Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patien...Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patient was found to have a duodenal tumor and enlarged regional lymph nodes. The patient initially underwent a pancreaticoduodenectomy to resect the tumor and involved lymph nodes completely. Histological and immunohistochemical analyses showed findings typical of GP. However, the distant metastatic lesions in the liver and pelvic cavity were rapidly observed after surgery. The patient underwent chemotherapy and radiotherapy, as well as a second surgery to partly remove the metastatic mass in the pelvic cavity. The histological examination revealed no significant difference in histological features between the primary duodenal tumor and the metastatic pelvic mass. However, the patient finally died of the tumor due to the recurrence of the residual pelvic lesion and increased liver mass. To our knowledge, this is the first report of lethal GP with multifocal metastases. Our case confirms that GP should be regarded as a malignant potential tumor with behavior code of “1”, rather than a benign tumor of “0”.展开更多
Gangliocytic paraganglioma(GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindlelike/susten...Gangliocytic paraganglioma(GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindlelike/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using Pub Med, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled(30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain(55%) and gastrointestinal bleeding or sequelae(42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure(except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin(SYN) stained all epithelioid endocrine cells(18/18). Neuron specific enolase(NSE) and SYN stained most ganglion-like cells(7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells(21/21). Our literature review of published cases of GP with lymph node metastasis underscores the excellent prognosis of GP regardless of specific treatment modality. We question the necessity of aggressive surgical intervention in select patients, and argue that local resection of the mass and metastasis may be adequate. We also emphasize the importance of pre-surgical assessment with imaging studies, as well as post-surgical follow-up surveillance for disease recurrence.展开更多
Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes.A 92-year-old female presented with o...Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes.A 92-year-old female presented with obstructive jaundice.On exam she had pale conjunctiva and icteric sclera.Abdominal examination revealed tenderness in the upper abdomen.Laboratory data was consistent with obstructive jaundice.Computed tomography of the abdomen revealed a dilated gall bladder and a common bile duct(CBD)with no evidence of liver lesions or pancreatic head mass.Endoscopic ultrasonography revealed a 1 cm isoechoic submucosal nodule at the periampullary area,dilated CBD(9 mm),a prominent pancreatic duct(4.1 mm)and a hydropic gall bladder with no stones.Endoscopic retrograde cholangiopancreaticography was performed to relieve obstruction and showed a 1 cm periampullary mass which underwent an en-bloc snare resection.Histopathology analyses with immunohistochemical stains were positive for cytokeratin,synaptophysin,S-100 protein,neuron specific enolase and negative for actin and desmin consistent with periampullary gangliocytic paraganglioma.Periampullary gangliocytic paraganglioma is a rare benign tumor of the small bowel.Common presentation includes abdominal pain and obstructive jaundice which should be included in differential diagnosis of obstructive jaundice.Endoscopic resection is a curative therapy in the absence of local invasion or distant metastasis.展开更多
Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or m...Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.展开更多
Gangliocytic paraganglioma(GP) is rare neuroendocrine tumor(NET) with a good prognosis that commonly arising from duodenum. Although the tumor is characterized by its unique triphasic cells(epithelioid, spindle, and g...Gangliocytic paraganglioma(GP) is rare neuroendocrine tumor(NET) with a good prognosis that commonly arising from duodenum. Although the tumor is characterized by its unique triphasic cells(epithelioid, spindle, and ganglion-like cells), the proportions of these three tumor cells vary widely from case to case,and occasionally, morphological and immunohistochemical similarities are found between GP and NET G1(carcinoid tumors). Further, GP accounts for a substantial number of duodenal NETs. Therefore, GP continues to be misdiagnosed, most often as NET G1. However, GP has a better prognosis than NET G1, and it is important to differentiate GP from NET G1. In this article, I wish to provide up-to-date clinicopathological information to help oncologists gain better insight into the diagnosis and clinical management of this tumor.展开更多
Gangliocytic paraganglioma (GP), a rare tumour that arises most frequently from the periampullary area, is considered to be a benign neoplasm with a potential for lymphatic spread. Distant metastases are rare [1]. We ...Gangliocytic paraganglioma (GP), a rare tumour that arises most frequently from the periampullary area, is considered to be a benign neoplasm with a potential for lymphatic spread. Distant metastases are rare [1]. We report a case of a 51 year old female who presented with abdominal pain, anaemia and melaena. Endoscopy, push enteroscopy and biopsy revealed a periampullary neuroendocrine tumour. Immunohistochemistry of the pancreatic duodenectomy specimen demonstrated a duodenal gangliocytic paraganglioma with no lymph node metastases. We review the literature on this rare tumour and the current treatment protocol.展开更多
Paragangliomas are extra‑adrenal neuroendocrine tumors that derive from neural crest.In general,they are benign tumors but few cases had shown a tendency to metastasize.Malignant forms have been reported previously wi...Paragangliomas are extra‑adrenal neuroendocrine tumors that derive from neural crest.In general,they are benign tumors but few cases had shown a tendency to metastasize.Malignant forms have been reported previously with intracranial metastasis from duodenal origin,but primary intracranial origin represents a rare and unusual location for such tumors.Here,we report a rare case of a 48‑year‑old lady who presented with symptomatic right‑sided insular mass with negative metastatic work up.A complete surgical resection had been done with an unexpected diagnosis of primary gangliocytic paraganglioma with malignant features.展开更多
文摘Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patient was found to have a duodenal tumor and enlarged regional lymph nodes. The patient initially underwent a pancreaticoduodenectomy to resect the tumor and involved lymph nodes completely. Histological and immunohistochemical analyses showed findings typical of GP. However, the distant metastatic lesions in the liver and pelvic cavity were rapidly observed after surgery. The patient underwent chemotherapy and radiotherapy, as well as a second surgery to partly remove the metastatic mass in the pelvic cavity. The histological examination revealed no significant difference in histological features between the primary duodenal tumor and the metastatic pelvic mass. However, the patient finally died of the tumor due to the recurrence of the residual pelvic lesion and increased liver mass. To our knowledge, this is the first report of lethal GP with multifocal metastases. Our case confirms that GP should be regarded as a malignant potential tumor with behavior code of “1”, rather than a benign tumor of “0”.
