scholarly journals Cushing Syndrome due to Ectopic ACTH Secretion Presenting With Lower Limb Edema and Pulmonary Nocardia Infection: Case Report and Review of the Literature

2016 ◽  
Vol 6 (5) ◽  
pp. 158-161
Author(s):  
Marylene Samia El Hayek ◽  
Mahmoud Choucair ◽  
Asma Arabi
Keyword(s):  
Case Report ◽  
Lower Limb ◽  
Cushing Syndrome ◽  
Review Of The Literature ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Limb Edema

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.

Case report: Ectopic ACTH secretion due to a metastatic atypical lung carcinoid tumor. From diagnosis to treatment

2019 ◽  
Vol 80 (2) ◽  
pp. 137-139
Author(s):  
Ellen Hoornaert ◽  
Laurence Jacqmin ◽  
Luc Montfort ◽  
Dominique Maiter ◽  
Luc Derdelinckx
Keyword(s):  
Case Report ◽  
Carcinoid Tumor ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Lung Carcinoid

scholarly journals Possible pitfalls in the workup of ectopic ACTH secretion illustrated by four rare cases

2019 ◽  
Vol 12 (11) ◽  
pp. e231067
Author(s):  
Jorianne Boers ◽  
P Christine Oldenburg-Ligtenberg ◽  
Aline ME Stades ◽  
Mark JC van Treijen
Keyword(s):  
Case Report ◽  
Adrenocorticotropic Hormone ◽  
Progressive Disease ◽  
Diagnostic Workup ◽  
Morbidity And Mortality ◽  
Significant Morbidity ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Ectopic Adrenocorticotropic Hormone

In this case report, we highlight four different cases of ectopic adrenocorticotropic hormone (ACTH) secretion with different pitfalls in the diagnostic workup. Ectopic ACTH secretion (EAS) is an uncommon cause of Cushing’s syndrome that accompanies a variety of tumours. It is associated with significant morbidity and mortality. This underlines the importance of early and adequate diagnosis. We will review the causes of EAS and their presentation to increase awareness of this rare and progressive disease.

scholarly journals Ectopic ACTH Secretion in a Child With Metastatic Ewing's Sarcoma: A Case Report

2020 ◽  
Vol 10 ◽  
Author(s):  
Valentina Di Ruscio ◽  
Giada Del Baldo ◽  
Maria Debora De Pasquale ◽  
Rita De Vito ◽  
Evelina Miele ◽  
...  
Keyword(s):  
Case Report ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion

scholarly journals Lung Carcinoid Tumors and Paraneoplastic Cushing's Syndrome: Diagnostic and Therapeutic Difficulties - A Case Report

2021 ◽  
Vol 2021 ◽  
Author(s):  
Hela Kamoun ◽  
Aymen Ayari ◽  
Hadhemi Rejeb ◽  
Sarra Zairi ◽  
Hanene Smadhi ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Lung Nodule ◽  
Cushing's Syndrome ◽  
Middle Lobe ◽  
Pathological Examination ◽  
Acth Secretion ◽  
Typical Carcinoid ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion

Introduction. - Paraneoplastic Cushing's syndrome is a rare cause of endogenous hypercorticism. It is caused by ectopic secretion of ACTH by a non-pituitary endocrine tumor. The aetiological biological and radiological data are often inconclusive, which creates a problem of differential diagnosis with Cushing's disease. In addition, these tumors are often small and their location is extremely variable. As a result, the difficulties of localization require the use of specific imaging techniques.Observation. - We report the observation of a 44-year-old man suffering from diabetes and high blood pressure, presenting a severe and rapidly progressive Cushing syndrome, in connection with a hypercorticism caused by an ectopic ACTH secretion. The thoracic computed tomography performed within the framework of a search for a neoplastic origin objectified a 15 mm nodule isolated at the level of the middle lobe, the scintigraphy with octreotide marked with indium-111 found a significant fixation at the level of the lung nodule. The patient had a middle lobe lobectomy. The outcome was favorable with regression of Cushing's syndrome. Pathological examination was in favor of a typical carcinoid tumor, and the immunohistochemical complement showed tumor cell positivity for ACTH, CD56, chromogranin, and synaptophysin.Conclusion. - This observation illustrates the dilemma between the need to locate an ectopic ACTH secretion and the control of aggressive and threatening Cushing's syndrome. Early use of the octreotide scintigraphy should be considered if a topographic diagnosis of the causative tumor cannot be done through conventional imaging techniques.

Sign in / Sign up

Export Citation Format

Share Document