Abstract
Introduction
Adrenal myelolipoma is an invariably benign neoplasm of the adrenal gland that is the second most common primary adrenal incidentaloma following adrenocortical adenomas. It is composed of elements of adipose tissue and extramedullary hematopoiesis. Hypotheses on stem cells and hormonal factors have been formulated regarding its pathogenesis that is still obscure. Despite its benign behavior, adrenal myelolipoma is clinically relevant as it might cause significant difficulties in the differential diagnosis of adrenal tumors.
Methods
We have reviewed 420 cases reported between 1957 and 2017 on adrenal myelolipoma retrieved from PubMed and Scopus databases and also 20 of our case series to provide a comprehensive analysis of their pathology, epidemiological and clinical features.
Results and Conclusions
The average age for its diagnosis was 51 years, and no gender difference was observed. The average size of tumors was 10.2 cm. Congenital adrenal hyperplasia was associated to 10% of all cases analyzed, while other adrenal hypersecretory disorders (cortisol, aldosterone) were found in 7.5% of cases. Computed tomography and magnetic resonance imaging can be reliably used for its differential diagnosis. If the diagnosis of an adrenal myelolipoma is unambiguous, and no associated symptoms or hormonal activity are established, surgical intervention is usually not necessary.
Similar content being viewed by others
Abbreviations
- ACTH:
-
adrenocorticotropic hormone
- AML:
-
adrenal myelolipoma
- CAH:
-
congenital adrenal hyperplasia
- EMH:
-
extramedullary hematopoiesis
References
A.K. Lam, Update on adrenal tumours in 2017 World Health Organization (WHO) of Endocrine Tumours. Endocr. Pathol. 1–15 (2017). https://doi.org/10.1007/s12022-017-9484-5
C.A. Olsson, R.J. Krane, R.C. Klugo, S.M. Selikowitz, Adrenal myelolipoma. Surgery 73, 665–670 (1973)
J.H. Song, F.S. Chaudhry, W.W. Mayo-Smith, The incidental adrenal mass on CT: Prevalence of adrenal disease in 1,049 consecutive adrenal masses in patients with no known malignancy. Am. J. Roentgenol. 190, 1163–1168 (2008). https://doi.org/10.2214/AJR.07.2799
X. Bin, Y. Qing, W. Linhui, G. Li, S. Yinghao, Adrenal incidentalomas: Experience from a retrospective study in a Chinese population. J. Urol. 29, 270–274 (2011). https://doi.org/10.1016/j.urolonc.2009.03.027
F. Mantero, A.M. Masini, G. Opocher, M. Giovagnetti, G. Arnaldi, Tumors, on behalf of the N.I.S.G. on A., Adrenal incidentaloma: An overview of hormonal data from the National Italian Study Group. Horm. Res. 47, 284–289 (1997). https://doi.org/10.1159/000185478
Y. Minamiya, S. Abo, M. Kitamura, K. Izumi, Mediastinal extraadrenal myelolipoma: report of a case. Surg. Today 27, 971–972 (1997)
Y. Zeng, Q. Ma, L. Lin, P. Fu, Y. Shen, Q.-Y. Luo, L.-H. Zhao, J.-H. Mou, H.-L. Xiao, Giant myelolipoma in the spleen: A rare case report and literature review. Int. J. Surg. Pathol. 24, 177–180 (2016). https://doi.org/10.1177/1066896915617027
M. Ghaouti, K. Znati, A. Jahid, F. Zouaidia, Z. Bernoussi, N. Mahassini, Renal myelolipoma: a rare extra-adrenal tumor in a rare site: a case report and review of the literature. J. Med. Case Rep. 7, 92 (2013). https://doi.org/10.1186/1752-1947-7-92
