BACKGROUND Adrenocorticotropic hormone(ACTH)-independent Cushing's syndrome(CS) is mostly due to unilateral tumors, with bilateral tumors rarely reported. Its common causes include primary pigmented nodular adreno...BACKGROUND Adrenocorticotropic hormone(ACTH)-independent Cushing's syndrome(CS) is mostly due to unilateral tumors, with bilateral tumors rarely reported. Its common causes include primary pigmented nodular adrenocortical disease,ACTH-independent macronodular adrenal hyperplasia, and bilateral adrenocortical adenomas(BAAs) or carcinomas. BAAs causing ACTHindependent CS are rare; up to now, fewer than 40 BAA cases have been reported. The accurate diagnosis and evaluation of BAAs are critical for determining optimal treatment options. Adrenal vein sampling(AVS) is a good way to diagnose ACTH-independent CS.CASE SUMMARY A 31-year-old woman had a typical appearance of CS. The oral glucose tolerance test showed impaired glucose tolerance and obviously increased insulin and Cpeptide levels. Her baseline serum cortisol and urine free cortisol were elevated and did not show either a circadian rhythm or suppression with dexamethasone administration. The peripheral 1-deamino-8-D-arginine-vasopressin(DDVAP)stimulation test showed a delay of the peak level, which was 1.05 times as high as the baseline level. Bilateral AVS results suggested the possibility of BAAs.Abdominal computed tomography showed bilateral adrenal adenomas with atrophic adrenal glands(right: 3.1 cm × 2.0 cm × 1.9 cm; left: 2.2 cm × 1.9 cm × 2.1 cm). Magnetic resonance imaging of the pituitary gland demonstrated normal findings. A left adenomectomy by retroperitoneoscopy was performed first,followed by resection of the right-side adrenal mass 3 mo later. Biopsy results of both adenomas showed cortical tumors. Evaluations of ACTH and cortisol showed a significant decrease after left adenomectomy but could still not be suppressed, and the circadian rhythm was absent. Following bilateral adenomectomy, this patient has been administered with prednisone until now,all of her symptoms were alleviated, and she had normal blood pressure without edema in either of her lower extremities.CONCLUSION BAAs causing ACTH-independent CS are rare. AVS is of great significance for obtaining information on the functional state of BAAs before surgery.展开更多
BACKGROUND Ectopic Cushing syndrome(ECS)is a rare condition commonly associated with neuroendocrine tumors(NET),mainly bronchial carcinoids.The association of paraneoplastic syndrome with Merkle cell carcinoma(MCC)is ...BACKGROUND Ectopic Cushing syndrome(ECS)is a rare condition commonly associated with neuroendocrine tumors(NET),mainly bronchial carcinoids.The association of paraneoplastic syndrome with Merkle cell carcinoma(MCC)is limited to individual case reports.CASE SUMMARY In this article we report an unusual and striking presentation of ECS in a patient with known metastatic MCC.An elderly patient presented with new onset severe hypertension,hyperglycemia and hypokalemia,muscle wasting,and peripheral edema.A diagnosis of adrenocorticotropic hormone dependent,non-pituitary,Cushing syndrome was established.Medical therapy inhibiting adrenal function was promptly started but unfortunately the patient survived only a few days after diagnosis.CONCLUSION The occurrence of an aggressive form of ECS in patients with NET should be recognized as an ominous event.To our knowledge,the association of this complication in a patient with MCC had not been reported.展开更多
In order to understand the effect of surgery in the treatment of the bronchial carcinoid tumor and the factors affecting prognosis, 18 cases of bronchial carcinoid tumor are presented, including 5 cases with Cushing&#...In order to understand the effect of surgery in the treatment of the bronchial carcinoid tumor and the factors affecting prognosis, 18 cases of bronchial carcinoid tumor are presented, including 5 cases with Cushing's syndrome. There were lobectomy in 10, lung wedge resection in 3, excision of intraluminal tumor of bronchus in 3, exploratory thoracotomy in 2 cases. No operation death. Pathological examination revealed 14 cases were typical carcinoid tumor and 4 cases were atypical carcinoid tumor. By 2-13 years follow-up . 3 , 5 and 10 years survival rate were 82% , 78%and 70% respectively. Bronchial carcinoid tumor is often confused with small cell carcinoma of lung, the correct diagnosis can be obtained by light microscopic, electron microscopic and immunohistochemistry studies. Those patients accompanied with ectopic-ACTH secretion always have Cushing,s syndrome , resection of tumor can produce gho result. Proper operation method depends on the location of the tumor and patient's extent of cardiac and pulmonary reserve.Atypical carcinoid tumor had high malignancy and poor prognosis. The size of tumor, lymph node involvement and adjuvant therapy seem no definite effect on the patients' survival rate.展开更多
