A Retroperitoneal Sympathetic Paraganglioma Invading the Duodenum and Mimicking a Submucosal Tumor

We report a case of an autopsy of unusual retroperitoneal sympathetic paraganglioma (SPG) that directly invaded the duodenum and showed expansive growth mimicking a submucosal tumor. The tumor was clinically suspected to be a gastrointestinal stromal tumor (GIST) of the duodenum because of its locat...

Full description

Saved in:
Bibliographic Details
Published in:Endocrine pathology 2008-06, Vol.19 (2), p.128-132
Main Authors: Kimura, Noriko, Ishidate, Takuzo, Kogawa, Takahiro, Miura, Yukio, Ishizaka, Masanori, Ogita, Masami
Format: Article
Language:English
Subjects:
Aged, 80 and over
Autonomic Nervous System Diseases - metabolism
Autonomic Nervous System Diseases - pathology
Autopsy
Cancer
Catecholamines - metabolism
Cell Proliferation
Diagnosis, Differential
Dopamine beta-Hydroxylase - metabolism
Duodenal Neoplasms - metabolism
Duodenal Neoplasms - pathology
Duodenal Neoplasms - secondary
Endocrinology
Fatal Outcome
Humans
Immunohistochemistry
Ki-67 Antigen - metabolism
Male
Medicine
Medicine & Public Health
Nervous System Neoplasms - metabolism
Nervous System Neoplasms - pathology
Oncology
Paraffin Embedding
Paraganglioma - metabolism
Paraganglioma - pathology
Paraganglioma - secondary
Pathology
Phenylethanolamine N-Methyltransferase - metabolism
Pneumonia - etiology
Pneumonia - pathology
Retroperitoneal Neoplasms - metabolism
Retroperitoneal Neoplasms - pathology
Retroperitoneal Neoplasms - secondary
Tyrosine 3-Monooxygenase - metabolism
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:We report a case of an autopsy of unusual retroperitoneal sympathetic paraganglioma (SPG) that directly invaded the duodenum and showed expansive growth mimicking a submucosal tumor. The tumor was clinically suspected to be a gastrointestinal stromal tumor (GIST) of the duodenum because of its location and extension to the retroperitoneum without catecholamine-associated symptoms. However, a small biopsy specimen of the tumor showed diffuse proliferation of large basophilic cells that were negative for C-kit and CD34, ruling out GIST and indicating an epithelial malignancy. An autopsy revealed that the tumor was mainly in the retroperitoneum, measuring 7.5 × 9.5 cm, weighing 600 g and extending into the duodenum, adjacent to the pancreas but free of the adrenal glands. On cut section, the tumor involved the entire wall of the duodenum. There were no metastases in any organs. For differential diagnosis, endocrine tumors of the duodenum or pancreas and extra-adrenal SPG were considered. The tumor cells were immunohistochemically strongly positive for chromogranin A and were surrounded by cells positive for S100 protein. The Ki67-labeling index was under 1%. The four catecholamine-synthesizing enzymes were detected in the tumor cells. We report this case of SPG with emphasis on differential diagnosis and the significance of its local invasion.
ISSN:1046-3976
1559-0097
DOI:10.1007/s12022-008-9025-3