scholarly journals The role of 68Ga-DOTA derivatives PET-CT in patients with ectopic ACTH syndrome

2020 ◽  
Vol 9 (4) ◽  
pp. 337-345
Author(s):  
Filippo Ceccato ◽  
Diego Cecchin ◽  
Michele Gregianin ◽  
Giacomo Ricci ◽  
Cristina Campi ◽  
...  
Keyword(s):  
Small Cell Lung Carcinoma ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Cell Lung Carcinoma ◽  
Ectopic Acth Secretion ◽  
Bronchial Carcinoids ◽  
Pet Ct ◽  
Pancreatic Net ◽  
Acth Secreting

Introduction and aim Ectopic ACTH secretion (EAS) is mostly secondary to thoracic/abdominal neuroendocrine tumours (NETs) or small cell-lung carcinoma (SCLC). We studied the diagnostic accuracy of CT with 68Ga-Dota derivatives (68Ga-SSTR) PET in localizing ACTH-secreting tumor in patients with EAS. Materials and methods 68Ga-SSTR-PET/CT was performed and compared with the nearest enhanced CT in 18 cases (16 primary and 2 recurrent neoplasms). Unspecific, indeterminate and false-positive uptakes were assessed using conventional imaging, follow-up or histology. Results We diagnosed 13 thoracic (9 primary and 2 recurrent bronchial carcinoids, 2 SCLCs) and 1 abdominal (pancreatic NET) tumors. Eight ACTH-secreting tumors were promptly identified at EAS diagnosis (’overt’, four pulmonary carcinoids with two recurrences and two SCLC); six EAS have been discovered during the subsequent follow-up (’covert’, five bronchial carcinoids and one pancreatic NET). At the time of EAS diagnosis, imaging was able to correctly detect the ACTH-secreting tumour in 8/18 cases (6 new diagnosis and 2 recurrences). During the follow-up, six out of initially ten ‘occult’ cases became ‘covert’. At last available follow-up, CT and 68Ga-SSTR-PET/CT were able to diagnose 11/18 and 12/18 ACTH-secreting tumours, respectively (11/14 and 12/14 considering only overt and covert cases, respectively). Four cases have never been localized by conventional or nuclear imaging (’occult EAS’), despite an average follow-up of 5 years. Conclusion The 68Ga-SSTR-PET/CT is useful in localizing EAS, especially to enhance positive prediction of the suggestive CT lesions and to detect occult neoplasms.

scholarly journals Case Report: A Challenging Localization of a Pulmonary Ectopic ACTH-Secreting Tumor in a Patient With Severe Cushing’s Syndrome

2021 ◽  
Vol 12 ◽  
Author(s):  
Andreea Liliana Serban ◽  
Lorenzo Rosso ◽  
Paolo Mendogni ◽  
Arianna Cremaschi ◽  
Rita Indirli ◽  
...  
Keyword(s):  
Ct Scan ◽  
Rectal Carcinoma ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Pet Ct ◽  
The Right ◽  
Acth Secreting

BackgroundEctopic adrenocorticotropic syndrome (EAS) is a rare cause of endogenous ACTH-dependent Cushing’s syndrome, usually associated with severe hypercortisolism as well as comorbidities. Tumor detection is still a challenge and often requires several imaging procedures. In this report, we describe a case of an ectopic ACTH secretion with a misleading localization of the responsible tumor due to a concomitant rectal carcinoma.Case presentationA 49-year-old man was referred to our Endocrinology Unit due to suspicion of Cushing’s syndrome. His medical history included metastatic rectal adenocarcinoma, diagnosed 5 years ago and treated with adjuvant chemotherapy, radiotherapy and surgical resection. During follow-up, a thoracic computed tomography scan revealed two pulmonary nodules located in the superior and middle lobes of the right lung with a diameter of 5 and 10 mm, respectively. However, these nodules remained radiologically stable thereafter and were not considered relevant. All biochemical tests were suggestive of EAS (basal ACTH levels: 88.2 ng/L, nv 0–46; basal cortisol levels: 44.2 µg/dl, nv 4.8–19.5; negative response to CRH test and high dose dexamethasone suppression test) and radiological localization of the ectopic ACTH-secreting tumor was scheduled. The CT scan revealed a dimensional increase of the right superior lung nodule (from 5 to 12 mm). [68Ga]-DOTA-TOC PET/CT scan was negative, while [18F]-FDG-PET/CT showed a tracer accumulation in the superior nodule. After a multidisciplinary consultation, the patient underwent thoracic surgery that started with two atypical wedge resections of nodules. Frozen section analyses showed a neuroendocrine tumor on the right middle lobe nodule and a metastatic colorectal adenocarcinoma on the superior lesion. Then, a right superior nodulectomy and a right middle lobectomy with mediastinal lymphadenectomy were performed. The final histopathological examination confirmed a typical carcinoid tumor, strongly positive for ACTH. A post-surgical follow-up showed a persistent remission of Cushing’s syndrome.ConclusionsThe present report describes a case of severe hypercortisolism due to EAS not detected by functional imaging methods, in which the localization of ACTH ectopic origin was puzzled by a concomitant metastatic rectal carcinoma. The multidisciplinary approach was crucial for the management of this rare disease.

