scholarly journals Prognostic factors in ectopic Cushing’s syndrome due to neuroendocrine tumors: a multicenter study

2017 ◽  
Vol 176 (4) ◽  
pp. 453-461 ◽  
Author(s):  
Maria Vittoria Davi’ ◽  
Elisa Cosaro ◽  
Serena Piacentini ◽  
Giuseppe Reimondo ◽  
Nora Albiger ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Prognostic Factors ◽  
Neuroendocrine Tumors ◽  
Cushing’S Syndrome ◽  
Distant Metastases ◽  
Cushing's Syndrome ◽  
Primary Role ◽  
Ectopic Acth ◽  
Term Survival ◽  
Ectopic Cushing’S Syndrome

Objective Evidence is limited regarding outcome of patients with ectopic Cushing’s syndrome (ECS) due to neuroendocrine tumors (NETs). Design We assessed the prognostic factors affecting the survival of patients with NETs and ECS. Methods Retrospective analysis of clinicopathological features, severity of hormonal syndrome, treatments from a large cohort of patients with NETs and ECS collected from 17 Italian centers. Results Our series included 110 patients, 58.2% female, with mean (±s.d.) age at diagnosis of 49.5 ± 15.9 years. The main sources of ectopic ACTH were bronchial carcinoids (BC) (40.9%), occult tumors (22.7%) and pancreatic (p)NETs (15.5%). Curative surgery was performed in 56.7% (70.2% of BC, 11% of pNETs). Overall survival was significantly higher in BC compared with pNETs and occult tumors (P = 0.033) and in G1-NETs compared with G2 and G3 (P = 0.007). Negative predictive factors for survival were severity of hypercortisolism (P < 0.02), hypokalemia (P = 0.001), diabetes mellitus (P = 0.0146) and distant metastases (P < 0.001). Improved survival was observed in patients who underwent NET removal (P < 0.001). Adrenalectomy improved short-term survival. Conclusions Multiple factors affect prognosis of ECS patients: type of NET, grading, distant metastases, severity of hypercortisolism, hypokalemia and diabetes mellitus. BCs have the highest curative surgical rate and better survival compared with occult tumors and pNETs. Hypercortisolism plays a primary role in affecting outcome and quality of life; therefore, prompt and vigorous treatment of hormonal excess by NET surgery and medical therapy should be a key therapeutic goal. In refractory cases, adrenalectomy should be considered as it affects outcome positively at least in the first 2 years.

scholarly journals Occult Carcinoid Causes Cushing’s Syndrome

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A594-A595
Author(s):  
Sabrina Meftali ◽  
Rebecca Unterborn ◽  
Amanda Gifford ◽  
Bankim Bhatt
Keyword(s):  
Weight Gain ◽  
Carcinoid Tumor ◽  
Neuroendocrine Tumors ◽  
Cushing’S Syndrome ◽  
Pulmonary Nodule ◽  
Cushing's Syndrome ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Ectopic Cushing’S Syndrome

