PSAT387 An Unusual Case of Sporadic Metastatic Medullary Thyroid Cancer Without a Primary Tumor Presenting with Paraneoplastic Cushing's Syndrome

Autor: Sanjay Jumani, Yamini Sterett, Henrik Elenius, Padmasree Veeraraghavan, Ryan Carrie, Jaydira del Rivero, Samira Sadowski, Naris Nilubol, Freddy Escorcia, Lynnette Nieman, Evgenia Globa, Marina Zemskova, Joanna Klubo-Gwiezdzinska, Sriram Gubbi
Rok vydání: 2022
Předmět:
Zdroj: Journal of the Endocrine Society. 6:A849-A850
ISSN: 2472-1972
DOI: 10.1210/jendso/bvac150.1756
Popis: Introduction Medullary thyroid carcinoma (MTC) accounts for 5-10% of all thyroid cancers and up to 30% of MTCs are hereditary. Only 0.6% of MTCs are associated with a paraneoplastic ACTH-dependent Cushing's syndrome (CS) (PMID: 16029131). Even rarer are MTCs without a primary intra-thyroidal tumor. Here we describe a patient with metastatic MTC without a known primary intrathyroidal tumor presenting with paraneoplastic ACTH-dependent CS. Clinical Case A 47-year-old Ukrainian man was referred to our center for management of metastatic MTC. During evaluation of refractory diarrhea two years earlier, an elevated serum calcitonin (1758 pg/mL, normal: Biochemical testing revealed elevated serum calcitonin (158,535 pg/mL, normal: The patient was unfortunately not eligible for any ongoing local clinical trials on RET-mutated cancer therapies. After extensive discussion with the patient, a palliative approach was pursued. The cervical lymphadenopathy and right femoral shaft metastasis were treated with external beam radiation. Diarrhea was controlled with loperamide. Cushing's syndrome was initially managed with metyrapone and ketoconazole, followed by bilateral adrenalectomy and hydrocortisone and fludrocortisone replacement. Conclusion Sporadic metastatic MTC can rarely present without an intrathyroidal primary tumor and may originate from ectopic C-cells. Paraneoplastic syndromes such as Cushing's syndrome can be observed in MTC due to ectopic-ACTH production. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
Databáze: OpenAIRE