Inter-aorta-caval located tumor: a case report.
ABSTRACT A paraganglioma is a rare tumor that develops out of extra-adrenal chromaffin cells and pheochromocytomas originating from the adrenal medulla. Early diagnosis and surgical planning are crucial, since the tumor secretes catecholamine and is adjacent to large vessels in the abdomen. Furthermore, since complete resection improves the prognosis, we recommend a meticulous surgical technique. Here, we present a case of paraganglioma in a 32-year-old male patient who initially presented with a stomachache. After conducting the required tests, we resected the tumor that was pressing against the vena cava in the interaortocaval region.
- SourceAvailable from: ncbi.nlm.nih.gov[Show abstract] [Hide abstract]
ABSTRACT: Paragangliomas are extra-adrenal chromaffin tumors that arise from neuroectodermal cells of the autonomous nervous system. It is difficult to make an accurate preoperative clinical diagnosis of silent paraganglioma. The best choice of treatment is complete surgical resection. However, it is important to note that in patients with functional paragangliomas, the tumor's ability to produce catecholamines may cause abrupt changes in blood pressure. Thus, surgery may induce life-threatening complications. In the present study, we present a case of functional retroperitoneal multiple paraganglioma in a 39-year-old male patient who was diagnosed during surgery. Four years after the operation, the patient remains asymptomatic and free of disease.Oncology letters 10/2012; 4(4):829-831. · 0.24 Impact Factor
Advance Online Publication
Inter-aorta-caval Located Tumor: A Case Report
Mustafa Kemal Demirag, MD,1 Hakki Kahraman, MD,2 Kenan Erzurumlu, MD,3
Onur Doyurgan, MD,1 Ugur Alp Goksu, MD,2 and Hasan Tahsin Keceligil, MD1
1Depertment of Cardiovascular Surgery, Ondokuz Mayis
University Medical School, Samsun, Turkey
2Division of Endocrinology, Ondokuz Mayis University Medical
School, Samsun, Turkey
3Depertment of General Surgery, Ondokuz Mayis University
Medical School, Samsun, Turkey
Received: August 31, 2009; Accepted: March 31, 2010
Corresponding author: Mustafa Kemal Demirag, MD. Department
of Cardiovascular Surgery, Ondokuz Mayis University Medical
School, 55139, Atakum, Samsun, Turkey
©2011 The Editorial Committee of Annals of Thoracic and
Cardiovascular Surgery. All rights reserved.
A paraganglioma is a rare tumor that develops out of extra-adrenal chromaffin cells and
pheochromocytomas originating from the adrenal medulla. Early diagnosis and surgical
planning are crucial, since the tumor secretes catecholamine and is adjacent to large vessels
in the abdomen. Furthermore, since complete resection improves the prognosis, we recom-
mend a meticulous surgical technique. Here, we present a case of paraganglioma in a
32-year-old male patient who initially presented with a stomachache. After conducting the
required tests, we resected the tumor that was pressing against the vena cava in the interaor-
Key words: pheochromocytoma, aortography, surgery
A Pheochromocytoma is a rare tumor that stems from
chromaffin cells of the sympathetic nervous system. The
tumors secrete catecholamine and cause hypertensive
episodes. In 90% of patients, the cells stem from the
adrenal medulla, and in 10%, from the extra-adrenal
medulla, and are called paraganglioma.1) Hypertension,
headache, sweating, weight loss and palpitation are com-
mon symptoms of these aggressive and malignant
tumors. Furthermore, since they are adjacent to major
vessels such as aorta and vena cava inferior, they must be
treated aggressively. It is possible to diagnose these
tumors with magnetic resonance imaging (MRI), scintig-
raphy and computerized tomography (CT).2, 3)
A 32-year-old male patient who had been diagnosed
with a tumor in his abdomen was subsequently referred
to the Department of Cardiovascular Surgery, Ondokuz
Mayis University Medical School, Turkey. The general
examination of the patient indicated no serious health
problems. His blood pressure was 130/75 mm Hg with a
pulse of 86 beats/min. We detected sensitivity on his
right quadrant with palpation. Other systemic examina-
tions produced normal results. Upper abdominal CT
examination showed a tumor on the anterior part of the
anteraortocaval region. The tumor occupied the area
from the inferior part of the liver to the iliac bifurcation
and pressed against the inferior vena cava. The tumor
also contained a necrotic part in its center, with dimen-
sions of 9 × 6 × 8 cm (Figs. 1 and 2). We suspected
paraganglioma due to the location of the tumor and the
level of contrast material, revealed by angiography (Fig.
2). Metaiodobenzylguanidine (I-131 MIBG) concentrated
in the right part of the mid line of the inferior liver in all
body scanning images, and the catecholamine level in
24-h urine examinations was high, which led to the diag-
nosis of paraganglioma. The serum counting blood cells
(CBC) and biochemical examinations were normal.
The patient exhibited no hypertensive episode or fever
during the clinical observations. Considering the diagno-
sis of paraganglioma, the cardiovascular surgical team
Demirag MK, et al.
Advance Online Publication
with specialists from the General Surgery Clinic resected
the tumor. During the operation, we could see a tumor of
7 × 7 cm that was adherent to the aorta and vena cava.
