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

Abstract

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 in the ampulla of Vater: Case report and review of the literature

“…Metastasis to regional lymph nodes is rare and thus does not affect the otherwise excellent outcome [3].…”

“…Histologically, GPs are characterized by the presence of epithelioid cells, spindle-shaped cells and ganglion-like cells [3]. Although these tumors have a benign behavior, a small number of cases have been reported to recur or metastasize to lymph nodes [3].…”

“…Although these tumors have a benign behavior, a small number of cases have been reported to recur or metastasize to lymph nodes [3].…”

A Rare Cause of Melaena-Duodenal Gangliocytic Paraganglioma

“…There is limited use for prognostic immunohistochemistry markers like p53 or bcl-2 expression [15]. Although serum chromogranin A monitoring has not been documented to be part of standard follow up in duodenal GP per se, it is beneficial in follow up of neuroendocrine tumours [16].…”

“…The role of adjuvant treatment in locally metastatic GP remains debatable, given its low likelihood of distant metastases and the curative effect of radical resection [15]. Following pancreaticoduodenectomy, follow up imaging is recommended to check for recurrence.…”