scholarly journals MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

2020 ◽  
Vol 182 (4) ◽  
pp. R29-R58 ◽  
Author(s):  
Jacques Young ◽  
Magalie Haissaguerre ◽  
Oceana Viera-Pinto ◽  
Olivier Chabre ◽  
Eric Baudin ◽  
...  
Keyword(s):  
Diagnostic Procedures ◽  
Therapeutic Strategies ◽  
Therapeutic Interventions ◽  
Ectopic Acth Syndrome ◽  
Endocrine Tumors ◽  
Severe Condition ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Acth Secreting ◽  
Specific Management

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.

scholarly journals Prevalence and clinical features of the ectopic ACTH syndrome in patients with gastroenteropancreatic and thoracic neuroendocrine tumors

2016 ◽  
Vol 174 (3) ◽  
pp. 271-280 ◽  
Author(s):  
K Kamp ◽  
R A Alwani ◽  
E Korpershoek ◽  
G J H Franssen ◽  
W W de Herder ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Small Cell Lung Carcinoma ◽  
Large Cohort ◽  
Ectopic Acth Syndrome ◽  
Gastroenteropancreatic Neuroendocrine Tumors ◽  
Ectopic Acth ◽  
Cell Lung Carcinoma ◽  
Tertiary Referral Center ◽  
Relative Contribution

ObjectiveSeveral series report on the relative contribution of ectopic ACTH syndrome (EAS) in the spectrum of Cushing's syndrome. However, prevalence of EAS in patients with thoracic or gastroenteropancreatic neuroendocrine tumors (GEP-NETs) is currently unknown.Design We assessed, in a tertiary referral center, the prevalence of EAS in a large cohort of thoracic and GEP-NET patients including clinical, biochemical, and radiological features; management; and treatment outcome.Methods In total, 918 patients with thoracic or GEP-NETs were studied (1993–2012). Multiple endocrine neoplasia type 1 and small cell lung carcinoma patients were excluded. Differentiation between synchronous, metachronous, and cyclic occurrence of EAS was made.Results Out of the 918 patients with thoracic and GEP-NETs (469 males and 449 females; median age 58.7 years (range: 17.3–87.3)), 29 patients (3.2%) had EAS (ten males and 19 females; median age 48.1 years (range: 24.7–77.9)). EAS occurred synchronously in 23 patients (79%), metachronously in four patients (14%), and cyclical in two patients (7%) respectively. NETs causing EAS included lung/bronchus (n=9), pancreatic (n=9), and thymic (n=4). In four patients, the cause of EAS was unknown (n=4). Median overall survival (OS) of non-EAS thoracic and GEP-NET patients was 61.2 months (range: 0.6–249.4). Median OS of EAS patients was 41.4 months (range: 2.2–250.9). After comparison, only the first 5-year survival was significantly shorter (P=0.013) in EAS patients.Conclusion Prevalence of EAS in this large cohort of patients with thoracic and GEP-NETs was 3.2%. EAS was mostly caused by thoracic and pancreatic NETs. First 5-year survival of EAS patients was shorter compared with non-EAS patients.