M. Sundaram, T. Bauer, A. von Hochstetter, H. Ilaslan, M. Joyce, Intraosseous myelolipoma. Skelet. Radiol. 36, 1181–1184 (2007). https://doi.org/10.1007/s00256-007-0347-x
M. Krismann, G. Reichle, K.M. Müller, [Thoracic bilateral myelolipoma]. Pneumologie 47, 501–503 (1993)
S.A. George, M.T. Manipadam, R. Thomas, Primary myelolipoma presenting as a nasal cavity polyp: a case report and review of the literature. J. Med. Case Rep. 6, 127 (2012). https://doi.org/10.1186/1752-1947-6-127
S.H. Stein, F. Latour, S.S. Frost, Myelolipoma arising from ectopic adrenal cortex: case report and review of the literature. Am. J. Gastroenterol. 81, 999–1001 (1986)
S.J. Cina, B.M. Gordon, N.S. Curry, Ectopic adrenal myelolipoma presenting as a splenic mass. Arch. Pathol. Lab. Med. 119, 561–563 (1995)
S.J. Newman, K. Inzana, W. Chickering, Extradural myelolipoma in a dog. J. Vet. Diagn. Investig. 12, 71–74 (2000). https://doi.org/10.1177/104063870001200115
G. Storms, G. Janssens, Intraocular myelolipoma in a dog. Vet. Ophthalmol. 16, 183–187 (2013). https://doi.org/10.1111/vop.12059
Taher, A., Vichinsky, E., Musallam, K., Cappellini, M.D., Viprakasit, V., Weatherall, S.D.: Guidelines for the management of non transfusion dependent thalassaemia (NTDT). Thalassaemia International Federation (2013). https://www.ncbi.nlm.nih.gov/books/NBK190453/
M.R. Fowler, R.B. Williams, J.M. Alba, C.R. Byrd, Extra-adrenal myelolipomas compared with extramedullary hematopoietic tumors: a case of presacral myelolipoma. Am. J. Surg. Pathol. 6, 363–374 (1982)
Chen, K.T.K., Felix, E.L., Flam, M.S.: Extraadrenal myelolipoma. Am. J. Clin. Pathol. 78, 386–389 (1982)
R. Sanders, N. Bissada, N. Curry, B. Gordon, Clinical spectrum of adrenal myelolipoma: Analysis of eight tumors in seven patients. J. Urol. 153, 1791–1793 (1995). https://doi.org/10.1016/S0022-5347(01)67307-5
V.G. Shenoy, A. Thota, R. Shankar, M.G. Desai, Adrenal myelolipoma: Controversies in its management. Indian. J. Urol. 31, 94–101 (2015). https://doi.org/10.4103/0970-1591.152807
S. De Navasquez, Case of myelo-lipoma (bone-marrow, heterotopia) pf suprarenal gland. Guys. Hosp. Rep. 88, 237–240 (1935)
K.-C. Chang, P.-I. Chen, Z.-H. Huang, Y.-M. Lin, P.-L. Kuo, Adrenal myelolipoma with translocation (3;21)(q25; p11). Cancer Genet. Cytogenet 134, 77–80 (2002). https://doi.org/10.1016/S0165-4608(01)00592-1
E. Bishop, J.N. Eble, L. Cheng, M. Wang, D.R. Chase, A. Orazi, D.P. O’Malley, Adrenal myelolipomas show nonrandom X-chromosome inactivation in hematopoietic elements and fat: Support for a clonal origin of myelolipomas. Am. J. Surg. Pathol. 30, 838–843 (2006). https://doi.org/10.1097/01.pas.0000202044.05333.17
C. Feng, H. Jiang, Q. Ding, H. Wen, Adrenal myelolipoma: A mingle of progenitor cells? Med. Hypotheses 80, 819–822 (2013). https://doi.org/10.1016/j.mehy.2013.03.021
H. Selye, H. Stone, Hormonally induced transformation of adrenal into myeloid tissue. Am. J. Pathol. 26, 211–233 (1950)
S.C. Sieber, N.A. Gelfman, R. Dandurand, F. Braza, Ectopic ACTH and adrenal myelolipoma. Conn. Med. 53, 7–10 (1989)
H. Hagiwara, T. Usui, T. Kimura, T. Tagami, M. Naruse, S. Minamiguchi, T. Kato, H. Okuno, A. Shimatsu, Lack of ACTH and androgen receptor expression in a giant adrenal myelolipoma associated with 21-hydroxylase deficiency. Endocr. Pathol. 19, 122–127 (2008). https://doi.org/10.1007/s12022-008-9034-2
