Table of Contents    
Case Report
 
Adrenal Myelolipoma: A case report
Sara Zaheri1, Iran Rashidi2, Mehrdad Dargahi Malamiri3, Seyed Nematollah Jazayeri4, Arshya Allahdin5
1Pathologist, Ahwaz-Jondishapour Medical University, Iran.
2MD Pathologist, Associate Professor of Medical School, Ahwaz-Jondishapour Medical University, Iran.
3Internal Medicine Specialist, Ahwaz-Jondishapour Medical University, Iran.
4Associate Professor of Medical School, Ahwaz-Jondishapour Medical University, Iran.
5Assistant of Pathology, Ahwaz-Jundishapour Medical University, Iran.

Article ID: 100005Z11SZ2016
doi:10.5348/Z11-2016-5-CR-1

Address correspondence to:
Sara Zaheri
Ahwaz- Jondishapour Medical University
Iran

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How to cite this article:
Zaheri S, Rashidi I, Malamiri MD, Jazayer SN, Allahdin A. Adrenal Myelolipoma: A case report. J Case Rep Images Pathol 2016;2:1–5.


Abstract
Introduction: Myelolipoma is a rare benign adrenal tumor. Usually it is small, asymptomatic and unilateral. It is mostly discovered as an incidentaloma during autopsy. The incident detection of the tumor is increasing in frequency with widespread use of cross-sectional imaging such ultrasonography and computed tomography (CT). The most consistent complaint is abdominal pain caused by hemorrhage the tumor when the lesion became larger than 5 cm. In this paper, we reported symptomatic adrenal myelolipoma because of rarity and its considerable size.
Case Report: We report a woman with obesity and mild hypertension presented with unilateral myelolipoma measuring 7x3.5x2 cm. The pathological study revealed adrenal consist of adipocytes associated with hematopoietic elements and blood clot.
Conclusion: Myelolipoma as an incidental diagnosis might kept in mind and evaluate accordingly validate protocol because of surgical emergencies such as spontaneous retroperitoneal hemorrhage.

Keywords: Adrenal tumor, Incidentaloma, Myelolipoma


Introduction

Myelolipoma is an uncommon benign tumor like lesion of the adrenal gland [1].

These tumors were initially described by Giercke in 1905, and 24 years later, Oberling coined the term 'Myelolipoma' [2]. They are omposed of mature adipocytes and normal hematopoietic tissue [1] [2][3]. The incidence of adrenal Myelolipoma is reported as being 0.08 to 0.4% at autopsy [4][5]. In the past, this tumor was primarily detected on autopsies [6] Lately, due to widespread use of radiological studies such as ultrasonography, CT, and magnetic resonance imaging (MRI), incidental discovery of indolent adrenal myelolipomas has become more common [7]. According to Akamatsu et al. result, its incidental detection has become more common, reaching up to 7% of the adrenal masses. The well-recognized complication of adrenal Myelolipoma is spontaneous retroperitoneal hemorrhage. No potential of malignancy for adrenal Myelolipoma has been proved [8]. If the diagnosis of adrenal myelolipoma cannot be made with confidence using noninvasive imaging, fine-needle aspiration (FNA) biopsy should be considered [9][10] [11]. Also in cases where expectant management is being considered, FNA can definitively rule out malignancy. The presence of mature adipocytes and hematopoietic elements is diagnostic of myelolipoma [10] [11][12].

The diagnosis of myelolipoma is based on the identification of macroscopic fat within the tumor with negative Hounsfield on CT (Figure 1), [13]. The differential diagnosis should include renal angiomyolipoma, retroperitoneal lipoma and liposarcoma [12]. It may present as bleeding or rupture. More severe symptoms include hematuria, renovascular hypertension, even surgical emergencies such as retroperitoneal hemorrhage of the masses presented with life-threatening cardiovascular shock was reported [14][15][16][17][18].

This paper report is a case of clinicopathological characteristic of adrenal myelolipoma with considerable size and bleeding when the tumor where diagnosis was made on the basis of pathological examination.


