tumor that had been excised as ganglioneuroma. Using imaging, histologic, and immunohistochemical studies, a comparative analysis of the primary and recurrent tumors was performed. RESULTS: A total of 44 histologic sections were examined for the primary and the recurrent tumors that had been excised surgically, with an 8-month interval between resections. Histologic and immunohistochemical findings in the primary tumor were consistent with ganglioneuroma, whereas those in the recurrent tumor indicated rhabdomyosarcoma. CONCLUSIONS: It is assumed that the rhabdomyosarcoma arose from a dumbbell ganglioneuroma as a component of malignant ectomesenchymoma.
BACKGROUND: Ganglioneuromas are rare benign tumors arising most commonly from the sympathetic nervous system. They occasionally grow in a dumbbell fashion extending into the spinal canal extradurally. However, ganglioneuromas of the cervical spine with intradural extension or multiple locations or in association with von Recklinghausen's disease are rare. CASE DESCRIPTION: A 35-year-old man with von Recklinghausen's disease presented with tetraparesis and respiratory dysfunction. Preoperative neuroimaging revealed an intradural mass extending from the foramen magnum to the C4 vertebral level, as well as bilateral extravertebral extension connecting it with bilateral paraspinal lesions in a dumbbell fashion. Four intradural tumors associated with the bilateral C2 and C3 nerves and located ventrally were removed, leaving the intraforaminal and extradural portion intact. The procedure resulted in postoperative symptomatic improvement. Second, extravertebral tumors of the left neck, which were not related to the cervical sympathetic nerve, were removed. The pathologic diagnosis of the tumors of both the intradural space and cervical neck was ganglioneuroma. CONCLUSION: We present an extremely rare case in an adult with von Recklinghausen's disease who had bilateral, symmetric and multiple dumbbell ganglioneuromas with intradural extension, and also multiple bilateral ganglioneuromas at the neck. The intradural ganglioneuromas were suspected to have originated from the posterior root ganglions of the bilateral C2 and C3 nerves and to have extended ventrally to the spinal cord involving not only sensory but also motor rootlets; the ganglioneuroma of the neck was suspected to have originated from the cervical nerve itself.
By means of immunohistochemistry we analysed the distribution of chromogranin A, secretogranin II and vasoactive intestinal peptide (VIP) in 16 phaeochromocytomas, two cases of combined phaeochromocytoma-ganglioneuroma and four adrenal ganglioneuromas. Chromogranin A was found in the majority of phaeochromocytes and in mixed phaeochromocytomas-ganglioneuromas. Secretogranin II was present to a lesser degree in phaeochromocytes, but strong immunostaining was found in most ganglion cells of phaeochromocytomas, in the ganglioneuroma component of combined tumours and in adrenal ganglioneuromas. Vasoactive intestinal peptide was present in some ganglion cells of phaeochromocytomas, in the ganglioneuroma component of mixed tumours and in three of four adrenal ganglioneuromas. On semi-adjacent sections a co-localization of VIP and secretogranin II was demonstrated. These results indicate that neuronal differentiation is accompanied by an increased immunohistochemical expression of secretogranin II. Therefore, secretogranin II may be a useful marker for ganglion cell differentiation.
Ganglioneuromas of the thyroid gland of Sprague-Dawley rats were found in 7.2% of 698 rats used in two, 2-year oncogenicity bioassays. The incidence of the tumor was unrelated to treatment or sex. Of the 52 ganglioneuromas of the thyroid gland identified in 50 animals, 40 (80%) had coincident C cell proliferations in the same or contralateral lobe. Ganglioneuromas were contiguous or commingled with C cell proliferations in 63.5% of the cases. The ganglioneuromas consisted of large ganglion cells and, in a few cases, cells thought to be less differentiated neuronal precursors, in a matrix of neurites and Schwann cells. They grew, infiltrating and expanding in the thyroid parenchyma, and did not metastasize. Immunohistochemical staining for calcitonin, S-100 protein, and neurofilaments, as well as electron microscopy, were used to further characterize the tumors. The close association of ganglioneuromas with C cell tumors supports the theory of neural crest origin of C cells and provides a parallel to the association of ganglioneuromas and pheochromocytomas in the adrenal medulla. This is the first report of ganglioneuromas occurring in the thyroid gland or occurring as a common entity in any species.