I. Motta, L. Boiocchi, P. Delbini, M. Migone De Amicis, E. Cassinerio, D. Dondossola, G. Rossi, M.D: Cappellini, A giant adrenal myelolipoma in a beta-thalassemia major patient: Does ineffective erythropoiesis play a role? Am. J. Hematol. 91, 1281–1282 (2016). https://doi.org/10.1002/ajh.24446
B.P. Rubin, K.H. Allison, G.N. Mann, T.H. Norwood, An unusual case of multiple giant myelolipomas: Clinical and pathogenetic implications. Endocr. Pathol. 14, 93–100 (2003)
Merchant, S.H., Herman, C.M., Amin, M.B., Ro, J.Y., Troncoso, P.: Myelolipoma associated with adrenal ganglioneuroma. Arch. Pathol. Lab. Med. 126, 736–737 (2002)
H. Lu, M. Gan, H. Chen, S. Huang, Adrenal myelolipoma within myxoid cortical adenoma associated with Conn’s syndrome. J. Zhejiang Univ. Sci. B. 9, 500–505 (2008). https://doi.org/10.1631/jzus.B0720014
U. Kumar, S. Priyadarshi, V. Tomar, R.R. Vohra, Adrenal myelolipoma with osseous metaplasia and hypercortisolism. Urol. Ann. 9, 71–73 (2017). https://doi.org/10.4103/0974-7796.198841
R. Armand, A.R. Cappola, R.B. Horenstein, C.B. Drachenberg, H. Sasano, J.C. Papadimitriou, Adrenal cortical adenoma with excess black pigment deposition, combined with myelolipoma and clinical Cushing’s syndrome: A case report and review of the literature. Int. J. Surg. Pathol. 12, 57–61 (2004). https://doi.org/10.1177/106689690401200111
B.F. Schwartz, L. Wasson, Hibernoma arising from the adrenal gland. Urology 61, 1035 (2003). https://doi.org/10.1016/S0090-4295(02)02586-4
I.A. Cardinalli, A.G. de Oliveira-Filho, M.J. Mastellaro, R.C. Ribeiro, S.S. Aguiar, A unique case of synchronous functional adrenocortical adenoma and myelolipoma within the ectopic adrenal cortex in a child with Beckwith–Wiedemann syndrome. Pathol. Res. Pract. 208, 189–194 (2012). https://doi.org/10.1016/j.prp.2011.12.011
M.C. Sebastià, M.O. Pérez-Molina, A. Alvarez-Castells, S. Quiroga, E. Pallisa, CT evaluation of underlying cause in spontaneous subcapsular and perirenal hemorrhage. Eur. Radiol. 7, 686–690 (1997). https://doi.org/10.1007/BF02742926
G. Low, H. Dhliwayo, D.J. Lomas, Adrenal neoplasms. Clin. Radiol. 67, 988–1000 (2012). https://doi.org/10.1016/j.crad.2012.02.005
K.Y. Lam, C.Y. Lo, Adrenal lipomatous tumours: a 30 year clinico- pathological experience at a single institution. J. Clin. Pathol. 54, 707–712 (2001)
A.K. Lam, Lipomatous tumours in adrenal gland: WHO updates and clinical implications. Endocr. Relat. Cancer 24, R65–R79 (2017). https://doi.org/10.1530/ERC-16-0564
M. Han, A.L. Burnett, E.K. Fishman, F.F. Marshall, The natural history and treatment of adrenal myelolipoma. J. Urol. 157, 1213–1216 (1997). https://doi.org/10.1016/S0022-5347(01)64926-7
Campbell, M.J., Obasi, M., Wu, B., Corwin, M.T., Fananapazir, G.: The radiographically diagnosed adrenal myelolipoma: what do we really know? Endocrine. 1–6 (2017). doi:https://doi.org/10.1007/s12020-017-1410-6
H.-P. Liu, W.-Y. Chang, S.-T. Chien, C.-W. Hsu, Y.-C. Wu, W.-C. Kung, C.-M. Su, P.-H. Liu, Intra-abdominal bleeding with hemorrhagic shock: a case of adrenal myelolipoma and review of literature. BMC Surg. 17, 74 (2017). https://doi.org/10.1186/s12893-017-0270-6
