|Year : 2014 | Volume
| Issue : 3 | Page : 127-130
A rare case of extra - Adrenal pheochromocytoma with coexisting thyrotoxicosis
Lingudu Brahmanandam, Bongi Vivekanand, Ayyagari Mythili, Kandregula Subrahmanyam
Department of Endocrinology, Andhra Medical College, King George Hospital, Visakhapatnam, Andhra Pradesh, India
|Date of Web Publication||13-Aug-2014|
Senior Resident, Department of Endocrinology, Andhra Medical College, King George Hospital, Visakhapatnam, Andhrapradesh - 530 002
Source of Support: None, Conflict of Interest: None
We report a rare case of extra-adrenal pheochromocytoma with coexisting thyrotoxicosis. A 52 year old female presented with hypertension, diabetes, hyperadrenergic symptoms, paroxysmal spells and computed tomography abdomen revealing a left extra adrenal mass. Evaluation showed normal 24 hour urinary epinephrine and metanephrines with an elevation of urinary norepinephrine. Thyroid function tests revealed thyrotoxicosis. The diagnosis of pheochromocytoma is delayed and the case illustrates the similarity of both the disorders. Despite existence of two similar disorders the patient recovered with treatment of both pheochromocytoma and thyrotoxicosis.
Keywords: Catecholamines, pheochromocytoma, thyrotoxicosis
|How to cite this article:|
Brahmanandam L, Vivekanand B, Mythili A, Subrahmanyam K. A rare case of extra - Adrenal pheochromocytoma with coexisting thyrotoxicosis. Thyroid Res Pract 2014;11:127-30
|How to cite this URL:|
Brahmanandam L, Vivekanand B, Mythili A, Subrahmanyam K. A rare case of extra - Adrenal pheochromocytoma with coexisting thyrotoxicosis. Thyroid Res Pract [serial online] 2014 [cited 2019 Aug 19];11:127-30. Available from: http://www.thetrp.net/text.asp?2014/11/3/127/138562
| Introduction|| |
Pheochromocytoma is a rare disorder, difficult to diagnose and only few cases are recognized during life. The hyperadrenergic features of the disorder mimic those of thyrotoxicosis. The coexistence of thyrotoxicosis and Pheochromocytoma has been reported in earlier studies. We report a rare case of extra-adrenal pheochromocytoma with associated thyrotoxicosis.
| Case History|| |
A 52 year old post-menopausal woman hailing from tribal region of Visakhapatnam presented with headache, palpitations since 1 year. There is history of excessive sweating for the last 5 years, recurrent abdominal pain and chest pain since 2 years. On questioning, there is history of heat intolerance, weight loss, dizziness, tremor, proximal muscle weakness for the last one year. She is a known hypertensive for the last 5 years and is on Atenolol 50 mg once daily, to which she is noncompliant. She is diagnosed to have Diabetes Mellitus (D M) 1 year back and takes premixed insulin 25 units and 15 units before breakfast and before dinner respectively. There is no history suggestive of microvascular or macrovascular complications.
Her history revealed recurrent attacks of paroxysmal spells. She had three episodes which are as follows: The first episode was 7 years back when she had vomiting with severe abdominal pain. The second episode was 2 months back and she presented with palpitations and dizziness. The third episode was 1 month back and she had vomiting, severe abdominal pain and altered sensorium. During the third episode she was admitted in medical unit for abdominal pain and an ultrasound abdomen done showed a mass at paraaortic region. A contrast-enhanced computed tomography (CECT) abdomen confirmed the mass at left renal hilum between renal vein and renal artery suggestive of extra-adrenal pheochromocytoma [Figure 1]. The patient was referred to department of endocrinology.
Her family history is negative for DM, HTN, thyroid disorders and pheochromocytoma.She is married and has three children. There is history of two intrauterine deaths during her reproductive period.
On general examination, she has acanthosis, skin tags, warm, moist skin and grade 1 diffuse soft goiter. Pulse Rate is 120/min, regular, high volume, blood pressure (BP) 150/90 mm Hg in supine position and 120/90 in standing position with a postural fall of 30 mm Hg of systolic BP suggestive of orthostatic hypotension. Cardiovascular examination was normal except for tachycardia. Central nervous examination revealed fine tremors. Abdomen was soft with no organomegaly.
