Cushing's syndrome caused by giant Ewing's sarcoma of the kidney:A case report and review of literature

BACKGROUND Primary renal Ewing’s sarcoma(ES)is extremely rare,and only two cases causing Cushing’s syndrome(CS)have been reported to date.We report that the case of an 18-year-old patient is diagnosed primary renal ES with typical CS characterized by purple stripes,weight gain,and hypertension.CASE SUMMARY CS was first diagnosed by laboratory testing.A huge tumor was revealed in the kidney following an imaging examination.Moreover,brain and bone metastases were observed.After comprehensive treatment,primarily based on surgery,primary renal ES was pathologically diagnosed with a typical EWSR1-FLI1 genetic mutation through genetic testing.Furthermore,the glucocorticoid level returned to normal.By the ninth postoperative month of follow-up,the patient was recovering well.Cushing-related symptoms had improved,and a satisfactory curative effect was achieved.CONCLUSION Primary renal ES,a rare adult malignant tumor,can cause CS and a poor prognosis.

Primary aldosteronism due to bilateral micronodular hyperplasia and concomitant subclinical Cushing’s syndrome:A case report

BACKGROUND Adrenal incidentaloma(AI)has been frequently encountered in the clinical setting.It has been shown that primary aldosteronism(PA)or subclinical Cushing’s syndrome(SCS)are the representative causative diseases of AI.However,the coexistence of PA and SCS has been reportedly observed.Recently,we encountered a case of AI,in which PA and SCS coexisted,confirmed by histopathological examinations after a laparoscopic adrenalectomy.We believe that there were some clinical implications in the diagnosis of the present case.CASE SUMMARY A 58-year-old man presented with lower right abdominal pain with a blood pressure of 170/100 mmHg.A subsequent computed tomography scan revealed right ureterolithiasis,which was the cause of right abdominal pain,and right AI measuring 22 mm×25 mm.After the disappearance of right abdominal pain,subsequent endocrinological examinations were performed.Aldosterone-related evaluations,including adrenal venous sampling,revealed the presence of bilateral PA.In addition,several cortisol-related evaluations showed the presence of SCS on the right adrenal adenoma.A laparoscopic right adrenalectomy was then performed.The histopathological examination of the resected right adrenal revealed the presence of a cortisol-producing adenoma,while CYP11B2 immunoreactivity was absent in this adenoma.However,in the adjacent nonneoplastic adrenal,multiple CYP11B2-positive adrenocortical micronodules were detected,showing the presence of aldosterone-producing adrenocortical micronodules.CONCLUSION Careful clinical and pathological examination should be performed when a patient harboring AI presents with concomitant SCS and PA.

Metabolic profile differences in ACTH-dependent and ACTH-independent Cushing syndrome

Background:The most common etiologies of Cushing’’s syndrome(CS)are adrenocorticotropic hormone(ACTH)-producing pituitary adenoma(pitCS)and primary adrenal gland disease(adrCS),both of which burden patients with metabolic disturbance.The aim of this study was to compare the metabolic features of pitCS and adrCS patients.Methods:A retrospective review including 114 patients(64 adrCS and 50 pitCS)diagnosed with CS in 2009-2019 was performed.Metabolic factors were then compared between pitCS and adrCS groups.Results:Regarding sex,females suffered both adrCs(92.2%)and pitCS(88.0%)more frequently than males.Regarding age,patients with pitCS were diagnosed at a younger age(35.40±11.94 vs.39.65±11.37 years,p=0.056)than those with adrCS,although the difference was not statistically significant.Moreover,pitCS patients had much higher ACTH levels and more serious occurrences of hypercortisolemia at all time points(8 AM,4 PM,12 AM)than that in adrCS patients.Conversely,indexes,including body weight,BMI,blood pressure,serum total cholesterol,low density lipoprotein cholesterol(LDL-C),high density lipoprotein cholesterol(HDL-C),triglycerides,fasting plasma glucose,and uric acid,showed no differences between adrCS and pitCS patients.Furthermore,diabetes prevalence was higher in pitCS patients than in adrCS patients;however,there were no significant differences in hypertension or dyslipidemia prevalence between the two.Conclusions:Although adrCS and pitCS had different pathogenetic mechanisms,different severities of hypercortisolemia,and different diabetes prevalences,both etiologies had similar metabolic characteristics.

