Adrenal Myelolipomas in Patients with Congenital Adrenal Hyperplasia: Review of the Literature and A Case Report |
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| Institution: | 1. Section of Endocrinology and Metabolism, Department of Internal Medicine, Louisiana State University Health Sciences Center.;2. Department of Surgery, Division of Transplantation, Louisiana State University Health Sciences Center, Shreveport, Louisiana.;1. Department of Radiology, University of Pennsylvania, 3400 Spruce Street, Philadelphia, Pennsylvania 19104;2. Department of Orthopaedic Surgery, University of Pennsylvania, Philadelphia, Pennsylvania 19104;3. Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104;4. Department of Genetics, University of Pennsylvania, Philadelphia, Pennsylvania 19104;5. The Center for Research in FOP and Related Disorders, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104;6. Department of Radiology, NYU Langone Medical Center, New York, New York;7. Division of Geriatric Medicine & Gerontology, Mayo Clinic College of Medicine, Rochester, Minnesota;1. Department of Public Health, Texas Tech University Health Sciences Center, Lubbock, Texas;2. Division of Endocrinology, Metabolism and Diabetes. University of Louisville, Louisville, Kentucky;1. Department of Endocrinology, Metabolism and Internal Medicine, Poznań University of Medical Sciences, Poznań, Poland;2. Department of Clinical Pathology, Poznań University of Medical Sciences, Poznań, Poland;1. American Memorial Hospital, Pediatric Surgery Unit, 51100 Reims France;2. Hopital Gatien de Clocheville, Pediatric Surgery Unit, 37000 Tours France;3. CHRU Anger, Pediatric Surgery Unit, 49000 Angers France;1. Department of Obstetrics and Gynecology, Division of Reproductive Endocrinology and Infertility, McGill University, Montréal Quebec H3A 0G4, Canada;2. McGill University Health Center, 1001 Decarie Boulevard, Room D05.2519, Montreal QC H4A 3J1;3. Faculty of Medicine, McGill University, Montréal Quebec H3G 2M1, Canada;4. Division of Maternal-Fetal Medicine, Obstetrics & Gynecology Department, University of Toronto, McGill University, Montreal, QC, Canada;5. MUHC Reproductive Centre, McGill University, Montréal Quebec H2L 4S8, Canada |
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| Abstract: | ObjectiveTo review the association between congenital adrenal hyperplasia (CAH) and adrenal myelolipomas and report a case of bilateral, giant adrenal myelolipomas in a patient with untreated CAH due to 21-hydroxylase deficiency.MethodsWe describe the patient’s clinical presentation, imaging findings, and laboratory test results and review the relevant English-language literature concerning patients with both CAH and myelolipomas.ResultsA 45-year-old man with untreated CAH due to 21-hydroxylase deficiency presented with increasing abdominal girth and abdominal pain. Computed tomography of the abdomen demonstrated very low-density adrenal masses (22 × 11 cm on the left side and 6 × 5.5-cm on the right side) consistent with adrenal myelolipomas. The left adrenal myelolipoma was resected (24.4 × 19.0 × 9.5 cm; 2557 g). The mass was composed of mature adipose tissue with areas of hematopoietic cells of myeloid, erythroid, and megakaryocytic cell lines. Islands of adrenal cortical cells were scattered between the adipose and hematopoietic tissue. Including the present case, we identified 31 patients with both CAH and myelolipomas who have been described in the English-language literature. The details of these cases were reviewed.ConclusionsPersons with CAH may be at increased risk of developing adrenal myelolipomas, particularly if their CAH is poorly controlled. How and whether chronic exposure of the adrenal glands to high corticotropin levels increases the risk of developing myelolipomas remains a matter of speculation. (Endocr Pract. 2011;17:441-447) |
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