BACKGROUND Ganglioneuroma(GN) is a rare and benign tumor that originates from autonomic nervous system ganglion cells. The most frequently involved sites are the posterior mediastinum, the abdominal cavity, and the re...BACKGROUND Ganglioneuroma(GN) is a rare and benign tumor that originates from autonomic nervous system ganglion cells. The most frequently involved sites are the posterior mediastinum, the abdominal cavity, and the retroperitoneal space. It rarely occurs in the cervical area, compressing the spinal cord. Neurofibromatosis type 1(NF-1) is an autosomal dominant inheritance disorder, whose prevalence rate approximates one per 3000.CASE SUMMARY We report an extremely rare case of bilateral and symmetric dumbbell GNs of the cervical spine with NF-1. A 27-year-old man with NF-1 presented with a one-year history of gradually progressive right upper extremity weakness and numbness in both hands. Magnetic resonance imaging showed bilateral and symmetric dumbbell lesions at the C1-C2 levels compressing the spinal cord. We performed total resection of bilateral tumors, and the postoperative histopathological diagnosis of the resected mass was GN. After operation, the preoperative symptoms were gradually relieved without complications. To our knowledge,this is the sixth report of cervical bilateral dumbbell GNs.CONCLUSION In some cases, cervical bilateral dumbbell GNs could be associated with NF-1.The exact diagnosis cannot be obtained before operation, and pathological outcome is the current gold standard. Surgical resection is the most effective option, and disease outcome is generally good after treatment.展开更多
BACKGROUND Ganglioneuromas are rare tumors of the sympathetic nervous system that originate from neural crest sympathogonia.Since the cervical spine has rarely been reported as a site for ganglioneuroma,we present a c...BACKGROUND Ganglioneuromas are rare tumors of the sympathetic nervous system that originate from neural crest sympathogonia.Since the cervical spine has rarely been reported as a site for ganglioneuroma,we present a case report on this uncommon manifestation.CASE SUMMARY A 34-year-old male presented with a 4-month history of progressive paralysis of both upper limbs along with an unsteady gait.The touch sensitivity of both hands was reduced,and there was conspicuously high muscle tonus in his upper and lower limbs,along with hyperactive physiological reflection and deep reflexes.Magnetic resonance imaging showed several nodules around the C2-7 intervertebral foramena,among which the masses lying between C1 and C2 were obviously bilaterally compressing the spinal cord.Successful posterior decompression was performed without fixation and the tumors in the upper cervical spine were removed intact,with rapid relief of symptoms.The pathological diagnosis was ganglioneuroma.CONCLUSION Multiple and bilateral ganglioneuroams are a rare occurrence in the cervical spine.In this case report,timely resection of the neoplasms around C1 and C2 resulted in spinal cord decompression,with rapid relief of symptoms and a good prognosis.Including the current case,we are aware of only seven such cases in the literature,of which four arose from Japan,one from China,and one from Sp-ain.We suppose that ethnicity and geographic associations with this rare disease presentation may be an aspect for future consideration and investigation.展开更多
Purpose: Relationship between CT or MR images and histological findings, especially vascularity, has not been adequately evaluated. The purpose of this study was to investigate correlation between contrast enhancement...Purpose: Relationship between CT or MR images and histological findings, especially vascularity, has not been adequately evaluated. The purpose of this study was to investigate correlation between contrast enhancement on CT and MRI and histological findings in posterior mediastinal ganglioneuromas. Materials and Methods: Contrast-enhanced (CE) CT (n = 11) and CE MRI (n = 5) of 12 patients with ganglioneuroma were reviewed. The attenuation, signal intensity, and dynamic enhancement pattern of the tumors were evaluated. The vascularity was histologically evaluated by the numbers of vessels. Results: Enhancement on CE-CT was none, mild, moderate, and high enhancement in 5 (45%), 2 (18%), 3 (27%), and 1 (9%) of the 11 lesions, respectively. Dynamic MRI showed mild, moderate and high enhancement in 3 (60%), 1 (20%) and 1 (20%) cases, respectively. The level of contrast enhancement correlated well only with the number of capillary vessels (r = 0.79, P = 0.0037). Conclusion: The posterior mediastinal ganglioneuromas sometimes show insufficient enhancement particularly on CE-CT. The level of enhancement might correlate with the amount of capillary vessels.展开更多
