scholarly journals An Ectopic ACTH Secreting Metastatic Parotid Tumour

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Thomas Dacruz ◽  
Atul Kalhan ◽  
Majid Rashid ◽  
Kofi Obuobie
Keyword(s):  
Neuroendocrine Tumours ◽  
Case Reports ◽  
Surgical Excision ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Delay In Diagnosis ◽  
Parotid Tumour ◽  
Original Tumour ◽  
Ectopic Adrenocorticotropic Hormone ◽  
Acth Secreting

A 60-year old woman presented with features of Cushing’s syndrome (CS) secondary to an ectopic adrenocorticotropic hormone (ACTH) secreting metastatic parotid tumour 3 years after excision of the original tumour. She subsequently developed fatal intestinal perforation and unfortunately died despite best possible medical measures. Ectopic ACTH secretion accounts for 5–10% of all patients presenting with ACTH dependent hypercortisolism; small cell carcinoma of lung (SCLC) and neuroendocrine tumours (NET) account for the majority of such cases. Although there are 4 previous case reports of ectopic ACTH secreting salivary tumours in literature, to our knowledge this is the first published case report in which the CS developed after 3 years of what was deemed as a successful surgical excision of primary salivary tumour. Our patient initially had nonspecific symptoms which may have contributed to a delay in diagnosis. Perforation of sigmoid colon is a recognised though underdiagnosed complication associated with steroid therapy and hypercortisolism. This case demonstrates the challenges faced in diagnosis as well as management of patients with CS apart from the practical difficulties faced while trying to identify source of ectopic ACTH.

scholarly journals The value of corticotropin-releasing hormone (CRH) test for differential diagnosis of Cushing’s syndrome

2007 ◽  
Vol 135 (1-2) ◽  
pp. 31-37 ◽  
Author(s):  
Zorana Penezic ◽  
Milos Zarkovic ◽  
Svetlana Vujovic ◽  
Miomira Ivovic ◽  
Biljana Beleslin ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Roc Curve ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Test Sensitivity ◽  
Basal Cortisol ◽  
Area Under Roc Curve ◽  
Curve Method ◽  
Acth Secreting

Introduction: Diagnosis and differential diagnosis of Cushing?s syndrome (CS) remain considerable challenge in endocrinology. For more than 20 years, CRH has been widely used as differential diagnostic test. Following the CRH administration, the majority of patients with ACTH secreting pituitary adenoma show a significant rise of plasma cortisol and ACTH, whereas those with ectopic ACTH secretion characteristically do not. Objective The aim of our study was to assess the value of CRF test for differential diagnosis of CS using the ROC (receiver operating characteristic) curve method. Method A total of 30 patients with CS verified by pathological examination and postoperative testing were evaluated. CRH test was performed within diagnostic procedures. ACTH secreting pituitary adenoma was found in 18, ectopic ACTH secretion in 3 and cortisol secreting adrenal adenoma in 9 of all patients with CS. Cortisol and ACTH were determined -15, 0, 15, 30, 45, 60, 90 and 120 min. after i.v. administration of 100?g of ovine CRH. Cortisol and ACTH were determined by commercial RIA. Statistical data processing was done by ROC curve analysis. Due to small number, the patients with ectopic ACTH secretion were excluded from test evaluation by ROC curve method. Results In evaluated subgroups, basal cortisol was (1147.3?464.3 vs. 1589.8?296.3 vs. 839.2?405.6 nmol/L); maximal stimulated cortisol (1680.3?735.5 vs. 1749.0?386.6 vs. 906.1?335.0 nmol/L); and maximal increase as a percent of basal cortisol (49.1?36.9 vs. 9.0?7.6 vs. 16.7?37.3 %). Consequently, basal ACTH was (100.9 ?85.0 vs. 138.0?123.7 vs. 4.8?4.3 pg/mL) and maximal stimulated ACTH (203.8 ?160.1 vs. 288.0?189.5 vs. 7.4?9.2 pg/mL). For cortisol, determination area under ROC curve was 0.815?0.083 (CI 95% 0.652-0.978). For cortisol increase cut-off level of 20%, test sensitivity was 83%, with specificity of 78%. For ACTH, determination area under ROC curve was 0.637?0.142 (CI 95% 0.359-0.916). For ACTH increase cut-off level of 30%, test sensitivity was 70%, with specificity of 57%. Conclusion Determination of cortisol and ACTH levels in CRH test remains reliable tool in differential diagnosis of Cushing?s syndrome.

