Endocrine Abstracts

Introduction: Cells of the neuroendocrine system are spread throughout the body, and they can give rise to various neuroendocrine tumors with unpredictable evolution. It has been observed that they are often associated with the development of fibrosis, both local and distant.

Case presentation: We present the case of an 81 year-old, normal-weight woman, who firstly presented in our clinic in 2014 for multinodular goiter (normal calcitonin). Fine needle aspiration biopsy from a mixed thyroid nodule (measuring 22.2/13.1 mm) indicated a well-differentiated follicular adenoma. Patient associated stage 3 hypertension and asthma. Also, routine abdominal ultrasound revealed a tumor in the right adrenal lodge. Consequent native computed tomography (CT) only described a well-defined dense mass measuring 5/3.6/2.6 cm in the right adrenal lodge. Further testing dismissed a secreting adrenal adenoma and catecholamines were normal at that time. In 2015, the patient underwent emergency biliary decompression surgery for mechanical jaundice due to a pancreatic tumor of the head and body. On-site evaluation rendered the tumor as unresectable, thus a side-to-side bilio-gastric anastomosis and an Omega loop gastro-enteric anastomosis were performed. Pancreatic tumor biopsy showed fibrous tissue and chronic inflammatory cell infiltrate (immunohistochemistry unavailable). 1-year follow-up magnetic resonance imaging showed the pancreatic solid tumor of the head, measuring 2/1.7/1.5 cm, and a stationary retro-caval solid tumor, without a clear demarcation from the right adrenal gland. In 2017, the patient returned with paroxysmal hypertension, partially responsive to treatment, flush, diaphoresis and fatigue. Contrast CT described the retro-caval tumor as a hypodense, iodophile, slightly unhomogenous nodule, measuring 5.6/3.5/2.2 cm, not well delimited from the right adrenal gland, which started to compress the posterior-medial side of the intrahepatic portion of the inferior vena cava. She also developed diabetes mellitus. Further testing identified elevated Chromogranin A (366 μg/l) and catecholamines (metanephrines=445.3 pg/ml; normetanephrines=1018.6 pg/ml), thus patient was diagnosed with a catecholamine-secreting tumor (either pheochromocytoma or paraganglioma). Currently, patient is under observation and antihypertensive treatment, as she refuses another surgical intervention.

Conclusion: Although the tumor in the right adrenal lodge was initially non-functional, catecholamines and symptoms became evident 3 years later. Also, the pancreatic tumor could be a ‘sclerosing variant’ of pancreatic neuroendocrine tumor, in which the associated fibrotic reaction usually involves the main pancreatic duct, resulting in ductal stenosis and upstream duct dilatation and/or pancreatic atrophy. Yet, immunohistochemistry is required for accurate diagnosis. Comorbidities, age and patient’s unwillingness represent a challenge in the diagnostic process and management of neuroendocrine tumors, especially pheochromocytoma.