Abstract Introduction: Lung neuroendocrine tumors (NETs) are a common cause of ectopic ACTH-secretion. Only 3% of NETs cause Cushing’s syndrome1; the majority are small and may be radiographically occult. Additionally, the responses to dexamethasone, metyrapone, and oCRF can be indistinguishable from that observed in pituitary Cushing’s disease2. These challenges lead to misdiagnosis and unnecessary procedures. Case Description: 45-year-old male presented for resection of right-sided pulmonary carcinoid tumor. He was diagnosed with Cushing’s syndrome in 2011 when he experienced uncontrolled hypertension and excessive weight gain with elevations in cortisol and ACTH levels. He underwent transsphenoidal pituitary resection in May 2011 for a possible pituitary microadenoma. Post-operative worsening hypertension, weight gain, and striae led to bilateral adrenalectomy in November 2011, complicated by retroperitoneal hemorrhage and resuscitation-induced pulmonary edema. On chest CT in early 2012, an 8 mm incidental right pulmonary nodule was documented. 8 years later, chest imaging for mild COVID-19 infection again revealed a right-sided pulmonary nodule. He underwent CT-guided biopsy of the nodule in July 2020 with pathology demonstrating carcinoid histology. Right lower lobectomy was performed. Pre-operative ACTH was 1673 pg/mL (normal: 10-60 pg/mL) and post-operative ACTH was 16 pg/mL. The resected tumor stained positive for ACTH, confirming that carcinoid tumor was the source of Cushing’s for the past decade. Conclusion: The diagnosis of ectopic Cushing’s syndrome can be elusive, leading to surgeries with significant morbidity, as seen in our patient. In one retrospective review, 14% of patients with Cushing’s had transsphenoidal resection before they were diagnosed with an ectopic source3. Ectopic Cushing’s syndrome can be a challenging diagnosis to make and a multidisciplinary approach with close collaboration between endocrinologist, radiologist, surgeon and pathologist may increase the diagnosis accuracy. Citations: 1Kamp, K, Alwani, R A, Korpershoek, E, Franssen, G., de Herder, W W, & Feelders, R A. (2016). Prevalence and clinical features of the ectopic ACTH syndrome in patients with gastroenteropancreatic and thoracic neuroendocrine tumors, European Journal of Endocrinology, 174(3), 271-280. Retrieved Jan 15, 2021, from https://eje.bioscientifica.com/view/journals/eje/174/3/271.xml 2Malchoff CD, Orth DN, Abboud C, Carney JA, Pairolero PC, Carey RM. Ectopic ACTH syndrome caused by a bronchial carcinoid tumor responsive to dexamethasone, metyrapone, and corticotropin-releasing factor. The American Journal of Medicine. 1988;84(4):760-764. doi:10.1016/0002-9343(88)90116-7 3Ioannis Ilias, David J. Torpy, Karel Pacak, Nancy Mullen, Robert A. Wesley, Lynnette K. Nieman,Cushing’s Syndrome Due to Ectopic Corticotropin Secretion: Twenty Years’ Experience at theNational Institutes of Health, The Journal of Clinical Endocrinology & Metabolism, Volume 90, Issue8, 1 August 2005, Pages 4955–4962, https://doi.org/10.1210/jc.2004-2527

scholarly journals Ectopic Cushing’s Syndrome Secondary to Metastatic Paraganglioma

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
R. Daya ◽  
C. Wingfield ◽  
P. Sotshononda ◽  
F. Seedat ◽  
S. Bulbulia ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Cushing’S Syndrome ◽  
Case Reports ◽  
Small Cell Lung Carcinoma ◽  
Cushing's Syndrome ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Cell Lung Carcinoma ◽  
Ectopic Acth Secretion ◽  
Ectopic Cushing’S Syndrome

Paraneoplastic or ectopic Cushing’s syndrome (CS) is a rare cause of endogenous hypercortisolism. It is due to ectopic adrenocorticotropic hormone (ACTH) secretion and has been reported in association with a variety of neuroendocrine tumors such as small-cell lung carcinoma, carcinoid tumors, and medullary carcinoma of the thyroid. Paragangliomas (PGLs) are rare neuroendocrine tumors that can secrete catecholamines. Case reports and reports of ectopic ACTH secretion from metastatic PGLs causing CS are exceedingly rare. We present a case of a 38-year-old female, who presented with typical signs, symptoms, and complications of CS, secondary to a PGL with widespread metastases, which eventually led to her demise.

scholarly journals A unique case of ectopic Cushing’s syndrome from a thymic neuroendocrine carcinoma

2019 ◽  
Vol 2019 ◽  
Author(s):  
Lima Lawrence ◽  
Peng Zhang ◽  
Humberto Choi ◽  
Usman Ahmad ◽  
Valeria Arrossi ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Cushing’S Syndrome ◽  
Opportunistic Infections ◽  
Cushing's Syndrome ◽  
Uncontrolled Hypertension ◽  
Ectopic Acth Syndrome ◽  
Metabolic Abnormalities ◽  
Unique Case ◽  
Ectopic Acth ◽  
Ectopic Cushing’S Syndrome

