Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01189 (beta-endorphin)
 21,003 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 34-year-old man was diagnosed with clinical Cushing's syndrome based on circadian fluctuation of plasma adrenocorticotropin and serum cortisol levels and Liddle's-method. The presence of ectopic adrenocorticotropin production was suspected. Urine 5-hydroxyindoleaceturic acid level was high. Chest computed-tomography scan revealed a mass in the right upper lung. 111In-pentetrotide scintigraphy demonstrated marked accumulation in the right upper lung. We suspected an adrenocorticotropin-producing bronchial carcinoid. Plasma adrenocorticotropin and serum cortisol levels decreased immediately following resection of the tumor. Adrenocorticotropin production by tumor cells was confirmed by immunohistochemistry. This case indicates 111In-pentetrotide scintigraphy could be successfully used to identify and localize ectopic adrenocorticotropin-producing bronchial carcinoid.
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PMID:Successful use of 111In-pentetrotide scintigraphy for localizing ectopic adrenocorticotropin-producing bronchial carcinoid tumor in a patient with Cushing's syndrome. 1460 15

A case of pulmonary tumorlets with ectopic adrenocorticotropin (ACTH) production and lymph node metastasis in a bronchiectatic patient is reported. A 65-year-old man underwent right lower lobe lobectomy because of a sudden attack of cough and hemoptysis. Histological study revealed multiple discrete uniform small nests of tumor cells surrounding dilated bronchioles. Tumor nests were also found in the hilar lymph node. Immunohistochemically, the proliferating cells were confirmed to be neuroendocrine in origin with ectopic ACTH production, despite being clinically silent. The findings in the present case suggest that the clinical behavior of pulmonary tumorlets may be like a carcinoid, and need to be treated as tumor-like lesions. Ectopic hormonal production in the present case suggests pulmonary tumorlets should be considered in the differential diagnosis of Cushing's syndrome.
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PMID:Adrenocorticotropin-producing pulmonary tumorlets with lymph node metastasis. 1462 55

Malignant pulmonary tumors are rarely accompanied by Cushing syndrome. We encountered 4 patients with adrenocorticotropic hormone (ACTH)-producing pulmonary tumors (3 with carcinoid and 1 with small cell carcinoma). All patients were females aged 33-76 years. In the 4 patients, diagnostic methods, surgical procedures, stage, outcome, and immunohistological findings were evaluated. Subtotal resection of the anterior lobe of the pituitary gland had been performed in 3 patients. However, ACTH did not decrease, and further examination revealed pulmonary tumors. Preoperative blood sampling by catheter examination was performed in 2 patients, of whom 1 showed increased ACTH. Intraoperative blood sampling showed increased ACTH in 1 patient. Lobectomy combined with mediastinal lymph node dissection was performed in 3 patients and lobectomy alone (video-assisted thoracoscopic surgery) in 1. All patients showed a decrease in ACTH after operation without tumor recurrence. Diagnosis is possible by measuring ACTH in pulmonary arterial wedge blood obtained by catherization or in pulmonary venous blood obtained during operation. Surgical resection is effective for ectopic ACTH-producing pulmonary tumors.
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PMID:Diagnosis and surgical treatment of ectopic adrenocorticotropic hormone-producing pulmonary tumors accompanied by Cushing syndrome. 1471 19

Bronchopulmonary carcinoids are one of the most common cause of ectopic secretion of corticotropin (ACTH) and account for approximately 1% of all the patients in whom Cushing's syndrome develops. We reviewed 98 cases described in the World Literature and we report on two new cases. A 60-year old woman affected by Cushing's syndrome underwent to surgical wedge resection of a peripheral pulmonary nodule and a 30-year old woman with similar clinical features underwent to middle lobectomy for a small hilar neoplasm. Histopathologic examination of the tumours defined them as typical bronchopulmonary carcinoids. The patients are asymptomatic and with no sign of recurrence 72 and 30 months after surgery. According to our review we found no clear evidence that bronchial carcinoids associated with Cushing's syndrome should be considered a more aggressive variant or subtype of the typical carcinoid. If Cushing's syndrome does not disappear after surgery, the presence of residual disease (often a nodal involvement) should be investigated. A long-term relapse of the syndrome requires a careful search for local or distant neoplastic recurrence.
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PMID:Cushing's syndrome induced by bronchopulmonary carcinoid tumours: a review of 98 cases and our experience of two cases. 1503 49

