PMON123 Diagnostically Challenging Case of Cushing's Syndrome from a Pancreatic Neuroendocrine Tumor Treated with Radiofrequency Ablation

BACKGROUND: The delayed diagnosis of Cushing's syndrome (CS) results in advanced disease, treatment delays and poor outcomes. We present a patient with ectopic ACTH-syndrome (EAS) from a pancreatic neuroendocrine tumor (NET) whose care posed significant diagnostic and therapeutic challenges. CL...

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Autores principales: Gugger, Aristea Sideri, Fang, Jiali, Virk, Renu, Ahmad, Umar, Sagredo, Jorge Ariazza, Visrodia, Kavel H, Wilson, Gabrielle Page
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2022
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9625753/
http://dx.doi.org/10.1210/jendso/bvac150.1116
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author Gugger, Aristea Sideri
Fang, Jiali
Virk, Renu
Ahmad, Umar
Sagredo, Jorge Ariazza
Visrodia, Kavel H
Wilson, Gabrielle Page
author_facet Gugger, Aristea Sideri
Fang, Jiali
Virk, Renu
Ahmad, Umar
Sagredo, Jorge Ariazza
Visrodia, Kavel H
Wilson, Gabrielle Page
author_sort Gugger, Aristea Sideri
collection PubMed
description BACKGROUND: The delayed diagnosis of Cushing's syndrome (CS) results in advanced disease, treatment delays and poor outcomes. We present a patient with ectopic ACTH-syndrome (EAS) from a pancreatic neuroendocrine tumor (NET) whose care posed significant diagnostic and therapeutic challenges. CLINICAL CASE: 59F with classic Cushing's stigmata (including striae, plethora, supraclavicular and dorsocervical fat pads, muscle wasting), and two-year history of rapid weight gain, hypertension, T2DM, hyperlipidemia, severe osteoporosis complicated by multiple vertebral compression fractures, presented for workup and management of ACTH dependent CS. CS was confirmed (Baseline: ACTH 96 [7.2-63.3 pg/ml], serum cortisol 19.6 [4.8 - 19.5 mcg/dL]; LDSST: cortisol 18.5 [<1.8 mcg/dL], dexamethasone 547 [>200 ng/dL]; 24-hour Urine Free Cortisol: 191.60 [<45 mcg/d], 24-hour Cr 484 mg/day; LNSC: 436, 464 [29-101 ng/dL]; 8 mg ODST cortisol 7.2 mcg/dL). Pituitary MRI showed possible 0.5 cm left anterior pituitary lesion. Inferior petrosal sinus sampling (IPSS) to differentiate between Cushing's disease and EAS was initially planned, however, her course was complicated by an NSTEMI and acute/subacute lacunar infarcts in the left parietal and frontal lobes, and IPSS was deemed contraindicated. Additional workup was pursued including POMC 154 [<40 fmol/ml], Chromogranin A 538 [0-103 ng/ml] (on pantoprazole), pancreatic polypeptide 798 [0 - 435 pg/mL], 24-hour 5HIAA/Creatinine 3 [0 - 15 mg/24hr]. Chest/Abdominal/Pelvis CT showed a 1.5 cm incidental lipid rich adrenal adenoma; workup was negative for pheochromocytoma/ Conn's tumor/ adrenocortical carcinoma. Given elevated POMC and clinical suspicion for EAS, the patient underwent (68)Ga-DOTATATE PET-CT scan which revealed an avid 7 mm lesion (SUVmax 14.5) in the pancreatic tail suspicious for a pancreatic NET- the presumed ectopic source of ACTH. Given the patient's poor functional status she was not a candidate for tumoral resection via distal pancreatectomy. Ketoconazole was initiated for treatment of hypercortisolism with good biochemical control, but the patient developed liver toxicity. The decision was made to pursue endoscopic ultrasound guided biopsy and radiofrequency ablation of the lesion. Pathology confirmed ACTH immunoreactive low-grade pancreatic NET. Post-procedure, morning ACTH was 8 pg/ml and cortisol was 9.1 mcg/dL, and the patient required steroids for relative adrenal insufficiency. Further follow up with repeat POMC, cortisol, ACTH levels, 24-hour UFC is required to monitor response. CONCLUSION: IPSS is the gold standard test for differentiating Cushing's disease from EAS but is only available in select centers and for suitable candidates. This case supports the potential utility of POMC peptide measurements in the differential diagnosis of CS and the use of advanced nuclear imaging modalities for tumor localization. For patients with functional pNET who are poor surgical candidates or intolerant of pharmacotherapy, novel endoscopic ablation may offer a lower risk therapeutic option and its use should be further investigated. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.
