scholarly journals A case of an ACTH-secreting typical carcinoid tumor resected 12 years after a diagnosis of ectopic ACTH syndrome was made

2021 ◽  
Vol 35 (6) ◽  
pp. 736-741
Author(s):  
Takashi Indo ◽  
Akihiro Aoyama ◽  
Shigeo Hara ◽  
Kanta Fujimoto ◽  
Hiroshi Hamakawa ◽  
...  
Author(s):  
Wei Yang ◽  
David Pham ◽  
Aren T Vierra ◽  
Sarah Azam ◽  
Dorina Gui ◽  
...  

Summary Ectopic ACTH-secreting pulmonary neuroendocrine tumors are rare and account for less than 5% of endogenous Cushing’s syndrome cases. We describe an unusual case of metastatic bronchial carcinoid tumor in a young woman presenting with unprovoked pulmonary emboli, which initially prevented the detection of the primary tumor on imaging. The source of ectopic ACTH was ultimately localized by a Gallium-DOTATATE scan, which demonstrated increased tracer uptake in a right middle lobe lung nodule and multiple liver nodules. The histological diagnosis was established based on a core biopsy of a hepatic lesion and the patient was started on a glucocorticoid receptor antagonist and a somatostatin analog. This case illustrates that hypercogulability can further aggravate the diagnostic challenges in ectopic ACTH syndrome. We discuss the literature on the current diagnosis and management strategies for ectopic ACTH syndrome. Learning points: In a young patient with concurrent hypokalemia and uncontrolled hypertension on multiple antihypertensive agents, secondary causes of hypertension should be evaluated. Patients with Cushing’s syndrome can develop an acquired hypercoagulable state leading to spontaneous and postoperative venous thromboembolism. Pulmonary emboli may complicate the imaging of the bronchial carcinoid tumor in ectopic ACTH syndrome. Imaging with Gallium-68 DOTATATE PET/CT scan has the highest sensitivity and specificity in detecting ectopic ACTH-secreting tumors. A combination of various noninvasive biochemical tests can enhance the diagnostic accuracy in differentiating Cushing’s disease from ectopic ACTH syndrome provided they have concordant results. Bilateral inferior petrosal sinus sampling remains the gold standard.


2015 ◽  
Vol 62 (4) ◽  
pp. 202-204
Author(s):  
Irene Acevedo-Báñez ◽  
Juan Luis Tirado-Hospital ◽  
Ovidio Muñiz-Grijalvo ◽  
Miguel Angel Mangas-Cruz ◽  
Francisco Javier García-Gómez

2010 ◽  
Vol 57 (8) ◽  
pp. 679-686 ◽  
Author(s):  
Yuji Tani ◽  
Toru Sugiyama ◽  
Shinichi Hirooka ◽  
Hajime Izumiyama ◽  
Yukio Hirata

2016 ◽  
Vol 31 (4) ◽  
pp. 794-797 ◽  
Author(s):  
Sung Yong Han ◽  
Bo Hyun Kim ◽  
Hee Ryeong Jang ◽  
Won Jin Kim ◽  
Yun Kyung Jeon ◽  
...  

2020 ◽  
Vol 182 (4) ◽  
pp. R29-R58 ◽  
Author(s):  
Jacques Young ◽  
Magalie Haissaguerre ◽  
Oceana Viera-Pinto ◽  
Olivier Chabre ◽  
Eric Baudin ◽  
...  

Ectopic ACTH syndrome (EAS) is rare but is frequently a severe condition because of the intensity of the hypercortisolism that may be dissociated from the tumoral condition. EAS should often be considered as an endocrine emergency requiring an emergency response both in terms of diagnostic procedures and therapeutic interventions. Patient management is complex and necessitates dual skills, in the diagnosis and treatment of CS and in the specific management of neuroendocrine tumors (NET). Therefore, initial management should be performed ideally by experienced endocrinology teams in collaboration with specialized hormonal laboratory, modern imaging platforms and intensive care units. Diagnostic procedures vary according to the endocrine and tumoral contexts but should be reduced to a minimum in intense hypercortisolism. Preventive and curative treatments of cortisol-induced comorbidities, non-specific management of hypercortisolism and etiological treatments should be considered simultaneously. Therapeutic strategies vary according to (1.) the intensity of hypercortisolism, the general condition of the patient and associated comorbidities and (2.) the tumoral status, ranging from resectable ACTH secreting tumors to non-resectable metastatic endocrine tumors or occult tumors. The ideal treatment is complete excision of the ACTH-secreting tumor that can be performed rapidly or after preoperative preparation using cortisol-lowering drugs. When this is not possible, the therapeutic strategy should be discussed by a multidisciplinary experienced team in a personalized perspective and include variable combinations of pharmacological agents, bilateral adrenalectomy and non-specific tumoral interventions. Here we discuss the diagnosis and therapeutic strategies including the modern, currently available tools and emphasize on the operational effectiveness of care.