scholarly journals An atypical case of ectopic ACTH syndrome in an adolescent boy

2020 ◽  
Vol 2020 (3) ◽  
Author(s):  
Shreya Sharma ◽  
Rajesh Joshi
Keyword(s):  
Abdominal Pain ◽  
Adrenocorticotropic Hormone ◽  
Neuroendocrine Tumours ◽  
Ectopic Acth Syndrome ◽  
Ectopic Acth ◽  
Atypical Case ◽  
Intermittent Abdominal Pain ◽  
Pancreatic Net ◽  
Pancreatic Neuroendocrine Tumours ◽  
Ectopic Adrenocorticotropic Hormone

Abstract Ectopic adrenocorticotropic hormone (ACTH) syndrome (EAS) is exceedingly rare in children and scarcely reported. Pancreatic neuroendocrine tumours (NETs) can rarely lead to secretion of ectopic ACTH. A 14-year-old boy presented with hyperpigmentation, hypertension and intermittent abdominal pain, and was diagnosed with endogenous hypercortisolism. An incidental pancreatic mass discovered on routine ultrasonogram (USG) revealed the source of ACTH. He underwent successful excision of the mass with resolution of hypercortisolism. The histopathology revealed a Pancreatic NET and immunohistochemistry was positive for ACTH stain.

scholarly journals SUN-938 Rare Case of Ectopic Cushing Syndrome Caused by ACTH Secreting Thymic Neuroendocrine Tumor in a Patient with Multiple Endocrine Neoplasia Type 1

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Yamuna Gorantla ◽  
Jorge Soria Moncada ◽  
Juan Sarmiento ◽  
Ambika Amblee ◽  
Malini Ganesh
Keyword(s):  
Neuroendocrine Tumor ◽  
Poor Prognosis ◽  
Rare Case ◽  
Cushing Syndrome ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Multiple Tumors ◽  
Men 1 ◽  
Acth Secreting

Abstract Introduction Cushing syndrome (CS) represents an uncommon manifestation of MEN1 and can be caused by both ACTH dependent or independent etiologies. Among them, ectopic ACTH secretion from a Thymic neuroendocrine tumor (TNET) in MEN1 is rare, with very few cases reported so far in literature. We report a case of Ectopic Cushing syndrome (ECS) in a MEN1 patient (pt) with multiple tumors, secondary to ACTH-secreting TNET. Case description: A 44 year old male presented to our institution for nausea, vomiting, dizziness. He had initial workup which revealed multiple tumors (papillary thyroid cancer, thymic mass, parathyroid adenomas, bilateral adrenal nodules, macroprolactinoma, peripancreatic nodules). Given concern for MEN 1, genetic testing was performed which was confirmative. Hormonal workup at this time for adrenal nodules was negative including low dose dexamethasone suppression test(DST). The immobile thymic mass was found to be poorly differentiated NET on biopsy with Ki-67 >50% with vascular invasion and adhesions to lung/chest wall on VATS, not amenable to surgery. The pt declined chemotherapy and radiotherapy due to poor social support. Six months later, he presented with complaints of shortness of breath, proximal muscle weakness, anasarca. Evaluation revealed AM cortisol >60 ug/dL(range 6.7-22), high-dose DST Cortisol >60 ug/dL, 24hr urine free cortisol: 8511mcg (range 4-50) and ACTH level: 278pg/mL(range 6-50) confirming ACTH-dependent CS. Special stains from the previous TNET biopsy demonstrated positive staining for ACTH confirming ectopic ACTH secretion. Ketoconazole and chemotherapy with Etoposide and Carboplatin was started, however he clinically deteriorated and expired a few weeks after diagnosed of ECS. Discussion: TNET in MEN 1 is rare, with a prevalence of 3-8%. TNET are unusual neoplasms that account for 2% to 7% of all mediastinal tumors. TNET in MEN1 rarely secrete functional hormones with very few reported Ectopic ACTH secretion. MEN1 associated ECS from TNET is an aggressive disease with local invasion of adjacent mediastinal structures or metastasis being common, resulting in poor prognosis as demonstrated in few case reports including our case. Radical surgery of involved adjacent structures and adjuvant local RT can provide local disease control. Conclusion: Our pt is a rare case of ECS from TNET in MEN1 with poor prognosis. A special feature of this case is that the patient had initial negative evaluation for hypercortisolemia, however 6 months later he presented with signs and symptoms of severe hypercortisolism, with evaluation confirming transformation into ACTH producing TNET. This conversion is very rarely found in literature and adds to the unique presentation of the case.

