內科學誌 2018:29:169-174
A Case Report of Retroperitoneal Mass:
Paraganglioma
Chin-Chou Yang
Division of General Medicine, Department of General Psychiatry, Tsaotun Psychiatric Center, Ministry of Health and Welfare, Nantou, Taiwan
Abstract
Pheochromocytoma and paraganglioma are rarely seen cathecolamine-secreting neuroendocrine tumors.
We reported a retroperitoneal tumor with a diameter of 5 cm in a 71 years old man. After admission, he was found loss of consciousness suddenly. Myocarditis related to pheochromocytoma was the most possible reason of sudden collapse. The diagnosis of paraganglioma was made later in ICU (intensive care unit).
Pheochromocytoma or paraganglioma may present with life-threatening cardiovascular manifestations, such as acute myocarditis, myocardial infarction, and hypertensive emergency. It should never been neglected in the differential diagnosis of retroperitoneal mass lesions. (J Intern Med Taiwan 2018; 29: 169-174)
Key Words: Paraganglioma, Pheochromocytoma, Adrenal tumor
Present Illness
A 71 years old man presented with acute pal- pitation, abdominal pain, headache and dizziness in ER (emergency room). He had history of hyperten- sion for 4 years, and his condition remained stable while taking daily combination therapy with Amlo- dipine, Irbesartan, and Propranolol. At ER, his body temperature was 36.4°C, heart rate was 96 beats per minute, and blood pressure was 87/47mmHg. The ECG (Electrocardiogram) revealed normal sinus rhythm. The chest X-ray revealed negative find- ings except atherosclerosis and tortuosity of tho- racic aorta. Abdominal CT was performed in ER.
It revealed a 5cm retroperitoneal mass, considered suspicious of a pancreatic tumor (Figure 1). He was
then admitted on Aug 20, 2014. On Aug 22 early morning, he was found loss of consciousness sud-
Reprint requests and correspondence:Dr. Chin-Chou Yang
Address:Division of General Medicine, Department of General Psychiatry, Tsaotun Psychiatric Center, Ministry of Health and Welfare, Nantou, Taiwan, No. 161, Yuping Rd., Caotun Township, Nantou County 542, Taiwan (R.O.C.)
DOI:10.6314/JIMT.201806_29(3).07
Figure 1. A 5cm heterogeneous soft tissue mass occu-
pied at right para-aortic region with anterior
displacement of duodenum and pancreas
head.
denly. After cardiopulmonary resuscitation and intubation, his vital sign recovered. He was then transferred to ICU (intensive care unit). Elevation of cardiac enzyme was noted. (Troponin-I: 12.67ng/
mL, CK: 966IU/L, CKMB: 74.5ng/mL). ECG revealed QS pattern and disappearance of R wave over anterior wall (Figure 2). Diagnostic cardiac catheterization was performed on Sep 10 and it revealed one vessel CAD (coronary artery disease) (Figure 3). Fluctuating blood pressure was noted in ICU. We checked 24hr-Urine VMA (Vanillylman- delic acid) and catecholamine for 2 days. The data revealed elevation of both VMA and catecholamine (Table 1). Correlated the clinical condition with lab- oratory data and CT findings, functional paragan- glioma was highly suspected. Surgery is indicated.
However, considering the patient’s age, his family
refused operation after discussion. The patient was discharged on Sep 27 with Terazosin and Labetalol for blood pressure control.
Discussion
The terms of “pheochromocytomas” and “cat- echolamine-secreting paragangliomas” are referred to as catecholamine-secreting tumors. Pheochromo- cytomas arise from adrenal medulla whereas para- gangliomas arise from extra-adrenal paraganglia.
The histology of paragangliomas and pheochromo- cytomas are similar. Sometimes, pheochromocyto- mas may be considered as intra-adrenal sympathetic paragangliomas. Because of similar clinical presen- tations and treatments, some clinicians might use
“pheochromocytoma” to refer to both of them. The
classic triad of pheochromocytomas or paragan-
Figure 2. The EKG revealed QS pattern and disappearance of R wave over anterior wall.