2015 ◽  
Vol 62 (4) ◽  
pp. 202-204
Author(s):  
Irene Acevedo-Báñez ◽  
Juan Luis Tirado-Hospital ◽  
Ovidio Muñiz-Grijalvo ◽  
Miguel Angel Mangas-Cruz ◽  
Francisco Javier García-Gómez

Author(s):  
K.-L. Lin ◽  
C.-Y. Chen ◽  
H.-H. Hsu ◽  
P.-F. Kao ◽  
M.-J. Huang ◽  
...  

2002 ◽  
Vol 57 (1) ◽  
pp. 23-25 ◽  
Author(s):  
S. Van Schaeybroeck ◽  
S. Van Imschoot ◽  
P. Cochez

2011 ◽  
Vol 152 (10) ◽  
pp. 403-406
Author(s):  
Attila Szabó ◽  
Péter Igaz ◽  
Róbert Kiss ◽  
Gergely Lakatos ◽  
Ibolya Varga ◽  
...  

The authors report a case of an ectopic ACTH-syndrome that resulted in severe hypercortisolism, hypokalemia, diabetes mellitus and osteoporosis. The ACTH-secreting tumor tissue was localized in the lung. The tumor was removed by segmentectomy and histological evaluation revealed an ACTH-secreting neuroendocrine tumor. After surgery, however, plasma cortisol and ACTH levels failed to decrease significantly due to subtotal tumor removal. Long-acting somatostatin analogue therapy resulted in a normalization of both plasma cortisol and ACTH levels and the clinical symptoms improved significantly. Residual tumor was removed by repeat surgery and the patient was permanently cured. Orv. Hetil., 2011, 152, 403–406.


1988 ◽  
Vol 68 (2) ◽  
pp. 205-210 ◽  
Author(s):  
John Zovickian ◽  
Edward H. Oldfield ◽  
John L. Doppman ◽  
Gordon B. Cutler ◽  
D. Lynn Loriaux

✓ Bilateral and simultaneous sampling of the inferior petrosal sinuses in patients with Cushing's disease has been used to establish the presence and laterality of adrenocorticotropic hormone (ACTH)-producing microad-enomas prior to transsphenoidal surgery. Successful preoperative lateralization depends upon equivalent dilution of pituitary venous blood on the two sides since samples which are diluted by unequal amounts of non-pituitary blood may lead to erroneous results. To assure valid sampling results, the use of other pituitary hormones, measured simultaneously, has been proposed to correct the ACTH concentrations from the inferior petrosal sinuses against unequal dilution by non-pituitary venous blood. This proposal presumes that ACTH-secreting microadenomas will not cause unequal delivery of the other pituitary hormones into the two inferior petrosal sinuses. The inferior petrosal sinus concentrations of prolactin (PRL), thyrotropin (TSH), and the alpha subunit of human chorionic gonadotropin (α-HCG) were evaluated as indicators of pituitary venous blood dilution in 11 patients with Cushing's disease. Four patients with ectopic ACTH syndrome served as controls. Blood was withdrawn simultaneously from catheters in both inferior petrosal sinuses and from a peripheral vein for measurement of ACTH, PRL, TSH, and α-HCG. The ACTH concentrations were then corrected for dilution by non-pituitary blood by dividing the ACTH concentration from each side by the ratio of the inferior petrosal sinus to peripheral blood concentrations of PRL, TSH, and α-HCG for that side. At surgery, all 11 patients had ACTH-secreting microadenomas on the side predicted by the uncorrected ACTH concentrations. However, in three patients the corrected ACTH values would have led to erroneous results. Among the 18 sets of corrected inferior petrosal sinus measurements in these three patients, the corrected ACTH values failed to show an inferior petrosal sinus gradient in six and localized the tumor to the side opposite the adenoma in four. Incorrect lateralization was obtained with each of the hormones (PRL, TSH, and α-HCG) used for correction. Furthermore, the ipsilateral (side of tumor)-to-contralateral inferior petrosal sinus gradient of ACTH in patients with Cushing's disease was generally paralleled by a significant inferior petrosal sinus gradient of PRL, TSH, and α-HCG to the side of the tumor, whereas patients with the ectopic ACTH syndrome tended not to exhibit lateralizing (side-to-side) gradients. These findings indicate that the simultaneous measurement of inferior petrosal sinus concentrations of PRL or TSH or of the glycoprotein hormone α subunit does not improve preoperative localization of ACTH-secreting microadenomas and may lead to incorrect lateralization of the tumor. The results also suggest that ACTH-secreting microadenomas cause enhanced delivery of PRL, TSH, and α subunit into the inferior petrosal sinus ipsilateral to the tumor which, in turn, may reflect paracrine stimulation of hormone secretion in the anterior pituitary gland surrounding ACTH-secreting microadenomas.


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