Ganglioneuroma is an extremely rare tumor that is derived from neural crest. Many ganglioneuroma cases are detected incidentally unless they are large enough to cause compressive symptoms. We report an 18-year-old pat...Ganglioneuroma is an extremely rare tumor that is derived from neural crest. Many ganglioneuroma cases are detected incidentally unless they are large enough to cause compressive symptoms. We report an 18-year-old patient with posterior mediastinal ganglioneuroma which was abutting the descending aorta. The patient underwent successful resection by thoracoscopic approach and was followed up for one year with no complications. In summary, a detailed review with experts in both radiology and pathology is mandated to diagnose these tumors. Informed consent was obtained from the patient.展开更多
Adrenal ganglioneuromas(GNs) constitute rare,differentiated tumors which originate from neural crest cells. GNs are usually hormonally silent and tend to be discovered incidentally on imaging tests. Adrenalectomy is t...Adrenal ganglioneuromas(GNs) constitute rare,differentiated tumors which originate from neural crest cells. GNs are usually hormonally silent and tend to be discovered incidentally on imaging tests. Adrenalectomy is the gold standard for the treatment of primary adrenal GNs. Nevertheless,preoperative differential diagnosis of GNs remains extremely challenging,and thus histopathological examination is required in order to confirm the diagnosis of GN. Overall,prognosis after surgical resection seems to be excellent,without any recurrences or need for adjuvant therapy.展开更多
BACKGROUND Ganglioneuroma (GN) is a rare neurogenic tumor that accounts for about 0.1%- 0.5% of all tumors of the nervous system.It originates from neural crest cells.GN has no specific clinical symptoms or laboratory...BACKGROUND Ganglioneuroma (GN) is a rare neurogenic tumor that accounts for about 0.1%- 0.5% of all tumors of the nervous system.It originates from neural crest cells.GN has no specific clinical symptoms or laboratory findings,which leaves it easily overlooked and misdiagnosed as other tumors.Retroperitoneal GN with very large volume and vascular penetration is extremely rare.CASE SUMMARY We present the imaging and pathological findings of a giant retroperitoneal GN in a child.A 4-year-old boy had suffered from postprandial vomiting for more than 6 mo with no precipitating factors.Abdominal computerized tomographic examination showed a giant cystic mass in the retroperitoneal area.After injection of contrast agent,the mass showed heterogeneous enhancement.Surgery with local excision of the mass was performed to address the embedded abdominal blood vessels,and the histopathological and immunohistochemical diagnosis of the mass was GN.Postprandial vomiting was relieved,and no complications occurred after the operation.CONCLUSION In the diagnosis of giant retroperitoneal hypodense masses in children,GN should be considered if the mass presents delayed enhancement,punctate calcification,and vascular embedding but no invasion.Pathology is the golden standard for the diagnosis of GN,and surgical excision is the optimal treatment for GN.展开更多
BACKGROUND Ganglioneuromas are mature, benign neurogenic tumors that arise from neural crest-derived cells. Given the rarity of these tumors and their often close proximity to major vessels, there is a paucity of repo...BACKGROUND Ganglioneuromas are mature, benign neurogenic tumors that arise from neural crest-derived cells. Given the rarity of these tumors and their often close proximity to major vessels, there is a paucity of reports in the literature of minimally invasive resections of ganglioneuromas near the celiac plexus. We report a case of laparoscopic resection of a retroperitoneal ganglioneuroma adhering to the portal vein and celiac axis.CASE SUMMARY A 27-year-old female was referred to our medical center with a symptomatic retroperitoneal mass. Using high quality preoperative imaging and biopsies, we confirmed the diagnosis of a 4 cm ganglioneuroma abutting the celiac axis, portal vein, and the caudate lobe of the liver. We elected for laparoscopic resection after careful preoperative planning and discussions with the patient. Laparoscopy enhanced visualization