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 ¤  Abstract
 ¤ Introduction
 ¤ Case report
 ¤ Discussion
 ¤ Conclusion
 ¤  References
 ¤  Article Figures

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UNUSUAL CASE
Year : 2014  |  Volume : 10  |  Issue : 4  |  Page : 213-215
 

Adrenal angiomyolipoma: A case report and review of literature


Department of Minimal Access, Metabolic and Bariatric Surgery, Max Super Specialty Hospital, Saket, New Delhi, India

Date of Submission08-Jun-2014
Date of Acceptance14-Jun-2014
Date of Web Publication23-Sep-2014

Correspondence Address:
Anil Sharma
1-2, Press Enclave Road, Saket, New Delhi - 110 017
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-9941.141531

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 ¤ Abstract 

Angiomyolipoma (AML) is a rare mesenchymal tumour arising from perivascular epithelioid cells. It is most commonly seen in kidney, but rarely AML can arise in extra renal sites. Adrenal AML is a very rare clinical entity, and very few cases have been reported so far. We present our experience with a 43-year-old female, who presented with right flank pain. Magnetic resonance imaging showed a right adrenal mass. Laparoscopic adrenelectomy was performed, and the histopathology report confirmed the diagnosis of AML. Patient was discharged uneventfully.


Keywords: Adrenal, angiomyolipoma, laparoscopy


How to cite this article:
Goswami A, Sharma A, Khullar R, Soni V, Baijal M, Chowbey P. Adrenal angiomyolipoma: A case report and review of literature . J Min Access Surg 2014;10:213-5

How to cite this URL:
Goswami A, Sharma A, Khullar R, Soni V, Baijal M, Chowbey P. Adrenal angiomyolipoma: A case report and review of literature . J Min Access Surg [serial online] 2014 [cited 2020 Sep 20];10:213-5. Available from: http://www.journalofmas.com/text.asp?2014/10/4/213/141531



 ¤ Introduction Top


Angiomyolipoma (AML) is a benign mesenchymal tumour consisting of varying amounts of mature adipose tissue, smooth muscle, and thick walled blood vessels. It is relatively rare benign tumour appearing in about 0.3% of general population and accounts for 3% of solid renal masses. [1] Extra renal AMLs (ERAMLs) are rare tumours that present as incidentalomas upon imaging for other conditions. Case reports of extra renal AML are rare with <60 reported cases since they were first described by Friis and Hjortrup in 1982. Liver is the most common site for extra-renal AML. Only few cases of adrenal AML are reported in literature. [2],[3],[4] Our case described one of the largest ever reported myolipomas, which despite its size, can be rather asymptomatic.


 ¤ Case report Top


A 43-year-old female presented with right loin pain. A vague lump was palpable in right hypochondrium. Routine haematological parameters were normal. Serum catecholamine, cortisol, and urinary, vanillylmandelic acid were within normal limits. Magnetic resonance imaging (MRI) revealed 9.5 cm × 8 cm × 2 cm right adrenal mass [Figure 1]. The tumour was predominantly hyperintense with signal intensity similar to fat on T1/T2-weighted images and lost signal on the fat-suppressed sequences.
Figure 1: Magnetic resonance imaging abdomen showing the adrenal mass

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Laparoscopic right adrenalectomy was performed. A well-encapsulated, firm globular mass separate from the right kidney and no definable adrenal was found [Figure 2] and [Figure 3]. Gross post-operative evaluation of the adrenal mass showed approximately 10 cm × 8 cm × 2 cm greyish white solid, smooth, and firm mass [Figure 4]. Histological examination revealed benign adrenal cortical elements, with a predominance of mature benign adipose tissue interspersed with pockets of haemopoietic (erythro-myeloid) intermediates, and megakaryocytes which was confirmatory for the diagnosis of adrenal AML.

