Cushing's syndrome caused by mixed adrenal tumor of cortical origin associated with myelolipoma

2017 ◽  
Vol 148 (6) ◽  
pp. 285-286
Author(s):  
Andreu Simó-Servat ◽  
Manuel Pérez Maraver ◽  
Jordi Caballero Corchuelo



PEDIATRICS ◽  
1970 ◽  
Vol 46 (2) ◽  
pp. 217-229
Author(s):  
Michel G. Gilbert ◽  
William W. Cleveland

Cushing's syndrome in infancy is usually due to adrenal tumor which is often malignant. Three cases are described in which the disorder was due to adenoma and surgical cure was achieved. The clinical picture is strongly suggestive if not diagnostic. Its hallmark is generalized obesity with typical facies; also present are hypertension and signs of virilization. Striae were not found in our patients. Increased excretion of both 17-hydroxysteroids and 17-ketosteroids is characteristic; this excretion was not suppressed by dexamethasone in two of these patients in whom it was tested. Intravenous pyelogram was distinctly abnormal in all cases and indicated the presence and location of the tumor. The diagnosis can be made efficiently and surgical treatment can be promptly instituted with reasonable expectation of cure. Careful attention must be paid to preoperative and postoperative administration of adrenal steroids.



2000 ◽  
Vol 14 (1) ◽  
pp. 50-54 ◽  
Author(s):  
M. J. M. Bugalho ◽  
X. Li ◽  
Ch. V. Rao ◽  
J. Soares ◽  
L. G. Sobrinho




2016 ◽  
Vol 63 (4) ◽  
pp. 334-337
Author(s):  
Ana Valea ◽  
◽  
Dan Nicolae Paduraru ◽  
Adriana Elena Nica ◽  
Maria Iuliana Oprisor ◽  
...  

Introduction. Scleroderma induces heterogeneous skin changes due to collagen anomalies, including facial. The recommended therapy is, among others, topic cortisol derived products, so an issue of diagnosis differential regarding the etiological type of Cushing’s syndrome (CS) is raised if a patient develops a suggestive phenotype. Case presentation. This is a 64-year female presenting (since the last few months) red face, high blood pressure, central obesity. She has a 6-year history of scleroderma, intermittently treated with local corticotherapy. On admission, she associated metabolic disturbances as lipid profile anomalies, high uric acid, insulin resistance and increased number of white blood cells (which were not connected with a relapse of scleroderma, neither to an inflammatory syndrome). Low baseline plasma ACTH and morning serum cortisol level (not high, yet detectable) with abnormal diurnal rhythm suggested an adrenal source of CS. A 3 cm right adrenal tumor was found on computer tomography and later removed through a classical intervention. Two weeks after, the patient voluntarily reduced her prednisone dose causing an adrenal crisis which required re-admission as an emergency. After discharge, daily oral adrenal replacement therapy is needed for the moment. Conclusions. Scleroderma, especially with skin involvement, and some of associated therapies may mask an endogenous CS as adrenal tumor derivate, thus delaying the adequate diagnosis and therapy. Patient’s education regarding a potential life threatening condition as chronic adrenal insufficiency is necessary since post-operatory recovery of adrenal function is expected within 6 to 24 months.







2017 ◽  
Vol 177 (5) ◽  
pp. K13-K20 ◽  
Author(s):  
C E Andreescu ◽  
R A Alwani ◽  
J Hofland ◽  
L H J Looijenga ◽  
W W de Herder ◽  
...  

Cushing syndrome (CS) during pregnancy is a rare condition with only a few cases reported in the literature. Misdiagnosis of CS is common because of overlapping features like fatigue, weight gain, striae and emotional changes that can occur during normal pregnancy. Changes in maternal hormones and their binding proteins complicate assessment of glucocorticoid hormone levels during gestation. CS during pregnancy is most frequently due to an adrenal adenoma and to a lesser degree to adrenocorticotropic hormone (ACTH) hypersecretion by a pituitary adenoma. Furthermore, aberrant expression of luteinizing hormone (LH) receptors in the adrenal cortex has been suggested to be involved in the pathogenesis of adrenal CS during pregnancy. We report three pregnant women with ACTH-independent Cushing’s syndrome and an adrenal tumor. After uncomplicated delivery, patient 1 underwent in vivo testing for aberrant hormone receptor expression by the adenoma. Cortisol responses were found after administration of luteinizing hormone-releasing hormone (LHRH), human chorionic gonadotropin (hCG), glucagon, vasopressin and a standard mixed meal. All patients were treated with laparoscopic adrenalectomy. Adrenal tumor tissue of two patients showed positive immunohistochemical staining of LH receptors. Considering the cortisol responses to LHRH and hCG, and the development of CS during pregnancy in these patients, it is likely that ACTH-independent hypercortisolism was induced by the pregnancy-associated rise in hCG levels that activated aberrantly expressed LH receptors in the adrenal adenoma. Remarkably, adrenal adenomas may simultaneously express multiple aberrant receptors and individual ligands may play a role in the regulation of cortisol production in CS during pregnancy.



eLife ◽  
2021 ◽  
Vol 10 ◽  
Author(s):  
Xuebin Zhang ◽  
Penghu Lian ◽  
Mingming Su ◽  
Zhigang Ji ◽  
Jianhua Deng ◽  
...  

Ectopic Cushing's syndrome due to ectopic ACTH&CRH-secreting by pheochromocytoma is extremely rare and can be fatal if not properly diagnosed. It remains unclear whether a unique cell type is responsible for multiple hormones secreting. In this work, we performed single-cell RNA sequencing to 3 different anatomic tumor tissues and 1 peritumoral tissue based on a rare case with ectopic ACTH&CRH-secreting pheochromocytoma. And in addition to that, 3 adrenal tumor specimens from common pheochromocytoma and adrenocortical adenomas were also involved in the comparison of tumor cellular heterogeneity. A total of 16 cell types in the tumor microenvironment were identified by unbiased cell clustering of single-cell transcriptomic profiles from all specimens. Notably, we identified a novel multi-functionally chromaffin-like cell type with high expression of both POMC (the precursor of ACTH) and CRH, called ACTH+&CRH+ pheochromocyte. We hypothesized that the molecular mechanism of the rare case harbor Cushing's syndrome is due to the identified novel tumor cell type, that is, the secretion of ACTH had a direct effect on the adrenal gland to produce cortisol, while the secretion of CRH can indirectly stimulate the secretion of ACTH from the anterior pituitary. Besides, a new potential marker (GAL) co-expressed with ACTH and CRH might be involved in the regulation of ACTH secretion. The immunohistochemistry results confirmed its multi-functionally chromaffin-like properties with positive staining for CRH, POMC, ACTH, GAL, TH and CgA. Our findings also proved to some extent the heterogeneity of endothelial and immune microenvironment in different adrenal tumor subtypes.



2015 ◽  
Vol 2 (3) ◽  
pp. 182-184
Author(s):  
Puskal Kumar Bagchi ◽  
Somor Jyoti Bora ◽  
Sasanka Kumar Barua ◽  
Rajeev Thekumpadam Puthenveetil


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