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ABDOMINAL IMAGING Table of Contents   
Year : 2002  |  Volume : 12  |  Issue : 3  |  Page : 355-356
Multicentric pheochromocytoma involving the adrenal gland and thorax


Mandakini Imaging Centre, 90 Feet Road, Ghatkopar (E), Mumbai-400077, India

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Keywords: Pheochromocytoma, Adrenal gland, MIBG Scintigraphy

How to cite this article:
Varma M S, Mashankar A S, Parihar A L, Salkade P R, Dialani V M. Multicentric pheochromocytoma involving the adrenal gland and thorax. Indian J Radiol Imaging 2002;12:355-6

How to cite this URL:
Varma M S, Mashankar A S, Parihar A L, Salkade P R, Dialani V M. Multicentric pheochromocytoma involving the adrenal gland and thorax. Indian J Radiol Imaging [serial online] 2002 [cited 2019 Nov 18];12:355-6. Available from: http://www.ijri.org/text.asp?2002/12/3/355/28481
Pheochromocytoma is a rare neoplasm of the adrenal gland. It is thought to be the cause in approximately 0.1 to 0.5% of patients with newly diagnosed hypertension [1]. This tumor occurs with equal frequency in men and women. It appears to be more common in the third and fourth decades of life [1]. It is rare in children and when it does occur in children, it tends to be with a high familial incidence [1].

We are presenting a case of multicentric pheochromocytoma in a 13 year old adolescent male. There is no case report so far, to our knowledge, describing a multicentric pheochromocytoma involving the adrenal gland and the thorax.


   Case Report Top


A 13 year old adolescent male patient presented with labile hypertension, left sided chest pain and breathlessness. Electrocardiogram showed short PR interval with signs of left ventricular hypertrophy. The plasma renin levels were normal. The urinary vanillylmandelic acid (VMA) levels were raised to 29.5 mg/24 hours (normal - upto 8-mg/24 hour). A routine chest radiograph was obtained which showed a right paravertebral mass. Subsequently contrast enhanced helical CT scan of the abdomen and thorax was performed. The scan showed oval, hypodense minimally enhancing mass lesions in both the adrenal glands, larger on the right side [Figure - 1]a. Another discrete, well defined, intra-thoracic, extra-parenchymal mass lesion in the right paravertebral region was also observed [Figure - 1]b. Metaiodobenzylguanidine (MIBG) scintigraphy was performed later on which was reported as MIBG concentrating neoplasms in the right adrenal and right thoracic regions. Familial and other syndromes like MEN, NE were excluded.

Later on the patient underwent surgery with removal of the nodular lesions in both the adrenal glands and resection of the right paravertebral lesion. Post operatively the patient became normotensive and was asymptomatic Histopathology report showed pheochromocytoma characterized by nests and balls of medium sized cells supported by vascular fibrous network in the materials resected from the right adrenal gland and the right paravertebral region. The cells showed granular acidophilic cytoplasm and pleomorphic nuclei. The material from the left adrenal gland showed hyperplasia of the adrenal medulla. No malignant changes were noted.


   Discussion Top


Pheochromocytoma is a catecholamine-secreting tumor that arises from the neuroectodermal tissue. Most pheochromocytomas are unilateral and arise in the adrenal medulla. Some of these catecholamine secreting tumors are extra-adrenal and are referred to as paragangliomas [2]. Although paragangliomas arise anywhere along the sympathetic chain from the pubis to the neck, they are usually located in the abdomen [2].

The incidence of pheochromocytoma in the general population is approximately 0.001-0.01% [3]. Approximately 10% are bilateral, 10% are extra-adrenal, 10% are familial, 10% are multicentric, 10% are seen in children and 10% are malignant [4],[5]. Hence pheochromocytoma is called a "Ten percent tumor".

Adult pheochromocytoma is more frequently located on the right side [6]. In children the right to left predominance is 2:1 [7].

