711/1097. ACTH-secreting medullary carcinoma of the thyroid: monitoring of clinical course with calcitonin and cortisol assays and immunohistochemical studies. The clinical course of a patient with Cushing's syndrome secondary to metastatic medullary carcinoma of the thyroid was documented with serial calcitonin and cortisol assays and tumor immunohistochemistry studies. Cortisol levels were originally markedly elevated but returned to normal after total thyroidectomy. These levels rose again when the patient developed liver metastases but normalized during chemotherapy, never to rise again despite the appearance of cervical lymph node metastasis. calcitonin levels remained elevated throughout the course. The original tumor was composed of two cell lines: one containing both calcitonin and ACTH and another containing only calcitonin-reacting cells. However, the cervical metastasis showed a marked decrease in both cell lines with fever than 1% of cells reacting to ACTH, and only 25% to calcitonin. The almost total disappearance of ACTH-reacting cells may have been therapeutically induced or may represent a consequence of tumor progression. ( info) |
712/1097. A clinico-pathological study of two patients with Cushing's disease. The clinical and autopsy findings are described in two patients with Crushing's disease who died five days and 18 years following bilateral adrenalectomy. In each case tomography of the pituitary fossa was normal but examination of the pituitary revealed a 5 to 6 mm basophilic adenoma in the antero-inferior aspect of the gland. The first patient failed to show suppression of urinary oxogenic steroids during administration of high dose dexamethasone. The second patient had functioning adrenal autografts in skeletal muscle 18 years after adrenalectomy. The clinico-pathological correlation of these two patients supports modern re-emphasis on the aetiology of Cushing's disease as a primary pituitary tumour and its treatment by selective trans-sphenoidal hypophysectomy. ( info) |
713/1097. Relationship of ectopic ACTH production to tumor differentiation: a morphologic and immunohistochemical study of prostatic carcinoma with Cushing's syndrome. A case of Cushing's syndrome in a patient with an ACTH-producing prostatic carcinoma is presented. Initial transurethral biopsy showed a moderately well-differentiated adenocarcinoma. The development of Cushing's syndrome was associated with a change in the morphology of the tumor; repeat transurethral prostatic biopsy and autopsy showed an admixture of moderately well-differentiated adenocarcinoma and anaplastic carcinoma. Immunoperoxidase studies revealed ACTH only within the anaplastic component of the tumor, which also showed strong argyrophilia. Electron microscopic studies revealed abundant dense-core granules within the anaplastic portion of the tumor with only very scanty granules in the differentiated portion. This case strongly supports the contention that cells with APUD characteristics can differentiate within epithelial tissues of multiple embryologic origins, and that the possession of APUD features does not necessarily imply origin from any specific neuroendocrine progenitor cell. ( info) |
714/1097. Cushing's syndrome due to an ACTH-secreting adrenal medullary tumour. A 35-year-old man with Cushing's syndrome due to ectopic ACTH production is described. The six-week history and severe proximal myopathy were highly suggestive of a malignant tumour, but a solitary phaeochromocytoma of the left adrenal gland was the source of ACTH production. The surgical outcome was successful and three months post-operatively the hypothalamic-pituitary-adrenal axis was normal. At 12 months follow-up he remains well with complete regression of the features of Cushing's syndrome. ( info) |
715/1097. Successful therapy of an ACTH-producing gastric carcinoid APUD tumor: report of a case and review of the literature. A case of a patient with a metastatic ACTH (adrenocorticotrophic hormone)-producing gastric carcinoid APUD (amine precursor uptake and decarboxylation) tumor is presented. The patient presented with Cushing's syndrome and very high levels of ACTH. The Cushing's syndrome was controlled medically, and when the patient deteriorated clinically she received treatment with a combination of BCNU, adriamycin, and ftorafur. She responded dramatically with marked clinical improvement and normalization of plasma ACTH levels. The current status of the APUD cell concept is discussed, with its overall implications for treatment of this group of malignancies. ( info) |
716/1097. amenorrhea-galactorrhea associated with Cushing's disease due to pituitary tumor. Two patients with amenorrhea-galactorrhea and Cushing's disease due to pituitary tumor are presented. In both cases, a diagnosis of prolactin-secreting tumor with moderate hyperprolactinemia was established, then Cushing's disease developed years later. Despite the typical clinical features of Cushing's disease, a dexamethasone suppression test inhibited both blood ACTH and cortisol concentrations in the 2 patients. hypophysectomy was performed in 1 case, and the histologic study revealed the presence of a cromophobe adenoma. The other case was treated by external radiation which improved the clinical situation. Clinical and endocrinological studies in these 2 cases were compared with previous reports in the literature in order to discuss the etiology of this unusual association. It is concluded that patients with pituitary tumors presenting moderate hyperprolactinemia may also have excessive secretion of ACTH. ( info) |
717/1097. Feminizing adrenocortical carcinoma with Cushing's syndrome and pseudohyperparathyroidism. A patient with adrenocortical carcinoma had three major endocrine abnormalities attributable directly to the tumor: hypercortisolism (Cushing's syndrome), hyperestrogenism (feminization), and hypercalcemia (pseudohyperparathyroidism). There were higher levels of immunoreactive parathyroid hormone in venous effluent from the tumor or its abdominal metastases compared to that found in the veins draining the parathyroid glands. This, together with the presence of normal parathyroid glands on autopsy, established the diagnosis of pseudohyperparathyroidism as the cause of hypercalcemia in this patient. ( info) |
| A patient with Cushing's disease was treated with cyproheptadine with concomitant remission of the disease for 60 months. Despite clinical improvement and achievement of normal levels of cortisol excretions, her menstruation-related cyclic surge of cortisol secretion was supranormal. The mode of action of cyproheptadine was studied by the administration of metyrapone. Long-term administration of cyproheptadine appeared to normalize excessive adrenocorticotropic hormone (ACTH) production by reducing the frequency and the peak levels of episodic secretion of ACTH. ( info) |
719/1097. Pituitary ACTH dependency of nodular adrenal hyperplasia in Cushing's syndrome. Report of two cases and review of the literature. Cushing's syndrome due to nodular adrenal hyperplasia comprises a clinically and biochemically heterogeneous group of disorders whose pathogenesis is unclear. We describe two patients with atypical steroid dynamics and large unilateral adrenal nodules who had pituitary ACTH-dependent disease. In the differential diagnosis of Cushing's syndrome, we recommend repeated ACTH measurement and selective venous sampling-particularly in those patients with impaired dexamethasone suppressibility and abnormal findings on computerized tomography. ( info) |
720/1097. Cushing's syndrome secondary to a neuroendocrine tumor: relapse after bilateral adrenalectomy. Bilateral adrenalectomy results in the removal of the site of glucocorticosteroid production thereby eliminating the signs and symptoms of Cushing's syndrome. A case is reported of a middle-aged man with Cushing's syndrome due to an ACTH-producing tumor. The patient initially responded to adrenalectomy but had a fatal relapse. autopsy showed that the right adrenal bed containing remnants of adrenal tissue. An islet cell carcinoma of the pancreas was thought to be the primary tumor. ( info) |