Endocrine Abstracts

Background: Pheochromocytoma and paragangliomas (PPGLs) are rare disorders with a rate of malignancy of 10–20%. The control of the hormonal symptoms caused by the catecholaminergic burden in the metastatic disease is a challenging task. Hypertension is the most recognized feature, but gastrointestinal manifestations can be hard to manage and life-threatening.

Cases Presentation: We present three cases of metastatic PPGLs who developed intestinal pseudo-obstruction. The first case refers to a 39-year-old man, SDHC-positive, with a plurimetastatic PPGL that progressed after different lines of therapy, who had several hospital admissions due to intestinal pseudo-obstruction. Laxative therapy, with osmotic agents, lubricants, emolients and gastrointestinal stimulants were ineffective; a trial with neostigmine and erythromycin was also unsuccessful. He was submitted to ileostomy that also failed to resolve the severe constipation. In the second case, a 34-year-old man, SDHB-positive, was admitted to ICU because of congestive heart failure due to cathecolamine-induced myocardial injury. After 2 months in the ICU, with slight improvement of the heart condition, intestinal pseudo-obstruction was similarly difficult to manage, with partial response to repeated enemas and optimized oral therapy. The last case was the easiest to manage: a 70-year-old woman, with negative genetic screening, presented with acute severe pseudo-obstruction that was managed by aggressive medical therapy.

Conclusions: Management of intestinal pseudo-obstruction in PPGLs is challenging because it is usually refractory to medical therapy in the advanced cases, being poorly defined in the literature with very few cases reported. Occasionally it can lead to megacolon, perforation and sepsis portending high morbidity and mortality.