BACKGROUND Ectopic adrenocorticotropic hormone(ACTH)-secreting neuroendocrine tumors are rare diseases.Patients with ACTH-secreting pancreatic neuroendocrine carcinomas have a poor prognosis.Infections and coagulopath...BACKGROUND Ectopic adrenocorticotropic hormone(ACTH)-secreting neuroendocrine tumors are rare diseases.Patients with ACTH-secreting pancreatic neuroendocrine carcinomas have a poor prognosis.Infections and coagulopathies have been reported as the cause of death.However,detailed clinical descriptions of the morbid complications of ACTH-secreting neuroendocrine carcinomas have not been reported.CASE SUMMARY A 78-year-old Japanese woman consulted a medical center due to systemic edema and epigastric discomfort.Laboratory analysis revealed hypercortisolemia with increased ACTH secretion without diurnal variation in serum cortisol level.An enhanced computed tomography(CT)scan revealed a 3-cm tumor in the pancreatic head.The cytological material from endoscopic ultrasound-guided fine-needle aspiration was compatible with ACTHsecreting pancreatic neuroendocrine carcinoma.The Ki-67 index was 40%.She was transferred to Mie University Hospital for surgical treatment.The patient was diagnosed with urinary tract infection,cytomegalovirus hepatitis,esophageal candidiasis,pulmonary infiltrates suspicious for Pneumocystis carinii pneumonia,peripheral deep vein thrombosis,pulmonary embolism,and disseminated intravascular coagulation.The multiple organ infections and thromboses responded well to antimicrobial and anticoagulant therapy.Radioisotope studies disclosed a pancreatic tumor and a metastatic lesion in the liver,whereas somatostatin receptor scintigraphy showed negative findings,suggesting the primary and metastatic tumors were poorly differentiated.A CT scan before admission showed no metastatic liver lesion,suggesting that the pancreatic tumor was rapidly progressing.Instead of surgery,antitumor chemotherapy was indicated.The patient was transferred to another hospital to initiate chemotherapy.However,she died four months later due to the rapidly progressive tumor.CONCLUSION ACTH-secreting pancreatic neuroendocrine neoplasm is a rare disease with a very poor prognosis.The clinical course and acute complications of the tumor remain unreported.Here we report the clinical course of a rapidly progressive case of ACTH-secreting pancreatic neuroendocrine tumor that developed infectious complications due to many types of pathogens in multiple organs,widespread thromboses,pulmonary embolism,and disseminated intravascular coagulation.展开更多
Objective To investigate the methods and efficacy of treatment on severe adrenal Cushing's syndrome.Methods The clinical data of 22 cases with severe adrenal Cushing's syndrome(severe group),and 136 cases with...Objective To investigate the methods and efficacy of treatment on severe adrenal Cushing's syndrome.Methods The clinical data of 22 cases with severe adrenal Cushing's syndrome(severe group),and 136 cases with mild or moderate adrenal Cushing's syndrome(non-severe group)were reviewed.The clinical features were analyzed by comparing the differences between these two groups when patients were admitted to hospital.We discussed the clinical managements of patients with severe adrenal Cushing's syndrome by comparing the differences with non-severe group after preoperative preparation,and with themselves before and after preoperative preparation.The effects of surgery were evaluated by comparing the differences between pre-operation and post-operation on patients with severe adrenal Cushing's syndrome.Results At admission,serum/urine cortisol,disease course,and blood pressure were significantly higher in the severe group than those in non-severe group(P<0.05 or P<0.01),serum potassium and ACTH level were decreased significantly in the severe group than those in non-severe group [(3.01±0.75 vs 3.62±0.48)mmol/L,P<0.01;(6.47±2.91 vs 8.21±3.22)pg/ml,P<0.01].However,no significant difference was observed in diastolic blood pressure,serum potassium,and fasting plasma glucose between these two groups after preoperative preparation(all P>0.05).And then,we performed adrenalectomy.The symptoms of 22 cases with severe adrenal Cushing's syndrome were obviously alleviated after 3 months.During follow-up,5 cases of primary bilateral macronodular adrenal hyperplasia(BMAH)and 1 case of primary pigmented nodular adrenocortical disease(PPNAD)were treated with contralateral adrenalectomy.Conclusion Sufficient preoperative preparation is essential for patients with severe adrenal Cushing's syndrome because of its high level serum cortisol with severe complications.If preparation fails before surgery,cortisol lowering medication or emergency unilateral adrenalectomy is necessary.Severe patients with BMAH and PPNAD were firstly performed unilateral adrenalectomy and followed-up closely,and then,contralateral adrenalectomy is needed when the recurrence of hypercortisolism appeared.展开更多
