WORLD JOURNAL OF
SURGICAL ONCOLOGY
Kwon et al. World Journal of Surgical Oncology 2010, 8:42
http://www.wjso.com/content/8/1/42
Open Access CASE REPORT
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Case report
A case of gangliocytic paraganglioma in the
ampulla of Vater
Junsik Kwon, Seung Eun Lee, Mee Joo Kang, Jin-Young Jang* and Sun-Whe Kim
Abstract
Background: Duodenal gangliocytic paraganglioma is an extremely rare tumor and few cases have been reported to
date.
Case presentation: The authors report a case of gangliocytic paraganglioma verified by post-op pathology after
pancreaticoduodenectomy for a tumor in the ampulla of Vater. The 56-year-old male patient concerned visited our
emergency room with melena that started one week prior to hospitalization. The patient was diagnosed to have a
tumor in the ampulla of Vater with bleeding on its surface. However post-op, he was diagnosed as having gangliocytic
paraganglioma by immunohistochemistry.
Conclusion: This tumor has precise clinical implications, and if continuous follow up is conducted after careful
diagnosis and surgical treatment, invasive major operations, such as, radical pancreaticoduodenectomy can be
avoided.
Background
Gangliocytic paragangliomas are rare tumors which are
usually encountered in the second portion of the duode-
num. They can be diagnosed histologically by the pres-
ence of epithelioid, spindle, and ganglion cells, which is
similar to that observed for paraganglioma [1]. Although
gancliocytic paragangliomas have no specific accompany-
ing symptoms, they are sometimes found due to bleeding
caused by mucosal ulceration, and rarely because of huge
mass effect, such as, abdominal pain or obstruction.
However, they are usually detected incidentally during
radiologic imaging conducted for different purposes [2].
Here, we report a gangliocytic paraganglioma in the sec-
ond portion of the duodenum in a patient hospitalized for
melena, which was removed by pancreaticoduodenec-
tomy. We also include a review of the literature.
Case presentation
A 56-year-old male patient visited our emergency room
due to melena of duration one week. History taking
revealed no particular issues other than antihypertensive
medication after a diagnosis of hypertension five years
previously. He did no smoke, but consumed a small
amount of alcohol regularly. No specific features arose
from his family or social history. He did not experience
nausea or vomiting at the time of hospitalization, and
only complained of mild indigestion. Furthermore, he
showed no epigastric soreness, abdominal pain, or weight
loss, and his vital signs at hospitalization were stable. His
physical examination was uneventful. His hemoglobin
was 10.4 g/dL, and renal and liver function, as deter-
mined by blood tests, were also normal. No lesions were
found in the esophagus or stomach by esophagogastrodu-
odenoscopy. However, an exophytic tumor with a bleed-
ing surface ulcer was observed luminally in the ampulla
of Vater in the second portion of the duodenum (Figure
1). An endoscopic biopsy was performed on the tumor
and bleeding from the ulcer was controlled endoscopi-
cally. And abdominal computer tomography (CT) and
magnetic resonance imaging (MRI) revealed a hypoatten-
uating mass of diameter 1.6 cm in the second portion of
the duodenum. The pathological result later revealed
atypical chronic inflammation and regenerative atypia.
Although no malignant cells were observed, surgery was
performed based on the judgment that gross findings
indicated that the possibility of malignancy was high.
During surgery, a papillary 2.5 × 2.0 × 0.7 cm sized mass
was found in the ampulla of Vater. Distant metastasis or
* Correspondence: jangjy4@snu.ac.kr
1
Department of Surgery, Seoul National University College of Medicine, Seoul,
Korea
Full list of author information is available at the end of the article