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.
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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)