of the tumor and its relationships to surrounding vital structures for optimal dissection. Ultrasonic energy devices and adjusting liver retraction to allow for manipulation of the mass facilitated a safe and effective resection in a tight space. There were no operative complications and the patient was discharged home on postoperative day 1 with no residual symptoms upon follow-up. With sufficient experience in laparoscopic surgery and preoperative imaging and diagnostics, a minimally invasive approach for removing this celiac plexus ganglioneuroma was successful.CONCLUSION In carefully selected patients, laparoscopic ganglioneuroma resection is appropriate, reducing postoperative morbidity, hospital length of stay, and recovery time.展开更多
Pheochromocytoma and ganglioneuroma form rare composite tumours of the adrenal medulla comprising less than 3% of all sympathoadrenal tumours. We present a case of intraoperatively detected adrenal medullary tumour of...Pheochromocytoma and ganglioneuroma form rare composite tumours of the adrenal medulla comprising less than 3% of all sympathoadrenal tumours. We present a case of intraoperatively detected adrenal medullary tumour of composite pheochromocytoma and ganglioneuroma diagnosed on histopathology, in a normotensive patient. A 50-year-old male with a past history of chronic obstructive pulmonary disease presented with abdominal pain and significant weight loss since one month. Ultrasound and contrast-enhanced computed tomography abdomen revealed a large lobulated lesion in the distal body and tail of pancreas suggestive of solid and papillary neoplasm of body and tail of pancreas. Intra-operatively, a 15 cm × 10 cm solid lesion with cystic areas was seen arising from the left lower pole of the adrenal gland pushing the pancreas which appeared unremarkable. In our case, exploratory laparotomy with tumour excision was done. Extensive sectioning and microscopic examination of this adrenal tumour confirmed a diagnosis of composite Pheochromocytoma with Ganglioneuroma on histopathology. Immunophenotyping with S-100 further supported the diagnosis. The goal of this report is to increase the awareness of this rare disease and to further identify its variable presentation.展开更多
GANGLIONEUROMA is considered as the most mature and noninvasive form of neuroblastic tumors.It derives from neural crest cells,and can arise from wherever sympathetic tissue exists,1 including neck,posterior mediastin...GANGLIONEUROMA is considered as the most mature and noninvasive form of neuroblastic tumors.It derives from neural crest cells,and can arise from wherever sympathetic tissue exists,1 including neck,posterior mediastinum,adrenal gland,retroperitoneum and pelvis.The two most common locations for this tumor are retroperitoneum and展开更多
Ganglioneuromas (GN) are rare, benign neurogenic tumors that arise from sympathetic ganglia. They are often asymptomatic. However, in rare cases, they may secrete catecholamines, vasoactive intestinal polypeptides (VI...Ganglioneuromas (GN) are rare, benign neurogenic tumors that arise from sympathetic ganglia. They are often asymptomatic. However, in rare cases, they may secrete catecholamines, vasoactive intestinal polypeptides (VIP), or androgenic hormones and manifest with flushing, symptoms of catecholamine excess or chronic diarrhea. We report a young Saudi girl with chronic secretory diarrhea, severe hypokalemia and abdominal distension. After an extensive work up, a well-defined hypodense heterogeneous mediastinal mass was noted in radio imaging. A complete recovery was seen after surgical removal of that mediastinal mass which turned to be a ganglioneuroma.展开更多
Embryologically, chromaffin cells of the pheo-chromocytoma and ganglion cells of the ganglioneuroma are both derived from neural crest cells. Composite pheochromocytoma-ganglioneuroma (Pheo-GN) in a single adrenal gla...Embryologically, chromaffin cells of the pheo-chromocytoma and ganglion cells of the ganglioneuroma are both derived from neural crest cells. Composite pheochromocytoma-ganglioneuroma (Pheo-GN) in a single adrenal gland is very rare. A case report of a patient with composite Pheo-GN of the adrenal gland and renal pelvic cancer is presented. Laparoscopic left adrenalectomy, nephroureterectomy and para-aortic lymphadenectomy were performed. This represents the first report of simultaneous surgical treatment for composite Pheo-GN and renal pelvic cancer.展开更多