A.A. Reza-Albarran, F.J. Gomez-Perez, J.C. Lopez, M. Herrera, A. Gamboa-Dominguez, C. Keirns, A. Aranda, J.A. Rull, Myelolipoma: A new adrenal finding in Carney’s complex? Endocr. Pathol. 10, 251–257 (1999)
P. Kalafatis, Bilateral giant adrenal myelolipoma and polycystic ovarian disease. Urol. Int. 63, 139–143 (1999)
M. Yoshioka, K. Fujimori, M. Wakasugi, N. Yamazaki, M. Kuroki, T. Tsuchida, H. Seki, M. Sekiya, T. Tamura, Cushing’s disease associated with adrenal myelolipoma, adrenal calcification and thyroid cancer. Endocr. J. 41, 461–466 (1994)
S. Banik, P.S. Hasleton, R.L. Lyon, An unusual variant of multiple endocrine neoplasia syndrome: a case report. Histopathology 8, 135–144 (1984)
I. Maschler, E. Rosenmann, E.N. Ehrenfeld, Ectopic functioning adrenocortico-myelolipoma in longstanding Nelson’s syndrome. Clin. Endocrinol. (Oxf.). 10, 493–7 (1979)
F. Gemmel, H. Bruinsma, P. Oomen, J. Collins, PET/CT incidental detection of bilateral adrenal myelolipomas in a patient with a huge maxillary sinus carcinoma. Clin. Nucl. Med. 35, 132–133 (2010). https://doi.org/10.1097/RLU.0b013e3181c7c007
V. Ludwig, M.H. Rice, W.H. Martin, M.C. Kelley, D. Delbeke, 2-Deoxy-2-[18F]fluoro-D-glucose positron emission tomography uptake in a giant adrenal myelolipoma. Mol. Imaging Biol. 4, 355–8 (2002)
P. Boraschi, G. Braccini, R. Gigoni, F. Cartei, A. Campatelli, A. Di Vito, G. Perri, [Adrenal myelolipomas: their magnetic resonance assessment]. Clin. Ter. 147, 549–557 (1996)
Musante, F., Derchi, L.E., Bazzocchi, M., Avataneo, T., Gandini, G., Pozzi Mucelli, R.S.: MR imaging of adrenal myelolipomas. J. Comput. Assist. Tomogr. 15, 111–114 (1991)
D.J. Wale, K.K. Wong, B.L. Viglianti, D. Rubello, M.D. Gross, Contemporary imaging of incidentally discovered adrenal masses. Biomed. Pharmacother. 87, 256–262 (2017). https://doi.org/10.1016/j.biopha.2016.12.090
M. Fassnacht, M. Kroiss, B. Allolio, Update in Adrenocortical Carcinoma. J. Clin. Endocrinol. Metab. 98, 4551–4564 (2013). https://doi.org/10.1210/jc.2013-3020
M.L. Nunes, A. Rault, J. Teynie, N. Valli, M. Guyot, D. Gaye, G. Belleannee, A. Tabarin, 18F-FDG PET for the identification of adrenocortical carcinomas among indeterminate adrenal tumors at computed tomography scanning. World J. Surg. 34, 1506–1510 (2010). https://doi.org/10.1007/s00268-010-0576-3
M. Fassnacht, W. Arlt, I. Bancos, H. Dralle, J. Newell-Price, A. Sahdev, A. Tabarin, M. Terzolo, S. Tsagarakis, O.M. Dekkers, Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur. J. Endocrinol. 175, G1–G34 (2016). https://doi.org/10.1530/EJE-16-0467
Zhong, Y., Shen, Y., Pan, J., Wang, Y., An, Y., Guo, A., Ma, L., Ye, H., Wang, H.: Renal epithelioid angiomyolipoma: MRI findings. Radiol. Med. (2017). doi:https://doi.org/10.1007/s11547-017-0788-9
S. Krishna, C.A. Murray, M.D. McInnes, R. Chatelain, M. Siddaiah, O. Al-Dandan, S. Narayanasamy, N. Schieda, CT imaging of solid renal masses: pitfalls and solutions. Clin. Radiol. 72, 708–721 (2017). https://doi.org/10.1016/j.crad.2017.05.003
W.D. Craig, J.C. Fanburg-Smith, L.R. Henry, R. Guerrero, J.H. Barton, Fat-containing lesions of the retroperitoneum: Radiologic-pathologic correlation. RadioGraphics 29, 261–290 (2009). https://doi.org/10.1148/rg.291085203
M. Terzolo, S. Bovio, A. Pia, G. Reimondo, A. Angeli, Management of adrenal incidentaloma. Best. Pract. Res. Clin. Endocrinol. Metab. 23, 233–243 (2009). https://doi.org/10.1016/j.beem.2009.04.001
