Malignant paraganglioma of the mesentery: a case report and review of literature

Michael Chetrit,Virginie Royal,Guy Leblanc,Lucas Sideris,Pierre Dubé

doi:10.1186/1477-7819-10-46

Abstract

Paragangliomas represent only 10% of chromaffin tissue tumors and those arising from the mesentery seem to be a rare occurrence. We report a case of a 55 year old man in whom an abdominal mass was discovered fortuitously by ultrasonography during a routine health exam. He presented occasional heart palpitations and diaphoresis as well as a well-demarcated mass upon abdominal physical examination. CT scan revealed a solid polylobulated mass in the right lower quadrant. Exploration laparotomy revealed a voluminous multi-nodular tumoral mass, which contained hemorrhagic spots. Histopathological studies confirmed the presence of a paraganglioma. The excision of the mass as well as the surrounding intestine and mesentery also revealed two lymphatic metastases, the first among 14 documented cases to be described concerning mesenteric paragangliomas. One year follow up and CT scan revealed neither recurrence nor the presence of distant metastases.

Highlights

With an annual incidence estimated at 1/100,000, paragangliomas represent ten percent of catecholamine secreting tumors
Some paragangliomas have been described in the Gastrointestinal System, the majority of which were associated with the duodenum
A very select few were described to arise from the mesentery [2]. Given their catecholamine secreting properties, paragangliomas have the potential to present as a mass with paroxystic symptoms of palpitations, pallor, tremor, headache and diaphoresis as well as hypertension [3]

Summary

Background

With an annual incidence estimated at 1/100,000, paragangliomas represent ten percent of catecholamine secreting tumors. A very select few were described to arise from the mesentery [2] Given their catecholamine secreting properties, paragangliomas have the potential to present as a mass with paroxystic symptoms of palpitations, pallor, tremor, headache and diaphoresis as well as hypertension [3]. This is only the case in 25% of the paragangliomas. The ultrasound, which revealed an asymptomatic hypoechogenous polylobulated solid mass in the right supraumbilical territory, was followed up and confirmed with a CT scan (Figure 1). The patient has been followed for almost a year and a follow up abdominal scan was conducted, all indicating the patient was free from any signs of recurrence including the palpitations and diaphoresis

Discussion

Findings

Conclusion

Young WF Jr