Duodenal Gangliocytic Paraganglioma: Clinicopathologic and Immunocytochemical Study of 11 Cases

Scheithauer, Bernd W.; Nora, Frederick E.; Lechago, Juan; Wick, Mark R.; Crawford, Barbara G.; Weiland, Louis H.; Carney, J. Aidan

doi:10.1093/ajcp/86.5.559

Cited by 118 publications

(83 citation statements)

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“…GPs are usually encountered in the periampullary region [2] of duodenum and rarely in the jejunum, pylorus, esophagus, pancreas and appendix. There have also been reported three cases of pulmonary GPs, that presented with chest pain, pneumonia and Cushing's syndrome respectively [8].…”

Section: B Amentioning

confidence: 99%

“…Mitotic figures are usually scanty and necrosis is absent. Immunohistochemistry is essential for the identification of the three cellular components [2,4]. The epithelioid cells are positive for neuron-specific enolase (NSE), synaptophysin and chromogranin.…”

Section: B Amentioning

confidence: 99%

“…The ganglion cells express NSE and synaptophysin, whereas spindle cells are positive for S100p. [2,7]. Some authors have reported that epithelioid and ganglion cells stain for neuroendocrine peptides such as somatostatin, pancreatic polypeptide and serotonin [9,10].…”

Section: B Amentioning

confidence: 99%

“…Gangliocytic paragangliomas (GPs) are extremely rare benign neuroendocrine tumors of the gastrointestinal tract [1] which occur primarily in the periampullary region in the second part portion of the duodenum [2], as small submucosal lesions. The clinical manifestations of the disease include abdominal pain, nausea, vomiting and gastrointestinal bleeding [2].The latter is due to possible mucosal erosion.…”

Section: Introductionmentioning

confidence: 99%

“…The clinical manifestations of the disease include abdominal pain, nausea, vomiting and gastrointestinal bleeding [2].The latter is due to possible mucosal erosion.…”

Section: Introductionmentioning

confidence: 99%

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

Zizi-Sermpetzoglou¹,

Marinis²,

Myoteri³

et al. 2012

J Cancer Ther Res

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Gangliocytic paraganglioma (GP) is a rare tumor. Until today, only few cases have been reported. Usually GPs are encountered in the second portion of the duodenum, commonly occurring as small submucosal lesions. Histologically, they are characterized by the presence of three different types of cells: epithelioid cells with endocrine growth pattern, resembling paraganglioma or carcinoid tumor cells, spindle cells reminiscent of Schwann cells and ganglia or ganglion-like cells. Generally this tumor has a benign clinical course. Rarely, it may recur or metastasize to regional lymph nodes. We present a case of gangliocytic paraganglioma of the ampulla of Vater in a patient who underwent partial pancreaticoduodenectomy.

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Section: B Amentioning

confidence: 99%

Section: B Amentioning

confidence: 99%

Section: B Amentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…The clinical manifestations of the disease include abdominal pain, nausea, vomiting and gastrointestinal bleeding [2].The latter is due to possible mucosal erosion.…”

Section: Introductionmentioning

confidence: 99%

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

Zizi-Sermpetzoglou¹,

Marinis²,

Myoteri³

et al. 2012

J Cancer Ther Res

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Duodenal gangliocytic paraganglioma with lymph node metastasis in a 17-year-old boy

Inai

Kobuke

Yonehara

et al. 1989

Cancer

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A case of duodenal gangliocytic paraganglioma (DGP) in a 17-year-old boy is presented. In this case a lymph node in the peripancreatic region was involved by a metastatic tumor. A review of the literature on DGP indicates that this case represents the youngest patient and is the second case of DGP with metastasis. Immunohistochemical staining for neuron-specific enolase (NSE), neurofilament (NF), pancreatic polypeptide, and somatostatin showed positive results for epithelioid and ganglion-like cells, whereas spindle cells showed immunoreactivities for S-100 protein, NSE, and NF. The histogenesis of DGP is discussed.Cancer 63:2540-2545, 1989.UODENAL gangliocytic paraganglioma (DGP) is an D extremely rare tumor and its name is derived from the peculiar histologic pattern with a mixed feature of carcinoid tumor, paraganglioma, and ganglioneuroma. In 1957, Dahl et al.' described the first case which was diagnosed as ganglioneuroma and to date about 60 cases of DGP have been reported in the literature. Although almost all of the reported cases were considered to be benign, it has not been clear why a malignant counterpart of this tumor is very rare.* Many theories have been postulated concerning the histogenesis of DGP, but they have so far failed to fully explain this. More recently, some immunohistochemical have been performed using antibodies against a range of general and specific markers for neuroendocrine cells, but the histogenesis of this tumor still remains unclear.We present a case of DGP which occurred in a 17-yearold boy. To our knowledge, this is the youngest reported case and the second case with metastasis to a regional lymph node. We also review briefly the literature on DGP. Case ReportA 17-year-old boy was hospitalized with a chief complaint of massive hematoemesis. His past history was not contributory. On the admission his consciousness was clear, although his systolic blood pressure was 60 mmHg, compatible with hypovolemic shock status. He received blood transfusion (1200 ml). Because of the second episode of hematoemesis, he underwent an exploratory laparotomy. A polypoid tumor was found involving Vater's papilla, and the surface was overlaid with duodenal mucosa, which was ulcerated and bleeding. The tumor was resected and a plastic surgery of choledochus and pancreatic duct was performed.Two months later, he underwent a pancreaticoduodenectomy (Child's procedure) with lymph node dissection. His postoperative course was uneventful and he was in good health without recurrence or metastasis 32 months after the surgery. Pathologic Examination Morphologic MethodsSections from routine, formalin-fixed and paraffinembedded tissues were examined light microscopically. Special staining procedures including periodic acid-Schiff (PAS), alcian blue, mucicarmine, Grimelius and FontanaMasson were performed.For immunohistochemistry, the paraffin sections were deparaffinized and processed for immunohistochemical technique using the avidin-biotin-peroxidase complex. Rabbit antibodies against the following antig...

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The world health organization's histologic classification of gastrointestinal tumors: A commentary on the second edition

Jass

Sobin

Watanabe

1990

Cancer

159

100

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The World Health Organization's (WHO) histologic classification of gastrointestinal tumors has been revised. Although the general basis of classification and the overall outline remain similar to the first edition, advances in the last decade justified changes in classifying certain entities; among them were malignant lymphomas, endocrine tumors, and dysplasias. Several newly recognized entities have also been added.

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Duodenal Gangliocytic Paraganglioma: Clinicopathologic and Immunocytochemical Study of 11 Cases

Cited by 118 publications

References 0 publications

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

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

Duodenal gangliocytic paraganglioma with lymph node metastasis in a 17-year-old boy

The world health organization's histologic classification of gastrointestinal tumors: A commentary on the second edition

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