Exp Clin Endocrinol Diabetes 2009; 117(8): 440-445
DOI: 10.1055/s-0029-1202274
Short Communication

© J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New York

Myelolipomatous Adrenal Masses Causing Cushing's Syndrome

C. Lamas1 , L. M. López1 , E. Lozano2 , M. Atienzar3 , R. Ruiz-Mondéjar4 , J. J. Alfaro1 , F. Botella1
  • 1Department of Endocrinology, Complejo Hospitalario Universitario de Albacete, Universidad de Castilla-La Mancha, Albacete, Spain
  • 2Department of Radiology, Complejo Hospitalario Universitario de Albacete, Universidad de Castilla-La Mancha, Albacete, Spain
  • 3Department of Pathology, Complejo Hospitalrio Universitario de Albacete, Universidad de Castilla-La Mancha, Albacete, Spain
  • 4Department of Urology, Complejo Hospitalario Universitario de Albacete, Universidad de Castilla-La Mancha, Albacete, Spain
Further Information

Publication History

received 18.10.2008 first decision 05.12.2008

accepted 22.01.2008

Publication Date:
16 April 2009 (online)

Abstract

Adrenal myelolipomas are uncommon benign tumors, composed of mature adipose tissue and haematopoietic elements in varying proportions. They are usually asymptomatic, non-functioning adrenal incidentalomas, but there have been a few reports of myelolipomatous masses associated with adrenocortical hypersecretion. We report two cases of large mixed adrenal tumors, with heterogeneous appearance and areas of fat density in imaging techniques, and with autonomous cortisol production leading to Cushing's syndrome. Both underwent adrenalectomy and the histological study showed an adrenocortical adenoma with widespread myelolipomatous metaplasia. Hypercortisolism resolved in the one patient that could be evaluated after surgery. We review all the previous reported cases of hypercortisolism associated with adrenal myelolipomas. We also discuss the recommended diagnostic approach and therapeutic management of adrenal masses of lipomatous appearance.