文摘BACKGROUND Adrenocorticotropic hormone(ACTH)-independent Cushing's syndrome(CS) is mostly due to unilateral tumors, with bilateral tumors rarely reported. Its common causes include primary pigmented nodular adrenocortical disease,ACTH-independent macronodular adrenal hyperplasia, and bilateral adrenocortical adenomas(BAAs) or carcinomas. BAAs causing ACTHindependent CS are rare; up to now, fewer than 40 BAA cases have been reported. The accurate diagnosis and evaluation of BAAs are critical for determining optimal treatment options. Adrenal vein sampling(AVS) is a good way to diagnose ACTH-independent CS.CASE SUMMARY A 31-year-old woman had a typical appearance of CS. The oral glucose tolerance test showed impaired glucose tolerance and obviously increased insulin and Cpeptide levels. Her baseline serum cortisol and urine free cortisol were elevated and did not show either a circadian rhythm or suppression with dexamethasone administration. The peripheral 1-deamino-8-D-arginine-vasopressin(DDVAP)stimulation test showed a delay of the peak level, which was 1.05 times as high as the baseline level. Bilateral AVS results suggested the possibility of BAAs.Abdominal computed tomography showed bilateral adrenal adenomas with atrophic adrenal glands(right: 3.1 cm × 2.0 cm × 1.9 cm; left: 2.2 cm × 1.9 cm × 2.1 cm). Magnetic resonance imaging of the pituitary gland demonstrated normal findings. A left adenomectomy by retroperitoneoscopy was performed first,followed by resection of the right-side adrenal mass 3 mo later. Biopsy results of both adenomas showed cortical tumors. Evaluations of ACTH and cortisol showed a significant decrease after left adenomectomy but could still not be suppressed, and the circadian rhythm was absent. Following bilateral adenomectomy, this patient has been administered with prednisone until now,all of her symptoms were alleviated, and she had normal blood pressure without edema in either of her lower extremities.CONCLUSION BAAs causing ACTH-independent CS are rare. AVS is of great significance for obtaining information on the functional state of BAAs before surgery.
文摘BACKGROUND Ectopic Cushing syndrome(ECS)is a rare condition commonly associated with neuroendocrine tumors(NET),mainly bronchial carcinoids.The association of paraneoplastic syndrome with Merkle cell carcinoma(MCC)is limited to individual case reports.CASE SUMMARY In this article we report an unusual and striking presentation of ECS in a patient with known metastatic MCC.An elderly patient presented with new onset severe hypertension,hyperglycemia and hypokalemia,muscle wasting,and peripheral edema.A diagnosis of adrenocorticotropic hormone dependent,non-pituitary,Cushing syndrome was established.Medical therapy inhibiting adrenal function was promptly started but unfortunately the patient survived only a few days after diagnosis.CONCLUSION The occurrence of an aggressive form of ECS in patients with NET should be recognized as an ominous event.To our knowledge,the association of this complication in a patient with MCC had not been reported.
文摘In order to understand the effect of surgery in the treatment of the bronchial carcinoid tumor and the factors affecting prognosis, 18 cases of bronchial carcinoid tumor are presented, including 5 cases with Cushing's syndrome. There were lobectomy in 10, lung wedge resection in 3, excision of intraluminal tumor of bronchus in 3, exploratory thoracotomy in 2 cases. No operation death. Pathological examination revealed 14 cases were typical carcinoid tumor and 4 cases were atypical carcinoid tumor. By 2-13 years follow-up . 3 , 5 and 10 years survival rate were 82% , 78%and 70% respectively. Bronchial carcinoid tumor is often confused with small cell carcinoma of lung, the correct diagnosis can be obtained by light microscopic, electron microscopic and immunohistochemistry studies. Those patients accompanied with ectopic-ACTH secretion always have Cushing,s syndrome , resection of tumor can produce gho result. Proper operation method depends on the location of the tumor and patient's extent of cardiac and pulmonary reserve.Atypical carcinoid tumor had high malignancy and poor prognosis. The size of tumor, lymph node involvement and adjuvant therapy seem no definite effect on the patients' survival rate.
