Ectopic Functioning Adrenocortico‐myelolipoma in Longstanding Nelson's Syndrome

Maschler, Itzhak; Rosenmann, E.; Ehrenfeld, E. N.

doi:10.1111/j.1365-2265.1979.tb02106.x

Cited by 24 publications

(11 citation statements)

References 7 publications

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“…A growing body of evidence indicates that ACTH has a role of ACTH in the development of adrenal tumors. A review of the literature reveals the relative frequency of myelolipomas in patients with excessive ACTH secretion, such as in congenital adrenal hyperplasia [3,4,9,25], Nelson's syndrome [18,21], and Addison's disease [2]. To date, approximately 20 cases of myelolipoma with congenital adrenal hyperplasia have been reported, approximately half of which are bilateral, and the left side tumor is usually larger than the right side tumor, probably due to anatomic features (Table 1) [3][4][5][6][7][8][9][10][11][12][13].…”

Section: Discussionmentioning

confidence: 99%

“…Adrenal myelolipomas sometimes arise in patients with endocrine disorders that cause adrenocorticotropic hormone (ACTH) hypersecretion, such as congenital adrenal hyperplasia. Although the pathogenesis of myelolipoma is unclear, prolonged stimulation of the adrenal cortex with high levels of ACTH or subsequent adrenal androgens may have a causative role in myelolipoma associated with congenital adrenal hyperplasia [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17], Cushing's disease [18][19][20], Nelson's syndrome [21], and Addison's disease [2]. In this paper, we report a case of myelolipoma in a patient with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, whose glucocorticoid replacement therapy was poorly controlled.…”

Section: Introductionmentioning

confidence: 99%

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Lack of ACTH and Androgen Receptor Expression in a Giant Adrenal Myelolipoma Associated with 21-hydroxylase Deficiency

et al. 2008

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Myelolipomas of the adrenal gland are benign, nonfunctioning tumors. Patients with congenital adrenal hyperplasia sometimes develop large and bilateral myelolipomas. Although the precise pathogenesis of myelolipomas remains unclear, prolonged stimulation with high levels of adrenocorticotropic hormone (ACTH) or adrenal androgens are assumed to have a causative role. To clarify the role of ACTH and androgen in the pathogenesis of myelolipoma, we report a case of giant adrenal myelolipoma in a patient with poorly controlled congenital adrenal hyperplasia. A 43-year-old female was diagnosed with congenital adrenal hyperplasia at 6 years of age because of ambiguous genitalia. She had high plasma ACTH and 17-hydroxyprogesterone levels. Abdominal computed tomography showed a huge mass on the left adrenal gland, and an enlarged right adrenal mass. Genetic testing for CYP21A2 was performed and revealed that her genotype was IVS2-13A/C>G/I172N. Adrenalectomy for the left-side tumor was performed. Histological study revealed that the tumor consisted of fat cells and myeloid components, findings compatible with adrenal myelolipoma. Neither ACTH receptors nor androgen receptor was over-expressed in the tumor. Our finding that the tumor did not over-express ACTH or androgen receptor suggests a limited direct role for these hormones in the development of the myelolipoma.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Lack of ACTH and Androgen Receptor Expression in a Giant Adrenal Myelolipoma Associated with 21-hydroxylase Deficiency

et al. 2008

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“…Adrenal myelolipoma has also been associated with endocrine disorders, other than congenital adrenal hyperplasia such as Cushing syndrome, and chronic stressful conditions such as obesity, diabetes mellitus, hypertension, chronic inflammatory processes and even malignancy (19,(47)(48)(49)(50)(51)(52)(53)(54). A review by Hisamatsu et al reported 26 cases of myelolipomas associated with endocrine dysfunction (16).…”

Section: Discussionmentioning

confidence: 97%

Bilateral Adrenal Myelolipoma in a 46 XX DSD patient with Congenital Adrenal Hyperplasia due to 21-hydroxylase Deficiency. case report

Sancak¹

2013

Acta Endo (Buc)

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Increased frequency of adrenal tumours and adrenal myelolipoma has been reported in patients with 21-hydroxylase deficiency (21-OHD). Adrenal myelolipoma is an uncommon, benign, biochemically nonfunctioning tumor and occasionally reported in association with endocrine disorders. Diagnosis of myelolipomas is based on imaging with ultrasonography, CT or MRI being effective in more than 90% of cases.We present a 34-year-old man with massive bilateral adrenal masses which was detected on computed tomography and was diagnosed as 21-hydroxylase deficiency (21-OHD) based on biochemical findings. Computerized tomography of the abdomen demonstrated bilaterally very low-density adrenal masses (16x28 mm on the right side and 91x88 and 33x30 mm on the left side) consistent with adrenal myelolipomas.Since myelolipomas are considered as benign tumors, he was not operated. Tumor size did not increase during two year follow-up periods.It is recommended to the physicians to be aware of increased frequency of benign adrenal tumors that occur frequently in patients with 21-OHD. Untreated Congenital Adrenal Hyperplasia CAH with prolonged excessive ACTH stimulation might contribute to the growth of adrenal masses. CAH should always be ruled out in incidentally detected adrenal masses to avoid unnecessary surgical procedures.

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“…Another rare complication of ectopic adrenal tissue is the possible arising of a neoplasm such as neuroblastoma (usually malignant), myelolipomas and oncocytomas [2,5,8,9]. Even testicular tumours are described as presumably originating from adrenal cells [5].…”

Section: Discussionmentioning

confidence: 98%