A Rare Case of Cushing Syndrome Caused by an Ectopic ACTH-Secreting Pancreatic Neuroendocrine Tumor in a Patient with Von Hipple-Lindau Disease Abstract #416

Introduction: Von Hipple-Lindau (VHL) disease is a rare autosomal dominant disorder, that causes the susceptibility to various tumors, both benign and malignant. The most frequent tumors are central nervous system haemangioblastomas, especially cerebellum and retinal haemangioblastomas, renal cell carcinoma /renal cysts and pheocromocytoma.
Aim(s): We report a case of a 36-year-old woman diagnosed with VHL disease characterized by the uncommon association with ACTH-dpendent Cushing's syndrome.
Materials and methods: Phisycal examination, laboratory findings and traditional and radioreceptorial imaging were performed.
Conference: 9th Annual ENETS Conference (2012)
Category: Clinical
Presenting Author: Andrea Lania

To read results and conclusion, please login ...

Further abstracts you may be interested in

#1924 Multimodal Treatment of a Metastatic Pancreatic NeuroEndocrine Tumor in Von Hippel Lindau Disease
Introduction: Von Hippel-Lindau disease (VHL) is an inherited neoplastic syndrome: pancreatic lesions in 35-77% of cases, usually serous cystic adenoma (SCA), but 5-17% are non-functioning (NF) NeuroEndocrine tumors (NETs).
Conference: 14th Annual ENETS conference (2017)
Category: Medical treatment - Targeted therapies
Presenting Author: Dr Anna Caterina Milanetto
#858 Metastatic Pancreatic Neuroendocrine Tumors with Ectopic Cushing Syndrome: Could Everolimus Be a Safe Therapeutic Option?
Introduction: Everolimus(E) has proved effective in prolonging PFS in advanced P-NETs. As E is a substrate of CYP3A4 co-administration with ketoconazole (k), a strong inhibitor of CYP3A4 used to control hypercortisolism, should be avoided.
Conference: 11th Annual ENETS Conference (2014)
Category: Clinical cases/reports
Presenting Author: Maria Vittoria Davì
#1266 Three Cases of Ectopic Adrenocorticotropic Hormone Syndrome Due to Medullary Thyroid Carcinoma
Introduction: Сushing's syndrome (CS) in medullar thyroid carcinoma (MTC) is rare. Among patients with MTC 0,7 % developed ectopic ACTH-syndrome (EAS). EAS due to MTS occurs in 2,2-8% of cases.
Conference: 13th Annual ENETS conference (2016)
Category: Pathology, grading, staging
Presenting Author: Iya Voronkova
#1206 Two Cases of Ectopic ACTH-Secreting Neoplasm of Occult Origin Becoming Overt After Bilateral Adrenalectomy
Introduction: Cushing's syndrome (CS) is an endocrine disorder associated with serious consequences if not adequately treated. Nearly 80% of the cases of endogenous hypercortisolism are adrenocorticotropic hormone (ACTH) dependent, the ACTH source being pituitary in about 70-80% and ectopic in 10-15%. The ACTH source may remain occult in few cases for many years in spite of extensive investigations.
Conference: 12th Annual ENETS Conference (2015)
Category: Clinical cases/reports
Presenting Author: MD Donato Iacovazzo
#2193 Advanced Gastric Neuroendocrine Carcinoma: A Diagnostic and Management Conundrum
Introduction: Gastric neuroendocrine carcinomas are rare tumors with a high malignant trait and poor prognosis. Endogenous hypercortisolism associated with neuroendocrine carcinomas can have a negative impact on patients' survival.
Conference: 15th Annual ENETS conference (2018)
Category: Case reports
Presenting Author: MD Ioana Maria Lambrescu