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spelling pubmed-96257532022-11-14 PMON123 Diagnostically Challenging Case of Cushing's Syndrome from a Pancreatic Neuroendocrine Tumor Treated with Radiofrequency Ablation Gugger, Aristea Sideri Fang, Jiali Virk, Renu Ahmad, Umar Sagredo, Jorge Ariazza Visrodia, Kavel H Wilson, Gabrielle Page J Endocr Soc Neuroendocrinology and Pituitary BACKGROUND: The delayed diagnosis of Cushing's syndrome (CS) results in advanced disease, treatment delays and poor outcomes. We present a patient with ectopic ACTH-syndrome (EAS) from a pancreatic neuroendocrine tumor (NET) whose care posed significant diagnostic and therapeutic challenges. CLINICAL CASE: 59F with classic Cushing's stigmata (including striae, plethora, supraclavicular and dorsocervical fat pads, muscle wasting), and two-year history of rapid weight gain, hypertension, T2DM, hyperlipidemia, severe osteoporosis complicated by multiple vertebral compression fractures, presented for workup and management of ACTH dependent CS. CS was confirmed (Baseline: ACTH 96 [7.2-63.3 pg/ml], serum cortisol 19.6 [4.8 - 19.5 mcg/dL]; LDSST: cortisol 18.5 [<1.8 mcg/dL], dexamethasone 547 [>200 ng/dL]; 24-hour Urine Free Cortisol: 191.60 [<45 mcg/d], 24-hour Cr 484 mg/day; LNSC: 436, 464 [29-101 ng/dL]; 8 mg ODST cortisol 7.2 mcg/dL). Pituitary MRI showed possible 0.5 cm left anterior pituitary lesion. Inferior petrosal sinus sampling (IPSS) to differentiate between Cushing's disease and EAS was initially planned, however, her course was complicated by an NSTEMI and acute/subacute lacunar infarcts in the left parietal and frontal lobes, and IPSS was deemed contraindicated. Additional workup was pursued including POMC 154 [<40 fmol/ml], Chromogranin A 538 [0-103 ng/ml] (on pantoprazole), pancreatic polypeptide 798 [0 - 435 pg/mL], 24-hour 5HIAA/Creatinine 3 [0 - 15 mg/24hr]. Chest/Abdominal/Pelvis CT showed a 1.5 cm incidental lipid rich adrenal adenoma; workup was negative for pheochromocytoma/ Conn's tumor/ adrenocortical carcinoma. Given elevated POMC and clinical suspicion for EAS, the patient underwent (68)Ga-DOTATATE PET-CT scan which revealed an avid 7 mm lesion (SUVmax 14.5) in the pancreatic tail suspicious for a pancreatic NET- the presumed ectopic source of ACTH. Given the patient's poor functional status she was not a candidate for tumoral resection via distal pancreatectomy. Ketoconazole was initiated for treatment of hypercortisolism with good biochemical control, but the patient developed liver toxicity. The decision was made to pursue endoscopic ultrasound guided biopsy and radiofrequency ablation of the lesion. Pathology confirmed ACTH immunoreactive low-grade pancreatic NET. Post-procedure, morning ACTH was 8 pg/ml and cortisol was 9.1 mcg/dL, and the patient required steroids for relative adrenal insufficiency. Further follow up with repeat POMC, cortisol, ACTH levels, 24-hour UFC is required to monitor response. CONCLUSION: IPSS is the gold standard test for differentiating Cushing's disease from EAS but is only available in select centers and for suitable candidates. This case supports the potential utility of POMC peptide measurements in the differential diagnosis of CS and the use of advanced nuclear imaging modalities for tumor localization. For patients with functional pNET who are poor surgical candidates or intolerant of pharmacotherapy, novel endoscopic ablation may offer a lower risk therapeutic option and its use should be further investigated. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m. Oxford University Press 2022-11-01 /pmc/articles/PMC9625753/ http://dx.doi.org/10.1210/jendso/bvac150.1116 Text en © The Author(s) 2022. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (https://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Neuroendocrinology and Pituitary
Gugger, Aristea Sideri
Fang, Jiali
Virk, Renu
Ahmad, Umar
Sagredo, Jorge Ariazza
Visrodia, Kavel H
Wilson, Gabrielle Page
PMON123 Diagnostically Challenging Case of Cushing's Syndrome from a Pancreatic Neuroendocrine Tumor Treated with Radiofrequency Ablation
title PMON123 Diagnostically Challenging Case of Cushing's Syndrome from a Pancreatic Neuroendocrine Tumor Treated with Radiofrequency Ablation
title_full PMON123 Diagnostically Challenging Case of Cushing's Syndrome from a Pancreatic Neuroendocrine Tumor Treated with Radiofrequency Ablation
title_fullStr PMON123 Diagnostically Challenging Case of Cushing's Syndrome from a Pancreatic Neuroendocrine Tumor Treated with Radiofrequency Ablation
title_full_unstemmed PMON123 Diagnostically Challenging Case of Cushing's Syndrome from a Pancreatic Neuroendocrine Tumor Treated with Radiofrequency Ablation
title_short PMON123 Diagnostically Challenging Case of Cushing's Syndrome from a Pancreatic Neuroendocrine Tumor Treated with Radiofrequency Ablation
title_sort pmon123 diagnostically challenging case of cushing's syndrome from a pancreatic neuroendocrine tumor treated with radiofrequency ablation
topic Neuroendocrinology and Pituitary
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9625753/
http://dx.doi.org/10.1210/jendso/bvac150.1116
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