Adrenal myelolipoma: Controversies in its management

Abstract

Adrenal myelolipomas (AMLs) are rare, benign neoplasms of the adrenal gland with varied clinical presentations. The rarity of these tumors precludes any case-controlled or randomized study into their management. The available literature is limited to case reports and short series from referral centers. This review is an effort to put the available literature into perspective such that clinical decision making can be done with some clarity. The PubMed and Cochrane databases were searched with key words Adrenal Myelolipoma, Adrenal Incidentaloma (AI) and Adrenal Collision Tumor (ACT). From over 1300 search results, 547 relevant publications dating from 1954 to 2014 were reviewed. Details of about 1231 AMLs in the indexed literature were analyzed. Increasing usage of imaging studies has significantly increased the discovery of AMLs. Although AMLs are benign tumors, those measuring larger than 6 cm are prone to rupture and hemorrhage. Thorough endocrine work-up may benefit a selected group of patients, especially those who are hypertensive, diabetic/pre-diabetic, young patients (<50 years) and those with bilateral AML. Regular observation is needed for AML patients who are being treated non-operatively, as many of them may require surgery during follow-up. Although the AACE/AAES guidelines for AI (2009) exclude AML from mandatory metabolic work-up for a newly discovered AI, we feel that a significant number of patients with AML would benefit from metabolic work-up. In the literature, endocrine dysfunction in AML is 7% as compared with 11% in AI. Endocrine dysfunction in AML is probably underdiagnosed.

Lipomatous tumours in adrenal gland: WHO updates and clinical implications

“…More than 1200 cases of adrenal myelolipomas were reported in the literature with more than 75% of the cases reported after 2000 (Shenoy et al 2015). The increase in incidence of reporting the entity in the recent years is assumed to be due to the increased use of imaging modalities.…”

“…In the English literature, approximately 40 cases of bilateral myelolipomas were noted (Jung et al 2007, Yang et al 2015, Zattoni et al 2015, Kale et al 2015, Chakraborty et al 2016, Soveid & Rais-Jalali 2016. Shenoy and coworkers analysed the literature and reported that 12% of cases reported in the literature are bilateral (Shenoy et al 2015). This may be related to the bias in reporting cases of bilateral myelolipomas.…”

“…On review of the literature, Shenoy and coworkers reported that adrenal myelolipoma comprised 6% of adrenal incidentaloma detected on radiological examination (Shenoy et al 2015). For the symptomatic cases, the symptoms reported are often abdominal pain or discomfort.…”

“…The stress may be related to prolong increase in endogenous adrenocorticotropic hormone (ACTH) stimulation (Shenoy et al 2015). Myelolipoma has been reported to be associated with many diseases as it was commonly being detected incidentally during workup for other diseases.…”

“…It is a benign tumour in the adrenal cortex composed of mixture of adipose tissue and bone marrow (haematopoietic) elements. In large series, adrenal myelolipoma comprises approximately 3-4% of primary adrenal tumour (Lam & Lo 2001, Kulis et al 2012, Shenoy et al 2015.…”