Case Report

An obese 54-year-old woman referred to surgical department with intermittent, dull aching, vague abdominal pain from a few months and history of mild hypertension. The physical examination was unremarkable. Computed tomography scan showed a right adrenal mass measuring 7x3.5x2 cm. It was labeled as a fat containing mass, raising the possibility of Lipoma, fat-rich adrenal adenoma or liposarcoma. The patient was subjected to multiplanar, multi sequential magnetic resonance imaging (MRI) of the adrenal glands. The findings were consistent with the heterogeneous solid mass. Some hormonal laboratory investigations were done before surgery, for rolling out pheochromocytoma, Cushing syndrome, aldosteronism and Cortico adrenal hyperplasia (CAH) (Table 1). Patients underwent unilateral laparoscopic adrenalectomy, Pathological investigation of tumor in grossly shows well defined mass measure about 7 cm, weighing 310 grams. In cut section, foci of solid yellow tissue and blood clot area are noted (Figure 2). Microscopic histopathology revealed myelolipoma composed of adipocytes with interspersed hematopoietic elements, consist of myeloid and erythroid precursors (Figure 3).


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Figure 1: Unenhanced computed tomography scan in an asymptomatic myelolipoma revealed a 6-cm right adrenal mass with density measurements that range from -14 to -27 Hounsfield units.



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Figure 2: Gross of myelolipoma, well defined mass measure 7x3.5x2 cm which, on cut section showing foci of solid yellow tissue and brownish blood clot like area.



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Figure 3: Microscopy of lesion revealed mature fat cell mixed with hematopoietic elements.



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Table 1: Symmetry of the useful laboratory data from patient to rule out differential diagnosis



Discussion

Myelolipoma in the patient were found with history of mild hypertension and obesity. Occasionally, there are clinical symptoms such as abdominal pain or flank pain due to excess growth, bleeding within tumor as our patient and spontaneous retroperitoneal tumor bleeding [15] [16]. The diagnosis can be established by US, CT or MRI examination based on the identification of negative Hounsfield values for fat within the tumor on CT, but visual comparison to visceral or subcutaneous fat is insufficient in most cases [11] [17]. In the majority of the cases, adrenal myelolipomas are unenhanced foci inT1-weighted imaging [12] . The tumor is usually unilateral and rarely exceed 4 cm. However, very large and bilateral myelolipomas have been reported [13]. Based on the other literature, one of the largest adrenal myelolipoma reported weighted 6 kg and measured 31×24.5×11.5 cm [16] [18][14]. Most patient with myelolipoma underwent surgical resection for a suspected neoplasm, recently with increased frequent detection of myelolipoma, the treatment has been a matter in debt. According to Cristofaro result asymmetric, small tumors < 5 cm are treated conservative with 6–9 months interval whereas symptomatic and large tumor >10 cm should be underwent surgery [5]. The pain responded to an intercostals nerve block, which was done by pain specialist [19]. Since the risk of spontaneous rupture or bleeding is minimal in these small myelolipoma, observation can avoid lifelong steroid substitution [5] [16]. However, according to result of Daneshmand et al. surgery indicate if tumor exceeds 7 cm [6]. Additionally, myelolipomas have been reported to grow significantly during observation and there are number of case reports spontaneous hemorrhage or bleeding with minor trauma [20] [21].


Conclusion

We conclude due to the recent increase in the number of incidentally found adrenal myelolipomas, as the matter of fact because of unawareness patient for following or rarely insufficient medical facilities, because surgical emergencies, tight guidelines are now needed to help decided between indolent watch full masses to waiting versus surgical removal of these benign tumors.


