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Table of Contents
Year : 2013  |  Volume : 17  |  Issue : 7  |  Page : 249-251

Functioning adrenal myelolipoma: A rare cause of hypertension

Department of Endocrinology, Osmania Medical College, Osmania General Hospital, Afzalgunj Hyderabad, Andhra Pradesh, India

Date of Web Publication11-Oct-2013

Correspondence Address:
Jayanthy Ramesh
Professor and Head,Department of Endocrinology, Osmania Medical College,Osmania General Hospital, Afzalgunj - 500 028, Hyderabad, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2230-8210.119588

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Co-occurrence of adrenal incidentaloma with hypertension calls for evaluation of endocrine causes including pheochromocytoma, Cushing's disease, and primary aldosteronism. We are reporting 40-years-old man who presented with hypertension and adrenal mass. He had elevated metanephrines, histology of resected adrenal mass revealed adrenal myelolipoma, and immuno-histochemistry was positive for chromogranin A. Both his blood pressure and urinary metanephrines returned to normal after surgery. The association of hypertension and adrenal myelolipoma may not be entirely coincidental, as it may be associated with secreting catecholamine. Literature on such an uncommon association is reviewed briefly as well.

Keywords: Adrenal myelolipoma, chromogranin A, hypertension, metanephrines, pheochromocytoma

How to cite this article:
Jakka N, Venkateshwarlu J, Satyavani N, Neelaveni K, Ramesh J. Functioning adrenal myelolipoma: A rare cause of hypertension. Indian J Endocr Metab 2013;17, Suppl S1:249-51

How to cite this URL:
Jakka N, Venkateshwarlu J, Satyavani N, Neelaveni K, Ramesh J. Functioning adrenal myelolipoma: A rare cause of hypertension. Indian J Endocr Metab [serial online] 2013 [cited 2021 Jun 24];17, Suppl S1:249-51. Available from: https://www.ijem.in/text.asp?2013/17/7/249/119588

   Introduction Top

Gierke, in 1905, described a lesion of adrenal gland containing fat and myeloid elements. [1] Myelolipomas are uncommon, small, asymptomatic benign lesions of adrenal cortex. [2] Incidence at autopsy has been reported from 0.08 to 0.4%. [3] Most of the lesions are discovered incidentally and are non-functional.

   Case Report Top

A 40-year-old man referred to department of endocrinology with adrenal mass and hypertension. He was diagnosed with hypertension 3 years back, initial BP was 180/110 mm Hg, was started on anti-hypertensive treatment. On examination, there were no neurocutaneous markers or marfanoid habitus, no features of Cushing's syndrome, 24-hour urine metanephrines level was 3000 micrograms/day (normal < 900 micrograms/day, the test was done after stopping all interfering drugs). Ultrasonography revealed 9.8 × 8.5 cms well-defined predominantly hyperechoic lesion, faint hypoechogenicity originating from right suprarenal region abutting the upper pole of right kidney and lower surface of right lobe of liver suggestive of right adrenal mass. CECT of abdomen showed 9.8 × 8.5 cm well-defined, well-circumscribed heterogenous hypoattenuated mass lesion noted in right suprarenal region and minimal enhancement on contrast with −80 to −100 HF units of attenuation suggestive of myelolipoma of right adrenal gland [Figure 1]. Baseline hematological and biochemical investigations and urinalysis were normal. Low-dose dexamethasone suppression test was normal.
Figure 1: Abdominal contrasted computerized tomography showing well‑defined non‑homogeneous mass of right adrenal origin

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In view of hypertension, adrenal mass, and elevated 24-hour urine metanephrines (>3 times), possibility of pheochromocytoma was considered. Hypertension was brought under control. Patient underwent surgery, and well encapsulated right adrenal tumor (weight: 500 gm) was excised [Figure 2]. Biopsy of specimen was suggestive of adrenal myelolipoma [Figure 3]. Immuno-histochemistry of specimen revealed positive for chromogranin A, suggestive of catecholamine-secreting granules in the tissue [Figure 4].
Figure 2: Gross specimen of removed mass

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Figure 3: H and E staining revealing features of myelolipoma with mature fat cells, suspended with plenty of normal hematopoitic marrow elements with congested blood vessels. (Original magnification, 100)

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Figure 4: Immuno‑histochemistry of specimen revealed positive for chromogranin A

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The patient had remission in hypertension. Metanephrine levels became normal after surgery. The patient is normal at the 3-month follow-up.

