Cases reported "Cushing Syndrome"

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1/152. 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.
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ranking = 1
keywords = endocrine
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2/152. Cushing's syndrome caused by nodular adrenal hyperplasia in children with McCune-Albright syndrome.

    McCune-Albright syndrome consists of fibrous dysplasia of bone, cafe-au-lait skin pigmentation, and endocrine dysfunction (usually precocious puberty). Other endocrine abnormalities occur in a minority of patients, and of these, Cushing's syndrome is the least often recognized. We present 5 children (4 girls) with features of McCune-Albright syndrome who had Cushing's syndrome in the infantile period (<6 months). In 2 children spontaneous resolution occurred, but the remaining 3 required bilateral adrenalectomy. In addition, all 4 girls have experienced precocious puberty, and 3 children demonstrated radiologic evidence of nephrocalcinosis. Understanding of the underlying defect causing McCune-Albright syndrome emphasizes the importance of searching for other endocrine dysfunction in these children.
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ranking = 3.0016061535118
keywords = endocrine, bone
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3/152. Extrapituitary parasellar microadenoma in Cushing's disease.

    Negative sellar exploration (despite the results of endocrine evaluation indicating Cushing's disease), the high incidence of failure of total hypophysectomy, and remission of Cushing's syndrome after unsuccessful hypophysectomy and sellar irradiation suggest that the etiology of refractory Cushing's disease, in some patients, lies near the sella but not in the pituitary gland. We present 5 patients, out of 626 who received surgery for Cushing's disease, in whom an ACTH-secreting extrapituitary parasellar adenoma was identified: 2 after unsuccessful total hypophysectomy for the treatment of refractory Cushing's disease, 2 after unsuccessful hemihypophysectomy (the first, 2 yr before treatment at the NIH for Nelson's syndrome; and the second, with recurrent Cushing's disease 5 yr after negative transsphenoidal exploration), and 1 with a preoperative diagnosis of an intraclival microadenoma, which was cured by resection of the tumor. In all cases, an extrapituitary parasellar microadenoma was confirmed unequivocally as the cause of the disease, by negative pathology of the resected pituitary gland (patients 1, 2, 3, and 5), and/or the remission of the disease after selective resection of the extrasellar adenoma (patients 3, 4, and 5). Three of 5 patients had a partial empty sella. These patients support the thesis that ACTH-secreting tumors can arise exclusively from remnants of Rathke's pouch, rather than from the adenohypophysis (anterior lobe or pars tuberalis of the pituitary gland) and can be a cause of Cushing's disease. In the sixth presented case, an extrapituitary tumor was suspected at surgery after negative pituitary exploration, but serial sections of the hemihypophysectomy specimen revealed a microscopic focus of tumor at the margin of the resected gland. This case demonstrates the importance of negative pituitary histology to establish the presence of an extrapituitary parasellar tumor as an exclusive source of ACTH, and it supports the value of clinical outcome to establish the diagnosis with selective adenomectomy of an extrapituitary parasellar tumor. In patients with negative pituitary magnetic resonance imaging, especially in the presence of a partial empty sella, the diagnostic and surgical approach in Cushing's disease should consider the identification and resection of extrapituitary parasellar adenoma, which can avoid total hypophysectomy, as was possible in 3 of our 5 patients.
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ranking = 1
keywords = endocrine
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4/152. Lack of response to octreotide in Cushing's syndrome due to metastatic adrenocortical carcinoma.

    Functional metastatic adrenocortical carcinoma is an uncommon cause of Cushing's syndrome, which rarely responds to conventional treatment. A patient presenting with Cushing's syndrome secondary to adrenocortical carcinoma underwent surgical resection. Postoperatively, she developed metastatic disease resistant to conventional chemotherapy. octreotide, a somatostatin analogue which is effective in the treatment of several types of neuroendocrine tumour, was tried to ameliorate her secretory symptoms, but without any therapeutic effect.
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ranking = 1
keywords = endocrine
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5/152. A choroidal amyloid-rich neuroendocrine tumor: initial manifestation of cushing syndrome.