Cushing syndrome caused by adrenocorticotropic hormone (ACTH) production from solid tumors can result in life-threatening hypercortisolemia. Ectopic ACTH production is most commonly associated with bronchial carcinoids and squamous cell carcinoma of the lung. We report a case of Cushing syndrome caused by ectopic ACTH production from a carcinoid of the duodenum. The patient presented to an outside hospital in hypertensive crisis and diabetic ketoacidosis. After stabilization, diagnostic studies including a serum cortisol level, and computed tomography (CT) scans of the head, chest, abdomen, and pelvis revealed hypercortisolemia and a large mass in the head of the pancreas. Pancreaticoduodenectomy was performed. Pathologic investigation revealed a 1-cm carcinoid of the duodenum with two large metastatic lymph nodes near the head of the pancreas. This is the first reported case in the English literature of Cushing syndrome caused by ectopic ACTH production from a carcinoid of the duodenum.
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PMID:A case of Cushing syndrome secondary to ectopic adrenocorticotropic hormone producing carcinoid of the duodenum. 1598 79

A 43-year-old man presented with fluctuating symptoms of weight gain, shortness of breath, pretibial oedema, associated with anxiety and memory disturbances. Laboratory investigation revealed an adrenocorticotropin (ACTH)-dependent cyclical Cushing's syndrome characterised by remarkable variations in urinary cortisol excretions ranging from 27 to 28,050 nmol/ 24 h. Magnetic resonance imaging (MRI ) of the pituitary was normal and ectopic ACTH production was suspected. A tumour in the right anterior mediastinum was revealed on octreotide receptor scintigraphy, which had initially been overlooked on computed tomography (CT) scanning. A thymic carcinoid tumour was suspected, which was supported by increased levels of urinary serotonin, while platelet serotonin and urinary 5-hydroxyindoleacetic acid levels were normal. The tumour was removed surgically and histological examination revealed an atypical thymic carcinoid tumour. Postoperatively, the patient's symptoms disappeared rapidly. He underwent external radiotherapy and is still free of symptoms after almost two years of follow-up. For clinical practice, a cyclical Cushing's syndrome should be suspected in any patient with clinical signs of Cushing's syndrome but normal biochemistry. Repeated measurement of urinary cortisol excretion is then required to establish or rule out the diagnosis.
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PMID:Cyclical Cushing's syndrome due to an atypical thymic carcinoid. 1642 39

Adrenocorticotropic hormone (ACTH)-producing bronchial carcinoid tumours are a rare cause of Cushing's syndrome. Cushing's syndrome is frequently complicated by osteoporosis, which results in an increased tendency for the development of vertebral compression fractures. Percutaneous vertebroplasty has been shown to be an effective treatment option in the setting of painful osteoporotic compression fractures refractory to conservative therapies. We report a case where vertebroplasty was performed on a 36-year-old woman with osteoporosis and compression fractures secondary to hypercorticolism. A bronchial carcinoid tumour was found to be the source of excess ACTH production. Three-level percutaneous vertebroplasty resulted in a marked improvement in pain.
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PMID:Vertebroplasty for vertebral compression fractures secondary to Cushing's syndrome induced by an ACTH-producing bronchial carcinoid tumour. 1942 71

