Intestinal pseudo-obstruction as a complication of paragangliomas: case report and literature review

Neth J Med. 2013 Dec;71(10):512-7.

Abstract

Intestinal pseudo-obstruction is a rare and relatively unknown complication of phaeochromocytoma÷ paraganglioma (PCC÷PGL). Its pathophysiology can be explained by the hypersecretion of catecholamines, which may reduce the peristaltic activity of the gastrointestinal tract. Clinically, this can result in chronic constipation, intestinal pseudo-obstruction or even intestinal perforation. We conducted a comprehensive literature search and retrieved 34 cases of pseudo-obstruction caused by either benign or malignant PCC÷PGL. We also included a case from our centre that has not been described earlier. We conclude that intestinal pseudo-obstruction is a rare but potentially life-threatening complication of PCC÷PGL. Intravenous administration of phentolamine is the most frequently described treatment when surgical resection of the PCC÷PGL is not feasible.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Aged
  • Diagnosis, Differential
  • Diagnostic Imaging
  • Disease Progression
  • Fatal Outcome
  • Female
  • Humans
  • Intestinal Pseudo-Obstruction / diagnosis
  • Intestinal Pseudo-Obstruction / etiology*
  • Intestinal Pseudo-Obstruction / therapy
  • Middle Aged
  • Paraganglioma, Extra-Adrenal / complications*
  • Paraganglioma, Extra-Adrenal / diagnosis
  • Paraganglioma, Extra-Adrenal / therapy
  • Radiography, Abdominal
  • Succinate Dehydrogenase / genetics
  • Treatment Outcome

Substances

  • Succinate Dehydrogenase