1/176. indium-111 pentetreotide lung uptake in infectious lung disease. Bilateral diffuse lung uptake of In-111 pentetreotide (OCT) was observed during a whole-body scan performed in a 68-year-old woman with Cushing's syndrome and suspected ectopic adrenocorticotropic hormone secretion. A few days later, she was found to have bilateral bacterial pneumonia (of mixed anaerobic origin). Cushing's syndrome was finally proved to be of pituitary origin. The OCT lung uptake in pneumonia probably resulted from tracer binding by somatostatin receptors on the inflammatory leukocytes. Although the rapid wash-out from experimentally induced abscesses does not make OCT a suitable tracer for detecting acute infections, the images and data here reported suggest that infectious lung disease should be excluded before diagnosing lung involvement by neuroendocrine tumors. ( info) |
2/176. Severe muscle weakness due to hypokalemia as a manifestation of small-cell carcinoma. We describe the case of a 56-year-old man with severe muscle weakness due to heavy hypokalemia (serum potassium 1.44 mmol/l) associated with inappropriate kaliuria and alkalemia. Subsequent investigation revealed the presence of ectopic ACTH hypersecretion due to a small-cell lung carcinoma. A significant clinical/laboratory improvement was achieved following chemotherapy-induced regression of the primary tumor. The profound hypokalemia was probably the result of cortisol hypersecretion, which in concert with the ACTH-induced decreased 11beta-hydroxysteroid dehydrogenase activity can exhibit an increased mineralocorticoid activity. In addition, other ACTH-dependent mineralocorticoids may play a contributory role in the development of severe hypokalemia. ( info) |
3/176. Ectopic ACTH syndrome due to thymic carcinoid tumor in a girl. An 8 year-old girl had a cushingoid appearance for six months. Hormone study showed extremely high serum levels of cortisol (> 60 micrograms/dl) and adrenocorticotropic hormone (930 pg/ml). Initial chest X-ray showed nothing unusual, but a technetium-99mm MIBI scan showed an accumulation lesion in the left upper chest cavity. Chest magnetic resonance imaging demonstrated that the mass was in the superior anterior mediastinum. She had complete removal of the tumor with partial thymectomy. The pathology revealed a thymic carcinoid tumor. Carcinoid tumors of the thymus are extremely rare in children and they usually present with Cushing's syndrome. To our knowledge, this is the youngest patient who has ever been reported with this disease. ( info) |
4/176. Carcinoid-associated ectopic ACTH syndrome with variable response to octreotide. The case is presented of a 31-year-old woman who developed florid clinical and biochemical Cushing's syndrome due to metastatic hepatic carcinoid tumour from a probable pancreatic primary. Hypercortisolaemia was controlled with metyrapone and ketoconazole, but high doses of octreotide failed to affect plasma cortisol and urinary 5-hydroxyindole acetic acid (5HIAA) levels, or prevent rapid tumour growth. Hepatic polystyrene embolisation failed, and she was treated by liver transplantation with initial excellent results, and normalisation of cortisol and 5HIAA levels. Ten months later, however, she relapsed with bony and pelvic tumour recurrence, and high and symptomatic levels of cortisol and 5HIAA. At this time, octreotide in similar doses to those used previously appeared to normalise her biochemically, although she died soon after. This variable responsiveness to octreotide could be related to somatostatin receptor changes, or cyclical tumour secretion patterns. ( info) |
5/176. Marked increase in plasma ACTH with tumor reduction after chemotherapy in ectopic ACTH syndrome. We report on a case of rapid and marked hormone release as a result of rapid tumor reduction due to chemotherapy in a 36-year-old woman with ectopic ACTH syndrome due to small cell lung cancer. Treatment of the cancer with cisplatin and etoposide resulted in an 80% reduction in tumor size on computed tomographic scan within two weeks. Concurrently, plasma ACTH exhibited an unexpected and astonishing increase from 373 pg/ml before treatment to more than 1200 pg/ml. There were no biochemical characteristics observed in tumor lysis syndrome of solid tumors such as azotemia, increased LDH and hyperkalemia. The present case indicates that anticancer chemotherapy instituted in patients with ectopic ACTH syndrome could result in an acute increase of plasma ACTH and exacerbation of hypercortisolism, similar to tumor lysis syndrome, which is a potentially fatal complication following anti-cancer chemotherapy. ( info) |
