From 1984 to 1990, six patients with histologically and endocrinologically proven ectopic ACTH syndrome were treated in our department. The lesion in five patients was clinically evident, but in the other was occult. ...From 1984 to 1990, six patients with histologically and endocrinologically proven ectopic ACTH syndrome were treated in our department. The lesion in five patients was clinically evident, but in the other was occult. All lesions were located within the chest. Four were thvmic carcinoid and the other two were bronchial carcinoids. Most of the patients had typical clinical manifestations of Cushing’s syndrome. Laboratory tests including histopathological examination confirmed the diagnosis of ectopic ACTH syndrome. The treatment consisted of surgical removal of the mass supplemented by radiotherapy. The results showed that after treatment all patients had satisfactory remission. Two bronchial carcinoid patients have been living for 40 and 88 months respectively without recurrence. Among 4 thymic carcinoid patients, only one has been living with no tumor for 34 months. One patient had recurrence and the other 2 died 32 and 50 months after the treatment respectively. However, the symptoms of two thymic carci展开更多
BACKGROUND Small cell lung cancer(SCLC)accounts for 15%of lung cancers,and it commonly expresses peptide and protein factors that are active as hormones.These secreting factors manifest as paraneoplastic disorders,suc...BACKGROUND Small cell lung cancer(SCLC)accounts for 15%of lung cancers,and it commonly expresses peptide and protein factors that are active as hormones.These secreting factors manifest as paraneoplastic disorders,such as ectopic adrenocorticotropic hormone(ACTH)syndrome(EAS).The clinical features are abnormalities in carbohydrate metabolism,hypokalemia,peripheral edema,proximal myopathy,hypertension,hyperpigmentation,and severe systemic infection.However,it is uncommon that EAS has an influence on hypothalamus-pituitary function.CASE SUMMARY A 62-year-old man presented with complaints of haemoptysis,polyuria,polydipsia,increased appetite,weight loss,and pigmentation.Following a series of laboratory and imaging examinations,he was diagnosed with SCLC,EAS,hypogonadism,hypothyroidism,and central diabetes insipidus.After three rounds of chemotherapy,levels of ACTH,cortisol,thyroid hormone,gonadal hormone,and urine volume had returned to normal levels.In addition,the pulmonary tumor was reduced in size.CONCLUSION We report a rare case of SCLC complicated with panhypopituitarism due to EAS.We hypothesize that EAS induced high levels of serum glucocorticoid and negative feedback for the synthesis and secretion of antidiuretic hormone from the paraventricular nucleus,and trophic hormones from the anterior pituitary.Therefore,patients who present with symptoms of hypopituitarism,or even panhypopituitarism,with SCLC should be evaluated for EAS.展开更多
Ectopic mineralization- inappropriate biomineralization in soft tissues- is a frequent finding in physiological aging processes and several common disorders, which can be associated with significant morbidity and mort...Ectopic mineralization- inappropriate biomineralization in soft tissues- is a frequent finding in physiological aging processes and several common disorders, which can be associated with significant morbidity and mortality. Further, pathologic mineralization is seen in several rare genetic disorders, which often present life-threatening phenotypes. These disorders are classified based on the mechanisms through which the mineralization occurs: metastatic or dystrophic calcification or ectopic ossification. Underlying mechanisms have been extensively studied, which resulted in several hypotheses regarding the etiology of mineralization in the extracellular matrix of soft tissue. These hypotheses include intracellular and extracellular mechanisms, such as the formation of matrix vesicles, aberrant osteogenic and chondrogenic signaling, apoptosis and oxidative stress. Though coherence between the different findings is not always clear, current insights have led to improvement of the diagnosis and management of ectopic mineralization patients, thus translating pathogenetic knowledge(variome) to the phenotype(phenome). In this review, we will focus on the clinical presentation, pathogenesis and management of primary genetic soft tissue mineralization disorders. As examples of dystrophic calcification disorders Pseudoxanthoma elasticum, Generalized arterial calcification of infancy, Keutel syndrome, Idiopathic basal ganglia calcification and Arterial calcification due to CD73(NT5E) deficiency will be discussed. Hyperphosphatemic familial tumoral calcinosis will be reviewed as an example of mineralization disorders caused by metastatic calcification.展开更多
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 th...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