Objective:to investigate the clinical and pathological characteristics of bronchial cyst of the posterior mediastinum misdiagnosed as a ganglioneuroma,and to improve the level of their diagnosis,diflerential diagnosis...Objective:to investigate the clinical and pathological characteristics of bronchial cyst of the posterior mediastinum misdiagnosed as a ganglioneuroma,and to improve the level of their diagnosis,diflerential diagnosis,and treatment.Methods:the clinical data and pathological findings of a young woman misdiagnosed with a ganglioneuroma was collected and analyzed,and the relevant literature were reviewed.Results:the patient had no specific clinical symptoms.The right posterior mediastinum was accidentally found due to a physical examination for COVID-19.The enhanced chest computed tomography(CT)showed a ganglioneuroma.After a thoracoscopic resection of the lesion,a pathological diagnosis revealed a posterior mediastinal bronchial cyst.Conclusion:bronchial cyst of the mediastinum is rare and their clinical symptoms are atypical and can be easily diagnosed as a ganglioneuroma.It can be preliminarily judged by laboratory and imaging examination and con-finned by pathological examination.The main treatment is surgical resection.展开更多
Retroperitoneal ganglioneuroma is a rare benign tumor that is challenging in terms of clinical diagnosis.Computed tomography and magnetic resonance imaging are usually performed for diagnosis rather than convenient an...Retroperitoneal ganglioneuroma is a rare benign tumor that is challenging in terms of clinical diagnosis.Computed tomography and magnetic resonance imaging are usually performed for diagnosis rather than convenient and inexpensive ultrasonography.Here,we present the case of a 21‐year‐old female patient who was diagnosed by multimodal ultrasound imaging and whose diagnosis was confirmed by ultrasound‐guided core needle biopsy before surgery.We hope that this rare case will help clinicians and radiologists realize the advantages of multimodal ultrasound imaging in the diagnosis of retropeitoneal solid tumors,and reduce misdiagnosis.展开更多
Background Adrenal ganglioneuroma is a rare adrenal pathogenic disease with difficult differential diagnosis from adrenal pheochromocytoma. Currently, very limited literature is available to allow a differential diagn...Background Adrenal ganglioneuroma is a rare adrenal pathogenic disease with difficult differential diagnosis from adrenal pheochromocytoma. Currently, very limited literature is available to allow a differential diagnosis of these two conditions from each other. This study aimed to evaluate the clinical profile, differential diagnosis and surgical treatments of both conditions. Methods Clinical characteristics of 36 patients with adrenal pheocheomocytoma and 18 patients with adrenal ganglioneuroma were analyzed. Data from CT scans and surgical treatments from 1999 to 2007 were collected. Endocrine hormone tests and ^131I-metaiodobenzylguanidine （MIBG） were performed. Neither ^131I-MIBG nor endocrine hormone tests were available in 9 cases of asymptomatic adrenal ganglioneuroma with tumor size less than 4 cm and there were negative findings from contrast enhanced CT scans. The level of urine catecholamine of patients was compared by one-way analysis of variance. Results The mean age of patients in the adrenal ganglioneuroma group was 41.2 years （16-67 year） and in the adrenal pheochromocytoma patients 38 years （17-74 year）. Contrast enhanced CT showed that the foci were intensified in 5 cases （27.8%） of adrenal ganglioneuroma and there were obvious contrast indications in 30 （83.3%） of the pheochromocytoma. Catecholamine levels in a 24-hour urine sample were normal in 4 patients with adrenal ganglioneuroma and increased in 36 （100%） cases with adrenal pheochromocytoma. ^131I-MIBG nuclear scan showed negative results in 4 patients （100%） with adrenal ganglioneuroma and positive results in 25 （96.2%） with adrenal pheochromocytom. Laparoscopy for adrenal tumors was performed through a transperitoneal or retroperitoneal approach during a follow-up period of （43±6） months, and all cases survived well. Conclusions CT, urinary catecholamine and ^131I-MIBG are standard and efficient tools for differential diagnosis of adrenal ganglioneuroma from pheochromocytoma. Laparoscopic surgery can be performed through a transperitoneal or retroperitoneal approach depending on the finding of CT scans. Open surgery is necessary for patients with blood loss of more than 800 ml and violent fluctuation of intraoperative blood pressure.展开更多