Of the 10% extra-adrenal pheochromocytomas only 1-2% are intra-thoracic and are frequently reported in the posterior mediastinum [3]. Of these, intra-pericardial pheochromocytomas are extremely rare [3].

Pheochromocytoma being a catecholamine-secreting tumor produces excess epinephrine and nor-epinephrine responsible for the signs and symptoms of the patient, which include episodic hypertension, headache, anxiety, palpitation and tachycardia [2]. At times the symptoms are atypical and may include a wide range of symptoms that cover the spectrum of labile hypertension to myocardial infarction and cerebral haemorrhage [1]. Even a simple per abdominal examination itself can cause hypertensive crisis. The same risk prevails in the handling of the tumor during surgery. In view of these problems, it becomes all the more necessary to be able to image these patients comprehensively with noninvasive techniques to map all the lesions in a patient including the rare extra-adrenal tumors before surgical excision of the lesions is planned. In majority of cases, symptomatic relief can be obtained if the tumor is excised [2].

The diagnosis of pheochromocytoma is usually made on the basis of clinical presentation and elevated catecholamine levels in serum or urine (metanephrine or VMA) [2]. Imaging is used primarily for localizing tumors prior to surgery [2].

CT and MRI are useful for pheochromocytoma located within the adrenals. MRI may be helpful in localizing extra-adrenal tumours due to its multi-planar capability. These images provide anatomical orientation and high quality images of adrenal structure [3]. However, they are limited by their inability to screen the entire body and fail to provide insight into the functional characterization of tumors. Hence, scintigraphy plays a vital role in identifying multicentric lesions with the capacity to screen a large portion of the body [8].

MIBG scintigraphy is the most widely used modality for localization of pheochromocytomas. However, about 10% of pheochromocytomas fail to be detected by this modality, as these tumors do not accumulate MIBG [8]. In this situation positron emission tomography (PET) performed after administration of 2-[fluorine - 18] - fluoro-2-deoxy-D-glucose (FDG) has been proved useful [8],[9]

 
   References Top

1.Isaac R. Francis, Melvyn Korobkin. Pheochromocytoma, RCNA 1996; 34:1101-1112  Back to cited text no. 1    
2.Thorsten L. Krebs, Brent J. Wagner, MR imaging of the adrenal gland: Radiologic - pathologic correlation. Radiographics 1998; 18: 1425-1440  Back to cited text no. 2    
3.Brian H. Hamilton, Isaac R. Francis, Barry H.   Back to cited text no. 3    
4.Gross, et al. Intrapericardial paragangliomas (pheochromocytomas): Imaging features. AJR 1997; 168:109-113  Back to cited text no. 4    
5.David Sutton. The adrenal glands. In: David Sutton, ed. Textbook of radiology and medical imaging vol 1, 4th edition. Churchill Livingstone, 1987:1033-1056  Back to cited text no. 5    
6.Akira Kawashima, Carl M. Sandler, Elliot K Fishman, et al. Spectrum of CT findings in nonmalignant disease of the adrenal gland. Radiographics 1998; 18:393-412  Back to cited text no. 6    
7.Thomas JL, Bernardino ME, Samaan VA, Hickey RC:CT of Pheochromocytoma. AJR 1980; 135:477  Back to cited text no. 7    
8.Radin D, Ralls P, Boswell W, et al. Pheochromocytoma detection by unenhanced CT. AJR 1986; 146: 741-744  Back to cited text no. 8    
9.Barry L. Shulkin, Robert A. Koeppe, Isaac R. Francis, et al. Pheochromocytomas that do not accumulate metaiodobenzylguanidine: Localization with PET and administration of FDG. Radiology 1993; 186:711-715  Back to cited text no. 9    

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Correspondence Address:
M S Varma
Mandakini Imaging Centre, 90 Feet Road, Ghatkopar (E), Mumbai-400077
India
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Source of Support: None, Conflict of Interest: None


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