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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 5  |  Issue : 4  |  Page : 220-223

Paraganglioma: A rare retroperitoneal tumor


1 Department of Surgery, Urology Unit, University of Benin Teaching Hospital, Benin City, Nigeria
2 Department of Pathology, University of Benin Teaching Hospital, Benin City, Nigeria

Date of Web Publication21-Jan-2016

Correspondence Address:
Dr. Edwin Oduagbon Obarisiagbon
Department of Surgery, Urology Unit, University of Benin Teaching Hospital, Benin City
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.174674

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  Abstract 

Paraganglioma is a rare catecholamine-secreting tumor, that is, found anywhere in the line of the sympathetic chain and that of the parasympathetic ganglia. They usually present with severe hypertension which can be challenging to the surgeon during the management. We successfully managed a 28-year-old lady who presented with a 6 year history of episodic hypertension, palpitation, insomnia, excessive sweating, and weight loss. An abdominal ultrasonography and computed tomography scan showed a retroperitoneal mass in front of the left kidney. She subsequently had exploratory laparotomy and excision of the tumor with resolution of symptoms. Histology of the mass revealed features consistent with a paraganglioma. She is presently being followed up in our surgical out-patient clinic.

Keywords: Abdomen, paraganglioma, retroperitoneal tumor


How to cite this article:
Obarisiagbon EO, Agbugui JO, Forae G. Paraganglioma: A rare retroperitoneal tumor. Arch Int Surg 2015;5:220-3

How to cite this URL:
Obarisiagbon EO, Agbugui JO, Forae G. Paraganglioma: A rare retroperitoneal tumor. Arch Int Surg [serial online] 2015 [cited 2019 Aug 24];5:220-3. Available from: http://www.archintsurg.org/text.asp?2015/5/4/220/174674


  Introduction Top


Pheochromocytoma, according to 2004, WHO classification of endocrine tumors was defined as tumor which arise from catecholamine-producing chromaffin cells in the adrenal medulla. Closely related tumors found in the extra-adrenal sympathetic and parasympathetic paraganglia were classified as extra-adrenal paragangliomas. [1] Paragangliomas may develop anywhere there are sympathetic nerve cells, and this usually means along any of the major arteries in the body. Paragangliomas can be found in the skull region, neck, chest cavity, abdomen, pelvis, and the bladder. The most common site is within the abdomen where approximately 85-90% is located. They have only about a 5% incidence of malignancy, these tumors are associated with a high risk of morbidity and mortality from cardiovascular complications. [2] Most paragangliomas occur as sporadic tumors. However, certain hereditary syndromes such as von Hippel-Lindau syndrome, multiple endocrines neoplasia Type 2, neurofibromatosis Type 1, and familial paraganglioma syndrome have been associated with the development of paragangliomas. [3] Because of their rarity, little information is available regarding the natural history of these tumors and patient outcome after resection, and especially regarding the diagnosis of malignant tumors. [4] We present here a case of a 28-year-old lady with features of abdominal paraganglioma managed in our unit.


  Case Report Top


NC is a 28-year-old lady who was referred to our outpatient clinic with 6 years history of intermittently elevated blood pressure (BP). There were associated tremulousness' anxiety insomnia palpitation, excessive sweating, and weight loss. There was no significant family history. At presentation, she was calm though sweating at rest, the BP was 260/130 mmHg, and pulse was 132/min. There was no neck or thyroid mass palpable. The abdominal examination did not reveal any abnormality.

