Undiagnosed Composite Pheochromocytoma Associated with Critical limb Ischemia -a Rare Entity of an Uncommon Disease

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-76
Shumei Meng*1, Pedram Argani2, Alan P B Dackiw2 and Douglas W Ball3
1John Hopkins Medicine, Baltimore, MD, 2Johns Hopkins University School of Medicine, Baltimore, MD, 3Johns Hopkins University School of Medicine
Background: Composite pheochromocytoma is a rare variant of pheochromocytoma typically diagnosed by pathologists. Most composite pheochromocytoma coexisted with ganglioneuromas, a tumor arising from neural crest cells. Infrequently, ganglioneuroma can secrete excess catecholamines causing hypertension.  Whether the regulation of pheochromocytoma cells by adjacent ganglion cells plays a role in the biological activity of the tumor is not clear. However, the biologic behavior of these tumors can be difficult to predict. Pheochromocytoma-associated peripheral limb ischemia is exceptionally rare. Composite pheochromocytoma related extremity ischemia has not been previously reported. 

Clinical case: A 54 year old woman with 3 year history of hypertension, episodic headache, went to ER for nausea, vomiting and abdominal pain.  Her Bp was noted to be 206/140 mmHg.  She was treated for hypertension urgency with labetalol, hydralazine, nitroglycerin and her BP dropped to 60/40 mmHg. She was intubated after she developed hypoxia and required transient pressor support for hypotension after fluid resuscitation failed. Subsequently she became hypertensive and developed profound gangrenous discoloration at four extremities shortly after off pressor. In addition, she also was found to have marked microangiopathic hemolytic anemia and acute renal failure. Her pan culture was negative as well as coagulation and vasculities studies. She had developed EKG changes and mild elevation of troponin. Eventually the 6 cm noncalcified adrenal mass detected on the abdominal CT obtained in ER pointed to the correct diagnosis of pheochromocytoma. It was further confirmed with 24 hour urinary metanephrine of 3583 mcg (less than 400) and normetanephrine of 2475 mcg (less than 900). She was properly treated with alpha blockade and followed with beta blocker. Her renal and cardiac functions were fully recovered. Ultimately she underwent left adrenalectomy with pathology diagnosis of composite pheochromocytoma and ganglioneuroma (2.5cm) with associated scarring and central necrosis. Unfortunately she had to have BKA amputation and her several distal fingers had autoamputation.

Conclusion: This is the first case reporting composite pheochromocytoma related catastrophic limb ischemia. This unique case illustrated the devastating damage related with unrecognized composite pheochromocytoma. Earlier diagnosis and treatment could lead to dramatic improvement of the outcome.

Nothing to Disclose: SM, PA, APBD, DWB

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