   Discussion Top

Adrenal myelolipomas are uncommon benign tumors of adrenals, composed of adipose and hematopoietic tissue in varying proportions, a result of metaplasia of reticuloendothelial cells. These occur most commonly in fifth to seventh decade with an equal sex distribution. [4] Most of adrenomyelolipomas are asymptomatic. As these tumors are usually more than 5 cms in diameter, they can be easily detected on ultrasound. CT scan is the most sensitive test for diagnosing myelolipoma. [5] The lesion is typically seen as a well-encapsulated heterogeneous supra-renal mass of low density with negative attenuation values, interspersed by dense myeloid tissue and with or without specks of calcification. [6] The diagnosis of adrenal myelolipoma in our case was suggested by the established CT scan criteria [Figure 1] and the typical histopathological features [Figure 3]. Functionality of the adrenal mass in this patient was suggested by the presence of hypertension and elevated metanephrines.Catecholamine secreting adrenal myelolipoma was confirmed by the absence of any evidence of pheochromocytoma on HPE and documentation of positive staining for chromogranin A on IHC [Figure 4]. Brogna et al., [7] reported a giant cortisol secreting adrenal myelolipoma,but our patient was clinically and biochemically eucortisolic. To the best of our knowledge, only two case reports are available on catecholamine-secreting adrenal myelolipoma in the world literature. Tamidari et al. have reported a case of a large, right-sided catecholamine, secreting adrenal myelolipoma with increased 24 hours urinary metanephrines. [8] Udupa et al. have reported a large adrenal myelolipoma with increased 24 hours urinary Vanillylmandelic Acid (VMA) levels. [9] All these patients became normotensive and biochemical abnormalities normalized following surgery similar to the patient in our case. The association of adrenal myelolipoma and hypertension may not be entirely coincidental, as it may be associated with catecholamine secretion, as seen in our case. Proper identification of underlying cause and appropriate treatment leads to resolution of hypertension.

   References Top

1.Gierke E. Ueber Knochenmarksgewebe in der Nebenniere Beitr. Pathol Anat 1905;37:311-25.   Back to cited text no. 1
2.Amendolara M, Barbarino C, Bucca D, Guarnieri F, Novello GB, Romano FM, et al. Giant and bilateral adrenal myelolipoma. G Chir 2008;29:85-8.  Back to cited text no. 2
3.Lam KY. Lipomatous tumors of the adrenal gland. J Urol Pathol 1995;3:95.  Back to cited text no. 3
4.Doddi S, Singhal T, Leake T, Sinha P. Management of an incidentally found large adrenal myelolipoma: A case report. Cases J 2009;2:8414.  Back to cited text no. 4
5.Akamatsu H, Koseki M, Nakaba H, Sunada S. Giant adrenal myelolipoma; Report of a case. Surg Today 2004;34:283-5.  Back to cited text no. 5
6.Haque F, Harish S, Ahmad I, Qamar A, Pandey H. Adrenal myelolipoma: A case report. Indian J Radiol Imaging 2004;14:301-2.  Back to cited text no. 6
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7.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. 7
8.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. 8
[PUBMED]  Medknow Journal  
9.Udupa S, Usha M, Visweswara RN, Desai MG. Left-sided giant adrenal myelolipoma secreting catecholamine. Indian J Pathol Microbiol 2012;55:389-91.  Back to cited text no. 9
[PUBMED]  Medknow Journal  


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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