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  Table of Contents    
CASE REPORT  
Year : 2022  |  Volume : 65  |  Issue : 3  |  Page : 689-691
Bilateral giant adrenal myelolipoma: A rare scenario


1 Department of Urology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
2 Department of Pathology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

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Date of Submission16-Feb-2021
Date of Acceptance16-Mar-2022
Date of Web Publication21-Jul-2022
 

   Abstract 


Adrenal myelolipoma is a benign tumor-like growth, composed of mature fat cells and bone marrow elements. We report a case of a 44-year lady who presented with a complaint of pain in the abdomen. The only positive finding was contrast-enhanced computed tomography (CECT) whole abdomen, which was suggestive of heterogeneously enhancing hypodense lesion of size 130 mm × 105 mm with few calcifications and 103 mm × 75 mm with intralesional fat attenuation in right and left adrenals. Rest laboratory parameters were normal.
Only a few cases so far have been reported for bilateral adrenal myelolipoma but what stands out in our case is its giant size and bilaterality, managed surgically without any complications.

Keywords: Adrenal, giant, mesenchymal

How to cite this article:
Gupta P, Mondal S, Datta C, Pal DK. Bilateral giant adrenal myelolipoma: A rare scenario. Indian J Pathol Microbiol 2022;65:689-91

How to cite this URL:
Gupta P, Mondal S, Datta C, Pal DK. Bilateral giant adrenal myelolipoma: A rare scenario. Indian J Pathol Microbiol [serial online] 2022 [cited 2022 Aug 15];65:689-91. Available from: https://www.ijpmonline.org/text.asp?2022/65/3/689/351595





   Introduction Top


Adrenal myelolipoma is a relatively rare, benign mesenchymal tumor with a benign biological behavior that is mainly composed of mature adipose and myeloid tissue (hematopoietic cells).[1]

First described in 1905 by Glerke, this lesion is most frequently located in the adrenal gland, more commonly right adrenal, but it may also occur as an isolated soft tissue mass in various sites, especially in the pelvic region. Extra-adrenal sites include the presacral area, stomach, lung, liver, spleen, testis, and retroperitoneum. About 12% of cases supposedly are bilateral.[2] To date, approximately 40 cases of bilateral myelolipomas are reported.[3] Both sexes are equally affected, most commonly occurring between the fifth and seventh decade. Incidence is 0.08%–0.4% based on autopsy series and accounts for 7%–15% of all adrenal incidentalomas.[4] This lesion generally exhibits benign biological activity though sometimes co-exists with primary aldosteronism, adenoma, and Cushing.


   Case Report Top


A 44-year type 2 diabetic lady presented with pain in the abdomen localized at the right flank, dull aching, and non-radiating type since 1 year, not associated with any other symptom such as fever, vomiting, or burning micturition. On clinical examination, there was no significant finding elicited. An ultrasonogram (USG) was suggestive of a bilateral 8 cm × 10 cm space-occupying lesion in the suprarenal area. Later on, contrast-enhanced computed tomography (CECT) was done which was suggestive of heterogeneously enhancing hypodense lesion size 130 mm × 105 mm with few calcifications and 103 mm × 75 mm with intralesional fat attenuation in right and left adrenal [Figure 1]. Laboratory study was done to evaluate the biological nature of the mass which was as follows: S. Potassium 4.6, S. Cortisol after ONDST: <0.1 ug/dl, S. DHEAS: 52. 6 ug/dl. Urinary metanephrines and normetanephrines were in the normal range. Bilateral open adrenalectomy was performed under general anesthesia (GA). Furthermore, histopathology was suggestive of encapsulated tumor composed of mature adipose tissue intermixed with islands of hematopoietic cells suggestive of myelolipoma [Figure 2].
Figure 1: CECT suggestive of heterogeneously enhancing hypodense lesion size 130 mm × 105 mm with few calcifications and 103 mm × 75 mm with intralesional fat attenuation in right and left adrenal

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Figure 2: Histopathology shows encapsulated tumor composed of mature adipose tissue intermixed with islands of hematopoietic cells including fair number of megakaryocytes, area of necrosis, hemorrhage, and cyst formation

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   Discussion Top


Adrenal myelolipoma is a rare, benign tumor having mature adipose tissue and bone marrow. They are generally unilateral and the size varies from 2 to 3 mm to up to 34 cm. In the literature, the largest myelolipoma (diameter 34 cm, weight 5900 g) has been described by Boudreaux et al.[5] Myelolipoma is defined as “giant” where its largest diameter is >10 cm.[6] We have reported bilateral giant adrenal myelolipoma which is a rare clinical occurrence.