Her clinical diagnosis is Hypertension, DM and diffuse soft goiter with hypermetabolic symptoms and left extra-adrenal mass and paroxysms. patient is investigated. [Table 1] shows the biochemical and hormonal investigations.
Ultrasound abdomen showed bulky kidneys with hypoechoic echopattern suggestive of pyelonephritis changes. Right kidney measured 12.3 × 5.3 cm and Left kidney 11.4 × 5.9 cm. There is a round hypoechoic lesion measuring 51 × 48 mm with few cystic areas at paraaortic region. The CECT abdomen [Figure 1] confirmed the presence of left extra-adrenal pheochromocytoma. Both adrenal glands are normal. Electrocardiogram, two dimensional echocardiography and CXR PA view were normal.
|Figure 1: Contrast enhanced computed tomography (CECT) abdomen showing well-circumscribed, intense heterogeneous, peripherally enhancing soft tissue mass lesion noted in left hilar region between renal vein anteriorly and renal artery posteriorly. Lesion has cystic, non enhancing necrotic areas within. Lesion appear to be separate from the left kidney, left adrenal gland and tail of pancreas. Size of the lesion measuring about 5.2 × 4.2 × 4.4 cm. No evidence of calcifications. Both adrenal glands are normal|
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Patient was started on Prazosin 2.5 mg per day, gradually increased to 7.5 mg twice daily. Metoprolol was added 2 weeks before surgery with 6.25 mg once daily and increased upto 12.5 mg once daily. Premixed insulin was given in a dose of 25 U before breakfast and 8 U before dinner. carbimazole was started with 20 mg twice daily. Intravenous (IV) fluids (normal saline) were given for volume replacement. Patient had three episodes of paroxysmal spells during hospital stay. Each episode lasted for three to four hours and was characterized by sudden onset of cough, dyspnea, severe sweating, vomiting, headache, palpitations, abdominal pain and backache. On examination, there were bilateral wheeze, bilateral crepitations, retractions, tachypnea, tachycardia, Pulse Rate: 140/min and blood pressure: 230/110 mm Hg. After cessation of spell blood pressure: 90/60 mm Hg. IV fluids and symptomatic treatment were given for spells.
The patient was planned for surgical management after achieving euthyroid state and preoperative management of pheochromocytoma. Exploratory laparotomy and excision of tumor (5 × 5 cm) was done. Postoperative period was uneventful and was managed with IV fluids, prazosin, metoprolol, carbimazole and insulin. Histopathology report [Figure 2] confirmed features consistent with that of pheochromocytoma. Follow up of the patient 1 month after surgery revealed euthyroid state and continued on carbimazole 20 mg once daily. She is euglycemic with six units basal insulin. Hypertension is under control with metoprolol 50 mg daily and she has no further paroxysmal spells.
|Figure 2: HPE shows uniform polygonal cells arranged as well-defined nests (zell ballen pattern) separated by thin fibrovascular septae. Individual cells show basophilic granular cytoplasm with round to oval nuclei. At places tumor cells show clear cytoplasm. The surrounding fibrofatty tissue shows congested vascular space|
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| Discussion|| |
The association between pheochromocytoma and thyrotoxicosis is rare, with only few case reports in literature. Snow and Burton  reported a case of associated thyrotoxicosis and pheochromocytoma in the year 1976, where pheochromocytoma was diagnosed only postmortem. Braverman and Sullivan, Dimova and Kiscleva , presented cases with pheochromocytoma and accompanying diffuse toxic goiter. Becker, Rosen and Engel man  have shown that 5 out of 36 cases with pheochromocytoma presented with thyroid disease other than medulllary carcinoma. In India, the association between severe thyrotoxicosis and extra adrenal pheochromocytoma has been reported by Bashir Ahmad Laway, Syed-ur Rehman et al.  from Kashmir. Though the coexistence of both the disorders is described, the exact relation between the two disorders is not known. Thyroid hormones produce the necessary intracellular environment without which the steady state and actions of catecholamines would be vitiated. In Thyrotoxicosis, the increased concentration of thyroid hormones results in lowering of the threshold for catecholamine action. Similarly, increased concentrations of catecholamines may aggravate/relapse thyrotoxicosis.