Pulmonary neuroendocrine tumors, especially small cell carcinoma and typical carcinoids, are the most common cause of ectopic Cushing syndrome (CS). Sometimes these adrenocorticotropic hormone (ACTH)-producing tumors are too small to localize, and it may take a long time to identify them. This report describes the case of a 27-year-old-man with CS. This CS was due to the ectopic ACTH produced by the pulmonary atypical carcinoids, which were recognized at the disseminated stage 5 years after the initial presentation of CS. Microscopically, multiple whitish nodules (up to 1.0 cm in diameter) of the wedge-resected lung were composed of small round cells appearing to be typical neuroendocrine but were diagnosed as atypical carcinoid, based on the findings of focal necrosis, high mitotic rates and multiple endolymphatic tumor emboli. Immunohistochemically, tumor cells robustly stained for ACTH. The delay of diagnosis is, in part, attributable to the pulmonary tuberculosis (one of the complications of hypercortisolism), because the pulmonary tuberculosis presented in the form of multiple nodules throughout the lung. In addition, this patient had a Crooke's cell hyperplasia of the pituitary gland, which represents the reactive change of corticotrophs exposed to the excess cortisol rather than neoplastic change leading to CS.
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PMID:Multiple pulmonary atypical carcinoids presenting with long-standing Cushing syndrome masked by pulmonary tuberculosis. 2039 1

Thymomas constitute majority of the thymic neoplasms. In contrast, neuroendocrine tumors (carcinoid and neuroendocrine carcinoma) of thymus are extremely rare. Thymic carcinoids may present rarely with Cushing's syndrome due to the ectopic production of adrenocorticotropic hormone (ACTH). Recognition of this association is imperative for appropriate management of patients. We describe three cases of rare atypical carcinoid tumor (neuroendocrine carcinoma) of the thymus. Case 1, of a 26-year-old man presenting with Cushing's syndrome, case 2--a 23-year-old female with Cushingoid features, and Case 3--a 39-year-old man complaining of progressively worsening dyspnea. Computed tomography (CT) scans of chest in all three patients revealed anterior mediastinal mass. Excision of tumors and histological examination of the three tumors showed a carcinoid tumor with nuclear pleomorphism, increased mitotic activity and focal necrosis. The features suggested a diagnosis of atypical carcinoid tumor in all the three cases. The tumor cells in Cases 1 and 2 showed focal immunohistochemical staining for ACTH. Atypical carcinoid (neuroendocrine carcinoma, well-differentiated and moderately-differentiated) of the thymus is a rare thymic tumor which carries a worse prognosis compared to thymoma and requires aggressive therapy. Hence, an accurate diagnosis is essential.
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PMID:Primary neuroendocrine carcinoma of thymus: a rare cause of Cushing's syndrome. 2009 Feb 49

A 56-year-old Japanese woman with adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (CS) was admitted to hospital, where she was diagnosed as having a mediastinal tumor with ectopic ACTH production. The tumor and associated lymph node metastases were resected endoscopically, and the pathological diagnosis was atypical thymic carcinoid. Radiation therapy and administration of metyrapone, an inhibitor of 11b-hydroxylase to decrease the cortisol level, were attempted, but the levels of ACTH and cortisol were unresponsive. Bilateral adrenalectomy and hydrocortisone replacement were performed to ameliorate the patient's hypercortisolism. She subsequently developed multiple vertebral metastases, but was unwilling to undergo chemotherapy. Her condition deteriorated progressively, and she died of heart and respiratory failure 3 years and 6 months after the first admission. Immunostaining for ACTH, chromogranin A, synaptophysin, and neuron-specific enolase was positive in the carcinoid cells. Since somatostatin (SS) and SS analogues inhibit the growth of carcinoid via the SS receptor (SSTR) 2, we evaluated the expression of SSTR2 in the carcinoid cells using reverse transcription-polymerase chain reaction, and this confirmed the expression of SSTR2 in the carcinoid cells. Our experience of this patient with CS due to an ectopic ACTH-producing atypical thymic carcinoid suggests that SS analogues may be useful for treatment of carcinoid showing expression of SSTR2.
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PMID:Atypical thymic carcinoid associated with Cushing's syndrome. 2131 31


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