CONTEXT: Pheochromocytomas of the adrenal gland are a common component of the multiple endocrine neoplasia type 2 (MEN2) syndromes. However, pure adrenal ganglioneuromas, an extremely rare pediatric tumor of neural crest origin composed of mature ganglion cells, have never been reported in association with MEN2 in humans. MEN2A is comprised of medullary thyroid carcinoma (MTC), pheochromocytoma, and parathyroid hyperplasia. MEN2B is characterized by MTC, pheochromocytoma, neural abnormalities of the gastrointestinal tract, and mucosal neuromas. EVIDENCE ACQUISITION: We report two pediatric patients, one with MEN2A and one with MEN2B, who developed isolated adrenal ganglioneuromas without evidence of pheochromocytomas. EVIDENCE SYNTHESIS: MEN2A and MEN2B are caused by activating mutations in the RET proto-oncogene, which encodes a tyrosine kinase receptor essential for signal transduction in neural crest-derived tissues, including the peripheral and enteric nervous systems, C cells of the thyroid gland, and chromaffin cells of the adrenal gland. Both pheochromocytomas and ganglioneuromas originate from neural crest cells. Interestingly, two mouse models of MEN2B exhibit adrenal ganglioneuroma formation. One mouse model develops only ganglioneuromas (but not pheochromocytomas) and expresses only one of the oncogenic RET isoforms. The other mouse model, created by site-directed mutagenesis to simulate the most common human mutation, develops both ganglioneuromas and pheochromocytomas. CONCLUSIONS: Given our two cases, our current understanding of the mouse models, and the common origins of all these tumor cell types, we recommend including ganglioneuromas as a rare, but not unexpected, component of the MEN2 syndromes.
Ganglioneuroma is a benign neoplasm arising from neural crest tissue and is composed of mature ganglion cells and Schwann's cells. Most ganglioneuromas arise in the posterior mediastinum followed by the retroperitoneum. Only a small proportion of ganglioneuromas are adrenal in origin and occur most commonly in children and young adults. Characteristically, ganglioneuromas do not secrete excess catecholamines or steroid hormones, and they are usually clinically silent lesions incidentally detected in imaging studies for unrelated reasons. Because it is an uncommon lesion, adrenal ganglioneuromas is not included in the differential diagnosis of adrenal incidentalomas in adults. We presents a new case of a 6.5 cm adrenal ganglioneuroma incidentally diagnosed in a 50-year old male patient. The exeresis of the adrenal mass and pathohistological study confirmed the diagnosis.
Ganglioneuroma is relatively rare, and difficult to distinguish from other tumors due to lack of image findings specific for ganglioneuromas. In this report, two cases of ganglioneuroma preoperatively diagnosed as non-functioning adrenal tumor and retroperitoneal tumor are reported. A 25-year-old male and a 29-year-old male visited our institute with chief complaints of upper abdominal pain and asymptomatic microscopic hematuria, respectively. Computed tomographic scan and magnetic resonance imaging showed a 7 x 6 x 5 cm solid tumor above the upper pole of the right kidney in the former case, 8 cm poorly enhanced tumor grown surrounding the left renal artery in the latter case. Surgical resection was performed in both cases. In the latder case, since intraoperative histological examination showed no malignant finding, renal vessels penetrating in the tumor were preserved by transecting the tumor. Postoperative histological examination revealed an adrenal ganglioneuroma and a retroperitoneal ganglioneuroma in the former and latter cases, respectively. A ganglioneuroma can be surgically dissected with favorable prognosis, but preoperative differential diagnosis is sometimes difficult due to few specific radiological and laboratory findings.
