Metastatic Paraganglioma Presenting With Hepatic Hematoma

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 37-82-Pheochromocytoma & Paraganglioma
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-74
Ella Rosenbloom*1, Amanda Laird2 and Martin I Surks3
1Montefiore Medical Center, Bronx, NY, 2Montefiore Medical Center, 3Montefiore Med Ctr, Bronx, NY
Background:  We describe a rare case of a retroperitoneal paraortic paraganglioma presenting with a subscapular hepatic hematoma.   

Case presentation: A 59 year old female with history of hypertension presented to the ED with right upper quadrant abdominal pain and chest pain. Her initial blood pressure was 138/74mmHg. Abdominal CAT scan showed a subscapular hepatic hematoma with active extravasation which was managed by embolization of the right hepatic artery. The CAT scan also showed a 5.4 by 5.2 by 8.1cm retroperitoneal mass with areas of necrosis abutting left aspect of the aorta and forth portion of the duodenum just below the level of the renal hilum. Plasma metanephrines and normetanephrines were within normal range, but the 24 hour urine for metanephrines was increased to 1607 ug/24hrs (19-315 ug/24hrs), and normetanephrines were 4008 ug/24hrs (122-678 ug/24hrs). An MIBG scan showed uptake in the left upper retroperitoneal area, a photopenic area in the right upper quadrant in the region of the subcapsular hepatic hematoma, as well as a focal area of uptake at the lower margin of the right lobe of the liver anteriorly.  After alpha and beta blockade, the patient had a radical resection of the retroperitoneal periaortic paraganglioma, and a right hepatectomy and splenectomy.  Intraoperatively, there was a significant hemodynamic response with a blood pressure of 272/256mmHg during tumor mobilization, followed by a drop to 72/67mmHg once the tumor was removed.  Operative histopathology revealed an encapsulated 8.8cm paraganglioma.  The right lobe of the liver revealed 25 cm of organizing hematoma without evidence of neoplasm. One month post operatively,  24 hour urine metanephrines were 36 ug/24hrs, urine normetanephrines  182 ug/24hrs, plasma  metanephrines <25 pg/ml (<58 pg/ml). Plasma normetanephrines elevated at 281 pg/ml(<149 pg/ml).  Patient’s blood pressure is currently maintained on two antihypertensive agents - Amlodipine and Labetalol.  She has been referred for genetic testing for mutations associated with extra adrenal paragangliomas.  

Conclusions:  Spontaneous hemorrhage from a pheochromocytoma, although rare, has been reported in the literature; correct diagnosis plays a large part in management. In this case we postulate there was metastasis of the paraganglioma to the liver which resulted in the patient’s  presentation, and the neoplasm was not identified in the liver due to its necrosis.

Kobayashi T, Iwai A, Takahashi R, Ide Y, Nishizawa K, Mitsumori K. Spontaneous rupture of adrenal pheochromocytoma: review and analysis of prognostic factors. J Surg Oncol. 2005;90:31-35.

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