A Rare Case Of Retroperitoneal Extra-Adrenal Paraganglioma Presented With Stroke: Case Report And Review Of Literature

摘要

Paragangliomas of the retroperitoneum arise from specialized neural crest cells distributed along the aorta in association with the sympathetic chain. We report a rare case of extra-adrenal paraganglioma between inferior vena cava and aorta in suprarenal portion with lateral displacement of inferior vena cava. This patient presented with left upper motor neuron facial palsy and left hemiparesis secondary to high blood pressure. The rarest presentation of paraganglioma with stroke has never been described to the best of our knowledge in the literature. Case Report A 19 year old man presented with sudden onset of weakness and parasthesia on the left side of body. He complained of severe pulsatile holocranial headache not associated with sweating, palpitations and visual symptoms. He was also recently diagnosed with hypertension.On examination his blood pressure was 200/108 mm Hg. His neurological examination confirmed left upper motor neuron facial palsy and left hemiparesis. His carotid doppler was reported as normal. There were no masses palpable per abdomen and auscultation did not disclose any bruits. Laboratory studies showed persistently elevated urinary nor-metanephrine levels. An ultrasound performed to rule out adrenal masses showed a well defined hypoechoic lesion between inferior vena cava and aorta in suprarenal portion just above origin of right renal artery, displacing inferior vena cava laterally with no evidence of calcification.CT scan abdomen revealed a 5.4 x 4 cm sized well defined heterogeneously enhancing lesion posterior to IVC at level of superior mesenteric artery. Mass effect was noted on IVC and right renal vein with no free fluid, no lymph nodes and normal right adrenal gland . These features were suggestive of paraganglioma in retroperitoneum (Fig1, 3).MRI brain showed right thalamic infarct and left frontal lobe infarct (Fig2).He underwent laparotomy and excision of the lump under general anaesthesia12 weeks after his intial admission (Fig 4). Histopathological diagnosis is consistent with paraganglioma (Fig 5, 6 ). His convalescence was uneventful.