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Editorial Comment to Adrenal myelolipoma associated with hyperandrogenemia

Myelolipoma is a rare, benign neoplasm composed of mature adipocytes and hematopoietic tissue. It was first described by Gierke in 1905 and subsequently by Oberling in 1929, who used the term “myelolipoma”. In the past, the finding of adrenal lesions was made possible by autopsy or by clinical presentation, related either to the massive growth of the gland or to altered hormone production. Today, with the widespread use of non-invasive imaging with ultrasonography, computed tomography and magnetic resonance imaging, incidental detection of myelolipoma has become more common, constituting up to 10–15% of incidental adrenal masses. Men and women seem to be equally affected by these tumors. Adrenal myelolipoma is most commonly found between the fifth and seventh decades of life. In the latter age group, its low incidence seems to have increased by 0.2% to 10% since the mid-1990s. Similar lesions have also been reported from extra-adrenal sites. The association of myelolipomas with obesity, hypertension and diabetes mellitus is well known. Most lesions are generally small, unilateral, nonfunctional and asymptomatic; nevertheless, a number of bilateral tumors have been described in the literature. An increase in mass is very slow. The most common symptom is non-specific abdominal pain, probably as a result of hemorrhage, tumor necrosis or mechanical compression of adjacent organs from large tumor bulk. Also, hematuria and renovascular hypertension have been seen. Although rare, surgical emergencies, such as a retroperitoneal hemorrhage, might be encountered. There are case reports of their association with overproduction of adrenal hormones, such us dehydroepiandrosterone-sulphate, and congenital adrenal hyperplasia caused by 21-hydroxylase deficiency, congenital adrenal 17 a-hydroxylase deficiency, Cushing disease, Conn’s syndrome, adrenal insufficiency, and pheochromocytoma. The differential diagnosis includes retroperitoneal lipoma or liposarcoma, upper pole renal angiomyolipoma and retroperitoneal teratoma. Pathogenesis of this unusual tumor is yet to be confirmed. According to a recent theory, myelolipoma originates from a metaplastic process of the reticuloendothelial cells within adrenal capillaries; however, some authors consider the development of adrenal myelolipomas to be a response to various endocrine stimulations, whereas other contemporary authors have speculated about a stressful lifestyle and an unbalanced diet as factors that might be involved in the pathogenesis of this tumor. In this issue of International Journal of Urology, Su et al. speculate that in the pathogenesis of myelolipoma, multiple factors are involved including adrenal cortical metaplasia, embolism of bone marrow cells and development from embryonic rests. They hypothesize that adrenal myelolipoma associated with hyperandrogenemia might be determined by the aforementioned multiple factors, extrinsic compression of the large tumor and the enzymes involved in the production of hormones, such as Melamin-A (specific marker of zona reticularis), that, in their case, is strongly expressed in the cells of the zona reticularis. So they suggest that immunohistochemical markers could help to discriminate the origin of this tumor. Biochemical studies are also useful and necessary in the typing of the mass. In conclusion, the prognosis of myelolipoma is excellent. Malignant change is still not documented. The treatment has been a matter of much debate. When the tumor is 4 cm or smaller and asymptomatic, follow up with ultrasonography or computed tomography is recommended. If symptoms occur, surgery should be carried out promptly, especially for a large myelolipoma, because spontaneous rupture of the lesion with hemorrhage is possible.