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  Table of Contents    
CASE REPORT  
Year : 2012  |  Volume : 55  |  Issue : 3  |  Page : 389-391
Left-sided giant adrenal myelolipoma secreting catecholamine


1 Department of Pathology, MSU-GEF International Medical School, MSRIT Post, Bangalore, Karnataka, India
2 Consulting Urologist, P.D. Hinduja Sindhi Hospital, and Department of Urology, Dr. B.R. Ambedkar Medical College, Bangalore, Karnataka, India

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Date of Web Publication29-Sep-2012
 

   Abstract 

Adrenal myelolipoma (AML) is a rare benign tumor composed of mature adipose and hematopoietic tissue. Most of these patients are asymptomatic and the tumors are non-secreting. We present a case with a large functional adrenal myelolipoma, wherein the patient was hypertensive and biochemistry revealed increase in 24 hours urinary Vanillylmandelic Acid (VMA), a metabolite of catecholamine. The mass was removed surgically and diagnosed as adrenal myelolipoma on histopathological examination. Both his blood pressure and urinary VMA returned to normal following surgery, which suggested that the mass was functioning and was secreting catecholamine. To the best of our knowledge, a catecholamine secreting adrenal myelolipoma has been reported in the literature only once previously. The association of hypertension and adrenal myelolipoma may not be entirely coincidental, as it may be associated with secreting catecholamine, as seen in our case. We also review the literature on functioning adrenal myelolipoma.

Keywords: Adrenal, catecholamine, myelolipoma, vanillylmandelic acid

How to cite this article:
Udupa S, Usha M, Visweswara R N, Desai M G. Left-sided giant adrenal myelolipoma secreting catecholamine. Indian J Pathol Microbiol 2012;55:389-91

How to cite this URL:
Udupa S, Usha M, Visweswara R N, Desai M G. Left-sided giant adrenal myelolipoma secreting catecholamine. Indian J Pathol Microbiol [serial online] 2012 [cited 2019 Jun 20];55:389-91. Available from: http://www.ijpmonline.org/text.asp?2012/55/3/389/101755



   Introduction Top


Adrenal myelolipoma (AML) is an uncommon benign tumor, composed of mature adipose tissue and hematopoietic elements that resemble bone marrow. AML constitutes about 9% of all incidentally discovered adrenal masses. [1] The tumor was first described by Gierke in 1905, and was given the name myelolipoma by Oberling, in 1929. Very few cases of giant adrenal myelolipoma have been described in literature. [2],[3],[4]

We describe a rare case of a left-sided giant adrenal myelolipoma, with increased 24 hours urinary Vanillylmandelic acid (VMA), which is one of the metabolites of the catecholamine metabolism. Our case is the second that we know of, with an adrenal myelolipoma presenting clinically with hypertension, associated with increased catecholamine, and clinically resembling a pheochromocytoma. [5]


   Case Report Top


A 58-year-old man came with history of pain in the left hypochondrium and lumbar region. The patient was a known case of diabetes mellitus and sustained hypertension, on medication.

On examination, he was found to be a hypertensive (blood pressure: 170 / 110 mmHg) and the body mass index (BMI) of the patient was 21.1 kg / m 2 .

Routine hematological parameters like hemoglobin, complete blood count, and peripheral smear were normal. The biochemical parameters like urea, creatinine, blood urea nitrogen, routine urine examination, and culture was normal. The random blood sugar level was 235 mg / dl. Ultrasonography showed a large hyperechoic lesion in the left suprarenal region measuring 18.4 × 10.1 cm. In view of the presence of a large suprarenal mass, pheochromocytoma was suspected clinically, and 24 hours urinary VMA was measured, which was markedly increased showing values to be 80.89 mg / day, (normal 1.6 - 4.2 mg / day), measured by the high performance liquid chromatography (HPLC) method. A computed tomography (CT) scan showed a large left suprarenal solid mass showing inter-mixed areas of fat and mildly enhancing soft tissue components, suggestive of adrenal myelolipoma, and the adrenal gland on the right side was normal. There was no family history of adrenal gland disease in any of the family members of the patient. Left adrenalectomy with left Double J stenting and nephropexy was performed. The patient had an uneventful recovery and became normotensive (blood pressure 130 / 80 mmHg) and the 24 hours urinary VMA returned to normal (4.13 mg / day) following surgery. He remains normotensive, without treatment, in the follow-up of three months after surgery.

Pathology

Gross: An oval-to-round well-encapsulated soft tissue mass measured 18.5 × 10.5 × 9 cm and weighed 2.5 kg. The cut section showed a yellowish appearance with focal gray white and hemorrhagic areas [Figure 1].
Figure 1: Gross appearance of the left suprarenal mass. (a) Shows a large capsulated mass measuring 18.5 × 10.5 × 9 cm and (b) demonstrates the cut section of the mass, showing yellowish adipose tissue and hemorrhagic areas

