Intraoperative diagnosis of a paraganglioma

Abstract

Introduction:
Paragangliomas are extra-adrenal chromaffin tumorsthat arise fromneuroectodermal cells ofthe autonomous
nervoussystem (1). They are rare tumors, and may be located in the skull base, neck, chest and abdomen.Also
they can be functioning secreting catecholamine and producing symptoms or non-functioning producing
no symptoms (silent). When found within the abdomen, a silent paraganglioma may be mistaken for other
retroperitoneal tumors, such as a lymphoma or a tumor of the pancreas.
Case presentation:
A 19-year-old female originally presented in September 2015 with complaints of upper abdominal pain
accompanied with pain radiating from the waist to the left lower limb, headaches and two attacks of
what had been thought of as a seizure. She had been evaluated and diagnosed as epilepsy and started
on treatment. But her abdominal pain and the headaches did not settle. A contrast-enhanced CT of the
abdomen demonstrated a 3X4cm left adrenal mass with predominantly peripheral enhancement and central
areas of low attenuation, presumed to represent necrosis fig 1.
The patient underwent surgical exploration. Unexpectedly, the patient became hypertensive with a systolic
blood pressure reaching 200 mmHg and supraventricular tachycardia (SVT) during the initial manipulation
of the tumor and were managed by phenoxybenzamine 40mg Iv and labetalol infusion 1mg/min by the
anaethetist. Paraganglioma was suspected to be the cause of this circulatory problem. The blood pressure
and SVT were rapidly controlled and the tumor was completely resected.
Pathological examination of the specimen revealed well-encapsulated tumor forming tubules , trabeculae
and solid sheets . The tumor was composed of polygonal cells displaying vesicular nuclei and acidophilic
cytoplasm. Pleomorphic and multinuclei, as well asfoci of necroses, were noted and confirmed the diagnosis
of paraganglioma(fig 2). The patient’s postoperative course was uneventful; her blood pressure returned to
normal and she was discharged 5 days after the operation.