Paraganglioma 171
gliomas includes symptoms of episodic headache, sweating, and tachycardia. The pathogenesis of sec- ondary hypertension and the role of catecholamines in the pathophysiology of pheochromocytomas or paragangliomas have been well documented
1. Indeed, sustained or paroxysmal hypertension is common, but about 5 to 15 percent of patients present with normal blood pressure
2. With high sen- sitivity, plasma metanephrines remains the first-line test as diagnosis of pheochromocytomas or para- gangliomas. However, it is not available in some hospital. Another reliable method for diagnosis is measuring metanephrines and catecholamines in a 24-hour urine collection, both of them have high sensitivity and specificity. Adrenalectomy by an experienced surgeon is suggested as treatment of pheochromocytomas or paragangliomas. However, surgical intervention does not always lead to long- term cure. Long-term monitoring is indicated in all patients, even those apparently cured
3.
The laboratory diagnostic cutoffs for pheo- chromocytomas or paragangliomas are based on normal reference of general population. However, false-positive results are common. According to
the UpToDate(Clinical presentation and diagnosis of pheochromocytoma, section on initial biochemi- cal tests), the diagnosis of pheochromocytomas or paragangliomas should be highly suspected if one or more of the following findings are found in 24hr- urine studies:
1. Normetanephrine >900 ug/24 hours or metanephrine >400 ug/24 hours
2. Norepinephrine >170 ug/24 hours 3. Epinephrine >35 ug/24 hours 4. Dopamine >700 ug/24 hours
If positive laboratory evidence is noted, image studies should be initiated to locate the tumor. In this case, a 5cm heterogeneous retroperitoneal mass occupied at right para-aortic region was found in CT scan. In the differential diagnosis of retroperitoneal Table 1. LAB Data
Items Day 1 Day 2 Normal range
Catecholamines
--Norepinephrine 205.6 168.7 11.1~85.5 (ug/dL) --Epinephrine 16.2 15.1 < 22.4 (ug/dL) --Dopamine 157.8 191.3 50~450 (ug/dL)
VMA 11.8 13 1.0~7.5 (mg/day)
Figure 3. Cardiac catheterization data: (A) Left ventriculography demonstrated preserved LV systolic function (EF
65.9%); (B) (C) RCA patent; (D) LAD-D 50% stenosis; LCX hypoplasia.
clinical presentation of myocarditis is highly vari- able. In patients who have elevated cardiac biomark- ers, ECG changes, arrhythmia, or other unexplained cardiac abnormalities, myocarditis should never be neglected. Although Troponin is the most common marker to diagnose acute myocardial infarction, it is not specific for acute thrombotic occlusion of a
catheterization revealed one vessel CAD. Echocar- diogram demonstrated preserved LV systolic func- tion (Table 2). Although it has not been proven by tissue biopsy, the combination of clinical presenta- tion and noninvasive and invasive diagnostic find- ings suggest the diagnosis of myocarditis related to paraganglioma. Also, the pathophysiology of car-
Table 2. Echocardiogram report
Data Normal range
AO (aortic root dimension) 31 20-38 mm
AV (aortic valve) 19 16-26 mm
LA (left atrium) 26 19-40 mm
RV (right ventricle) 10 5-21 mm
EF slope 43 80-150 mm/sec
IVS (Interventricular septum) 10 7-11 mm
LVPW (Left ventricle posterior wall) 11 9-11 mm
IVRT (Isovolumic relaxation time) 110 70-90 msec
LVIDs (Left ventricular internal diameter end systole) 29 24-36 mm
LVIDd (Left ventricular internal diameter end diastole) 43 37-53 mm
LVEDV (Left ventricular end-diastolic volume) 80.8 80-190 ml
LVESV(Left ventricular end-systolic volume) 30.9 16-83 ml
EF (Ejection fraction) 61.8 50-70 %
CO (Cardiac output) 3.4 2.5-7.2 (1/m)
DT (Deceleration time of mitral inflow) 260 160-220 msec
HR (Heart rate) 69 60-100 bpm
E (Early ventricular filling velocity) 64.2cm/s
A (Late ventricular filling velocity) 93.8cm/s
Conclusions:
.Normal sizes of cardiac chambers.