The clinical course of the present case was episodic spells, hyperadrenergic symptoms and signs mimicking both the disorders. Hypertension was sustained and associated with postural fall. The presence of thyrotoxicosis did not aggravate the clinical picture of pheochromocytoma in the present case. Spells were associated with severe hypertension, bronchospasm, sweating, headache, pallor and abdominal pain. Whether spells were more common/aggravated by coexisting thyrotoxicosis in this case could not be concluded as her BP was inadequately treated prior to hospitalization.
Biochemically, the diagnosis of pheochromocytoma in this patient was difficult, as evidenced by normal 24 hour urinary metanephrines and urinary epinephrine. The only biochemical abnormality was the elevation of urinary norepinephrine. Urinary normetanephrine estimation could not be done due to nonavailability. The presence of untreated thyrotoxicosis in the present case could have contributed to these biochemical findings by accelerating the metabolism of catecholamines. However, animal studies in thyroxine treated and euthyroid rats for altered catecholamine destruction did not show any difference in thyroxine treated rats as studied by Svedmyr, N.  Similar studies carried out into the possibility of increased production and release of catecholamines in the hyperthyroid state have generally been negative as given by Harrison, Siegel, Wilson and Weber.  It is thus clear that the urinary catecholamine excretion products are normal in hyperthyroidism. Therefore, it appears that thyroid hormones potentiating certain effects of catecholamines must be due to changes in the receptor cell, rather than to reduced uptake or breakdown of catecholamines locally as given by Victor parsons and Ian Ramsay. 
In patients with pheochromocytoma, the thyroid function tests are unaltered and the elevation of catecholamines do not have a significant direct effect on thyroid function. Goiter is present in this case and thyroid swelling occurs in 6% of patients with pheochromocytoma and can be infused experimentally by infusion of noradrenaline as studied by Herman and Momex.  There have been several case reports describing the association of Graves' disease and pheochromocytoma which suggest that increased catecholamines in pheochromocytoma act as an exacerbating factor for triggering autoimmunity in Graves' disease. Paschke et al.  reported relapse of Graves' disease in patients with pheochromocytoma. The diagnosis of the present case is most probably Graves' disease in view of diffuse goiter and thyrotoxicosis. However, we could not do TSH Receptor Antibodies in the present case for confirmation.
In conclusion, this is the second case report of extra adrenal pheochromocytoma with associated thyrotoxicosis from India. The case illustrates the eventual recovery despite coexistence of two hyperadrenergic disorders and a delay in the diagnosis.
| References|| |
|1.||Snow MH, Burton P. A case of associated thyrotoxicosis and pheochromocytoma. A diagnostic problem. Postgrad Med 1976; 52:288-91. |
|2.||Braverman LE, Sullivan R M. Another polyendocrine disorder: Pheochromocytoma and diffuse toxic goiter. Johns Hopkins Med J 1969;125:331-33. |
|3.||Dimova MN, Kiscleva TP. Case of combined diffuse toxic goiter and Pheochromocytoma. Probl Endokrinol Mosk 1987;33:42-43. |
|4.||Becker CE, Rosen SW, Engel man K. Pheochromocytoma and hyporesponsiveness to thyrotropin in a 46XY male with features of the Turner phenotype. Ann Intern Med 1969; 70:325-328. |
|5.||Bashir Ahmad Laway, Syed-ur Rehman, Mohd. Ashraf Ganie, Khursheed Alam Wani, Rauf A. Wani, Ajaz Hakeem, et al. Severe thyrotoxicosis in a patient with extra-adrenal pheochromocytoma: two disorders with a similar clinical presentation. Endocrinologist 2010;20:55-7. |
|6.||Svedmyr, N. Studies on the relationships between some meatabolic effects of thyroid hormones and catecholamines in animals and man. Acta physiol.scand 1966; 68, Suppl. 274,1. |
|7.||Harrison, T.S, Siegel, J.H, Wilson, W.S. and Weber, W.J. Adrenergic reactivity in hyperthyroidism. Arch Surg 1967; 94: 396. |
|8.||Victor parsons, Ian ramsay. Thyroid and adrenal relationships. Postgrad. Med j 1968;44:377-384. |
|9.||Herman, H. and Momex, R. Human tumors secreting catecholamines. Oxford, Pergamon press 1964. |
|10.||Paschke R, Enger J, Harsch I, Usadel KH. Relapse of Graves disease following development of pheochromocytoma. Thyroid 2:203-06,1992. |
[Figure 1], [Figure 2]