References
  1. Lack EE. Tumors of the adrenal gland and extra-adrenal paraganglia. Washington, DC: Armed Forces Institute of Pathology; 1970.    Back to citation no. 1
  2. Novitsky YW, Czerniach DR, Kercher KW, Perugini RA, Kelly JJ, Litwin DE. Feasibility of laparoscopic adrenalectomy for large adrenal masses. Surg Laparosc Endosc Percutan Tech 2003 Apr;13(2):106–10.   [CrossRef]   [Pubmed]    Back to citation no. 2
  3. Ersoy E, Ozdogan M, Demirag A, et al. Giant adrenal myelolipoma associated with small bowel leiomyosarcoma: a case report. Turk J Gastroenterol 2006 Jun;17(2):126–9.   [Pubmed]    Back to citation 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 Sep 3;2:8414.   [CrossRef]   [Pubmed]    Back to citation no. 4
  5. Cristofaro MG, Lazzaro F, Fava MG, Aversa C, Musella M. Giant adrenal myelolipoma: a case report and review of the literature. Ann Ital Chir 2004 Nov-Dec;75(6):677–81.   [Pubmed]    Back to citation no. 5
  6. Daneshmand S, Quek ML. Adrenal myelolipoma: diagnosis and management. Urol J 2006 Spring;3(2):71–4.   [Pubmed]    Back to citation no. 6
  7. Akamatsu H, Koseki M, Nakaba H, Sunada S, Ito A, Teramoto S, Miyata M. Giant adrenal myelolipoma: report of a case. Surg Today 2004;34(3):283–5.   [CrossRef]   [Pubmed]    Back to citation no. 7
  8. deBlois GG, DeMay RM. Adrenal myelolipoma diagnosis by computed-tomography-guided fine-needle aspiration. A case report. Cancer 1985 Feb 15;55(4):848–50.   [CrossRef]   [Pubmed]    Back to citation no. 8
  9. Gaboardi F, Carbone M, Bozzola A, Galli L. Adrenal incidentalomas: what is the role of fine needle biopsy? Int Urol Nephrol 1991;23(3):197–207.   [CrossRef]   [Pubmed]    Back to citation no. 9
  10. Galli L, Gaboardi F. Adrenal myelolipoma: report of diagnosis by fine needle aspiration. J Urol 1986 Sep;136(3):655–7.   [Pubmed]    Back to citation no. 10
  11. Wadih GE, Nance KV, Silverman JF. Fine-needle aspiration cytology of the adrenal gland. Fifty biopsies in 48 patients. Arch Pathol Lab Med 1992 Aug;116(8):841–6.   [Pubmed]    Back to citation no. 11
  12. Carlos AA, Westphalen MD, Bonnie N, Joe MD. CT and MRI of Adrenal Masses. Appl Radiol 2006;35(8):10–26.   [Pubmed]    Back to citation no. 12
  13. Tyritzis SI, Adamakis I, Migdalis V, Vlachodimitropoulos D, Constantinides CA. Giant adrenal myelolipoma, a rare urological issue with increasing incidence: a case report. Cases J 2009 Sep 1;2:8863.   [CrossRef]   [Pubmed]    Back to citation no. 13
  14. Polamaung W, Wisedopas N, Vasinanukorn P, Pak-art P, Snabboon T. Asymptomatic bilateral giant adrenal myelolipomas: case report and review of literature. Endocr Pract 2007 Oct;13(6):667–71.   [CrossRef]   [Pubmed]    Back to citation no. 14
  15. Répássy DL, Csata S, Sterlik G, Iványi A. Giant adrenal myelolipoma. Pathol Oncol Res 2001;7(1):72–3.   [Pubmed]    Back to citation no. 15
  16. Brogna A, Scalisi G, Ferrara R, Bucceri AM. Giant secreting adrenal myelolipoma in a man: a case report. J Med Case Rep 2011 Jul 9;5:298.   [CrossRef]   [Pubmed]    Back to citation no. 16
  17. McGeoch SC, Olson S, Krukowski ZH, Bevan JS. Giant bilateral myelolipomas in a man with congenital adrenal hyperplasia. J Clin Endocrinol Metab 2012 Feb;97(2):343–4.   [CrossRef]   [Pubmed]    Back to citation no. 17
  18. Ferreira F, Martins JM, do Vale S, Esteves R, Nunes G, Carmo Id. Rare and severe complications of congenital adrenal hyperplasia due to 21-hydroxylase deficiency: a case report. J Med Case Rep 2013 Feb 6;7:39.   [CrossRef]   [Pubmed]    Back to citation no. 18
  19. Al-Bahri S, Tariq A, Lowentritt B, Nasrallah DV. Giant bilateral adrenal myelolipoma with congenital adrenal hyperplasia. Case Rep Surg 2014;2014:728198.   [CrossRef]   [Pubmed]    Back to citation no. 19
  20. Albala DM, Chung CJ, Sueoka BL, Memoli VA, Heaney JA. Hemorrhagic myelolipoma of adrenal gland after blunt trauma. Urology 1991 Dec;38(6):559–62.   [CrossRef]   [Pubmed]    Back to citation no. 20
  21. Russell C, Goodacre BW, vanSonnenberg E, Orihuela E. Spontaneous rupture of adrenal myelolipoma: spiral CT appearance. Abdom Imaging 2000 Jul-Aug;25(4):431–4.   [CrossRef]   [Pubmed]    Back to citation no. 21

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Author Contributions
Sara Zaheri – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Iran Rashidi – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Mehrdad Dargahi Malamiri – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Seyed Nematollah Jazayeri – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Arshya Allahdin – Analysis and interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
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None
Conflict of interest
Authors declare no conflict of interest.
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© 2016 Sara Zaheri et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.