A working diagnosis of pheochromocytoma, to rule out a paraganglioma was made. An abdominal ultrasonography showed a large retroperitoneal mass in the region of the left kidney. This was supported by an abdominal computed tomography scan that also revealed a heterogeneous mass (5 cm × 8 cm) lying anterior to the lower pole of the left kidney [Figure 1]. A 24 h urine vanillylmandelic acid (VMA) was 12 mg/day. She was subsequently commenced on prazosin, and propranolol to control the BP in conjunction with the endocrinology unit of the hospital. Her thyroid function test, serum calcium, phosphate, alkaline phosphatase, and other parameters were all within normal limits. Her hematocrit at admission was 36%. She was transfused with 2 units of whole blood and adequately hydrated with normal saline few days before surgery. She subsequently had exploratory laparotomy and excision of a retroperitoneal mass. The findings at operation include a large retroperitoneal mass that measured 8 cm × 5 cm × 4 cm intimately related to the lower pole of the left kidney and the abdominal aorta but not attached to them. No enlarged lymph nodes were seen, and both adrenal glands were normal. The excised mass weighed 124.1 g [Figure 2].
Figure 1: A coronal section of the abdomen showing the tumor X on computed tomography scan

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Figure 2: Gross specimen of the excised tumor (bisected)

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Intraoperatively her BP was closely monitored and controlled with sodium nitroprusside. Microscopically, sections showed benign neoplastic lesions composed of large polyhedral cells with granular cytoplasm and pleomorphic nuclei. The cells were disposed in cords and alveolar pattern, and oriented around, and closely opposed to thin walled sinusoidal vascular channels. This was consistent with a paraganglioma [Figure 3].
Figure 3: Microscopic section of the tumor

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Her postoperative period was uneventful. Her BP gradually normalized over days. All other symptoms have resolved. She is presently being followed up as an outpatient. Her last 24 h urine VMA was 1.8 mg/day.


  Discussion Top


Catecholamine producing tumors of extra-adrenal origin are known as paragangliomas. Paraganglioma may arise in any portion of the paraganglion system though they most commonly occur below the diaphragm, frequently in the organ of Zuckerkandl. They represent at least 15% of adult and 30% of childhood pheochromocytomas, as opposed to the traditional teaching that 10% of all pheochromocytomas are at the extra-adrenal site. [5] It most commonly occurs in the second and third decade of life with a slight male preponderance. This is in contrast to adrenal pheochromocytomas, which typically are diagnosed in the fourth and fifth decades with a slight propensity for women. [5] Episodic tachycardia, sweating, headache, and signs of paroxysmal hypertension are classic of pheochromocytoma or paraganglioma. [6],[7] These were all noted in our patient. These symptoms arise as a consequence of excessive catecholamine release. Between episodes, BP can be normal. However, clinical presentation can differ, depending on the catecholamine-releasing profile of the tumor. A tumor predominantly secreting epinephrine is usually associated with paroxysmal hypertension while the norepinephrine-secreting variant is associated with sustained hypertension. [8],[9]

Preoperative management requires appropriate pharmacologic blockade. In the case of our index patient, prazosin a selective α-blocker was used. Phenoxybenzamine (a nonselective α-blocker) can equally be used, but it is not readily available in our environment. A β-blocker is added if there is an α-blocker induced tachycardia. Alternatively, Salinas et al. recommended labetalol (a combined alpha- and beta-adrenoceptor blocker) or calcium-channel blockers (dihydropyridines), used either alone or in combination with adrenoceptor blockers. [10] It is also pertinent to carry out adequate hydration and volume expansion prior to surgery. This, we did by transfusing the patient despite a fairly satisfactory hematocrit.

Intraoperatively, the aim is usually complete surgical resection of the tumor even though this may be challenging because of the vascularization and its proximity to major vessels. Laparoscopic resection is preferred to open surgery, especially for small tumors. However, where the tumor is big, malignancy cannot be ruled out, and there is uncontrolled intraoperative bleeding one may resort to open retroperitoneal dissection. [11],[12] Open dissection is also done when facility for laparoscopy is not available. We were able to achieve a complete resection of the tumor macroscopically without vascular injury. The diagnosis of paraganglioma was made in this patient based on the clinical features, radiological, intraoperative, and histological findings. Although immunostaining for neuroendocrine markers such as chromogranin, synaptophysin, CD56, and CD57 was not done because they were not readily available, the hematoxylin and eosin staining was separately and independently read by five pathologists before the diagnosis was concluded.