Objectives: Ganglioneuroma is a rare, benign neurogenic tumor arising from the sympathetic ganglia. In this report, we reviewed and summarized the clinical features,treatment, and prognosis of a posterior mediastinal ...Objectives: Ganglioneuroma is a rare, benign neurogenic tumor arising from the sympathetic ganglia. In this report, we reviewed and summarized the clinical features,treatment, and prognosis of a posterior mediastinal ganglioneuroma. Case: Here, we report on a 29-year-old man referred to us with transient pain in the right side of the chest, lasting for three days. Physical examination revealed no abnormalities.The results of routine laboratory tests were within the normal ranges. Thoracic spinal magnetic resonance imaging showed a well-defined, solid mass in the right paravertebral region at the T5-T8 level, measuring 7.5 cm × 4.2 cm × 1.5 cm. To accurately locate the lesion during surgery, O-arm intraoperative imaging was used in conjunction with the Stealth Station navigation system. The tumor was completely excised and no related complications occurred. The tumor was an encapsulated mass with a solid, homogenous, grayish-tan cut surface. Histopathological examinations confirmed that the mass was a ganglioneuroma. Conclusions: Ganglioneuroma is a rare, benign tumor. Prior to treatment, a careful imaging evaluation is necessary in order to obtain an accurate diagnosis. A definitive diagnosis can be made by histological examination. En bloc resection is the preferred treatment for ganglioneuroma as it has an excellent prognosis.展开更多
文摘BACKGROUND Ganglioneuroma(GN) is a rare and benign tumor that originates from autonomic nervous system ganglion cells. The most frequently involved sites are the posterior mediastinum, the abdominal cavity, and the retroperitoneal space. It rarely occurs in the cervical area, compressing the spinal cord. Neurofibromatosis type 1(NF-1) is an autosomal dominant inheritance disorder, whose prevalence rate approximates one per 3000.CASE SUMMARY We report an extremely rare case of bilateral and symmetric dumbbell GNs of the cervical spine with NF-1. A 27-year-old man with NF-1 presented with a one-year history of gradually progressive right upper extremity weakness and numbness in both hands. Magnetic resonance imaging showed bilateral and symmetric dumbbell lesions at the C1-C2 levels compressing the spinal cord. We performed total resection of bilateral tumors, and the postoperative histopathological diagnosis of the resected mass was GN. After operation, the preoperative symptoms were gradually relieved without complications. To our knowledge,this is the sixth report of cervical bilateral dumbbell GNs.CONCLUSION In some cases, cervical bilateral dumbbell GNs could be associated with NF-1.The exact diagnosis cannot be obtained before operation, and pathological outcome is the current gold standard. Surgical resection is the most effective option, and disease outcome is generally good after treatment.
文摘BACKGROUND Ganglioneuromas are rare tumors of the sympathetic nervous system that originate from neural crest sympathogonia.Since the cervical spine has rarely been reported as a site for ganglioneuroma,we present a case report on this uncommon manifestation.CASE SUMMARY A 34-year-old male presented with a 4-month history of progressive paralysis of both upper limbs along with an unsteady gait.The touch sensitivity of both hands was reduced,and there was conspicuously high muscle tonus in his upper and lower limbs,along with hyperactive physiological reflection and deep reflexes.Magnetic resonance imaging showed several nodules around the C2-7 intervertebral foramena,among which the masses lying between C1 and C2 were obviously bilaterally compressing the spinal cord.Successful posterior decompression was performed without fixation and the tumors in the upper cervical spine were removed intact,with rapid relief of symptoms.The pathological diagnosis was ganglioneuroma.CONCLUSION Multiple and bilateral ganglioneuroams are a rare occurrence in the cervical spine.In this case report,timely resection of the neoplasms around C1 and C2 resulted in spinal cord decompression,with rapid relief of symptoms and a good prognosis.Including the current case,we are aware of only seven such cases in the literature,of which four arose from Japan,one from China,and one from Sp-ain.We suppose that ethnicity and geographic associations with this rare disease presentation may be an aspect for future consideration and investigation.