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Microscopy revealed adrenal tissue at the periphery, under which was mature adipose tissue with interspersed hematopoietic tissue, resembling bone marrow, consisting of erythroid, lymphoid, granulocytic, and megakaryocytic elements [Figure 2]. There was no evidence of pheochromocytoma or adrenal medullary hyperplasia. On the basis of gross and microscopic examination, a final diagnosis of adrenal myelolipoma, with no evidence of pheochromocytoma or adrenal medullary hyperplasia was made.
Figure 2: Photomicrograph of the adrenal myelolipoma. (a) Shows a capsulated tumor showing adipose tissue, hematopoitic elements, and a strip of adrenal tissue (Hematoxylin and eosin, ×40) and (b) shows a megakayocyte in the hematopoitic tissue (Hematoxylin and eosin, ×400)

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   Discussion Top


Adrenal myelolipoma is a tumor, which is commonly found between the fifth and seventh decades of life. [6] Most of the adrenal myelolipomas arise from the right adrenal gland and measure less than 4 cm in diameter. [7] The largest reported giant adrenal myelolipoma measured 31 × 24.5 × 11.5 cm and weighed 6 kg. [6]

Adrenal myelolipomas are generally non-secreting with normal values of VMA, cortisol, adrenaline, and noradrenaline. Endocrine dysfunction like hyperparathyroidism and Cushings disease have rarely been reported with AML, while medical conditions like obesity, diabetes mellitus, and hypertension are more commonly associated with AML. The association of hypertension is reportedly either coincidental or due to mechanical compression of the renal vasculature by the tumor. [8],[9]

There are few case reports in the literature of giant AML associated with secretion of hormones. Brogna et al.[10] have reported a case of a giant secreting adrenal myelolipoma measuring 25 × 20 × 20 cm, weighing 4.4 kg, associated with hypertension and increased level of cortisol. Tamidari et al.[5] have reported a case of a large, right-sided catecholamine, secreting adrenal myelolipoma measuring 13 × 11 cm, in which the patient was hypertensive with increased 24 hours urinary metanephrine, a metabolite of catecholamine. The patient became normotensive and urinary metanephrine returned to normal following surgery, similar to the patient in our case.

The case we have described here is that of a large functioning AML, which elaborates VMA, as evidenced by increased VMA levels and hypertension. Following resection of the tumor, the patient became normotensive and the VMA decreased to normal, clearly demonstrating the cause and effect relationship, and it suggests that the mass was functioning.

One interesting observation which can be made from above cases is functioning adrenal myelolipoma is usually associated with large size of tumor but we need more evidences to establish the relationship.

Another rare feature present in our case is the left-sided position of the AML, because AMLs have been reported to be commonly located on right side.


   Conclusion Top


The association of adrenal myelolipoma and hypertension may not be entirely coincidental as it may be associated with the secretion of catecholamine.


   Acknowledgement Top


A special thanks to Medical Director of P. D. Hinduja Sindhi Hospital & Dr. Nagaraju. A. R (Pathologist) for their kind support & encouragement.

 
   References Top

1.Boland GW, Blake MA, Hahn PF, Mayo-Smith WW. Incidental adrenal lesions: Principles, techniques and algorithms for imaging characterization. Radiology 2008;249:756-75.  Back to cited text no. 1
[PUBMED]    
2.Wilhelmus JL, Schrodt GR, Alberhasky MT, Alcorn MO. Giant adrenal myelolipoma: Case report and review of literature. Arch Pathol Lab Med 1981;105:532-5.  Back to cited text no. 2
[PUBMED]    
3.Boudreaux D, Waisman J, Skinner DG, Low R. Giant adrenal myelolipoma and testicular interstitial cell tumor in a man with congenital 21-hydroxylase deficiency. Am J Surg Pathol 1979;3:109-23.  Back to cited text no. 3
[PUBMED]    
4.O'Daniel-Pierce ME, Weeks JA, McGrath PC. Giant adrenal myelolipoma. South Med J 1996;89:1116-8.  Back to cited text no. 4
[PUBMED]    
5.Tamidari H, Mishra AK, Gupta S, Agarwal A. Catecholamine secreting adrenal myelolipoma. Indian J Med Sci 2006;60:331-3.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
6.Mukherjee S, Pericleous S, Hutchins RR, Freedman PS. Asymptomatic giant adrenal myelolipoma. Urol J 2010;7:66-8.  Back to cited text no. 6
[PUBMED]    
7.Daneshmand S, Quek ML. Adrenal myelolipoma: Diagnosis and management. Urol J 2006;3:71-4.  Back to cited text no. 7
[PUBMED]    
8.Han M, Burnett AL, Fishman EK, Marshall FF. The natural history and treatment of adrenal myelolipoma. J Urol 1997;157:1213-6.  Back to cited text no. 8
[PUBMED]    
9.Meaglia JP, Schmidt JD. Natural history of an adrenal myelolipoma. J Urol 1992;147:1089-90.  Back to cited text no. 9
[PUBMED]    
10.Brogna A, Scalisi G, Ferrara R, Bucceri AM. Giant secreting adrenal myelolipoma in a man: A case report. J Med Case Rep 2011;5:298.  Back to cited text no. 10
[PUBMED]    

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Correspondence Address:
Sujatha Udupa
Deparment of Pathology, International Medical School, M.S. Ramaiah Campus, New BEL Road, MSRIT Post, Bangalore- 560054
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.101755

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    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
   Acknowledgement
    References
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