Retroperitoneal paraganglioma manifesting as paralytic ileus: a case report

Journal of Medical Case Reports, Jun 2012

Introduction Retroperitoneal neoplasms are rare and easily misdiagnosed. These tumors are often discovered incidentally during imaging studies performed for other reasons. Paragangliomas are tumors that arise from extra-adrenal medullary neural crest derivatives. They are usually located in the head and neck but can be found in various body sites, including the chest cavity, abdomen, pelvis and bladder. We report the case of a patient who had a retroperitoneal paraganglioma manifested as paralytic ileus, which is an unusual presentation of a paraganglioma. Case presentation A 63-year-old Taiwanese woman was admitted to the emergency department of our hospital with progressive abdominal fullness for two days. Her medical history included medically controlled hypertension for 10 years and type 2 diabetes mellitus. Plain abdominal radiography showed a solitary loop of the air-filled dilated small bowel. Abdominal computed tomography did not show a mechanical obstruction; however, a retroperitoneal mass was incidentally detected. Histological analysis of the mass led to a diagnosis of a paraganglioma. Conclusions In cases of patients with hypertension presenting with an intestinal pseudo-obstruction, a paraganglioma may be considered as a possible differential diagnosis of retroperitoneal tumors to avoid risky therapeutic procedures or medication that may produce severe adverse effects.

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Retroperitoneal paraganglioma manifesting as paralytic ileus: a case report

Wei-Chen Lin 0 1 Horng-Yuan Wang 0 1 Chen-Wang Chang 0 1 Jiun-Lu Lin 1 3 Chung-Hsin Tsai 1 2 0 Division of Gastroenterology, Department of Internal Medicine, Mackay Memorial Hospital , Taipei , Taiwan 1 Mackay Medicine, Nursing and Management College , Taipei , Taiwan 2 Department of General Surgery, Mackay Memorial Hospital , Taipei , Taiwan 3 Division of Endocrinology and Metabolism, Department of Internal Medicine, Mackay Memorial Hospital , Taipei , Taiwan Introduction: Retroperitoneal neoplasms are rare and easily misdiagnosed. These tumors are often discovered incidentally during imaging studies performed for other reasons. Paragangliomas are tumors that arise from extra-adrenal medullary neural crest derivatives. They are usually located in the head and neck but can be found in various body sites, including the chest cavity, abdomen, pelvis and bladder. We report the case of a patient who had a retroperitoneal paraganglioma manifested as paralytic ileus, which is an unusual presentation of a paraganglioma. Case presentation: A 63-year-old Taiwanese woman was admitted to the emergency department of our hospital with progressive abdominal fullness for two days. Her medical history included medically controlled hypertension for 10 years and type 2 diabetes mellitus. Plain abdominal radiography showed a solitary loop of the air-filled dilated small bowel. Abdominal computed tomography did not show a mechanical obstruction; however, a retroperitoneal mass was incidentally detected. Histological analysis of the mass led to a diagnosis of a paraganglioma. Conclusions: In cases of patients with hypertension presenting with an intestinal pseudo-obstruction, a paraganglioma may be considered as a possible differential diagnosis of retroperitoneal tumors to avoid risky therapeutic procedures or medication that may produce severe adverse effects. - Introduction Retroperitoneal neoplasms are rare tumors and present several therapeutic challenges because of their rarity and relatively late presentation, with soft tissue sarcoma being the most common type. Paragangliomas are one of these retroperitoneal tumors that mainly affect adults who are in the fourth or fifth decade of life, and they have no sex predilection. Approximately 10% of patients with a paraganglioma have a significant family history and one-third of cases are associated with germ line mutations in at least nine genes (NF1, RET, SDHA, SDHB, SDHC, SDHD, TMEM127, MAX and VHL) [1]. Most paragangliomas are located in the head and neck and less than 5% of these tumors are endocrinologically active [2,3]. Except for the paragangliomas that originate from the organ of Zuckerkandl and produce various catecholamines, functional paragangliomas in various body sites secrete only noradrenaline owing to a lack of phenylethanolamine N-methyltransferase in those areas. Most paragangliomas with catecholamine hypersecretion are localized to the abdomen and pelvis [2]. Patients with secretory tumors experience paroxysmal episodic hypertension, as well as the typical triad of symptoms associated with pheochromocytomas, for example, palpitations, headache and sweating. Nonfunctional paragangliomas most commonly manifest as abdominal pain or a mass [4]. In this report, we present a rare case of a retroperitoneal paraganglioma that manifested as paralytic ileus. Case presentation A 63-year-old Taiwanese woman was admitted to our emergency department because she experienced progressive abdominal fullness, nausea and intermittent dull epigastric pain for two days. Relieving or aggravating factors were not present. Her past medical history included well controlled hypertension for 10 years; type 2 diabetes mellitus; and acute appendicitis that occurred after an operation performed 10 years ago. Her blood pressure was 177/100mmHg and heart rate was 86 beats/minute on arrival at our hospital. Her abdomen was mildly distended and tympanic with periumbilical tenderness on palpation. Bowel sounds were hypoactive and the remaining examination yielded normal results. Laboratory investigations showed leukocytosis (white blood cell count: 16,500 cells/uL) and unremarkable amylase and lipase values. Carcinoembryonic antigen and cancer antigen 199 levels were within normal limits. A plain radiograph showed a coiled-spring appearance of her small bowel in the left upper quadrant (Figure 1). Ultrasonography showed a mixed-echoic lesion (diameter approximately 4cm) near the pancreatic body (Figure 2). A computed tomography (CT) scan of her abdomen incidentally showed a hypervascular retroperitoneal mass that had central areas of low attenuation, caused displacement of the anterior aspect of the pancreatic body, and was in proximity to the caudate lobe of her liver (Figure 3). Our patient received surgical treatment after full fasting and adequate hydration. At the time of surgery, a well-demarcated, hypervascular, fragile mass (approximately 6.5cm 6.5cm 4cm) was observed in h (...truncated)


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Wei-Chen Lin, Horng-Yuan Wang, Chen-Wang Chang, Jiun-Lu Lin, Chung-Hsin Tsai. Retroperitoneal paraganglioma manifesting as paralytic ileus: a case report, Journal of Medical Case Reports, 2012, pp. 158, 6, DOI: 10.1186/1752-1947-6-158