| Objective: To provide first-hand clinical data which can help to research and reveal the common nature of this rare tumor by reporting a case of duodenal papilla Gangliocytic paraganglioma with local lymph node metastasis.Through analyzing the clinical data and reviewing the related literature,we can improve awareness of the tumor,promote the early diagnosis and early treatment,select the suitable way of diagnosis and treatment.Methods: Make a systematic analysis of the incidence,pathological changes,clinical manifestations,pathological nature,pathological characteristics,immune phenotype,source organization,differential diagnosis,treatment and follow-up of Gangliocytic paraganglioma.by analyzing the clinical data of a Gangliocytic paraganglioma case was confirmed by the pathologic examination postoperative who was treated in the First Affiliated Hospital of Dalian Medical University in October 2013,and reviewing some relevant documents at home and abroad from Pub Med and CNKI database.Results: A 40 years old man patient visited our hospital due to mild upper abdominal pain for more one month.Family history taking revealed no particular issues.His physical examination was uneventful.The abdominal computer tomography(CT)scans revealed a hypoattenuating mass in the third portion of the duodenum.An exophytic tumor was observed by upper gastrointestinal endoscopy in the ampulla of Vater in the second part of the duodenum.The endoscopic biopsy reminded some kind of neuroendocrine tumor.Surgery of pylorus preserving pancreaticoduodenectomy(PPPD)and local lymph node resection was performed.The pathological result as follows: high power fields showed that the tumor was composed of epithelioid cells,ganglion cells and spindle cells,and the epithelioid cells were the most.Immunohistochemistry showed that tumor cells were positive for S-100(supporting cells),Syn and Cg A.Based on the above features,the mass was diagnosed as a GP with submucosal invasion and lymph node metastasis.The patient didn't underwent any chemotherapy or radiotherapy after surgical intervention.About 3 years of follow-up,the patient still alive without recurrence or metastases.According to the literature survey,all the clinical manifestation,pathogenesis and imaging findings of GP are no specificity.Although gastrointestinal endoscopy is helpful in discovering,diagnosing and treating this tumor,the endoscopic biopsy diagnosis rate is only 11.4%.Thus,making a definite diagnosis depend on the pathologic histology after operation.The epithelioid cells,arranged in nests and gland-like structures;The spindle cells formed slender fascicles wrapping around nests of epithelioid cells.According to immunohistochemistry,spindle-shaped mainly showed positive for S-100,epithelioid cells mainly showed positive for neuron specific enolase(NSE)and pancreatic polypeptide(PP),and ganglion cells mainly showed positive for neuron specific enolase(NSE)and synaptophysin.Nither pathologic histology nor immunohistochemistry could identify the nature of the tumor.GP with submucosal invasion or distant metastasis may indicate malignant transformation.The origin of GP is controversial,tending to consider it comes from mucosa related stem cell at present.The tumor is easily misdiagnosed clinically,and should be distinguished from gastrointestinal stromal tumor,gastrointestinal leiomyoma,duodenal adenocarcinoma,neuroendocrine tumor grade 1,paraganglioma phase identification and so on.The main treatment include gastrointestinal endoscopy and surgery,not regular to radiation and chemotherapy.Long-term follow-up after surgery is very necessary.Conclusion: GP is extremely rare in clinic.There were only about 200 cases reported in domestic and overseas,mainly arising in the second portion of the duodenum.Most of this tumor are benign,but GP has some possibility to occur lymph node metastasis and distant metastasis.GP has typical pathologic histology and immunohistochemistry.Gastrointestinal endoscopy and surgery are the main treatment.Radiation or chemotherapy for malignant cases is alternative,but the curative effect is undefined.Long-term follow-up to notice the recurrence and metastasis after surgery is very necessary. |
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