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 Bowel perforation complicating an ACTH-secreting phaeochromocytoma

2016 ◽  
Vol 2016 ◽  
Author(s):  
Elise Flynn ◽  
Sara Baqar ◽  
Dorothy Liu ◽  
Elif I Ekinci ◽  
Stephen Farrell ◽  
...  
Keyword(s):  
Bowel Perforation ◽  
Diagnostic Workup ◽  
List Type ◽  
Ectopic Acth ◽  
Urinary Catecholamines ◽  
Unilateral Adrenalectomy ◽  
Gastrointestinal Complications ◽  
Ectopic Acth Secretion ◽  
Plasma Metanephrines ◽  
Acth Secreting

Summary ACTH-secreting phaeochromocytoma (ASP) is a rare cause of ACTH-dependent Cushing’s syndrome (CS). We report the case of a 63-year-old female presenting with CS secondary to an ASP complicated by bowel perforation. This case report highlights ASP as an uncommon but important cause of ectopic ACTH secretion (EAS). There have been 29 cases of ASP, all of which were unilateral and benign, but associated with significant complications. Patients presenting with ASP have the potential for cure with unilateral adrenalectomy. Given this promising prognosis if recognised, ASP should be considered in the diagnostic workup of ACTH-dependent CS. As this case demonstrates, gastrointestinal complications can arise from severe hypercortisolaemia associated with CS. Early medical and surgical intervention is imperative as mortality approaches 50% once bowel perforation occurs. Learning points Consider phaeochromocytoma in the diagnostic workup of ACTH-dependent CS; screen with plasma metanephrines or urinary catecholamines. Serial screening may be required if ACTH-secreting phaeochromocytoma is suspected, as absolute levels can be misleading. Early catecholamine receptor blockade and adrenal synthesis blockade may avoid the need for rescue bilateral adrenalectomy in ACTH-secreting phaeochromocytoma. Consider early medical or surgical management when gastrointestinal features are present in patients with CS, as bowel perforation due to severe hypercortisolaemia can occur and is associated with significant mortality.

scholarly journals MON-178 Cyclical Cushing’s Syndrome in 12 Patients with Ectopic ACTH Secretion

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Anna Zenno ◽  
Raven McGlotten ◽  
Atil Kargi ◽  
Lynnette Nieman
Keyword(s):  
Medical Treatment ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Inferior Petrosal Sinus ◽  
Acth Secretion ◽  
Diagnostic Challenge ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Inferior Petrosal Sinus Sampling ◽  
Acth Secreting