Patient recovered well and was discharged uneventfully on 2 nd post-operative day.
Figure 2: The adrenal mass intra-operatively

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Figure 3: Dissection with harmonic

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Figure 4: Resected adrenal mass specimen

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 ¤ Discussion Top


Angiomyolipoma is a rare clinical entity mostly involving kidney and is part of the group of tumour known as tumours of perivascular epithelioid cell origin. The most widely accepted theory of origin is adrenocortical cell metaplasia in response to stimuli, such as necrosis, inflammation, infection or stress (Meyer et al.). The presence of perivascular epithelioid cells is characteristic of AML since these cells show immunoreactivity for muscle markers (epithelial membrane antigen, keratin, vimentin, desmin, and actin) and HMB-45. Positive immunoreactivity for HMB-45, a monoclonal antibody, is characteristic of AMLs and can be used to differentiate AMLs from other similar appearing lesions. Most commonly involved organ is a kidney. Broadly two types have been described. One is isolated AML, and the other is that is associated with tuberous sclerosis. Male to female ratio in isolated cases ranges from 1:4 to 1:8. Most patients of isolated cases are in the age group of 27-72 years, mean age being 43 years.

Although the majority of ERAMLs are benign, two cases of metastatic and recurrent ERAMLs have been reported. The demonstration of fat density (hypodense) within an adrenal mass by computed tomography is virtually diagnostic of AML. MRI is sometimes required to demonstrate origin of the tumour, to define the tissue planes when the tumour is large and heterogeneous, and to distinguish benign from malignant lesions by comparing signal intensity ratios of adrenal to liver.

The management of adrenal myelolipomas should be individualised. A small, <5 cm, asymptomatic myelolipoma could be followed-up over 1-2 year period with imaging controls. On the contrary, asymptomatic lesion or a large >5 cm myelolipoma should be surgically excised, since there are reports of spontaneous rupture and haemorrhage of the mass presented with life-threatening cardiovascular shock. [5] Unstable patients may benefit from emergent tumour embolisation and a subsequently staged surgical resection.

Since it is a benign disease, its prognosis is good. Nevertheless, follow-up is recommended because of atypical morphology. Currently, there is no agreed protocol on follow-up but an ultrasound three to 6 months following the surgery with annual clinical examination for large tumours is recommended.


 ¤ Conclusion Top


The adrenal myolipoma is a rare urological entity, which seems to increase its frequency, probably due to causes affecting primarily the function and physiology of the adrenals.

Even with the contemporary imaging modalities, precise diagnosis is difficult. Ever since its introduction by Gagner in 1992, laparoscopic adrenalectomy has become the standard of care for the treatment of functioning and non-functioning adrenal tumours. Many authors have found a decrease in perioperative morbidity and convalescence after this procedure when compared with open surgery. [6],[7]

 
 ¤ References Top

1.Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma. J Urol 2002;168:1315-25.  Back to cited text no. 1
    
2.Sutter R, Boehler A, Willmann JK. Adrenal angiomyolipoma in lymphangioleiomyomatosis. Eur Radiol 2007;17:565-6.  Back to cited text no. 2
    
3.Lam KY, Lo CY. Adrenal lipomatous tumours: A 30 year clinicopathological experience at a single institution. J Clin Pathol 2001;54:707-12.  Back to cited text no. 3
    
4.Godara R, Vashist MG, Singla SL, Garg P, Sen J, Mathur SK, et al. Adrenal angiomyolipoma: A rare entity. Indian J Urol 2007;23:319-20.  Back to cited text no. 4
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5.Russell C, Goodacre BW, vanSonnenberg E, Orihuela E. Spontaneous rupture of adrenal myelolipoma: Spiral CT appearance. Abdom Imaging 2000;25:431-4.  Back to cited text no. 5
    
6.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;13:106-10.  Back to cited text no. 6
    
7.Vargas HI, Kavoussi LR, Bartlett DL, Wagner JR, Venzon DJ, Fraker DL, et al. Laparoscopic adrenalectomy: A new standard of care. Urology 1997;49:673-8.  Back to cited text no. 7
    


    Figures

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



 

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