R. Libè, C. Dall’Asta, L. Barbetta, A. Baccarelli, P. Beck-Peccoz, B. Ambrosi, Long-term follow-up study of patients with adrenal incidentalomas. Eur. J. Endocrinol. 147, 489–494 (2002)
M. Zeiger, G. Thompson, Q.-Y. Duh, A. Hamrahian, P. Angelos, D. Elaraj, E. Fishman, J. Kharlip, American Association of Clinical Endocrinologists, American Association of Endocrine Surgeons: American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons Medical Guidelines for the Management of Adrenal Incidentalomas: Executive Summary of Recommendations. Endocr. Pract. 15, 450–453 (2009). https://doi.org/10.4158/EP.15.5.450
M. Terzolo, A. Stigliano, I. Chiodini, P. Loli, L. Furlani, G. Arnaldi, G. Reimondo, A. Pia, V. Toscano, M. Zini, G. Borretta, E. Papini, P. Garofalo, B. Allolio, B. Dupas, F. Mantero, A. Tabarin, A.M.E. Position, Statement on adrenal incidentaloma. Eur. J. Endocrinol. 164, 851–870 (2011). https://doi.org/10.1530/EJE-10-1147
S. Yamashita, K. Ito, K. Furushima, J. Fukushima, S. Kameyama, Y. Harihara, Laparoscopic versus open adrenalectomy for adrenal myelolipoma. Ann. Med. Surg. 3, 34–38 (2014). https://doi.org/10.1016/j.amsu.2014.04.001
R. Chaudhary, A. Deshmukh, K. Singh, R. Biswas, Is size really a contraindication for laparoscopic resection of giant adrenal myelolipomas? BMJ. Case Rep. 2016, bcr2016215048 (2016). https://doi.org/10.1136/bcr-2016-215048
A. Agrusa, G. Romano, G. Frazzetta, D. Chianetta, V. Sorce, G. Di Buono, G. Gulotta, Laparoscopic adrenalectomy for large adrenal masses: Single team experience. Int. J. Surg. 12, S72–S74 (2014). https://doi.org/10.1016/j.ijsu.2014.05.050
A.K. Hemal, A. Singh, N.P. Gupta, Whether adrenal mass more than 5 cm can pose problem in laparoscopic adrenalectomy? An evaluation of 22 patients. World J. Urol. 26, 505–508 (2008). https://doi.org/10.1007/s00345-008-0270-3
X. Shen, Y. Qiu, Y. Zheng, S. Zhang, Retroperitoneal laparoscopic liposuction for large adrenal myelolipomas: A report of nine cases. J. Laparoendosc. Adv. Surg. Tech. 22, 578–580 (2012). https://doi.org/10.1089/lap.2012.0113
Nakajo, M., Onohara, S., Shinmura, K., Fujiyoshi, F., Nakajo, M.: Embolization for spontaneous retroperitoneal hemorrhage from adrenal myelolipoma. Radiat. Med. 21, 214–219 (2003)
S.M. Chng, M.B.K. Lin, F.C. Ng, H.C. Chng, T.K. Khoo, Adrenal myelolipoma presenting with spontaneous retroperitoneal haemorrhage demonstrated on computed tomography and angiogram—a case report. Ann. Acad. Med. Singap. 31, 228–230 (2002)
Acknowledgements
The study has been supported by a grant from the Hungarian National Research, Development and Innovation Office (NKFIH K115398) to Dr. Peter Igaz. The authors would like to thank Dr. Katalin Borka MD PhD for providing a microscopic picture of myelolipoma and pathologic data of our case series.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no competing interests
Electronic supplementary material
Rights and permissions
About this article
Cite this article
Decmann, Á., Perge, P., Tóth, M. et al. Adrenal myelolipoma: a comprehensive review. Endocrine 59, 7–15 (2018). https://doi.org/10.1007/s12020-017-1473-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12020-017-1473-4