.Preserved global contractility of left ventricle.
.LVEF by M-mode is 61.8 %.
.Grade II diastolic dysfunction.
.Mild tricuspid regurgitation.
.Trivial mitral regurgitation.
.Estimated systolic pulmonary artery pressure is 38 mmHg.
.EKG: Sinus rhythm.
Paraganglioma 173
diomyopathy due to pheochromocytoma has been tried to be explained
6,7. High catecholamine level may directly cause membrane damage and death with degeneration and necrosis of contraction band, interstitial fibrosis, myocyte infiltration, vasocon- striction and medial thickening of coronary arteries.
Long-term elevation of catecholamines may lead to cardiomyopathy, acute myocarditis, myocardial infarction, or acute hemodynamic collapse(8). Cor- relation with clinical findings, myocarditis related to paraganglioma was the most possible reason of sudden collapse in this case.
The laparoscopic adrenalectomy is the proce- dure of choice for pheochromocytoma or paragan- glioma treatment. It can be safely performed in more than 90 percent of cases. In this case, however, the patient’s family refused operation even though we had already explained the clinical importance and indication to them. All patients should accept alpha- adrenergic blockade before the procedure. Target blood pressure is less than 120/80 mmHg when sedated, and the systolic blood pressure should be greater than 90 mmHg when standing. After ade- quate blood pressure control, beta-adrenergic block- ade should then be given. In this case, we prescribed Terazosin and then Labetalol for the patient.
Although most clinicians could recognize the classic symptoms, the clinical presentation of pheo- chromocytomas or paragangliomas may mimic severe diseases. If fewer symptoms were presented,
early diagnosis may be more difficult. Patients with unrecognized pheochromocytomas or paraganglio- mas presenting with critical illness such as sepsis, shock, or acute myocardial infarction may have poor prognosis. For clinicians, pheochromocytoma or paraganglioma should always be kept in mind even though initial manifestations are not suggestive for the diagnosis.
References
1. Melmed S, Polonsky KS, Larsen R and Kronenberg HM.
Endorcrine Hypertension. In: William F. Young Jr, eds.
Williams Textbook of Endocrinology, 13th Edition. Canada:
Elsevier 2016; 556-73.
2. Bravo EL. Pheochromocytoma: new concepts and future trends. Kidney Int 1991; 40: 544-56
3. Amar L, Servais A, Gimenez-Roqueplo AP, et al. Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paragan- glioma. J Clin Endocrinol Metab 2005; 90: 2110-6.
4. Agarwal G, Sadacharan D, Kapoor A, et al. Cardiovascular dysfunction and catecholamine cardiomyopathy in pheochro- mocytoma patients and their reversal following surgical cure:
Results of a prospective case-control study. Surgery 2011;
150: 1202-11.
5. Wu XM, Chen JJ, Wu CK, et al. Pheochromocytoma Pre- senting as Acute Myocarditis with Cardiogenic Shock in Two CasesInter. Intern Med 2008; 47: 2151-55.
6. Kounatiadis P, Kolettas V, Megarisiotou A, et al. Cardiomy- opathy due to pheochromocytoma. Herz 2015; 40: 139-43.
7. Cho SK, Kim KH, Cho JY, et al. Pheochromocytoma as a Rare Hidden Cause of Inverted Stress Cardiomyopathy. J Cardiovasc Ultrasound 2014; 22: 80-3.
8. Fent GJ, Kamaruddin H, Garg P, et al. Hypertensive Emer- gency and Type 2 Myocardial Infarction Resulting From Pheochromocytoma and Concurrent Capnocytophaga Cani- morsus Infection. Open Cardiovasc Med J 2014; 8: 43-7.