Ectopic pheochromocytoma or paraganglioma of the ZUCKERKANDL organ: A case report and review of the literature

Abstract

Introduction: Paraganglioma of the ZUCKERKANDL organ are rare. Diagnosis is based on clinical, radiological and biological arguments. We report a case to describe our surgical procedure and insist on the necessity of preoperative diagnosis.

Presentation of case: BA, 52-years-old male patient was seen in consultation for left hypochondrium pains. The clinical examination had revealed a painful tumefaction in the left flank and the left hypochondrium. A deep mass was observed, but was difficult to be assessed, due to pain. Abdominal-pelvic CT scan with contrast injection had revealed a tissue mass, suggesting a tumor of the tail of the pancreas. Laparotomy showed this mass was not attached to the tail of the pancreas, and was along the abdominal aorta up to the aortic bifurcation. Upon touching the mass, blood pressure raised up to 240 mmHg. A least mobilization of the mass and the use of nicardipine helped maintain blood pressure below 180mmhg. Dissection was carried out from the aortic bifurcation to the TREITZ's angle and the mass was removed. The follow-ups were characterized by low blood pressure a few minutes following the resection of the mass.

Discussion: Pheochromocytoma is rare. The Clinical signs, Abdominal-pelvic CT scan and biology are the steps of the preoperative diagnosis. The surgery consists a lumpectomy. The resuscitation determines the patient's prognosis.

Conclusion: Pheochromocytoma is an unusual mass. Preoperative diagnosis can be difficult in pauci-symptomatic cases. One should consider this in the face of any abdominal mass, so as to improve planning of resuscitation which determines the patient's prognosis.

Keywords: Diagnosis; Pheochromocytoma; Surgery.