Summary Ectopic adrenocorticotropic hormone (ACTH) production leading to ectopic ACTH syndrome accounts for a small proportion of all Cushing’s syndrome (CS) cases. Thymic neuroendocrine tumors are rare neoplasms that may secrete ACTH leading to rapid development of hypercortisolism causing electrolyte and metabolic abnormalities, uncontrolled hypertension and an increased risk for opportunistic infections. We present a unique case of a patient who presented with a mediastinal mass, revealed to be an ACTH-secreting thymic neuroendocrine tumor (NET) causing ectopic CS. As the diagnosis of CS from ectopic ACTH syndrome (EAS) remains challenging, we emphasize the necessity for high clinical suspicion in the appropriate setting, concordance between biochemical, imaging and pathology findings, along with continued vigilant monitoring for recurrence after definitive treatment. Learning points: Functional thymic neuroendocrine tumors are exceedingly rare. Ectopic Cushing’s syndrome secondary to thymic neuroendocrine tumors secreting ACTH present with features of hypercortisolism including electrolyte and metabolic abnormalities, uncontrolled hypertension and hyperglycemia, and opportunistic infections. The ability to undergo surgery and completeness of resection are the strongest prognostic factors for improved overall survival; however, the recurrence rate remains high. A high degree of initial clinical suspicion followed by vigilant monitoring is required for patients with this challenging disease.

scholarly journals A rare case of duodenal carcinoid presenting as ectopic Cushing’s syndrome

2019 ◽  
Vol 6 (3) ◽  
pp. 959
Author(s):  
Siddharth Pugalendhi ◽  
Tarun Kumar Dutta ◽  
Dhivya . ◽  
Kiran Yadav
Keyword(s):  
Neuroendocrine Tumors ◽  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Somatostatin Analogues ◽  
Cushing's Syndrome ◽  
High Dose ◽  
Ectopic Acth ◽  
High Dose Dexamethasone ◽  
Ectopic Acth Production ◽  
Duodenal Carcinoid

ACTH-dependent Cushing syndrome (CS) due to an ectopic source is responsible for approximately 10-15% cases of Cushing’s syndrome. It is associated with various tumors such as small cell lung cancer and well-differentiated bronchial or gastrointestinal neuroendocrine tumors. Many a times ectopic ACTH production is difficult to manage, and identification of the source may take many years.  Hormonal diagnostics include assessments in basic conditions as well as dynamic tests, such as the high-dose dexamethasone suppression test and corticotrophin releasing hormone (CRH) stimulation test. Treatment selection depends on the type of tumor and its extent. In the case of neuroendocrine tumors, the main treatments are surgery and administration of somatostatin analogues or bilateral adrenalectomy in refractory cases and if the source remains unidentified. Here, we report a case who presented with features of Cushing’s syndrome which eventually through workup led us to a diagnosis of duodenal carcinoid producing ectopic ACTH which is extremely rare and was successfully treated.

Prospective Study of the Clinical Course, Prognostic Factors, Causes of Death, and Survival in Patients With Long-Standing Zollinger-Ellison Syndrome

1999 ◽  
Vol 17 (2) ◽  
pp. 615-615 ◽  
Author(s):  
Fang Yu ◽  
David J. Venzon ◽  
Jose Serrano ◽  
Stephan U. Goebel ◽  
John L. Doppman ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Liver Metastases ◽  
Bone Metastases ◽  
Cushing’S Syndrome ◽  
Clinical Course ◽  
Cushing's Syndrome ◽  
Ectopic Cushing’S Syndrome ◽  
Zollinger Ellison Syndrome ◽  
Large Primary

PURPOSE: The long-term clinical course of unselected patients with gastrinomas as well as other functional pancreatic endocrine tumors (PETs) in whom the excess-hormone state is controlled is largely unknown. To address this issue, patients with gastrinomas were assessed. PATIENTS AND METHODS: Two hundred twelve patients with Zollinger-Ellison syndrome (ZES) were prospectively studied. All had controlled acid hypersecretion and were assessed yearly, with a mean follow-up period of 13.8 ± 0.6 years (range, 0.1 to 31 years). Annual assessments of possible factors that might affect prognosis or treatment approaches were performed, such as those for tumor size and location; the presence, location, and extent of metastases; and the occurrence of ectopic Cushing's syndrome or another PET syndrome. Deaths were categorized as ZES-related or non–ZES-related and classified into different causes. RESULTS: Thirty-one percent of patients died, all of non–acid-related causes. One half died of a ZES-related cause; they differed from those who died of non–ZES deaths by having a large primary tumor, more frequently a pancreatic tumor; lymph node, liver, or bone metastases; ectopic Cushing's syndrome; or higher gastrin levels. The extent of liver metastases correlated with survival rate. The presence of liver metastases alone only moderately decreased survival time; however, the additional development of bone metastases or ectopic Cushing's syndrome markedly decreased survival rate. CONCLUSIONS: In ZES, gastrinoma growth is now the main single determinant of long-term survival, with one half of patients dying a gastrinoma-related death and none an acid-related death. Large primary tumors that are pancreatic in location, the development of liver metastases, (especially if associated with bone metastases or Cushing's syndrome), and the extent of liver metastases are all important prognostic factors. The identification of these factors allows the recognition of subgroups that can be used to tailor antitumor treatment approaches.

