Asymptomatic extra-adrenal paraganglioma masquerading as retroperitoneal sarcoma

Abstract

A 41-year-old man without clinical symptoms was referred for treatment of an enlarging retroperitoneal tumor. Enhanced computed tomography showed a well-defined and heterogeneously enhanced tumor, 4 cm in size, in the dorsal portion of the pancreas. A low-density nodule was detected in the left adrenal gland, 10 mm in diameter. Retroperitoneal sarcoma and nonfunctional left adrenal tumor were suspected, and surgical treatment was performed. During excision of the retroperitoneal tumor, blood pressure was extremely elevated when the tumor was compressed. Blood pressure normalized after excision of the tumor; thus, a diagnosis of paraganglioma was favored over that of retroperitoneal sarcoma. The left adrenal gland was resected together with the adrenal tumor. Microscopically, the tumor cells of the retroperitoneum had round to oval nuclei, and abundant granular amphophilic cytoplasm proliferated in nest-like fashion. Extra-adrenal retroperitoneal paraganglioma was considered, and the adrenal tumor was diagnosed as cortical adenoma. In patients with retroperitoneal tumor, even in the absence of clinical symptoms, we should keep in mind the possibility of extra-adrenal paraganglioma.

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

“…Paragangliomas are classified as functioning and non-functioning tumors defined by the production of catecholamine (9). Due to the deep location of retroperitoneal paraganglioma, especially the non-functional tumor, and the lack of specific clinical manifestations and imaging manifestations, early diagnosis is difficult, and it is easy to cause missed diagnosis and misdiagnosis (4). As in our case, asymptomatic tumor disguised as subserosa type of duodenal GIST, which was not confirmed as paraganglioma until postoperative pathology.…”

“…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.…”

Retroperitoneal Paraganglioma Mimicking Lymph Node Metastasis in an Endometrial Cancer Patient: Case Report

“…So far only a few cases were reported. 6 Our case is the first case of a paraaortic paraganglioma detected during paraaortic lymphadenectomy in an endometrium cancer patient. In our case radiologic appearance leads to suspicion of advanced stage endometrium cancer with lymphatic involvement, however the disease is low risk early stage in fact.…”

A rare case of retroperitoneal paraganglioma located in the neck of the pancreas: a case report and literature review

“…We choose cases diagnosed with PGL located in the pancreas from 14 articles through PubMed from 2001 to 2019 (Table 1) (11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24). These patients' age ranged from 30 to 72 years old and only 3/15 were males.…”

“…These patients' age ranged from 30 to 72 years old and only 3/15 were males. Eight patients (11,15,16,18,(20)(21)(22)(23) were presented to be abdominal discomfort and six (12,14,17,19,23,24) cases were asymptomatic. Only one special patient (13) presented with loss of appetite, weight loss, weakness, and breathlessness.…”