文摘Purpose: Relationship between CT or MR images and histological findings, especially vascularity, has not been adequately evaluated. The purpose of this study was to investigate correlation between contrast enhancement on CT and MRI and histological findings in posterior mediastinal ganglioneuromas. Materials and Methods: Contrast-enhanced (CE) CT (n = 11) and CE MRI (n = 5) of 12 patients with ganglioneuroma were reviewed. The attenuation, signal intensity, and dynamic enhancement pattern of the tumors were evaluated. The vascularity was histologically evaluated by the numbers of vessels. Results: Enhancement on CE-CT was none, mild, moderate, and high enhancement in 5 (45%), 2 (18%), 3 (27%), and 1 (9%) of the 11 lesions, respectively. Dynamic MRI showed mild, moderate and high enhancement in 3 (60%), 1 (20%) and 1 (20%) cases, respectively. The level of contrast enhancement correlated well only with the number of capillary vessels (r = 0.79, P = 0.0037). Conclusion: The posterior mediastinal ganglioneuromas sometimes show insufficient enhancement particularly on CE-CT. The level of enhancement might correlate with the amount of capillary vessels.
文摘Ganglioneuroma is an extremely rare tumor that is derived from neural crest. Many ganglioneuroma cases are detected incidentally unless they are large enough to cause compressive symptoms. We report an 18-year-old patient with posterior mediastinal ganglioneuroma which was abutting the descending aorta. The patient underwent successful resection by thoracoscopic approach and was followed up for one year with no complications. In summary, a detailed review with experts in both radiology and pathology is mandated to diagnose these tumors. Informed consent was obtained from the patient.
文摘Adrenal ganglioneuromas(GNs) constitute rare,differentiated tumors which originate from neural crest cells. GNs are usually hormonally silent and tend to be discovered incidentally on imaging tests. Adrenalectomy is the gold standard for the treatment of primary adrenal GNs. Nevertheless,preoperative differential diagnosis of GNs remains extremely challenging,and thus histopathological examination is required in order to confirm the diagnosis of GN. Overall,prognosis after surgical resection seems to be excellent,without any recurrences or need for adjuvant therapy.
文摘BACKGROUND Ganglioneuroma (GN) is a rare neurogenic tumor that accounts for about 0.1%- 0.5% of all tumors of the nervous system.It originates from neural crest cells.GN has no specific clinical symptoms or laboratory findings,which leaves it easily overlooked and misdiagnosed as other tumors.Retroperitoneal GN with very large volume and vascular penetration is extremely rare.CASE SUMMARY We present the imaging and pathological findings of a giant retroperitoneal GN in a child.A 4-year-old boy had suffered from postprandial vomiting for more than 6 mo with no precipitating factors.Abdominal computerized tomographic examination showed a giant cystic mass in the retroperitoneal area.After injection of contrast agent,the mass showed heterogeneous enhancement.Surgery with local excision of the mass was performed to address the embedded abdominal blood vessels,and the histopathological and immunohistochemical diagnosis of the mass was GN.Postprandial vomiting was relieved,and no complications occurred after the operation.CONCLUSION In the diagnosis of giant retroperitoneal hypodense masses in children,GN should be considered if the mass presents delayed enhancement,punctate calcification,and vascular embedding but no invasion.Pathology is the golden standard for the diagnosis of GN,and surgical excision is the optimal treatment for GN.
文摘BACKGROUND Ganglioneuromas are mature, benign neurogenic tumors that arise from neural crest-derived cells. Given the rarity of these tumors and their often close proximity to major vessels, there is a paucity of reports in the literature of minimally invasive resections of ganglioneuromas near the celiac plexus. We report a case of laparoscopic resection of a retroperitoneal ganglioneuroma adhering to the portal vein and celiac axis.CASE SUMMARY A 27-year-old female was referred to our medical center with a symptomatic retroperitoneal mass. Using high quality preoperative imaging and biopsies, we confirmed the diagnosis of a 4 cm ganglioneuroma abutting the celiac axis, portal vein, and the caudate lobe of the liver. We elected for laparoscopic resection after careful preoperative planning and discussions with the patient. Laparoscopy enhanced visualization of the tumor and its relationships to surrounding vital structures for optimal dissection. Ultrasonic energy devices and adjusting liver retraction to allow for manipulation of the mass facilitated a safe and effective resection in a tight space. There were no operative complications and the patient was discharged home on postoperative day 1 with no residual symptoms upon follow-up. With sufficient experience in laparoscopic surgery and preoperative imaging and diagnostics, a minimally invasive approach for removing this celiac plexus ganglioneuroma was successful.CONCLUSION In carefully selected patients, laparoscopic ganglioneuroma resection is appropriate, reducing postoperative morbidity, hospital length of stay, and recovery time.
