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

Abstract

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 x 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.

Diagnosis and surgical treatment of retroperitoneal paraganglioma: A single-institution experience of 34 cases

“…In our previous study, conducted between 1999 and 2009, the preoperative CT misdiagnosis rate was 89% (16). In addition, the low diagnosis rate of retroperitoneal paragangliomas using CT and MRI scans may be associated with the ability of these tumors to invade adjacent tissues, including the intestine and pancreas, mimicking intestinal or pancreatic tumors (17–19). In the present study, the tumors were misdiagnosed as intestinal stromal tumors in 2 cases due to the marked association of the tumor with the intestine on CT images.…”

A case of the large retroperitoneal solitary fibrous tumor

“…As the differential diagnosis for the large retroperitoneal tumors, the candidates for consideration would be liposarcoma, leiomyosarcoma, lymphoma [19,20], schwannoma, neurofibroma [20][21][22], leiomyoma [23], myolipoma [24], angiomyolipoma [25], sympathetic paraganglioma [26], desmoid tumor [27], and epithelioid sarcoma [28]. The histological [19][20][21][22] study with the CD34 immunohistochemistry will be helpful in the diagnosis.…”

Case Report: Totally Laparoscopic Resection of Retroperitoneal Paraganglioma Masquerading as a Duodenal Gastrointestinal Stromal Tumor

“…RPGL is a rare entity, which often lacks of typical clinical symptoms related to the excessive secretion of catecholamines and unique radiologic features, making its diagnosis unclear and easy to be misdiagnosed as other neoplasms, such as duodenal GIST, retroperitoneal sarcoma, neurofibroma, pancreatic neoplasm and lymphoma, etc. (2)(3)(4)(5). And as for surgical treatment, RPGLs are often found in close to the abdominal aorta or inferior vena cava, rendering removal difficult.…”