Abstract Background: Cyclical Cushing’s syndrome (CCS) is characterized by alternating periods of endogenous hypercortisolism and eucortisolism. A literature survey of 60 adult patients with CCS found 15 to have ectopic ACTH secretion (EAS) (1). The duration and frequency of hypercortisolemia are unpredictable, creating a diagnostic challenge. Objective: Describe biochemical and clinical characteristics of patients with CCS due to occult or histologically proven ectopic ACTH-secreting neuroendocrine tumor (NET). Methods: We conducted a retrospective medical record review of 12 adults with EAS admitted to our institution. Inclusion required 1) evidence of ectopic ACTH tumor from biochemical testing (CRH stimulation, 8 mg dexamethasone suppression [DST], and/or inferior petrosal sinus sampling [IPSS]) or pathology results and 2) cycles of hypercortisolism (Hi-F) to eucortisolism (Eu-F) off medical treatment. Results: Average age on admission was 61 (46-79) years; 58% were women. All 12 had biochemical evidence of ACTH-dependent Hi-F. IPSS results suggested EAS in 9 patients, 8 of whom had Hi-F for more than two months, and 1 whose cycles occurred every 5 - 7 days. IPSS was consistent with Cushing’s disease (CD) in 2 patients after Hi-F of only 6 -7 weeks and one with Eu-F on admission, estimated duration < 4 weeks. DST suggested EAS in 9 patients, and CD in the one with recent Eu-F. CRH was consistent with EAS in 10 patients, but suggested CD in 2 with marginal increases in ACTH (34.5%, 38%) but not cortisol. 7 patients had ACTH-secreting tumor on pathology (5 pulmonary, 1 pancreas, 1 appendix NET), and 5 had occult presumed EAS. Time from one Hi-F episode to the next ranged from 1 week to 6 years with Hi-F duration of 3 days to 5 years. 24-hour urine free cortisol (UFC) levels were 17 - 301 times the upper reference range (RR) during Hi-F periods. During Eu-F, lowest UFCs were within RR in 9 patients and subnormal in 3. Hypokalemia occurred in 11 patients with Hi-F; increasing values paralleled movement to Eu-F. Conclusion: Patients with possible ectopic ACTH-secretion and CCS may pose a diagnostic challenge: clinical and biochemical evidence of hypercortisolemia may not be present, depending on the timing and/or duration of hypercortisolism. Furthermore, test results may inappropriately suggest Cushing’s disease if performed after less than 8 weeks of hypercortisolism, or with recent eucortisolism. Thus, weekly UFC measurement may facilitate diagnosis of cyclical Cushing’s syndrome and determine appropriate timing of dynamic testing such as inferior petrosal sinus sampling. Potassium may be a useful marker to determine when medical treatment can be tapered or stopped. 1. Meinardi JR, et al. Eur J Endocrinol. 157:245, 2007.

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 Cushing's storm secondary to a rare case of ectopic ACTH secreting metastatic breast cancer

2015 ◽  
Vol 2015 ◽  
Author(s):  
Maura Bucciarelli ◽  
Ya-Yu Lee ◽  
Vasudev Magaji
Keyword(s):  
Breast Cancer ◽  
Intensive Care ◽  
Metastatic Breast Cancer ◽  
Cushing’S Syndrome ◽  
Metastatic Breast ◽  
Cushing's Syndrome ◽  
List Type ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Acth Secreting

Summary Ectopic ACTH secretion from breast cancer is extremely rare. We report a case of a 30-year-old woman with a history of breast cancer, who presented with psychosis and paranoid behaviour. CT of the head showed white matter disease consistent with posterior reversible encephalopathy syndrome (PRES). Despite using mifepristone with multiple antihypertensives including lisinopril, spironolactone and metoprolol, she was hypertensive. Transaminitis did not allow mifepristone dose escalation and ketoconazole utilization. Etomidate infusion at a non-sedating dose in the intensive care unit controlled her hypertension and cortisol levels. She was transitioned to metyrapone and spironolactone. She was discharged from the hospital on metyrapone with spironolactone and underwent chemotherapy. She died 9 months later after she rapidly redeveloped Cushing's syndrome and had progressive metastatic breast cancer involving multiple bones, liver and lungs causing respiratory failure. Learning points Cushing's syndrome from ectopic ACTH secreting breast cancer is extremely rare. Cushing's syndrome causing psychosis could be multifactorial including hypercortisolism and PRES. Etomidate at non-sedating doses in intensive care setting can be effective to reduce cortisol production followed by transition to oral metyrapone.

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.

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