文摘Pheochromocytoma and ganglioneuroma form rare composite tumours of the adrenal medulla comprising less than 3% of all sympathoadrenal tumours. We present a case of intraoperatively detected adrenal medullary tumour of composite pheochromocytoma and ganglioneuroma diagnosed on histopathology, in a normotensive patient. A 50-year-old male with a past history of chronic obstructive pulmonary disease presented with abdominal pain and significant weight loss since one month. Ultrasound and contrast-enhanced computed tomography abdomen revealed a large lobulated lesion in the distal body and tail of pancreas suggestive of solid and papillary neoplasm of body and tail of pancreas. Intra-operatively, a 15 cm × 10 cm solid lesion with cystic areas was seen arising from the left lower pole of the adrenal gland pushing the pancreas which appeared unremarkable. In our case, exploratory laparotomy with tumour excision was done. Extensive sectioning and microscopic examination of this adrenal tumour confirmed a diagnosis of composite Pheochromocytoma with Ganglioneuroma on histopathology. Immunophenotyping with S-100 further supported the diagnosis. The goal of this report is to increase the awareness of this rare disease and to further identify its variable presentation.
基金Supported by National Natural Science Foundation of China(81101034)
文摘GANGLIONEUROMA is considered as the most mature and noninvasive form of neuroblastic tumors.It derives from neural crest cells,and can arise from wherever sympathetic tissue exists,1 including neck,posterior mediastinum,adrenal gland,retroperitoneum and pelvis.The two most common locations for this tumor are retroperitoneum and
文摘Ganglioneuromas (GN) are rare, benign neurogenic tumors that arise from sympathetic ganglia. They are often asymptomatic. However, in rare cases, they may secrete catecholamines, vasoactive intestinal polypeptides (VIP), or androgenic hormones and manifest with flushing, symptoms of catecholamine excess or chronic diarrhea. We report a young Saudi girl with chronic secretory diarrhea, severe hypokalemia and abdominal distension. After an extensive work up, a well-defined hypodense heterogeneous mediastinal mass was noted in radio imaging. A complete recovery was seen after surgical removal of that mediastinal mass which turned to be a ganglioneuroma.
文摘Embryologically, chromaffin cells of the pheo-chromocytoma and ganglion cells of the ganglioneuroma are both derived from neural crest cells. Composite pheochromocytoma-ganglioneuroma (Pheo-GN) in a single adrenal gland is very rare. A case report of a patient with composite Pheo-GN of the adrenal gland and renal pelvic cancer is presented. Laparoscopic left adrenalectomy, nephroureterectomy and para-aortic lymphadenectomy were performed. This represents the first report of simultaneous surgical treatment for composite Pheo-GN and renal pelvic cancer.
文摘Objective:to investigate the clinical and pathological characteristics of bronchial cyst of the posterior mediastinum misdiagnosed as a ganglioneuroma,and to improve the level of their diagnosis,diflerential diagnosis,and treatment.Methods:the clinical data and pathological findings of a young woman misdiagnosed with a ganglioneuroma was collected and analyzed,and the relevant literature were reviewed.Results:the patient had no specific clinical symptoms.The right posterior mediastinum was accidentally found due to a physical examination for COVID-19.The enhanced chest computed tomography(CT)showed a ganglioneuroma.After a thoracoscopic resection of the lesion,a pathological diagnosis revealed a posterior mediastinal bronchial cyst.Conclusion:bronchial cyst of the mediastinum is rare and their clinical symptoms are atypical and can be easily diagnosed as a ganglioneuroma.It can be preliminarily judged by laboratory and imaging examination and con-finned by pathological examination.The main treatment is surgical resection.
