Corticomedullary mixed tumor of the adrenal gland

Ajmi, S.; Chaïeb, M.; Mokni, Moncef; Braham, Rafik; Ach, K.; Maaroufi, A.; Chaieb, L.

doi:10.1016/j.ando.2009.09.003

Cited by 16 publications

(10 citation statements)

References 8 publications

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“…Although only a few previous studies have examined the tumorigenesis of CMMT, several hypotheses have been proposed. For example, it has been hypothesized that ACTH or growth factors derived from pheochromocytoma components might cause ectopic adrenocortical neoplasia [ 28 ]. In the present case, ACTH expression was not detected in pheochromocytoma cells.…”

Section: Discussionmentioning

confidence: 99%

Adrenal Corticomedullary Mixed Tumor Associated With the FGFR4-G388R Variant

Kanzawa

Fukuoka

Yamamoto

et al. 2020

Journal of the Endocrine Society

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Adrenal corticomedullary mixed tumors (CMMTs) are extremely rare: with only 20 cases being reported to date the pathogenesis has remained elusive. A 31-year-old woman developed gestational hypertension with psychiatric disturbances persistent to postpartum, diagnosed with pheochromocytoma, for which adrenalectomy was performed. Histological findings showed mixed adrenocortical adenoma and pheochromocytoma. Double immunostaining of inhibin and INSM1 (insulinoma-associated protein 1) showed that the two tumor components had distinct functional properties. Exome analysis of peripheral leukocytes and tumor (singular as anatomically it is only one mass) revealed a homozygous germline FGFR4-G388R variant. As a readout of the variant, serine phosphorylation of signal transducer and activator of transcription 3 (STAT3) was detected only in the nucleus of adrenocortical adenoma component but not in the pheochromocytoma component. No tyrosine phosphorylation of STAT3 was detected. We report a case of CMMT with the germline FGFR4-G388R variant. Although additional studies are required, our immunohistochemical analysis suggests that the variant may play a role in the development of the adrenocortical component within the pheochromocytoma, leading to CMMT.

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Section: Discussionmentioning

confidence: 99%

Adrenal Corticomedullary Mixed Tumor Associated With the FGFR4-G388R Variant

Kanzawa

Fukuoka

Yamamoto

et al. 2020

Journal of the Endocrine Society

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“…Hypersecretion of cortical and medullary lesions causes 40% of diabetes cases, 80% of hypertension cases, and 53.33% of Cushing’s syndrome cases [ 20 ]. The typical clinical manifestations are easy to identify, however, the lack of specific clinical manifestations in many cases leads to them often being discovered accidentally, and ultimately a pathological diagnosis is required [ 13 ]. Cases similar to that of our patient may manifest with subclinical Cushing’s syndrome, including cortisol rhythm abnormalities, but no typical Cushing’s syndrome appearance [ 11 ].…”

Section: Discussionmentioning

confidence: 99%

“…The following mechanisms mainly have been proposed: Firstly, pheochromocytomas secreted ectopic corticotropin, which might caused adrenal cortical hyperplasia and/or adrenal cortical adenoma. Moreover, hyper-secreted catecholamines stimulated the anterior pituitary to secrete corticotropin, which may lead to similar adrenal cortical lesions [ 13 ]. Additionally, key enzymes of catecholamine synthesis, such as beta hydroxylase, tyrosine hydroxylase, and phenylethanolamine-N-methyltransferase might be regulated by high concentrations of glucocorticoid via the intra-adrenal portal system.…”

Section: Discussionmentioning

confidence: 99%

Corticomedullary mixed tumour resembling a small adrenal gland-involvement of cancer stem cells: case report

Duan

Fang

et al. 2017

BMC Endocr Disord

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BackgroundAdrenal corticomedullary mixed tumours are very rare. Its mechanism is rarely reported. Here we report the first case of a corticomedullary mixed tumour resembling a “small adrenal gland” with distinct arrangement of the cortical and medullary layers. We further hypothesize regarding the tumorigenic mechanism of this tumour.Case presentationA 58-year man had been diagnosed with diabetes and hypertension for 3 years. His 24-h urine vanillylmandelic acid (VMA) levels were slightly elevated. An abnormal circadian cortisol rhythm was noted, and his cortisol levels were not suppressed by dexamethasone. Abdominal computed tomography (CT) revealed a right adrenal gland lesion (diameter, 30 × 38 mm), while an enhanced CT showed enhancement and hypervascularization. The tumour was positive for adrenocorticotropic hormone, chromogranin A (CGA), and steroidogenic factor-1 (SF-1) on the tumour surface. Acetaldehyde dehydrogenase 1(ALDH1), CD44, CD133, Nestin, Nerve growth factor receptor (NGFR), and Sex determining region y-box 9(SOX9) staining were positive. Although administration of medications for diabetes and hypertension was stopped until surgery was performed, the blood sugar level and blood pressure were maintained after surgery.ConclusionsThis is the first report about a possible mechanism by which cancer stem cells induce adrenal corticomedullary tumours.

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“…Boyanton et al [2] also presented a man with an adrenal tumor and high catecholamine levels but the histopathologic examination revealed mature adipocytes and focal hematopoietic elements with normal com-pressed adrenal tissue. Finally, Ajmi et al [3] reported another patient with an adrenal mass and elevated cortisol and androgen levels; however, mixed components of fat and adrenal tissue were only seen on the pathological specimen.…”

Section: Introductionmentioning

confidence: 99%

Coexistence of the Hypersecretion of Catecholamine, Adrenal Cortical Nodular Hyperplasia, and Nephrotic-Range Proteinuria of Focal Segmental Scleronephrosis: Is It Fat-Induced Hypertension?

Lương¹,

Nguyễn²,

Nguyen³

et al. 2011

IJCM

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Two patients had a long history of hypertension and one of them also had a nephrotic-range proteinuria secondary to focal segmental scleronephrosis which had been diagnosed by kidney biopsy. They presented with uncontrolled hypertension. Laboratory examination suggested hypersecretion of catecholamines by the left adrenal glands in both patients and primary aldosteronism in one of them. A computed tomography scan revealed small nodules on left adrenal gland in both patients. Patients underwent laparoscopic left adrenalectomy. After surgery, blood pressure was normalized and proteinuria was resolved. Most interestingly, prominent adipocytes infiltrated were detected in the adrenal cortex and were associated with the presence of lymphocytes, which suggested that adipocytes might have a role in the pathogenesis of these diseases in our patients

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Corticomedullary mixed tumor of the adrenal gland

Cited by 16 publications

References 8 publications

Adrenal Corticomedullary Mixed Tumor Associated With the FGFR4-G388R Variant

Adrenal Corticomedullary Mixed Tumor Associated With the FGFR4-G388R Variant

Corticomedullary mixed tumour resembling a small adrenal gland-involvement of cancer stem cells: case report

Coexistence of the Hypersecretion of Catecholamine, Adrenal Cortical Nodular Hyperplasia, and Nephrotic-Range Proteinuria of Focal Segmental Scleronephrosis: Is It Fat-Induced Hypertension?

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