Background: Adrenal composite pheochromocytoma-ganglioneuroma is uncommon. The aim of this study is to investigate the characteristics of patients with this tumor. Methods: Histologic features of 46 pheochromocytomas diagnosed over a 28-year period (1971-1998) were reviewed. The clinical records, pathologic features, and ploidy patterns of patients with composite pheochromocytoma-ganglioneuroma were studied. Cases reported in the literature were also reviewed and compared with typical pheochromocytomas. Results: Four patients (two men; two women) with composite pheochromocytoma-ganglioneuromas were found. The tumors comprised 8.7% of pheochromocytomas. The two male patients with composite tumors were younger than the two female patients (age = 32, 52 vs 73, 75, respectively). The radiologic, gross, and microscopic appearances of the tumors were heterogeneous. One patient had malignant tumor with distant metastases. Flow cytometric analysis showed that the pheochromocytoma component was aneuploid and the ganglioneuroma portion was diploid. A review of the literature showed 31 adrenal composite pheochromocytoma-ganglioneuromas. Composite tumors were seen in older patients and were bigger than typical pheochromocytomas. Conclusion: Composite pheochromocytoma-ganglioneuromas had distinctive clinicopathologic features. Life-long clinical and biochemical follow-up of these patients is essential in view of the non-diploid DNA pattern.
BACKGROUND: Ganglioneuroma is a rare tumour of neural crest origin, which arises from maturation of a neuroblastoma. While previously considered to be non-functioning, they are now known to be frequently endocrinologically active. AIMS AND METHODS: We report a case of a massive retroperitoneal ganglioneuroma presenting with small bowel obstruction in an adult, 18 years after initial diagnosis. Urinary dopamine levels were elevated, but other catecholamines were within normal limits. This is the first report in the English-language literature of a retroperitoneal ganglioneuroma presenting with or causing intestinal obstruction. We also review the metabolic, radiological, and histological features of these tumours. Relevant publications were identified from a Medline search using the MeSH headings 'ganglioneuroma', 'retroperitoneal neoplasms' and 'intestinal obstruction', and also from the reference lists of retrieved articles. CONCLUSIONS: Ganglioneuroma can grow to a massive size and present in a varied manner. It should be included in the differential diagnosis of any large retroperitoneal or mediastinal mass, including those causing bowel obstruction.
Neuroblastomas and ganglioneuromas frequently produce somatostatin (SOM) and vasoactive intestinal peptide (VIP), and elevated concentrations in tumour tissue are associated with favourable outcome. Both somatostatin and VIP have been shown to have an autocrine effect on tumour growth and differentiation in vitro, and VIP may cause clinical symptoms when released systemically. Using gel-permeation chromatography and specific radioimmunoassays, we further characterised somatostatin-like immunoreactivity (SOM-LI) and VIP-like immunoreactivity (VIP-LI) in neuroblastoma and ganglioneuroma tumour tissue. The major part of SOM-LI and VIP-LI in both neuroblastoma and ganglioneuroma represents the biologically active forms SOM-28, SOM-14 and VIP-2, respectively. 21 children with neuroblastoma and ganglioneuroma were monitored with serial plasma samples during surgery. In 8 children with measurable concentrations of SOM-LI, all showed increased concentrations during tumour manipulation (P = 0.004) that subsequently decreased below preoperative levels in all but one case (P = 0.06). The only child presenting with diarrhoea showed the highest preoperative plasma VIP-LI in the study (54 pmol/l). 2 children with increased concentrations of VIP-LI preoperatively showed a rapid decrease after surgical tumour removal. These findings indicate a systemic release from the tumours. It is concluded that plasma and tumour tissue from children with neuroblastoma and ganglioneuroma contain biologically active molecular forms of somatostatin and vasoactive intestinal peptide. These peptides may bear significance both for specific symptoms in certain patients as well as influencing tumour growth and differentiation in vivo.