文摘Retroperitoneal ganglioneuroma is a rare benign tumor that is challenging in terms of clinical diagnosis.Computed tomography and magnetic resonance imaging are usually performed for diagnosis rather than convenient and inexpensive ultrasonography.Here,we present the case of a 21‐year‐old female patient who was diagnosed by multimodal ultrasound imaging and whose diagnosis was confirmed by ultrasound‐guided core needle biopsy before surgery.We hope that this rare case will help clinicians and radiologists realize the advantages of multimodal ultrasound imaging in the diagnosis of retropeitoneal solid tumors,and reduce misdiagnosis.
文摘Background Adrenal ganglioneuroma is a rare adrenal pathogenic disease with difficult differential diagnosis from adrenal pheochromocytoma. Currently, very limited literature is available to allow a differential diagnosis of these two conditions from each other. This study aimed to evaluate the clinical profile, differential diagnosis and surgical treatments of both conditions. Methods Clinical characteristics of 36 patients with adrenal pheocheomocytoma and 18 patients with adrenal ganglioneuroma were analyzed. Data from CT scans and surgical treatments from 1999 to 2007 were collected. Endocrine hormone tests and ^131I-metaiodobenzylguanidine （MIBG） were performed. Neither ^131I-MIBG nor endocrine hormone tests were available in 9 cases of asymptomatic adrenal ganglioneuroma with tumor size less than 4 cm and there were negative findings from contrast enhanced CT scans. The level of urine catecholamine of patients was compared by one-way analysis of variance. Results The mean age of patients in the adrenal ganglioneuroma group was 41.2 years （16-67 year） and in the adrenal pheochromocytoma patients 38 years （17-74 year）. Contrast enhanced CT showed that the foci were intensified in 5 cases （27.8%） of adrenal ganglioneuroma and there were obvious contrast indications in 30 （83.3%） of the pheochromocytoma. Catecholamine levels in a 24-hour urine sample were normal in 4 patients with adrenal ganglioneuroma and increased in 36 （100%） cases with adrenal pheochromocytoma. ^131I-MIBG nuclear scan showed negative results in 4 patients （100%） with adrenal ganglioneuroma and positive results in 25 （96.2%） with adrenal pheochromocytom. Laparoscopy for adrenal tumors was performed through a transperitoneal or retroperitoneal approach during a follow-up period of （43±6） months, and all cases survived well. Conclusions CT, urinary catecholamine and ^131I-MIBG are standard and efficient tools for differential diagnosis of adrenal ganglioneuroma from pheochromocytoma. Laparoscopic surgery can be performed through a transperitoneal or retroperitoneal approach depending on the finding of CT scans. Open surgery is necessary for patients with blood loss of more than 800 ml and violent fluctuation of intraoperative blood pressure.
基金Supported by the Beijing Tsinghua Changgung Hospital Fund(grant No.12015C1045)National Natural Science Foundation of China(grant No.81472817)Beijing Municipal Administration of Hospitals Incubating Program(grant No.PX2016066)
文摘Objectives: Ganglioneuroma is a rare, benign neurogenic tumor arising from the sympathetic ganglia. In this report, we reviewed and summarized the clinical features,treatment, and prognosis of a posterior mediastinal ganglioneuroma. Case: Here, we report on a 29-year-old man referred to us with transient pain in the right side of the chest, lasting for three days. Physical examination revealed no abnormalities.The results of routine laboratory tests were within the normal ranges. Thoracic spinal magnetic resonance imaging showed a well-defined, solid mass in the right paravertebral region at the T5-T8 level, measuring 7.5 cm × 4.2 cm × 1.5 cm. To accurately locate the lesion during surgery, O-arm intraoperative imaging was used in conjunction with the Stealth Station navigation system. The tumor was completely excised and no related complications occurred. The tumor was an encapsulated mass with a solid, homogenous, grayish-tan cut surface. Histopathological examinations confirmed that the mass was a ganglioneuroma. Conclusions: Ganglioneuroma is a rare, benign tumor. Prior to treatment, a careful imaging evaluation is necessary in order to obtain an accurate diagnosis. A definitive diagnosis can be made by histological examination. En bloc resection is the preferred treatment for ganglioneuroma as it has an excellent prognosis.