Giant adrenal tumor presenting as Cushing’s syndrome and pheochromocytoma:A case report

We report a case of a 35-year-old lady who presented with Cushingoid features and associated raised urinary metanephrine.The patient underwent open adrenelectomy.Histopathological examination revealed adreno-cortical carcinoma with microscopic lymphovascular invasion.Postoperative period was uneventful and is on follow-up for the last one year and is doing well.

Clinical,Biochemical,and Radiological Retrospective Analysis in Patients with Adrenal Incidentaloma-A Secondary Publication

Objective:To evaluate the epidemiological,demographic,clinical features,treatment approaches,and survival of patients followed up for adrenal incidentaloma.Methods:Data from 46 patients who were treated and followed up due to adrenal incidentaloma in the Endocrinology Department of Mersin University Health Research and Application Hospital between 2010 and 2014 were retrospectively analyzed.Results:Of the cases included in the study,13 were male,33 were female,and the mean age was 54.09±10.7 years.The most common reason for admission was abdominal pain in 34.78%of the patients,the most commonly diagnosed radiological method was dynamic adrenal CT in 60.87%,and the most common location was the left adrenal gland.The mean lesion diameter was between 26.8±16.5 mm.The frequency of hypertension was 50%,obesity 47.8%,type 2 diabetes 21.7%,osteoporosis 42.8%,and metabolic syndrome 41.3%.According to hormonal evaluation results,non-functional adrenal adenoma(NFAA)was found in 82.61%,subclinical Cushing’s syndrome(SCS)in 15.21%,and aldosteronoma in 2.1%.Myelolipoma,pheochromocytoma,and adrenocortical adenoma were diagnosed in 8 cases undergoing adrenalectomy.One patient died due to liver failure.No hormonal activation or growth in lesion size was detected during the follow-up of the patients.Conclusion:Due to the very different pathological and radiological appearances of adrenal incidentaloma,it is important to evaluate demographic,etiological,clinical,laboratory,and radiological data as a whole in the treatment and follow-up.

Bilateral inferior petrosal sinus sampling for the treatment of Cushing's disease Data from 52 cases from one institute over an eight-year period

The present study analyzed data from 108 Cushing＇s disease patients, who underwent transsphenoidal surgery in one hospital between January 2003 and April 2010, to investigate the performance value of bilateral inferior petrosal sinus sampling （BIPSS）. Of the 108 patients 52 underwent BIPSS prior to surgery and 56 did not. Results showed that BIPSS accuracy for the diagnosis of Cushing＇s disease was 86.5% （45/52）, and accuracy for adenoma lateralization during surgery was 76.9% （40/52）. In addition, early remission rate was significantly greater compared to patients without BIPSS. Results demonstrated that BIPSS facilitated localization and diagnosis of Cushing＇s disease and helped to predict adenoma lateralization.

Electrocardiographic Manifestations of Endocrine and Metabolic Disorders

Endocrine dysfunction has an adverse impact on the cardiovascular system that may be due to an endocrine abnormality that leads to electrocardiogram (EKG) changes. The EKG changes due to endocrine disorder can be reversible and irreversible and treating underlying disease can reverse EKG changes in some cases. In this article, we review the electrocardiogram manifestations of various endocrine disorders.

Carcinoembryonic antigen-producing adrenal adenoma resected using combined lateral and anterior transperitoneal laparoscopic surgery

A 74-year-old woman presented with symptoms consistent with hyperadrenocorticism and hyperca- techolaminism. She had a cushingoid appearance and her cortisol level was elevated. Her serum dopamine and noradrenalin levels were also elevated. Computed tomography detected a left adrenal mass measuring 3.5 cm x 3.0 cm in diameter. Metaiodobenzylguanidine scintigraphy was negative. Unexpectedly, the serum Serum carcinoembryonic antigen （CEA） level waselevated. Fluorodeoxyglucose positron emission tomography showed increased uptake in the adrenal tumor only, with a maximum standardized uptake value of 2.8. Selective venography and blood sampling revealed that the concentrations of cortisol, catecholamines and CEA were significantly elevated in the vein draining the tumor. A diagnosis of CEA-producing benign adenoma was made. After preoperative management, we performed a combined lateral and anterior transpedtoneal laparoscopic adrenectomy. Her vital signs remained stable during surgery. Histopathological examination revealed a benign adenoma. Her cortisol, catecholamine and CEA levels normalized immediately after surgery. We present, to the best of our knowledge, the first case of CEA-producing adrenal adenoma, along with a review of the relevant literature, and discuss our laparoscopic surgery techniques.