The etiology of myelolipoma is still not defined clearly. The embolization of hemopoietic stem cells and ectopic myeloid hyperplasia are implicated in the etiology.[7] The association with various clinical syndromes such as Cushing's syndrome, Conn's syndrome, Addison's disease, and obesity has led some authors to think of this tumor as a part of a rare variant of multiple endocrine neoplasias.[8] Selye and Stone found that the zona reticularis of mature rats when treated with hormones like testosterone and pituitary gland extracts, was transformed into bone marrow tissue.[9] Conversely, Boudreaux et al.[5] suggested that a variety of factors like tissue necrosis, adrenocorticotropic hormone, and hypersecretion of androgens might contribute to the development of adrenal myelolipoma.

Adrenal myelolipomas, which are usually asymptomatic and non-functioning, are usually diagnosed during surgical interventions and imaging procedures performed for various other purposes. The most frequent symptoms of giant myelolipomas are non-specific and include abdominal pain, hematuria, reno-vascular hypertension, and those other symptoms which are secondary to the mechanical compression of adjacent organs.[5],[7]

Four clinicopathological patterns were described by Kenney et al.[10] that includes isolated adrenal myelolipoma without any other disorders; myelolipoma admixed with hemorrhage, which is more common in the larger lesion (diameter >10 cm); extra-adrenal myelolipomas those being similar to adrenal myelolipomas, found most frequently in the retroperitoneum; and myelolipomatous foci within other adrenal pathologic conditions, which have smaller size, low-fat content, and are densely calcified.

As we have already discussed that only 40 cases so far have been reported regarding bilateral adrenal myelolipoma but what distinguishes the rest from our case is its giant size and bilaterality, i.e., 13 and 10.3 cm with the most unique feature of calcification on the right side managed surgically without complications.

Acute hemorrhage, though rare, is described as the most significant complication commonly in giant myelolipomas, and this can be manifested with pain in the back, epigastrium, or flanks, associated with nausea, vomiting, hypotension, and anemia. Surgical resection is recommended in such cases. Transarterial embolization with polyvinyl alcohol (PVA) particles or gelatin sponge particles can also be used for successful hemostasis before planning adrenalectomy in patients with retroperitoneal hemorrhage from these adrenal myelolipomas.

Due to the lack of formal and specified guidelines regarding the management of these adrenal myelolipomas, the decision should be made on a case-to-case or as a subjective basis. If surgical removal is required because of more growth, symptoms (e.g., abdominal discomfort), or hormonal activity, laparotomy or minimally invasive surgery can be performed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Lam AK. Update on adrenal tumours in 2017 World Health Organization (WHO) of endocrine tumours. Endocr Pathol 2017;28:213-27.  Back to cited text no. 1
    
2.
Shenoy VG, Thota A, Shankar R, Desai MG. Adrenal myelolipoma: Controversies in its management. Indian J Urol 2015;31:94-101.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Lam AK. Lipomatous tumours in adrenal gland: WHO updates and clinical implications. Endocr Relat Cancer 2017;24:R65–79.  Back to cited text no. 3
    
4.
Kloss RT, Gross MD, Francis IR, Korobkin M, Shapire B. Incidentally discovered adrenal masses. Endocr Rev 1995;16:460-84.  Back to cited text no. 4
    
5.
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. 5
    
6.
Decmann A, Perge P, Toth M, Igaz P. Adrenal myelolipoma: A comprehensive review. Endocrine 2018;59:7-15.  Back to cited text no. 6
    
7.
Vierna J, Laforga JB. Giant adrenal myelolipoma. Scand J Urol Nephrol 1994;28:301–4.  Back to cited text no. 7
    
8.
Banik S, Hasleton PS, Lyon RL. An unusual variant of MEN syndrome: A case report. Histopathology 1984;8:135–44.  Back to cited text no. 8
    
9.
Selye H, Stone H. Hormonally induced transformation of adrenal into myeloid tissue Am J Pathol 1950;26:211–33.  Back to cited text no. 9
    
10.
Kenney PJ, Wwagner BJ, Rao P, Heffess CS. Myelolipoma. CT and pathologic features. Radiology 1998;208:87-95.  Back to cited text no. 10
    

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Correspondence Address:
Dilip K Pal
Department of Urology, Institute of Post Graduate Medical Education and Research, Kolkata - 700 020, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpm.ijpm_182_21

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