    A 57-year-old man with a history of hepatic adenocarcinoma was referred 3 years after his diagnosis with a choroidal tumor in the right eye. Results of a transscleral excision biopsy revealed the tumor to be an amyloid-rich neuroendocrine metastasis. The patient subsequently developed cushingoid features and investigations revealed ectopic corticotropin syndrome, an elevated urinary 5-hydroxyindoleacetic acid level, and neuroendocrine metastasis in several locations. The choroidal neuroendocrine metastasis stained negative for serotonin and corticotropin. The source of the ectopic corticotropin and the location of the primary tumor have not been found. This case demonstrates that disseminated neuroendocrine tumors may rarely cause ocular lesions before systemic endocrine sequelae arise.
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ranking = 9
keywords = endocrine
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6/152. 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
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ranking = 2
keywords = endocrine
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7/152. Cushing's syndrome associated with a chemodectoma and a carcinoid tumour.

    We present a case of Cushing's syndrome where 111In-octreotide scanning provided evidence for the presence of two neuroendocrine tumours. Uptake in the right neck corresponded to a chemodectoma, but there was no change in the clinical condition or fall in ACTH levels following surgical resection. Uptake in the left chest was assumed to relate to a bronchial carcinoid, but a tumour could not initially be localized on magnetic resonance imaging (MRI), spiral CT scanning or on selective venous sampling. A 1 cm bronchial carcinoid tumour was identified post-mortem which immunostained for ACTH. This case demonstrates that 111ln-octreotide scanning is a useful technique for identifying the source of ectopic ACTH production in difficult cases of Cushing's syndrome. Reliance should not be placed solely on standard imaging techniques to localize the tumour prior to surgery. Although rare, the possibility of a non-ACTH secreting neuroendocrine tumour should also be considered in patients with ectopic ACTH syndrome, who have positive 111In-octreotide scans.
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ranking = 2
keywords = endocrine
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8/152. Cushing's syndrome presenting as a schizophrenia-like psychotic state.

    Neuropsychiatric abnormalities, of which depression is the most common, are frequently associated with Cushing's syndrome. The following paper describes a 58-year-old woman in whom a prolonged psychotic state--schizophrenia-like--was the presenting sign of Cushing's syndrome. The psychiatric symptoms disappeared shortly after the cortisol level has been normalized. Due to the variety of symptoms, both organic and psychiatric, which may occur with Cushing's syndrome, a high suspicion index is advisable. Suspect clinical findings should always prompt the appropriate endocrine work-up.
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ranking = 1
keywords = endocrine
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9/152. A case of Cushing's syndrome in ACTH-secreting mediastinal paraganglioma.

    Paragangliomas are unusual neuroendocrine cell tumors arising from paraganglia, of which ACTH-secreting cases in the mediastinum are extremely rare. A 51-year-old woman was admitted for generalized edema and weakness which began 5 months ago. Chest X-ray and CT scan revealed a tumor mass in the anterior mediastinum. The plasma cortisol and ACTH levels were very high. Other sources secreting ACTH, except mediastinal mass, were not found. Surgical excision of mediastinal mass and left supraclavicular lymph node was performed. The postoperative microscopic finding and immunohistochemical staining revealed organoid tumor cell nests (zellballen) and S-100 protein positive sustentacular cells which are characteristics of paraganglioma. This was thus a case of Cushing's syndrome resulting from ectopic ACTH production in anterior mediastinal paraganglioma.
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ranking = 1
keywords = endocrine
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10/152. Small cell neuroendocrine carcinoma of the thymus complicated by Cushing's syndrome. Report of a 58-year-old woman with a 3-year history of hypertension.

    A 58-year-old woman with a history of Cushing's syndrome for three years presented with a mediastinal mass and received the diagnosis of small cell neuroendocrine carcinoma of the thymus invading the pericardium. On immunohistochemical study, the neoplastic cells reacted with antibodies against cytokeratin, epithelial membrane antigen, neuron-specific enolase, chromogranin, synaptophysin, and ACTH. Clinicopathologic findings of this rare case of ectopic adrenocorticotropic hormone (ACTH) syndrome are discussed with a literature review.
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ranking = 5
keywords = endocrine
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