文摘Gangliocytic paraganglioma(GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindlelike/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using Pub Med, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled(30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain(55%) and gastrointestinal bleeding or sequelae(42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure(except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin(SYN) stained all epithelioid endocrine cells(18/18). Neuron specific enolase(NSE) and SYN stained most ganglion-like cells(7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells(21/21). Our literature review of published cases of GP with lymph node metastasis underscores the excellent prognosis of GP regardless of specific treatment modality. We question the necessity of aggressive surgical intervention in select patients, and argue that local resection of the mass and metastasis may be adequate. We also emphasize the importance of pre-surgical assessment with imaging studies, as well as post-surgical follow-up surveillance for disease recurrence.
文摘Gangliocytic paraganglioma is a rare neuroendocrine tumor predominantly arising in the second part of the duodenum with rare local recurrence or metastasis to regional lymph nodes.A 92-year-old female presented with obstructive jaundice.On exam she had pale conjunctiva and icteric sclera.Abdominal examination revealed tenderness in the upper abdomen.Laboratory data was consistent with obstructive jaundice.Computed tomography of the abdomen revealed a dilated gall bladder and a common bile duct(CBD)with no evidence of liver lesions or pancreatic head mass.Endoscopic ultrasonography revealed a 1 cm isoechoic submucosal nodule at the periampullary area,dilated CBD(9 mm),a prominent pancreatic duct(4.1 mm)and a hydropic gall bladder with no stones.Endoscopic retrograde cholangiopancreaticography was performed to relieve obstruction and showed a 1 cm periampullary mass which underwent an en-bloc snare resection.Histopathology analyses with immunohistochemical stains were positive for cytokeratin,synaptophysin,S-100 protein,neuron specific enolase and negative for actin and desmin consistent with periampullary gangliocytic paraganglioma.Periampullary gangliocytic paraganglioma is a rare benign tumor of the small bowel.Common presentation includes abdominal pain and obstructive jaundice which should be included in differential diagnosis of obstructive jaundice.Endoscopic resection is a curative therapy in the absence of local invasion or distant metastasis.
文摘Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.
基金JSPS KAKENHI,No.JP17K08713 from the Ministry of Education,Culture,Sports,Science and Technology of JapanKanagawa Prefectural Hospitals Cancer Fund,No.20182019-Nenndo
文摘Gangliocytic paraganglioma(GP) is rare neuroendocrine tumor(NET) with a good prognosis that commonly arising from duodenum. Although the tumor is characterized by its unique triphasic cells(epithelioid, spindle, and ganglion-like cells), the proportions of these three tumor cells vary widely from case to case,and occasionally, morphological and immunohistochemical similarities are found between GP and NET G1(carcinoid tumors). Further, GP accounts for a substantial number of duodenal NETs. Therefore, GP continues to be misdiagnosed, most often as NET G1. However, GP has a better prognosis than NET G1, and it is important to differentiate GP from NET G1. In this article, I wish to provide up-to-date clinicopathological information to help oncologists gain better insight into the diagnosis and clinical management of this tumor.
文摘Gangliocytic paraganglioma (GP), a rare tumour that arises most frequently from the periampullary area, is considered to be a benign neoplasm with a potential for lymphatic spread. Distant metastases are rare [1]. We report a case of a 51 year old female who presented with abdominal pain, anaemia and melaena. Endoscopy, push enteroscopy and biopsy revealed a periampullary neuroendocrine tumour. Immunohistochemistry of the pancreatic duodenectomy specimen demonstrated a duodenal gangliocytic paraganglioma with no lymph node metastases. We review the literature on this rare tumour and the current treatment protocol.
文摘Paragangliomas are extra‑adrenal neuroendocrine tumors that derive from neural crest.In general,they are benign tumors but few cases had shown a tendency to metastasize.Malignant forms have been reported previously with intracranial metastasis from duodenal origin,but primary intracranial origin represents a rare and unusual location for such tumors.Here,we report a rare case of a 48‑year‑old lady who presented with symptomatic right‑sided insular mass with negative metastatic work up.A complete surgical resection had been done with an unexpected diagnosis of primary gangliocytic paraganglioma with malignant features.