文摘BACKGROUND Ectopic adrenocorticotropic hormone(ACTH)-secreting neuroendocrine tumors are rare diseases.Patients with ACTH-secreting pancreatic neuroendocrine carcinomas have a poor prognosis.Infections and coagulopathies have been reported as the cause of death.However,detailed clinical descriptions of the morbid complications of ACTH-secreting neuroendocrine carcinomas have not been reported.CASE SUMMARY A 78-year-old Japanese woman consulted a medical center due to systemic edema and epigastric discomfort.Laboratory analysis revealed hypercortisolemia with increased ACTH secretion without diurnal variation in serum cortisol level.An enhanced computed tomography(CT)scan revealed a 3-cm tumor in the pancreatic head.The cytological material from endoscopic ultrasound-guided fine-needle aspiration was compatible with ACTHsecreting pancreatic neuroendocrine carcinoma.The Ki-67 index was 40%.She was transferred to Mie University Hospital for surgical treatment.The patient was diagnosed with urinary tract infection,cytomegalovirus hepatitis,esophageal candidiasis,pulmonary infiltrates suspicious for Pneumocystis carinii pneumonia,peripheral deep vein thrombosis,pulmonary embolism,and disseminated intravascular coagulation.The multiple organ infections and thromboses responded well to antimicrobial and anticoagulant therapy.Radioisotope studies disclosed a pancreatic tumor and a metastatic lesion in the liver,whereas somatostatin receptor scintigraphy showed negative findings,suggesting the primary and metastatic tumors were poorly differentiated.A CT scan before admission showed no metastatic liver lesion,suggesting that the pancreatic tumor was rapidly progressing.Instead of surgery,antitumor chemotherapy was indicated.The patient was transferred to another hospital to initiate chemotherapy.However,she died four months later due to the rapidly progressive tumor.CONCLUSION ACTH-secreting pancreatic neuroendocrine neoplasm is a rare disease with a very poor prognosis.The clinical course and acute complications of the tumor remain unreported.Here we report the clinical course of a rapidly progressive case of ACTH-secreting pancreatic neuroendocrine tumor that developed infectious complications due to many types of pathogens in multiple organs,widespread thromboses,pulmonary embolism,and disseminated intravascular coagulation.
文摘Objective To investigate the methods and efficacy of treatment on severe adrenal Cushing's syndrome.Methods The clinical data of 22 cases with severe adrenal Cushing's syndrome(severe group),and 136 cases with mild or moderate adrenal Cushing's syndrome(non-severe group)were reviewed.The clinical features were analyzed by comparing the differences between these two groups when patients were admitted to hospital.We discussed the clinical managements of patients with severe adrenal Cushing's syndrome by comparing the differences with non-severe group after preoperative preparation,and with themselves before and after preoperative preparation.The effects of surgery were evaluated by comparing the differences between pre-operation and post-operation on patients with severe adrenal Cushing's syndrome.Results At admission,serum/urine cortisol,disease course,and blood pressure were significantly higher in the severe group than those in non-severe group(P<0.05 or P<0.01),serum potassium and ACTH level were decreased significantly in the severe group than those in non-severe group [(3.01±0.75 vs 3.62±0.48)mmol/L,P<0.01;(6.47±2.91 vs 8.21±3.22)pg/ml,P<0.01].However,no significant difference was observed in diastolic blood pressure,serum potassium,and fasting plasma glucose between these two groups after preoperative preparation(all P>0.05).And then,we performed adrenalectomy.The symptoms of 22 cases with severe adrenal Cushing's syndrome were obviously alleviated after 3 months.During follow-up,5 cases of primary bilateral macronodular adrenal hyperplasia(BMAH)and 1 case of primary pigmented nodular adrenocortical disease(PPNAD)were treated with contralateral adrenalectomy.Conclusion Sufficient preoperative preparation is essential for patients with severe adrenal Cushing's syndrome because of its high level serum cortisol with severe complications.If preparation fails before surgery,cortisol lowering medication or emergency unilateral adrenalectomy is necessary.Severe patients with BMAH and PPNAD were firstly performed unilateral adrenalectomy and followed-up closely,and then,contralateral adrenalectomy is needed when the recurrence of hypercortisolism appeared.