scholarly journals Vandetanib induces a marked anti-tumor effect and amelioration of ectopic Cushing’s syndrome in a medullary thyroid carcinoma patient

2016 ◽  
Vol 2016 ◽  
Author(s):  
Hashem Bseiso ◽  
Naama Lev-Cohain ◽  
David J Gross ◽  
Simona Grozinsky-Glasberg
Keyword(s):  
Cushing’S Syndrome ◽  
Tumor Size ◽  
Cushing's Syndrome ◽  
Whole Body ◽  
List Type ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Ectopic Cushing’S Syndrome ◽  
Free Cortisol

Summary A 55-year-old woman diagnosed with sporadic MTC underwent total thyroidectomy 20 years ago. After the first surgery, elevated calcitonin levels in parallel with local disease persistence were noted and therefore she underwent repeated neck dissections. During follow-up, multiple foci of metastatic disease were noted in the neck and mediastinal lymph nodes, lungs and bones; however, the disease had an indolent course for a number of years, in parallel with a calcitonin doubling time of more than two years and without significant symptoms. During a routine follow-up visit 2 years ago, findings suggestive of Cushing’s syndrome were observed on physical examination. The biochemical evaluation demonstrated markedly elevated serum calcitonin level, in parallel with lack of cortisol suppression after an overnight 1 mg dexamethasone suppression test, lack of cortisol and ACTH suppression after high-dose IV dexamethasone 8 mg, elevated plasma ACTH up to 79 pg/mL (normal <46 pg/mL) and elevated 24-h urinary free cortisol up to 501 µg/24 h (normal 9–90 µg/24 h). After a negative pituitary MRI, she underwent IPSS, which was compatible with EAS. Whole-body CT demonstrated progressive disease at most of the tumor sites. Treatment with vandetanib at a dosage of 200 mg/day was commenced. The patient showed a significant, rapid and consistent clinical improvement already after two months of treatment, in parallel with biochemical improvement, whereas a decrease in tumor size was demonstrated on follow-up CT. Learning points: Ectopic Cushing’s syndrome due to ectopic ACTH secretion (EAS) by MTC is an uncommon and a poor prognostic event, being associated with significant morbidity and mortality. We demonstrate that vandetanib is effective in controlling the signs and symptoms related to the EAS in patients with advanced progressive MTC. We demonstrate that vandetanib is effective in decreasing tumor size and in inducing tumor control.

scholarly journals Fluconazole and acetazolamide in the treatment of ectopic Cushing's syndrome with severe metabolic alkalosis

2015 ◽  
Vol 2015 ◽  
Author(s):  
Verena Schwetz ◽  
Felix Aberer ◽  
Claudia Stiegler ◽  
Thomas R Pieber ◽  
Barbara Obermayer-Pietsch ◽  
...  
Keyword(s):  
Case Report ◽  
Surgical Resection ◽  
Cushing’S Syndrome ◽  
Metabolic Alkalosis ◽  
Cushing's Syndrome ◽  
List Type ◽  
Ectopic Acth ◽  
Ectopic Cushing’S Syndrome ◽  
Ectopic Acth Production ◽  
Learning Points

Summary Cushing's syndrome (CS) due to ectopic ACTH production accounts for about 10% of all types of CS and is frequently associated with metabolic alkalosis. Treatment of CS involves surgical resection and/or medical therapy to control hypercortisolism. We present the case of an 80-year-old woman affected by CS due to an unknown cause. The patient had severe metabolic alkalosis with refractory hypokalemia. To treat the underlying CS, fluconazole was initiated due to unavailability of ketoconazole. In spite of markedly decreasing cortisol levels, metabolic alkalosis persisted. Treatment of metabolic alkalosis with acetazolamide was thus initiated and pH levels successfully lowered. This case report shows that hypercortisolism can be effectively treated with fluconazole in cases where ketoconazole is unavailable or not tolerated and that persistent severe metabolic alkalosis caused by glucocorticoid excess can be safely and successfully treated with acetazolamide. Learning points Hypercortisolism can be effectively treated with fluconazole where ketoconazole is unavailable or not tolerated. Glucocorticoid excess can cause severe metabolic alkalosis. Persistent severe metabolic alkalosis can be safely and successfully treated with acetazolamide.

