scholarly journals Successful localization and treatment for ectopic adrenocorticotrophic hormone secretion in a rare case of possible Tx N2 M0 carcinoid tumor with Cushing syndrome

2002 ◽  
Vol 124 (6) ◽  
pp. 1237-1238 ◽  
Author(s):  
Takafumi Sugawara ◽  
Masami Sato ◽  
Keiichi Itoi ◽  
Akira Sugawara ◽  
Yasushi Matsuda ◽  
...  
Keyword(s):  
Carcinoid Tumor ◽  
Rare Case ◽  
Cushing Syndrome ◽  
Hormone Secretion ◽  
Adrenocorticotrophic Hormone

Cushing syndrome: a rare presentation of bronchial carcinoid tumor

2019 ◽  
Vol 27 (7) ◽  
pp. 603-605
Author(s):  
Pavneet Kohli ◽  
Prasanth Penumadu ◽  
K Chandrashekhar Rao ◽  
Sreevathsa KS Prasad ◽  
Sadishkumar Kamalanathan
Keyword(s):  
Carcinoid Tumor ◽  
Adrenocorticotropic Hormone ◽  
Cushing Syndrome ◽  
Imaging Techniques ◽  
Hormone Secretion ◽  
Clinical Feature ◽  
Bronchial Carcinoid ◽  
Rare Presentation ◽  
Pulmonary Carcinoid ◽  
Ectopic Adrenocorticotropic Hormone

Cushing syndrome is a very rare clinical feature of typical pulmonary carcinoid, caused by hypercorticism, and despite advances in imaging techniques, it poses a difficult diagnostic and therapeutic challenge. We report the case of a patient presenting with Cushing syndrome associated with ectopic adrenocorticotropic hormone secretion from a bronchial carcinoid, whose management presented diagnostic and therapeutic challenges.

scholarly journals A rare case of Cushing syndrome by cyclic ectopic-ACTH

2012 ◽  
Vol 56 (5) ◽  
pp. 324-330 ◽  
Author(s):  
Mariana Farage ◽  
Mario Alberto da Dantas Loures Costa ◽  
Amélio Fernando Godoy-Matos
Keyword(s):  
Primary Tumor ◽  
Rare Case ◽  
Glomus Tumor ◽  
Cushing Syndrome ◽  
Severe Hypertension ◽  
Abdominal Fat ◽  
Ectopic Acth ◽  
Mood Changes ◽  
Ectopic Acth Production

ACTH-dependent Cushing syndrome (CS) due to ectopic ACTH production is most times difficult to manage. The identification of the source of ACTH may take many years. Surgery or chemotherapy for the primary tumor is not always possible. Control of Cushing symptoms is many times achieved using medication, or bilateral adrenalectomy in refractory cases. This case presents a Brazilian male who showed severe hypertension, mood changes, muscle weakness, darkening of skin, and increased abdominal fat. An investigation for Cushing syndrome was carried out and, after a four-year follow-up, a carotid glomus tumor (chemodectoma) was confirmed, a rare ectopic ACTH-producing tumor. Besides, the patient presented cyclic Cushing syndrome that was exacerbated by diverticulitis episodes. This case presents interesting pitfalls on diagnosis and management of ACTH-dependent CS. This is the only report of a chemodectoma that produced ACTH in the literature.

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 Diagnosis and management of small pulmonary atypical carcinoid tumor associated with Cushing syndrome

2020 ◽  
Vol 9 (4) ◽  
Author(s):  
Dathe Z Benissan-Messan ◽  
Robert E Merritt ◽  
Konstantin Shilo ◽  
Desmond M D'Souza ◽  
Peter J Kneuertz
Keyword(s):  
Carcinoid Tumor ◽  
Adrenocorticotropic Hormone ◽  
Cushing Syndrome ◽  
Wedge Resection ◽  
Lung Nodule ◽  
Pet Scan ◽  
Atypical Carcinoid ◽  
Pulmonary Carcinoid ◽  
Ectopic Adrenocorticotropic Hormone

Ectopic adrenocorticotropic hormone ( ACTH) syndrome is rare and identification of its source is often challenging. We report the case of an ectopic Cushing syndrome in a young adult male secondary to an occult ACTH producing atypical carcinoid tumor. Extensive biochemical and imaging workup was unrevealing. The diagnosis was aided by Ga-DOTA PET scan demonstrating a suspicious left upper lobe lung nodule. The patient underwent video-assisted thoracoscopic exploration with wedge resection and mediastinal lymphadenectomy of a T2aN2M0 atypical carcinoid, resulting in the normalization of ACTH levels and complete resolution of symptoms. The role of a Ga-DOTA PET scan in diagnosing pulmonary carcinoid tumors and their management are discussed.

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