Interleukin-6 Producing Pheochromocytoma Presenting as Sepsis

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

Poster Board MON-48
Michelle Carey*1, Rodolfo Javier Galindo2, Ziqiang Yuan1, Steven K Libutti3 and Norman Fleischer4
1Albert Einstein College of Medicine, Bronx, NY, 2Jacobi Medical Center, Bronx, NY, 3Albert Einstein College of Med, Bronx, NY, 4Albert Einstein Coll of Med &, Bronx, NY
Background: Pheochromocytomas can present with a wide variety of clinical features, including fevers. A small number of patients have been described in whom recurrent fevers were attributed to interleukin (IL)-6 production by a pheochromocytoma.

Clinical Case: A 62-year-old male with longstanding hypertension was admitted with fever, leukocytosis, relative hypotension, and progressively worsening abdominal pain. An infectious workup and broad-spectrum antibiotics were initiated. A CT-scan of the abdomen revealed a large left adrenal mass (5.9 x 4.9 x 4.8cm) and plasma and urine metanephrines were dramatically elevated (plama metanephrines 1076pg/mL, n<25pg/mL; plasma normetanephrines 782pg/mL, n<148pg/mL; urine metanephrines 3296mcg/24hrs, n 90-315mcg/24hrs; urine normetanephrines 1492mcg/24hrs, n 122-676mcg/24hrs), consistent with a diagnosis of pheochromocytoma.

The patient initially responded to antibiotics with normalization of temperature and white blood cell count, but fever and leukocytosis recurred in a cyclical fashion. An extensive infectious workup failed to identify an etiology of the fevers, and serum IL-6 was elevated (69.76pg/mL, n 0.31-5.0pg/mL). After resection of the pheochromocytoma, fevers and leukocytosis completely resolved and serum IL-6 fell to 7.27pg/mL. Histologic examination revealed a chromaffin cell tumor, and immunohistochemistry staining of the tumor was strongly positive for IL-6. Two other spontaneous pheochromocytomas from our Neuroendocrine Tissue Bank were also stained for IL-6 as controls. These tumors were resected from patients who did not present with fevers or other infectious symptoms, and staining for IL-6 was negative.

Conclusion: This is the tenth reported case of suspected IL-6 production by a pheochromocytoma, and the first to provide immunohistochemistry staining of tumors from control pheochromocytoma subjects.  In patients with pheochromocytoma whose clinical presentation includes recurrent fevers without an identifiable etiology, IL-6 production by the tumor should be considered.

Nothing to Disclose: MC, RJG, ZY, SKL, NF

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