文摘From 1984 to 1990, six patients with histologically and endocrinologically proven ectopic ACTH syndrome were treated in our department. The lesion in five patients was clinically evident, but in the other was occult. All lesions were located within the chest. Four were thvmic carcinoid and the other two were bronchial carcinoids. Most of the patients had typical clinical manifestations of Cushing’s syndrome. Laboratory tests including histopathological examination confirmed the diagnosis of ectopic ACTH syndrome. The treatment consisted of surgical removal of the mass supplemented by radiotherapy. The results showed that after treatment all patients had satisfactory remission. Two bronchial carcinoid patients have been living for 40 and 88 months respectively without recurrence. Among 4 thymic carcinoid patients, only one has been living with no tumor for 34 months. One patient had recurrence and the other 2 died 32 and 50 months after the treatment respectively. However, the symptoms of two thymic carci
基金Supported by the National Science Foundation for Youth,No.30800533the Public Welfare Project of Science and Technology Department of Zhejiang Province,China,No.2017C33056
文摘BACKGROUND Small cell lung cancer(SCLC)accounts for 15%of lung cancers,and it commonly expresses peptide and protein factors that are active as hormones.These secreting factors manifest as paraneoplastic disorders,such as ectopic adrenocorticotropic hormone(ACTH)syndrome(EAS).The clinical features are abnormalities in carbohydrate metabolism,hypokalemia,peripheral edema,proximal myopathy,hypertension,hyperpigmentation,and severe systemic infection.However,it is uncommon that EAS has an influence on hypothalamus-pituitary function.CASE SUMMARY A 62-year-old man presented with complaints of haemoptysis,polyuria,polydipsia,increased appetite,weight loss,and pigmentation.Following a series of laboratory and imaging examinations,he was diagnosed with SCLC,EAS,hypogonadism,hypothyroidism,and central diabetes insipidus.After three rounds of chemotherapy,levels of ACTH,cortisol,thyroid hormone,gonadal hormone,and urine volume had returned to normal levels.In addition,the pulmonary tumor was reduced in size.CONCLUSION We report a rare case of SCLC complicated with panhypopituitarism due to EAS.We hypothesize that EAS induced high levels of serum glucocorticoid and negative feedback for the synthesis and secretion of antidiuretic hormone from the paraventricular nucleus,and trophic hormones from the anterior pituitary.Therefore,patients who present with symptoms of hypopituitarism,or even panhypopituitarism,with SCLC should be evaluated for EAS.
基金Supported by The Research Foundation Flanders(FWO)(FWO14/ASP/084),Vanakker OM is a senior clinical investigator at the Fund for Scientific Research-FlandersContract grant sponsor:FWO grant No G.0241.11NMethusalem grant No.08/01M01108
文摘Ectopic mineralization- inappropriate biomineralization in soft tissues- is a frequent finding in physiological aging processes and several common disorders, which can be associated with significant morbidity and mortality. Further, pathologic mineralization is seen in several rare genetic disorders, which often present life-threatening phenotypes. These disorders are classified based on the mechanisms through which the mineralization occurs: metastatic or dystrophic calcification or ectopic ossification. Underlying mechanisms have been extensively studied, which resulted in several hypotheses regarding the etiology of mineralization in the extracellular matrix of soft tissue. These hypotheses include intracellular and extracellular mechanisms, such as the formation of matrix vesicles, aberrant osteogenic and chondrogenic signaling, apoptosis and oxidative stress. Though coherence between the different findings is not always clear, current insights have led to improvement of the diagnosis and management of ectopic mineralization patients, thus translating pathogenetic knowledge(variome) to the phenotype(phenome). In this review, we will focus on the clinical presentation, pathogenesis and management of primary genetic soft tissue mineralization disorders. As examples of dystrophic calcification disorders Pseudoxanthoma elasticum, Generalized arterial calcification of infancy, Keutel syndrome, Idiopathic basal ganglia calcification and Arterial calcification due to CD73(NT5E) deficiency will be discussed. Hyperphosphatemic familial tumoral calcinosis will be reviewed as an example of mineralization disorders caused by metastatic calcification.
基金This study was supported by grants from the National Natural Science Foundation of China （No. 30270625）, and Science and Technology Foundation of the Science and Technology Commission of the Shanghai Municipality （No. 06DZ22030）.
文摘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
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.