scholarly journals Severe Cyclical Hypercortisolism Due to Metastatic Neuroendocrine Cancer Initially Diagnosed as Colon Cancer

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A156-A157
Author(s):  
John Chen Liu ◽  
Richa Patel ◽  
Rajani Gundluru ◽  
Joseph Theressa Nehu Parimi ◽  
Timur Gusov ◽  
...  
Keyword(s):  
Colon Cancer ◽  
Liver Biopsy ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Initial Diagnosis ◽  
Chemotherapy Response ◽  
Ectopic Acth ◽  
Neuroendocrine Cancer ◽  
Ectopic Cushing’S Syndrome ◽  
Cortisol Levels

Abstract Case Description: A 65 year-old male with initial diagnosis of metastatic colon cancer based on liver biopsy presented for evaluation of severe hypercortisolism with 24-hour urinary free cortisol (UFC) of 1184 mcg/day after presenting to his Oncologist in December 2019 requesting evaluation for muscle weakness and weight gain. On re-evaluation in March, his cortisol had dramatically improved with 24-hr UFC of 120 mcg/day and ACTH of 84 pg/mL and had resolved with 24-hr UFC of 32.6 mcg/day by May. A 2 cm pancreatic lesion was noted during cancer staging; a second opinion was obtained on the liver biopsy which was interpreted as metastatic neuroendocrine tumor, possibly of pancreatic origin. The patient was started on FOLFINIRNOX in December 2019. This was transitioned to carboplatin and etoposide after the revised diagnosis and was continued through May with regression of liver lesions on followup imaging. A 68 Ga-DOTATATE PET CT confirmed uptake in the pancreas, retroperitoneal lymph nodes, and thyroid. A thyroid ultrasound was obtained which did not meet FNA criteria. Given the chronologic association between cortisol levels and chemotherapy response we were suspicious for ectopic Cushing’s syndrome due to metastatic neuroendocrine cancer and the patient was followed for recurrence. In September, the patient endorsed leg weakness and hyperglycemia and was found to have a 24-hr UFC of 4731 mcg/day and an 8 am cortisol of 102 mcg/dL. The patient developed severe hypokalemia requiring admission and was restarted on chemotherapy with moderate improvement in cortisol levels and symptoms. Given the degree of cortisol elevation, we recommended bilateral adrenalectomy, for which the patient established care at a more specialized center. Discussion: Ectopic Cushing’s syndrome is an uncommon variety of ACTH-dependent Cushing’s syndrome caused by CRH or ACTH-producing tumors. The more aggressive form is associated with small-cell lung cancer while the chronic form is associated with indolent tumors. Cyclical Cushing’s syndrome is a rare cause of hypercortisolism characterized by cyclical variations in cortisol levels. The pathophysiology of the cyclical variation is unclear. About half of cases are caused by pituitary corticotrophs but up to 26% can be attributed to ectopic ACTH production, typically due to indolent tumors rather than aggressive malignancies. We present a case of cyclical hypercortisolism with an initial diagnosis of colon cancer. On further review, the cyclical hypercortisolism was likely associated with chemotherapy response. Reevaluation of surgical pathology was consistent with metastatic neuroendocrine cancer, the likely source of ectopic ACTH. Our case highlights the importance of maintaining a broad differential when considering cases of cyclical cortisol elevation, including consideration of concurrent chemotherapy for seemingly unrelated malignancies.

scholarly journals Demographic Characteristics, Etiology, and Comorbidities of Patients with Cushing’s Syndrome: A 10-Year Retrospective Study at a Large General Hospital in China

2019 ◽  
Vol 2019 ◽  
pp. 1-10 ◽  
Author(s):  
Jingya Zhou ◽  
Meng Zhang ◽  
Xue Bai ◽  
Shengnan Cui ◽  
Cheng Pang ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Fatty Liver ◽  
Cushing’S Syndrome ◽  
Osteoporotic Fractures ◽  
Cushing's Syndrome ◽  
Demographic Characteristics ◽  
Ectopic Acth Syndrome ◽  
Adrenocortical Adenoma ◽  
Ectopic Acth ◽  
Icd 10