A Small Solitary Pulmonary Nodule Discovered by ^(18)F-fluorodeoxyglucose Positron Emission Tomography and CT: Rare Infection Instead of Rare Tumor

THE causes of Cushing＇s syndrome are mainly di- vided into adrenocorticotropic hormone （ACTH） dependent and independent. ACTH dependent hypercortisolism represents excess ACTH se-creting by the pituitary or tumor outside the pituitary; and the latter one is also called as ectopic ACTH syndrome. Thorax is the most common location of causative lesions for ectopic ACTH syndrome, and the size of lesion is too small to be detected in some cases.1＇ 2 Cryptococcal pneumonia usually occurs in immunocompromised patients and excess cortisol production can theoretically produce a state of immunodeficiency. Development of cryptococcal pneumonia concomitant with Cushing syndrome （CS） was rare. Here, we report a case of pulmonary nodule in a patient with CS differentiated with ectopic ACTH-producing tumor. Crypto- coccal pneumonia was diagnosed followinglung resection.

Cushing＇s syndrome during pregnancy caused by adrenal cortical adenoma： a case report and literature review

Cushing＇s syndrome （CS） during pregnancy is a rare condition with Significant maternal and fetal complications. A case of CS during the third trimester of pregnancy secondary to adrenocortical adenoma was reported. Literature review revealed the disadvantages of different treatments in this period. Besides the conservative treatment, surgery is recommended for CS during the third trimester of pregnancy secondary to adrenal adenoma, if an experienced surgeon is available.

Characteristics of ectopic adrenocorticotropic hormone syndrome due to thymic carcinoid and analysis of the molecular mechanism

Ectopic adrenocorticotropic hormone （ACTH） secretion rfrom a non-pituitary tumor is a main cause of Cushing＇s syndrome, which is associated with significant morbidity and mortality. Although early studies showed that the predominant cause of ectopic Cushing＇s syndrome is small cell carcinomas of the lungs, the incidence of carcinoid, including thymic carcinoid, has increased substantially in recent surveys） This evidence suggested that the clinical syndrome of ectopic ACTH production only occurs in tumors with high proopiomelanocortin （POMC, the precursor to ACTH） transcripts generated from the start of the pituitary transcription initiation site.2

Adrenocorticotropic hormone-producing pheochromocytoma： a case report and review of the literature

Ectopic Cushing＇s syndrome caused by pheochromocytoma is rare. We reported a 15-year-old female patient who was admitted to hospital with typical Cushing＇s syndrome. She had not started menstruation. Her plasma adrenocorticotropic hormone （ACTH） and 24-hour urinary free cortisol levels were extremely high. Gonadal and progestational hormone levels were also abnormal. Abdominal computed tomography scans and enhanced scans revealed multiple irregular tumors in the right adrenal. Pelvic echogram showed an infantile uterus, while the ovaries were at an immature stage of development. Retroperitoneal laparoscopic right adrenalectomy was performed without intraoperative complications. Histology and immunohistochemistry of the tumor were consistent with pheochromocytoma. Retroperitoneal laparoscopic adrenalectomy is a safe procedure with satisfactory outcomes and allows for rapid recovery.

Pituitary-dependent hyperadrenocorticism in a terrier dog:A case report

We report a case of pituitary-dependent hyperadrenocorticism in a 10-year-old,female,terrier dog.The animal was admitted due to polyphagia,weight gain,polyuria,polydipsia,hair loss,exercise intolerance and panting at rest.On physical examination,abdominal distention,truncal and bilaterally symmetric alopecia,thin hypotonic skin,comedones,bruising,hyperpigmentation and calcinosis cutis on the dorsal midline were observed.Hematologic investigations showed stress leukogram,high serum alkaline phosphatase activity,mild to moderate alanine aminotransferase activity,hypercholesterolemia,hypertriglyceridemia and hyperglycemia.Mild generalized interstitial lung patterns and hepatomegaly were detected in the radiographs.Bilaterally symmetric normal-sized adrenal glands were also diagnosed in ultrasonography.Diagnosis of pituitary-dependent hyperadrenocorticism was confirmed with low-dose dexamethasone suppression test.The dog was successfully treated with mitotane.