The tumor was retroperitoneally located on the inferior
part of the duodenum. We carefully excised the tumor
from the edges of the aorta and vena cava, so as not to
damage the adjacent vessels. The patient had a complica-
tion-free post-operative period and was discharged on the
sixth day following the operation. The pathological
examination of the surgical specimen indicated pheo-
chromocytoma and confirmed paraganglioma, as well.
Pheochromocytoma stemming from extra-adrenal
chromaffin cells is called “extra-adrenal pheochromocy-
toma” or “paraganglioma.”4) The paraganglioma is
located in the extra-adrenal region and secretes cate-
cholamine; it stems from chromaffin cells of the sympa-
thetic nerve system,5) proliferating on the carotid mass,
jugular foramen, mediasten, Zuckerkandl organ (located
around the aortic bifurcation) and the peri-aortic area.
However, paraganglioma also proliferates in areas near
sympathetic ganglions.6) Eighty-five percent of tumors
are located beneath the thoracic diaphragm; they do not
usually invade between the abdominal aorta and inferior
In the case described here, the tumor was located on
the inferior part of the liver and anterior part of the
interaortacaval region. We carefully dissected the tumor
from the vessel walls and the surrounding tissues. In
comparison with adrenal pheochromocytoma, the metas-
tasis ratio for paraganglioma is higher at the time of
diagnosis,8) particularly in men in the 4th and 5th decades
of their lives; however, the tumor can be detected in peo-
ple of other ages as well.6, 9) Most of the retroperitoneal
paragangliomas are functional, leading to symptoms that
are similar to those of adrenal pheochromocytoma, such
as paroxysmal hypertension, sweating, headache, palpita-
tion springing from the increase in serum cate-
cholamine.10) Paraganglioma is generally observed spo-
radically; however, approximately 10% of the tumors are
associated with genetic diseases such as neurofibromato-
sis type 1, von Hippel-Lindau disease, carney triad syn-
drome and multiple endocrine neoplasia (MEN) type 2.1–
4) The serum and 24-hour urine catecholamine levels are
often high in such cases; however, our patient had normal
catecholamine levels and no history of a genetic disease.
Several methods are used in detecting the localization
of the tumor. Although CT and MRI have high sensitivi-
ties, their specificities are low. For I-131 MIBG, its sensi-
tivity is 77% for paraganglioma, and its specificity is
88%–100% for adrenal pheochromocytoma. Further-
more, MIBG is valuable because it can reveal areas of
tumor metastases; thus, it is an indispensable tool in the
arsenal of post-operative follow-up.7–10)
A Paraganglioma, a rare tumor, can pose a serious risk
in the patient since it usually presses against major ves-
sels in the abdomen. Thus, early diagnosis and immediate
surgical intervention are essential. Surgery is especially
preferred for large tumors, which require long-term fol-
low-up since they are malignant and can metastasize.
Fig. 1 Abdominal-CT scan of tumor mass.
Ao, aorta; Tm, tumor; IVC, inferior vena cava
Fig. 2 Abdominal DSA angiogram of hiper-
vascular mass, located to the right of
the aorta (black arrows).
Inter-aorto-caval Located Tumor
Advance Online Publication
1) Samaan NA, Hickey RC, Shutts PE. Diagnosis, local-
ization and management of pheochromocytoma.
Cancer 1988; 62: 2451-60.
2) Rha SE, Byun JY, Jung SE, Chun HJ, Lee HG, et al.
Neurogenic tumors in the abdomen: tumor types and
imaging characteristics. Radiographics 2003; 23:
3) Benn DE, Gimenez-Roqueplo AP, Reilly JR, Bertherat
J, Burgess J, et al. Clinical presentation and penetrance
of pheochromocytoma/paraganglioma syndromes. J
Clin Endocrinol Metab 2006; 91: 827-36.
4) Taneri F. Feokromositoma: Etiyoloji ve tanı. Turkiye
Klinikleri J Surg 2000; 5: 131-9. (in Turkish)
5) Dahia PL. Evolving concepts in pheochromocytoma
and paraganglioma. Curr Opin Oncol 2006; 18: 1-8.
6) Lee KY, Oh YW, Noh HJ, Lee YJ, Yong HS, et al.
Extraadrenal paragangliomas of the body: imaging
features. AJR 2006; 187: 492-504.
7) Lenders JWM, Eisenhofer G, Mannelli M, Pacak K.
Phaeochromocytoma. Lancet 2005; 366: 665-75.
8) Whitson BA, Tuttle TM. Laparoscopic resection of
periaortic paragangliomas. Am Surg 2005; 71: 450-4.
9) Cunningham SC, Sub HS, Winter JM, Montgomery E.
Retroperitoneal paraganglioma: single-institution
experience and rewiev of the literature. J Gastrointest
Surg 2006; 10: 1156-63.
10) Hayes WS, Davidson AJ, Grimley PM, Hartman DS.
Extraadrenal retroperitonel paraganglioma: clinical,
pathologic and CT findings. AJR Am J Roentgenol
1990; 155: 1247-50.