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 Clinical and laboratory characteristics and results of treatment of patients with ACTH-producing neuroendocrine tumors of various localization

2021 ◽  
Vol 93 (10) ◽  
pp. 1171-1178
Author(s):  
Olga O. Golounina ◽  
Zhanna E. Belaya ◽  
Liudmila Ya. Rozhinskaya ◽  
Evgeniya I. Marova ◽  
Michail Yu. Pikunov ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Fatal Outcome ◽  
Distant Metastases ◽  
Multiple Organ ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Results Of Treatment ◽  
Single Center Study ◽  
Pancreatic Net

Aim. To study the clinical, biochemical characteristics, treatment results and follow-up of patients with ectopic ACTH syndrome EAS (ACTH adrenocorticotropic hormone ). Materials and methods. A retrospective, observational, single-center study of 130 patients with EAS. Demographic information of patients, medical history, results of laboratory and instrumental investigations at the pre- and postoperative stages and follow-up of EAS were analyzed. Results. The mean age at the diagnosis ranged from 12 to 74 years (Me 40 years [28; 54]). The duration of the disease from the onset of symptoms to the verification of the diagnosis varied from 2 to 168 months (Me 17.5 months [7; 46]). Eighty-one (62,3%) patients had bronchopulmonary NET, 9 thymic carcinoid, 7 pancreatic NET, 5 pheochromocytoma, 1 cecum NET, 1 appendix carcinoid tumor, 1 medullary thyroid cancer and 25 (19.2%) had an occult source of ACTH. The median follow-up period of patients was 27 months [9.75; 61.0] with a maximum follow-up of 372 months. Currently, primary tumor was removed in 82 (63.1%) patients, bilateral adrenalectomy was performed in 23 (18%) patients, in 16 of them there was an occult source of ACTH-producing NET and in 7 patients in order to control hypercortisolism after non-successful surgical treatment. Regional and distant metastases were revealed in 25 (19.2%) patients. At the time of the last observation 59 (72%) patients were exhibited a full recovery, 12 (14.6%) had relapse of the disease and 26 (20%) died from multiple organ failure (n=18), pulmonary embolism (n=4), surgical complications (n=2), disseminated intravascular coagulation syndrome (n=1) or COVID-19 (n=1). Conclusion. In our cohort of patients bronchopulmonary NET are the most frequent cause of EAS (62.3%). Surgical treatment leads to remission of hypercortisolism in 72% cases; the proportion of relapse (14.6%) and fatal outcome (20%) remains frequent in EAS.

scholarly journals Imaging in Cushing's syndrome

2007 ◽  
Vol 51 (8) ◽  
pp. 1319-1328 ◽  
Author(s):  
Anju Sahdev ◽  
Rodney H. Reznek ◽  
Jane Evanson ◽  
Ashley B. Grossman
Keyword(s):  
Cushing’S Syndrome ◽  
Imaging Modality ◽  
Small Cell Lung Carcinoma ◽  
Cushing's Syndrome ◽  
Main Step ◽  
Ectopic Acth ◽  
Cell Lung Carcinoma ◽  
Non Invasive ◽  
Ectopic Acth Production ◽  
Acth Secreting

Once the diagnosis of Cushing's syndrome (CS) has been established, the main step is to differentiate between ACTH dependent and independent disease. In adults, 80% of CS is due to ACTH-dependent causes and 20% due to adrenal causes. ACTH-secreting neoplasms cause ACTH-dependent CS. These are usually anterior pituitary microadenomas, which result in the classic Cushing's disease. Non-pituitary ectopic sources of ACTH, such as a small-cell lung carcinoma or carcinoid tumours, are the source of the remainder of ACTH-dependent disease. In the majority of patients presenting with clinical and biochemical evidence of CS, modern non-invasive imaging can accurately and efficiently provide the cause and the nature of the underlying pathology. Imaging is essential for determining the source of ACTH in ectopic ACTH production, locating the pituitary tumours and distinguishing adrenal adenomas, carcinomas and hyperplasias. In our chapter we review the adrenal appearances in ACTH-dependent and ACTH-independent CS. We also include a discussion on the use of MRI and CT for the detection and management of pituitary ACTH secreting adenomas. CT of the chest, abdomen and pelvis with intravenous injection of contrast medium is the most sensitive imaging modality for the identification of the ectopic ACTH source and detecting adrenal pathology. MRI is used for characterising adrenal adenomas, problem solving in difficult cases and for detecting ACTH-secreting pituitary adenomas.