Purpose. To investigate the demographic characteristics, etiology, and comorbidities of Cushing’s syndrome (CS) patients at a large medical center in China. Methods. Records on CS patients discharged from 2008 to 2017 were retrieved from the hospital discharge abstract database (DAD) using ICD-10 codes. Demographic characteristics, etiology, and comorbidity data were analyzed. Results. Cushing’s disease (CD) accounted for 63.0% of CS patients, followed by adrenocortical adenoma (ACA) (20.9%), primary bilateral macronodular adrenal hyperplasia (BMAH) (6.2%), ectopic ACTH syndrome (EAS) (5.9%), primary pigmented nodular adrenocortical disease (PPNAD) (1.8%), and adrenocortical carcinoma (ACC) (1.0%). CD, ACA, ACC, and PPNAD presented marked preponderances in women (4.1 : 1, 10.5 : 1, 4.3 : 1, and 2.3 : 1, respectively), while BMAH (59.8%) and EAS (51.0%) showed slightly higher preponderances in men. CD patients were younger than ACA and EAS patients (36.1±12.9 years vs. 39.4±12.7 years and 36.1±12.9 years vs. 41.0±15.8, P<0.001); PPNAD patients were the youngest (24.2±10.8 years, P<0.001), and BMAH patients were the oldest (51.3±9.9 years, P<0.001). Hypertension, diabetes mellitus, osteoporosis without fractures, osteoporotic fractures, dyslipidemia, and fatty liver occurred more frequently in CD patients than in ACA patients (P<0.001 for all). Osteoporotic fractures were observed more frequently in PPAND than in ACA (26.7% vs. 9.0%, P<0.001) and BMAH (26.7% vs. 4.9%, P<0.001) patients. EAS patients had more severe and diverse comorbidities, with higher prevalences of hypokalemia (52.0%), diabetes mellitus (61.2%), and osteoporotic fractures (28.6%). When adjusted for age, male CD patients were associated with hypertension (OR = 2.266, 95% CI: 1.524–3.371, and P<0.001), osteoporotic fractures (OR = 2.274, 95% CI: 1.568–3.298, and P<0.001), fatty liver (OR = 1.435, 95% CI: 1.028–2.003, and P=0.034), and hypokalemia (OR = 1.944, 95% CI: 1.280–2.951, and P=0.002). Conclusions. The proposed method efficiently evaluates CS patients’ epidemiological profiles using hospital DADs with ICD-10 codes and thus may enrich the limited epidemiological data and contribute to clinical practice for CS.

Occult eutopic Cushing's syndrome--failure of simultaneous bilateral petrosal sinus sampling to diagnose pituitary-dependent Cushing's syndrome

1997 ◽  
pp. 74-78 ◽  
Author(s):  
C Heppner ◽  
K Becker ◽  
W Saeger ◽  
RW Gunther ◽  
B Allolio ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Carcinoid Tumour ◽  
Cushing's Syndrome ◽  
High Dose ◽  
Acth Secretion ◽  
Cell Adenoma ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Ectopic Cushing’S Syndrome ◽  
Lysine Vasopressin

Simultaneous bilateral inferior petrosal sinus (IPS) sampling has been repeatedly proposed to be a highly specific approach for the diagnosis of Cushing's disease and 100% sensitivity in detecting autonomous pituitary ACTH secretion by an adenoma has been reported in a large series. We now report on a patient suffering from ACTH-dependent Cushing's syndrome in whom repeated bilateral IPS sampling failed to detect a central/peripheral gradient diagnostic for autonomous pituitary ACTH secretion during initial evaluation. Applying lysine vasopressin as the corticotroph secretatogue, the maximum central/peripheral gradient was 1.0 before and 1.1 following stimulation. Moreover, results of high dose dexamethasone and corticotrophin releasing hormone administration suggested ectopic ACTH secretion. Since thorough diagnostic procedures failed to localise a suspected carcinoid tumour, occult ectopic Cushing's syndrome was diagnosed. Eight years later, a pituitary macroadenoma was detected by magnetic resonance imaging (MRI), IPS catheterisation then revealed a maximal central/ peripheral gradient of 9.3 before and 20.4 after the intravenous administration of lysine vasopressin. Resected tumour tissue was classified as a typical densely granulated ACTH cell adenoma. We conclude that repeated MRI scans should be included in the follow-up of patients with a diagnosis of occult ectopic Cushing's syndrome to avoid the risk of overlooking 'occult eutopic Cushing's syndrome'.

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