Neuroblastoma is an extremely malignant solid tumor in children, characterized by spontaneous differentiation and regression. An epidermal growth factor-like homeotic protein, delta-like (dlk), has been involved in differentiation of neuroblastoma cell lines, but is unknown in in vivo expression of neuroblastoma. By using in situ hybridization and immunohistochemistry, dlk mRNA and protein expression were studied in formalin-fixed archival tissues from 10 patients with neuroblastoma, five with ganglioneuroblastoma, and five with ganglioneuroma. Three adrenal tissues from children died of diseases other than adrenal tumors and one from an adult with pheochromocytoma were severed as normal and disease controls. The results showed strong immunoreactive dlk staining in endothelial cells in neuroblastoma, ganglioneuroblastoma and ganglioneuroma. Dlk was detectable in mature neuromatous stroma and gangliocytes of ganglioneuroma, but not in neuroblasts of neuroblastoma and ganglioneuroblastoma, neither in gangliocytes of ganglioneuroblastoma. In contrast, dlk mRNA expression was mainly observed in the gangliocytes, but was less intense in the neuroblasts and neuromatous stroma cells. Endothelial cells were essentially devoid of dlk mRNA expression. The findings indicated that there is differential expression of dlk gene and protein among neuroblastoma, ganglioneuroblastoma and ganglioneuroma. The stronger expression of dlk in gangliocytes in ganglioneuroma, in contrast to weaker or no expression in gangliocytes in ganglioneuroblastoma and neuroblasts in neuroblastoma, suggests upregulation of dlk during differentiation of neuroblastoma into more benign form. Furthermore, higher dlk protein expression in the tumor endothelium than in the endothelium of normal adrenal gland implies that dlk may regulate the endothelial function in neuroblastic tumors.
AIM: This study analyses the occurrence and distribution of neurotrophins and their receptors in some types of tumours of neural-crest derived cells. METHODS AND RESULTS: Light microscopy immunohistochemistry associated with quantitative image analysis was used to study the expression of neurotrophins (nerve growth factor, brain-derived neurotrophic factor and neurotrophin (NT)-3) and their cognate receptors (p75(LNGFR), TrkA, TrkB and TrkC) in histologically defined ganglioneuroma, phaeochromocytoma and paraganglioma. The material was fixed in 10% formaldehyde, paraffin-embedded and processed for indirect peroxidase immunohistochemistry using a battery of poly- and monoclonal antibodies to detect neurotrophins and their receptors, as well as some neuronal, endocrine and glial cell markers. A subpopulation of cells in phaeochromocytomas and ganglioneuromas expressed NT-3, but not other neurotrophins, while in paragangliomas no neurotrophins were detected. Regarding neurotrophin receptors, all tumours lacked p75(LNGFR), except for the ganglionic part of a case of mixed phaeochromocytoma, whereas they displayed TrkA (two of two ganglioneuromas, six of nine phaeochomocytomas and three of four paragangliomas). Furthermore, TrkC was regularly detected in a neuronal subpopulation in ganglioneuroma. Interestingly, the percentage of neurones expressing TrkA and TrkC was increased with respect to normal tissues in ganglioneuromas, as well as the percentage of the area occupied by TrkA-immunoreactive cells in the phaeochromocytomas. CONCLUSION: The pattern of expression of neurotrophins and neurotrophin receptors in the analysed tumours basically matches that of sympathetic neurones, adrenal chromaffin cells and paraganglionic cells, and suggests responsiveness of these cells to neurotrophins. Nevertheless, the function of TrkA and TrkC in regulating the biology of these tumours, if any, remains to be elucidated.
PURPOSE: The aim of this study was to demonstrate the typical appearance of ganglioneuromas in computer-assisted tomography (CT), and magnetic resonance imaging (MRI). MATERIAL AND METHODS: Retrospective analysis of diagnostic imaging (9 CT, 6 MRI) in 9 children aged 3 to 15 years with the histological diagnosis of ganglioneuroma. RESULTS: The tomographies showed large (max. 13.4 cm in diameter) round or oval tumors with sharp delineation. The sites of the tumors were the retroperitoneum (5), the mediastinum (3), and the adrenal gland (1). Intraspinal tumor involvement occurred in 4 cases. On comparing CT with MRI, MRI was more accurate in defining the intraspinal involvement. The ganglioneuromas were of hypodense appearance in the native CT scan and showed moderate enhancement upon administration of contrast media. In five patients tumor calcifications with a disseminated sprinkled pattern were seen in CT. In MRI T1-weighted scans the tumors were homogeneous and hypointense, after gadolinium application a marked enhancement was evident. In T2-weighted scans the tumors were hyperintense. CONCLUSION: At the time of diagnosis ganglioneuromas are generally large tumors which can be well detected by CT and MRI. Information towards the diagnosis is given by the appearance of the ganglioneuromas in CT and MRI. However, MRI is the modality of choice due to its superiority in documenting intraspinal tumor expansion.