Shift from CRH to ACTH Production in a Thymic Carcinoid with Cushing&amp;rsquo;s Syndrome

Ozawa, Yasunori; Tomoyasu, Hiroshi; Takeshita, Akira; Shishiba, Yoshimasa; Yamada, Shozo; Kovács, Kálmán; Matsushita, Hiroshi

doi:10.1159/000184803

Cited by 19 publications

(7 citation statements)

References 13 publications

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“…Indeed, patients with ectopic ACTH/CRH secretion may display an ACTH response to CRH and a petrosal-to-periphery ACTH gradient consistent with Cushing’s disease [10, 11, 12], as did our patient. In vitro studies showing the failure of primary tumor cultures to secrete ACTH or CRH, however, excluded this diagnosis.…”

Section: Discussionsupporting

confidence: 59%

Unusual Association of Adrenal Angiosarcoma and Cushing’s Disease

et al. 2001

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The occurrence of an angiosarcoma in the adrenal gland is exceedingly rare as only 21 patients have been reported in the literature since its first description 30 years ago. We report the clinicopathological features of a 34-year-old patient presenting with overt Cushing’s syndrome due to a ACTH-secreting pituitary adenoma and an adrenal spindle cell angiosarcoma. This association made the diagnosis of Cushing’s disease particularly puzzling in view of the results of biochemical tests which were compatible in part with adrenal and in part with paraneoplastic hypercortisolism and the negative pituitary imaging. The unique appearance of this tumor and its diagnostic implications are discussed.

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Section: Discussionsupporting

confidence: 59%

Unusual Association of Adrenal Angiosarcoma and Cushing’s Disease

et al. 2001

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“…In addition, the majority of tumor cells showed a positive expression of ACTH (Fig. 3d) (9), concomitantly with CRH (10), or with GHRH (11).…”

Section: F I G U R E 4 S E Q U E N C I N G C H R O Ma T O G R a M Dmentioning

confidence: 90%

“…The reported patient is a Japanese man with a smoking history of 10 i g u r e 2 . MRI o f t h e h e a d ( c o r o n a l s c a n , g a d o l i n i u m e nh a n c e me n t + ) .…”

Section: Case Reportmentioning

confidence: 99%

Multiple Endocrine Neoplasia Type I and Cushing's Syndrome Due to an Aggressive ACTH Producing Thymic Carcinoid

et al. 2006

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“…There have been two reports of thymic CRH production (16,17). In such cases functional imaging coupled with conventional imaging has recently been used to localize endocrine tumors.…”

Section: Discussionmentioning

confidence: 99%

[18F]fluoro-2-deoxy-d-glucose ([18F]FDG) positron emission tomography imaging of thymic carcinoid tumor presenting with recurrent Cushing’s syndrome

et al. 2005

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We report a case of a young woman with Cushing's syndrome (CS), in whom although endocrine investigations and negative pituitary imaging were suggestive of ectopic ACTH secretion, the results of inferior petrosal sinus (IPS) sampling after coricotropin-releasing hormone (CRH) stimulation were suggestive of pituitary ACTH hypersecretion.111 In-labelled octreotide and high-resolution computer tomography (CT) revealed a lesion possibly responsible for the ACTH source in the thymus. Thymectomy confirmed concomitant ectopic CRH and probable ACTH production by a thymic neuroendocrine carcinoma. After an 8-year remission period the patient developed a clinical and biochemical relapse. A high-resolution computed tomography (CT) scan of the thorax showed a 2-cm nodule in the thymic bed, which was positive on a [ Case reportA 25-year-old woman initially presented with recentonset depression, hirsuitism, acne and bruising suggestive of Cushing's syndrome (CS); clinical examination revealed arterial hypertension (170/120 mmHg) and mild proximal myopathy. Subsequent investigations showed hypokalemic alkalosis (serum K þ , 2.3 mmol/l (normal range 3.5 -5.0 mmol/l); HCO 3 , 32 mmol/l (normal levels 24^2 mmol/l)), marked hyperandrogenemia (testosterone, 12.5 nmol/l (normal range 0.5 -2.5 nmol/l)), normal fasting glucose concentrations and autonomous hypercortisolemia (elevated 24-h urinary free cortisol (UFC) of 4064 nmol per 24 h (normal range 20 -300 nmol per 24 h), serum midnight cortisol of 1161 nmol/l and inadequate cortisol suppression to a formal low-dose dexamethasone suppression test).An ectopic source of adrenocorticotropic hormone (ACTH) secretion was suggested by a marked elevated plasma ACTH concentration (1350 ng/l (normal values , 25 ng/l)), and a failure to suppress serum cortisol following a high-dose dexamethasone suppression test (HDDST). Inferior petrosal sinus (IPS) sampling after corticotropin-releasing hormone (CRH) stimulation, however, was more in keeping with Cushing's disease than with ectopic ACTH production as it showed a brisk ACTH increment following CRH administration (Table 1) (Fig. 1A). A further high-resolution CT showed a 2-cm thymic nodule in the upper mediastinum. Whole-body catheterization and sampling did not reveal an ectopic ACTH source. However, a greater than 3:1 CRH gradient suggestive of ectopic CRH production between the left brachiocephalic vein (BCV) and superior vena cava (SVC) and their simultaneous peripheral samples (BCV, 90 pg/ml and SVC, 100 pg/ml vs 30 pg/ml respectively) was shown. Based on the above findings a working diagnosis of a neuroendpcrine tumor of the thymus (thymic carcinoid tumor) secreting CRH was made.The patient underwent a mediastinotomy and thymectomy. A central firm nodule within the thymus

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Shift from CRH to ACTH Production in a Thymic Carcinoid with Cushing’s Syndrome

Cited by 19 publications

References 13 publications

Unusual Association of Adrenal Angiosarcoma and Cushing’s Disease

Unusual Association of Adrenal Angiosarcoma and Cushing’s Disease

Multiple Endocrine Neoplasia Type I and Cushing's Syndrome Due to an Aggressive ACTH Producing Thymic Carcinoid

[18F]fluoro-2-deoxy-d-glucose ([18F]FDG) positron emission tomography imaging of thymic carcinoid tumor presenting with recurrent Cushing’s syndrome

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