6/176. Cushing's syndrome secondary to ectopic ACTH secretion from metastatic breast carcinoma. breast carcinoma is a rare cause of ectopic ACTH syndrome. There are only two previously reported cases in which ACTH secretion is documented. We describe the case of a 56-year-old woman who presented with clinical and biochemical features of ectopic ACTH syndrome in the setting of metastatic breast carcinoma. Despite aggressive management of her ectopic ACTH syndrome, her course was complicated by opportunistic infection, respiratory failure and death. Immunostaining of the breast metastases for ACTH was positive and in situ hybridization revealed proopiomelanocortin gene expression. This is the first reported case of ectopic ACTH syndrome associated with metastatic breast cancer in which the technique of in situ hybridization has been used to confirm the breast cancer metastases as the source of ectopic ACTH secretion. ( info) |
7/176. Cyclical Cushing's syndrome--a trap for the unwary. A woman with cyclical Cushing's syndrome due to periodic hormonogenesis from a corticotropin (ACTH) secreting pituitary adenoma is discussed. This patient presented with acute steroid-induced psychosis but she was found to have subtle cushingoid features that went undetected for two years. Laboratory evaluation for Cushing's syndrome showed incongruous results due to periodic ACTH production by the tumour. Cyclical Cushing's syndrome may be an under-recognised phenomenon and incorrect interpretation of investigative results may lead to wrong tumour localisation and inappropriate surgery. This case highlights the subtleties and complexities that one may encounter in the diagnostic evaluation of patients with Cushing's syndrome, and emphasises that the laboratory results must always be interpreted in the appropriate clinical context. ( info) |
8/176. Ectopic corticotroph adenoma in the cavernous sinus: case report. OBJECTIVE AND IMPORTANCE: Adrenocorticotropin (ACTH)-secreting pituitary adenomas causing Cushing's disease are often difficult to identify because of their variable locations and their small size. This report presents histological evidence of an ectopic ACTH-secreting adenoma located entirely within the cavernous sinus. CLINICAL PRESENTATION: A 62-year-old woman presented with central obesity, hypertension, and osteoporosis. Endocrinological evaluation suggested the presence of an acth-secreting pituitary adenoma; however, imaging studies, including dynamic magnetic resonance imaging, did not reveal any visible lesions in the pituitary gland. Bilateral cavernous sinus sampling demonstrated a large central/peripheral ACTH gradient, with a right/left ACTH gradient. The patient was treated as having pituitary-dependent Cushing's disease, until she died suddenly as a result of acute respiratory failure. INTERVENTION: In a postmortem histological examination, an ACTH-secreting adenoma was found in the right cavernous sinus, which was completely surrounded by dura mater and had no direct connection with the pituitary gland. CONCLUSION: Although they are rare, such adenomas located in the cavernous sinus should be recognized as one of the reasons for inaccurate cavernous sinus sampling and the failure of transsphenoidal surgery for patients with ACTH-dependent Cushing's syndrome. ( info) |
9/176. The ectopic ACTH syndrome and superior vena cava obstruction due to a malignant carcinoid tumour of the thymus. We report the case of a 28-year-old African Caribbean woman with Cushing's syndrome and superior vena cava obstruction secondary to an ACTH-secreting carcinoid tumour of the thymus. The case highlights the problems which may be encountered in performing the 2-day high dose dexamethasone suppression test but clinicians are reminded that this test or any other dynamic test is absolutely essential for elucidating the cause of ACTH-dependent Cushing's Syndrome. ( info) |
10/176. Cushing's syndrome in prostate cancer. An aggressive course of prostatic malignancy. We report a case with an initial diagnosis of adenocarcinoma of the prostate in whom Cushing's syndrome developed. The disease did not respond to estrogen treatment and the patient died of severe septicemia. Histopathologic examination of the autopsy specimens revealed a small cell carcinoma intermingled with a moderately differentiated adenocarcinoma in the prostate and widespread metastases of small cell carcinoma. Immunoreactivity for neuroendocrine differentiation was found only in the small cell carcinoma. Determination of different tumor markers in plasma samples showed markedly elevated levels of prostate-specific antigen as well as carcinoembryonic antigen prior to treatment, with no significant changes after treatment. The concentration of the neuroendocrine marker chromogranin a was initially within the normal range, but increased during estrogen treatment, whilst neuron-specific enolase was moderately elevated throughout the observation period. copyright copyright 1999 S. Karger AG, Basel ( info) |