References

  • 1 Abecassis M, MacLoughlin MJ, Langer B. et al . Serendipitous adrenal masses: prevalence, significance, and management.  Am J Surg. 1985;  149 783-788
  • 2 Angeli A, Osella G, Ali A. et al . Adrenal incidentaloma: an overview of clinical and epidemiological data from the National Italian Study Group.  Horm Res. 1997;  47 279-283
  • 3 Armand R, Cappola A, Horenstein R. et al . Adrenal cortical adenoma with excess black pigment deposition, combined with myelolipoma and clinical Cushing's syndrome.  Int J Surg Pathol. 2004;  12 57-61
  • 4 Aso Y, Homma Y. A survey on incidental adrenal tumors in Japan.  J Urol. 1992;  147 1478-1481
  • 5 Barry MK, van Heerden JA, Farley DR. et al . Can adrenal incidentalomas be safely observed?.  World J Surg. 1998;  22 599-603
  • 6 Barzon L, Scaroni C, Sonino N. et al . Risk factors and long-term follow-up of adrenal incidentalomas.  J Clin Endocrinol Metab. 1999;  84 520-526
  • 7 Barzon L, Sonino N, Fallo F. et al . Prevalence and natural history of adrenal incidentalomas.  Eur J Endocrinol. 2003;  149 273-285
  • 8 Bennett BD, MacKenna TJ, Hough AJ. et al . Adrenal myelolipoma associated with Cushing's disease.  Am J Clin Pathol. 1980;  73 443-447
  • 9 Boronat M, Moreno A, Cajal S. et al . Subclinical Cushing's syndrome due to adrenal myelolipoma.  Arch Pathol Lab Med. 1997;  121 735-737
  • 10 Condom E, Villabona CM, Gomez JM. et al . Adrenal myelolipoma in a woman with congenital 17-hydroxylase deficiency.  Arch Pathol Lab Med. 1985;  109 1116-1117
  • 11 Cormio L, Ruutu M, Giardina C. et al . Combined adrenal adenoma and myelolipoma in a patient with Conn syndrome.  Case report. Panminerva Med. 1992;  34 209-212
  • 12 Grumbach MM, Biller BM, Braunstein GD. et al . Management of the clinically inapparent adrenal mass („incidentaloma”).  Ann Intern Med. 2003;  138 424-429
  • 13 Guo Y, Yang Z, Li Y. et al . Uncommon adrenal masses: CT and MRI features with histopathologic correlation.  Eur J Radiol. 2007;  62 359-370
  • 14 Han M, Burnett AL, Fishman EK. et al . The natural history and treatment of adrenal myelolipoma.  J Urol. 1997;  157 1213-1216
  • 15 Hisamatsu H, Sakai H, Tsuda S. et al . Combined adrenal adenoma and myelolipoma in a patient with Cushing's syndrome: case report and review of the literature.  Int.J Urol. 2004;  11 416-418
  • 16 Jaresch S, Kornely E, Kley HK. et al . Adrenal incidentaloma and patients with homozygous or heterozygous congenital adrenal hyperplasia.  J Clin Endocrinol Metab. 1992;  74 685-689
  • 17 Jenkins P, Chew S, Lowe D. et al . Adrenocorticotrophin-independent unilateral macronodular adrenal hyperplasia occuring with myelolipoma: an unusual cause of Cushing's syndrome.  Clin Endocrinol. 1994;  41 827-830
  • 18 Jung SI, Kim SO, Kang TW. et al . Bilateral adrenal myelolipoma associated with hyperaldosteronism: report of a case and review of the literature.  Urology. 2007;  70 1223 , e11–1223.e13
  • 19 Kanj HA, Noronha J, D’Aguillo AF. et al . Bilateral adrenal myelolipomas with Cushing's syndrome.  JAMA. 1988;  259 3034-3036
  • 20 Kasperlik-Zeluska AA, Roslonowska E, Slowinska-Srzednicka J. et al . Incidentally discovered adrenal mass (incidentaloma): investigation and management of 208 patients.  Clin Endocrinol (Oxf). 1997;  46 29-37
  • 21 Kenney PJ, Wagner BJ, Rao P. et al . Myelolipoma: CT and pathologic features.  Radiology. 1998;  208 87-95
  • 22 Kloos RT, Gross MD, Francis IR. et al . Incidentally discovered adrenal masses.  Endocr Rev. 1995;  16 460-484
  • 23 Lam KY, Lo CY. Adrenal lipomatous tumours: a 30 year clinicopathological experience at a single institution.  J Clin Pathol. 2001;  54 707-712
  • 24 Lazurova I, Sokol L, Trejbal D. et al . Aldosterone-producing adenoma associated with foci of myelolipoma.  Wien Klin Wochenschr. 1998;  110 379-381
  • 25 Liao CH, Chueh SC, Lai MK. et al . Laparoscopic adrenalectomy for potentially malignant adrenal tumors greater than 5 centimeters.  J Clin Endocrinol Metab. 2006;  91 3080-3083
  • 26 Mansmann G, Lau J, Balk E. et al . The clinically inapparent adrenal mass: update in diagnosis and management.  Endocr Rev. 2004;  25 309-340
  • 27 Mantero F, Terzolo M, Arnaldi G. et al . A survey on adrenal incidentaloma in Italy.  Study Group on Adrenal Tumors of the Italian Society of Endocrinology. J Clin Endocrinol Metab. 2000;  85 637-644
  • 28 Maschler I, Rosenmann E, Ehrenfeld E. Ectopic functioning adrenocortico-myelolipoma in longstanding NelsonŽs syndrome.  Clin Endocrinol. 1979;  10 493-497
  • 29 Matsuda T, Abe H, Takase M. et al . Case of combined adrenal cortical adenoma and myelolipoma.  Pathol Int. 2004;  54 725-729
  • 30 Meyer A, Behrend M. Presentation and therapy of myelolipoma.  Int J Urol. 2005;  12 239-243
  • 31 Olsson CA, Krane RJ, Klugo RC. et al . Adrenal myelolipoma.  Surgery. 1973;  73 665-670
  • 32 Rockall AG, Babar SA, Sohaib SA. et al . CT and MR imaging of the adrenal glands in ACTH-independent Cushing syndrome.  Radiographics. 2004;  24 435-452
  • 33 Sakaki M, Izaki H, Fukumori T. et al . Bilateral adrenal myelolipoma associated with adrenogenital syndrome.  Int J Urol. 2006;  13 801-802
  • 34 Sasano H, Masuda T, Ojima M. et al . Congenital 17 alpha-hydroxylase deficiency: a clinicopathologic study.  Hum Pathol. 1987;  18 1002-1007
  • 35 Schaeffer EM, Kavoussi LR. Adrenal myelolipoma.  J Urol. 2005;  173 1760
  • 36 Ukimura O, Inui E, Ochiai A. et al . Combined adrenal myelolipoma and pheochromocytoma.  J Urol. 1995;  154 1470
  • 37 Umpierrez MB, Fackler S, Umpierrez GE. et al . Adrenal myelolipoma associated with endocrine dysfunction: review of the literature.  Am J Med Sci. 1997;  314 338-341
  • 38 Vrezas I, Wentworth P, Borstein S. Myelolipomatous foci in an adrenal adenoma causing Cushing's syndrome?.  Endocr Res. 2003;  29 67-71
  • 39 Vyberg M, Sestoft L. Combined adrenal myelolipoma and adenoma associated with Cushing's syndrome.  Am J Clin Pathol. 1986;  86 541-545
  • 40 Wagnerova H, Lazurova I, Bober J. et al . Adrenal myelolipoma. 6 cases and a review of the literature.  Neoplasma. 2004;  51 300-305
  • 41 Whaley D, Becker S, Presbrey T. et al . Adrenal myelolipoma associated with Conn syndrome: CT evaluation.  J Comput Assist Tomogr. 1985;  9 959-960
  • 42 Young  WF Jr. Clinical practice.  The incidentally discovered adrenal mass. N Engl J Med. 2007;  356 601-610

Correspondence

C. Lamas

Department of Endocrinology

Complejo Hospitalario Universitario de Albacete

c/Hermanos Falcó

37. 02006 Albacete

Spain

Phone: +34/967/59 74 42

Phone: 34 647 94 22 26

Fax: +34/967/59 71 41

Email: clamaso@sescam.jccm.es

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