Case Of Duodenal Gangliocytic Paraganglioma Research Articles

A case of duodenal gangliocytic paraganglioma treated by endoscopic papillectomy

A case of duodenal gangliocytic paraganglioma treated by endoscopic papillectomy

内視鏡的切除術を施行した十二指腸乳頭部gangliocytic paragangliomaの1例

内視鏡的切除術を施行した十二指腸乳頭部gangliocytic paragangliomaの1例

Recurrent episodes of acute pancreatitis due to duodenal gangliocytic paraganglioma: report of a case.

Gangliocytic paraganglioma is a rare tumor, almost always located in the second portion of the duodenum, and manifested with upper gastrointestinal bleeding and abdominal pain. To date, only one case of duodenal gangliocytic paraganglioma presented with recurrent acute pancreatitis has been reported in the literature.We present a 72-year-old woman admitted to the hospital due to recurrent episodes of acute pancreatitis. Paraclinical examinations showed a polypoid mass in the second portion of duodenum which was removed surgically by local excision. The preoperative differential diagnosis was suggestive with gastrointestinal stromal tumor or adenoma. The histopathology examination revealed a duodenal gangliocytic paraganglioma. After a follow up period of seventeen months the patient remained without clinical evidence of tumor recurrence.Our case report draws attention to the need for including in our differential diagnosis of recurrent acute pancreatitis the mechanical obstruction of the pancreatic duct due to this tumor.

十二指腸carcinoid tumorと術前診断された十二指腸gangliocytic paragangliomaの1例

十二指腸carcinoid tumorと術前診断された十二指腸gangliocytic paragangliomaの1例

Duodenal gangliocytic paraganglioma showing lymph node metastasis: A rare case report

We describe a case of duodenal gangliocytic paraganglioma showing lymph node metastasis. A 61-year-old Japanese man underwent pylorus preserving pancreaticoduodenectomy to remove a tumor at the papilla of Vater. The section of the tumor extending from the mucosa to submucosa of the duodenum was sharply demarcated, solid, and white-yellowish. Neither necrosis nor hemorrhage was present. Histological examination confirmed the immunohistochemical identification of three components comprising epithelioid cells, spindle-shaped cells, and ganglion-like cells. Epithelioid cells showed positive reactivity for synaptophysin, somatostatin, and CD56. In contrast, spindle-shaped cells showed positive reactivity for S-100 protein, but not for synaptophysin, somatostatin or CD56. Furthermore, we found lymph node metastasis despite lack of bcl-2 and p53 expression. In addition to the rarity of the tumor, we are describing here the present case suggests the malignant potency of the tumor despite lack of acceptable prognostic indicators for neuroendocrine tumor.

Gangliocytic Paraganglioma: Case Report and Review of the Literature

Gangliocytic paraganglioma is a rare tumor, which occurs nearly exclusively in the second portion of the duodenum. Generally, this tumor has a benign clinical course, although rarely, it may recur or metastasize to regional lymph nodes. Only one case with distant metastasis has been reported. We present a case of duodenal gangliocytic paraganglioma treated first by local resection followed by pylorus-preserving pancreaticoduodenectomy. Examination of the first specimen revealed focal nuclear pleomorphism and mitotic activity, in addition to the presence of three characteristic histologic components: epithelioid, ganglion, and spindle cell. In the subsequent pancreaticoduodenectomy specimen, there was no residual tumor identified in the periampullary area, but metastatic gangliocytic paraganglioma was present in two of seven lymph nodes. This case report confirms the malignant potential of this tumor. We review the published literature on gangliocytic paragangliomas pursuing a malignant course. We conclude that surgical therapy of these neoplasms should not be limited to local resection, as disease recurrence, lymph node involvement, and rarely distant metastasis may occur.

Duodenal gangliocytic paraganglioma: a radiological-pathological correlation

Duodenal gangliocytic paraganglioma is a rare tumor that characteristically occurs in the second portion of the duodenum and typically presents with gastrointestinal bleeding. Gangliocytic paragangliomas have a characteristic triphasic microscopic appearance with epithelioid cells, spindle cells, and ganglion cells, resulting in a complex histology with features of paraganglioma, carcinoid, and ganglioneuroma. Duodenal gangliocytic paragangliomas have an excellent prognosis after surgical resection but metastatic spread to regional lymph nodes and recurrence may rarely occur. We report a case of duodenal gangliocytic paraganglioma and discuss the radiological and pathological differential diagnosis of this rare entity.