Severe Cushing's syndrome due to ectopic ACTH secretion from small cell lung carcinoma with adrenal metastases

2021 ◽  
Author(s):  
Aristidis Diamantopoulos ◽  
Marina Koulenti ◽  
Eirini Sampanioti ◽  
Eirini Evangelatou ◽  
Efstathios Dimitropoulos ◽  
...  
Keyword(s):  
Lung Carcinoma ◽  
Small Cell Lung Carcinoma ◽  
Cushing's Syndrome ◽  
Small Cell ◽  
Small Cell Lung ◽  
Adrenal Metastases ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Cell Lung Carcinoma ◽  
Ectopic Acth Secretion

scholarly journals Ectopic Cushing’s Syndrome Unveiling a Metastatic Parotid Carcinoma

2019 ◽  
Vol 2019 ◽  
pp. 1-7
Author(s):  
Sofia Castro Oliveira ◽  
João Sérgio Neves ◽  
Pedro Souteiro ◽  
Sandra Belo ◽  
Ana Isabel Oliveira ◽  
...  
Keyword(s):  
Parotid Gland ◽  
Clinical Course ◽  
Cushing Syndrome ◽  
Present Report ◽  
Small Cell Lung Carcinoma ◽  
Somatostatin Receptor ◽  
Ectopic Acth ◽  
Cell Lung Carcinoma ◽  
Ectopic Acth Secretion ◽  
First Case

Introduction. Adrenocorticotropic hormone (ACTH) ectopic production is a rare cause of Cushing syndrome (CS). The most commonly associated tumours are small-cell lung carcinoma along with bronchial and thymic carcinoids. To date, only 5 cases have been published in the literature featuring ectopic ACTH secretion from metastatic acinic cell carcinoma (ACC) of the parotid gland. We hereby describe a very uncommon case of ectopic CS (ECS) unveiling a metastatic parotid ACC. Case Presentation. A 46-year-old man with hypertension and dyslipidemia diagnosed 4-months before, as well as new-onset diabetes mellitus unveiled 1-month earlier, was referred to emergency department for hypokalemia. Hormonal study and dynamic biochemical tests performed indicated ECS. Imaging and cytological findings pointed toward a likely primary right parotid malignancy with liver metastases. Somatostatin receptor scintigraphy has shown an increased uptake in the parotid gland and mild expression in liver metastasis. The patient underwent right parotidectomy, and histopathologic examination confirmed ACC. Meanwhile, hypercortisolism was managed with metyrapone, ketoconazole, and lanreotide. Despite chemotherapy onset, a rapid disease progression and clinical course deterioration was observed. Conclusion. The present report highlights a rare ECS, exposing a metastatic parotid ACC, with an aggressive and challenging clinical course, representing the first case whose diagnosis of ECS came prior to ACC.

scholarly journals Ectopic ACTH syndrome

2007 ◽  
Vol 51 (8) ◽  
pp. 1217-1225 ◽  
Author(s):  
Andrea M. Isidori ◽  
Andrea Lenzi
Keyword(s):  
Small Cell Lung Carcinoma ◽  
Ectopic Acth Syndrome ◽  
Extensive Experience ◽  
Cross Sectional ◽  
Ectopic Acth ◽  
Cell Lung Carcinoma ◽  
Common Causes ◽  
Cross Sectional Imaging ◽  
The Common ◽  
Best Localization

Ectopic adrenocorticotropic secretion (EAS) is responsible for 12-17% of cases of Cushing's syndrome (CS) and covers a range of tumours, from undetectable benign lesions to widespread metastases. The syndrome is often associated with severe hypercortisolaemia, which aggravates the underlying condition. EAS requires a complete workup that includes the establishment of endogenous CS, diagnosis of adrenocorticotropic hormone (ACTH) dependency, localization of the source of ACTH secretion and rapid biochemical control of hypercortisolaemia. Dynamic endocrine tests should include inferior petrosal sinus sampling with CRH stimulation. Localization studies depend on the availability of reliable high-resolution cross-sectional imaging. This systematic review of the largest published series of patients with EAS (over 380 patients) reveals the common trends in the prevalence and management of this syndrome. The concept of 'occult' EAS has been revisited and the terms 'overt' and 'covert' EAS introduced. In addition to small cell lung carcinoma, the most common causes of ectopic EAS are bronchial carcinoids, thymic tumours, islet cell tumour of the pancreas, medullary thyroid carcinomas, and phaeochromocytomas. Their prevalence and the best localization modalities are presented. Medical and surgical management is discussed on the basis of the extensive experience of major referral centres.

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