Ectopic Cushing' syndrome caused by a neuroendocrine carcinoma of the mesentery

Faßhauer, Mathias; Lincke, Thomas; Witzigmann, Helmut; Kluge, Regine; Tannapfel, Andrea; Moche, Michael; Buchfelder, Michael; Petersenn, Stephan; Kratzsch, Juergen; Paschke, Ralf; Koch, Christian A.

doi:10.1186/1471-2407-6-108

Cited by 32 publications

(32 citation statements)

References 60 publications

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“…There are reports in literature on the concurrent CS and pheochromocytoma [3,4], sarcoidosis [5], pancreatic acinar cell carcinoma [6], pre-eclamptic findings [7], malignant gastrinoma [8], bronchial carcinoid lung tumor [9], pancreatic neuroendocrine tumor [10], Hippel-Lindau disease [10], or mesenteric neuroendocrine carcinoma [11]. Our patients had nothing of these associations.…”

Section: Discussionmentioning

confidence: 62%

Two Cases With Cushing Syndrome: What an Anesthesiologist Should Know

Domi¹

2011

J Endocrinol Metab

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Cushing syndrome is a multietiologic clinical situation, resulting in several features like obesity, hyperglycemia, hypertension, proximal muscle weakness, skin thinning, menstrual irregularities, buffalo hump, and purple striae. In perioperative period the anesthesiologist must deal with difficult ventilation and intubation, hemodynamic disturbances, volume overload and hypokalemia, glucose intolerance and diabetes, maintaining the blood cortisol level and preventing the glucocorticoid deficiency. This syndrome is quite rare and its features make these patients very difficult to the anaesthesiologist.

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Section: Discussionmentioning

confidence: 62%

Two Cases With Cushing Syndrome: What an Anesthesiologist Should Know

Domi¹

2011

J Endocrinol Metab

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“…Previous publications describing FDG PET or PET/CT scans in Cushingoid patients with ACTH-secreting tumors or adrenal hyperplasia have not reported altered FDG uptake patterns [18][19][20][21][22][23][24][25][26]. Our study is limited by the lack of a control population and the small number of children.…”

Section: Discussionmentioning

confidence: 84%

Altered FDG uptake patterns in pediatric lymphoblastic lymphoma patients receiving induction chemotherapy that includes very high dose corticosteroids

2011

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Children with lymphoblastic lymphoma on induction chemotherapy protocols including very high doses of corticosteroids transiently demonstrated altered FDG uptake patterns, including increased superficial facial uptake and reduced hepatic uptake. The facial uptake is probably the FDG PET equivalent of Cushingoid facies. Caution in interpreting scans with this altered FDG uptake pattern is suggested, as uptake at sites of lymphomatous involvement may potentially be affected.

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“…None of the dynamic biochemical tests achieves 100% accuracy, although BIPSS almost always shows absent central-toperipheral gradient (12). Patients with EAS tend to have higher ACTH levels than those with Cushing's disease, but there is no clear distinction between the two.…”

Section: Discussionmentioning

confidence: 95%

Ectopic Cushing's syndrome caused by a pulmonary ACTH-secreting tumor in a patient treated with octreotide

Rodrigues

Castedo

Damasceno

et al. 2012

Arq Bras Endocrinol Metab

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SUMMARYEctopic ACTH syndrome is a rare disease often associated with severe hypercortisolism. When feasible, optimal management is surgical excision of the tumor. A 33-year-old male patient was admitted to the hospital in 1993 with clinical manifestations suggestive of Cushing's syndrome. He presented high plasma ACTH and markedly elevated urinary free cortisol excretion that was not suppressed with high-dose dexamethasone administration. Pituitary MRI scan was normal. No central-to-peripheral ACTH gradient was present in bilateral inferior petrosal sinus sampling. Thoracic CT scan showed a 1.7 cm nodule at the left lung. Pulmonary fine needle cytology and immunocytochemical and ultrastructural studies, together with the presence of bone metastases, led to the diagnosis of an ACTH-producing neuroendocrine carcinoma. He was initially submitted to chemotherapy and has been on treatment with octreotide LAR since 1998, having shown a favorable clinical, biochemical and imaging response. We highlight the excellent longterm response to medical therapy with octreotide LAR, without tachyphylaxis, probably due to its antiproliferative effect. Arq Bras Endocrinol Metab. 2012;56(7):461-4 SUMÁRIO A secreção ectópica de ACTH é uma síndrome rara associada habitualmente à hipercortisolemia grave. A remoção cirúrgica do tumor é o tratamento de primeira linha, sempre que seja exequível. Homem com 33 anos, internado em 1993 com manifestações clínicas sugestivas de síndrome de Cushing, apresentava valores elevados de ACTH plasmática e excreção urinária de cortisol livre muito aumentada, sem supressão na prova com dose alta de dexametasona; RM hipofisária sem alterações; cateterismo bilateral dos seios petrosos inferiores sem gradiente central-periférico de ACTH. A CT de tórax mostrou um nódulo de 1,7 cm no pulmão esquerdo. O diagnóstico de carcinoma neuroendócrino produtor de ACTH foi feito com base nos resultados citológico, imunocitoquímico e ultraestrutural, juntamente com a presença de metástases ósseas. Foi inicialmente submetido à quimioterapia e encontra-se em tratamento com octreotide LAR desde 1998, apresentando resposta clínica, bioquímica e imagiológica favorável. Destacamos a excelente resposta a longo prazo à terapêutica com octreotide LAR, sem taquifilaxia, provavelmente devido ao seu efeito antiproliferativo. Arq Bras Endocrinol Metab. 2012;56(7):461-4

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Ectopic Cushing' syndrome caused by a neuroendocrine carcinoma of the mesentery

Cited by 32 publications

References 60 publications

Two Cases With Cushing Syndrome: What an Anesthesiologist Should Know

Two Cases With Cushing Syndrome: What an Anesthesiologist Should Know

Altered FDG uptake patterns in pediatric lymphoblastic lymphoma patients receiving induction chemotherapy that includes very high dose corticosteroids

Ectopic Cushing's syndrome caused by a pulmonary ACTH-secreting tumor in a patient treated with octreotide

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