Postoperatively the serum catecholamines levels will normalize and symptoms resolve. The urinary VMA in our patient fell to a normal level when it was repeated 4 weeks after surgery. The prognosis is excellent if the tumor is benign and completely excised, with a 5-year survival rate >95%. Feng et al. noted, however, that the exact survival in the malignant cases is difficult to determine. [4] Although the incidence is lower, the survival rate is related to the familial circumstance, the stage of the disease at the diagnosis, the therapeutic methods, and follow-up after the surgery. [13],[14] It is, therefore, pertinent that a long-term follow-up should be done using clinical, biochemical, and radiological modalities.


  Conclusion Top


The management of patients with paraganglioma should be performed by teams of experienced anesthesiologists, endocrinologists, and surgeons in order to prevent perioperative complications and reduce the morbidity. Although a laparoscopic approach is favored open surgery becomes an alternative when the facility is not available. Successful surgical excision is associated with the complete resolution of the symptoms. However, long-term follow-up is the rule.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Pacak K, Eisenhofer G, Ahlman He, Bornstein SR, Gimenez-Roqueplo AP, Grossman AB, et al. Pheochromocytoma: Recommendations for clinical practice from the First International Symposium. October 2005. Nat Clin Pract Endocrinol Metab 2007;3:92-102.  Back to cited text no. 1
    
2.
Baudin E, Habra MA, Deschamps F, Cote G, Dumont F, Cabanillas M, et al. Therapy of endocrine disease: Treatment of malignant pheochromocytoma and paraganglioma. Eur J Endocrinol 2014;171:R111-22.  Back to cited text no. 2
    
3.
Därr R, Lenders JW, Hofbauer LC, Naumann B, Bornstein SR, Eisenhofer G. Pheochromocytoma - Update on disease management. Ther Adv Endocrinol Metab 2012;3:11-26.  Back to cited text no. 3
    
4.
Feng N, Zhang WY, Wu XT. Clinicopathological analysis of paraganglioma with literature review. World J Gastroenterol 2009;15:3003-8.  Back to cited text no. 4
    
5.
Whalen RK, Althausen AF, Daniels GH. Extra-adrenal pheochromocytoma. J Urol 1993;147:1-10.  Back to cited text no. 5
    
6.
Stein PP, Black HR. A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institution′s experience. Medicine (Baltimore) 1991;70:46-66.  Back to cited text no. 6
    
7.
Bravo EL. Pheochromocytoma: New concepts and future trends. Kidney Int 1991;40:544-56.  Back to cited text no. 7
    
8.
Bravo EL, Tagle R. Pheochromocytoma: State-of-the-art and future prospects. Endocr Rev 2003;24:539-53.  Back to cited text no. 8
    
9.
Langer SZ. Presynaptic regulation of the release of catecholamines. Pharmacol Rev 1980;32:337-62.  Back to cited text no. 9
[PUBMED]    
10.
Salinas CL, Beltran OD, Sánchez-Hidalgo JM, Bru RC, Padillo FJ, Rufián S. Emergency adrenalectomy due to acute heart failure secondary to complicated pheochromocytoma: A case report. World J Surg Oncol 2011;9:49.  Back to cited text no. 10
    
11.
Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet 2005;366:665-75.  Back to cited text no. 11
    
12.
Humphrey R, Gray D, Pautler S, Davies W. Laparoscopic compared with open adrenalectomy for resection of pheochromocytoma: A review of 47 cases. Can J Surg 2008;51:276-80.  Back to cited text no. 12
    
13.
Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P, et al. Biochemical diagnosis of pheochromocytoma: Which test is best? JAMA 2002;287:1427-34.  Back to cited text no. 13
    
14.
Furuta N, Kiyota H, Yoshigoe F, Hasegawa N, Ohishi Y. Diagnosis of pheochromocytoma using [123I]-compared with [131I]-metaiodobenzylguanidine scintigraphy. Int J Urol 1999;6:119-24.  Back to cited text no. 14
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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