- Email: [email protected]
Carney's Complex
Journal of the American College of Cardiology © 2010 by the American College of Cardiology Foundation Published by Elsevier Inc.
Vol. 55, No. 13, 2010 ISSN 0735-1097/10/$36.00 doi:10.1016/j.jacc.2009.07.077
IMAGES IN CARDIOLOGY
Carney’s Complex Amit K. Pursnani, MD, Natalie K. Levy, MD, Maryellen Benito, BS, Susie N. Hong, MD, Monvadi B. Srichai, MD, Herman Yee, MD, Itzhak Kronzon, MD New York, New York
From the Division of Cardiology, New York University, New York, New York. Manuscript received June 22, 2009, accepted July 2, 2009.
A
22-year-old man with a palpable pelvic mass was admitted with left-sided weakness. Head computed tomography (CT) revealed right posterocerebral infarction (A). Pelvic CT unveiled an extensive retroperitoneal soft tissue mass (B). Echocardiography demonstrated a left atrial mass (C), which was hypointense on T1-weighted and hyperintense on T2-weighted magnetic resonance imaging (D). Biopsy analysis of the retroperitoneal mass revealed spindle-shaped cells with immunostaining consistent with a schwannoma (E). The atrial mass was surgically removed and had histologic features of an atrial myxoma (F, ovoid cells, low cellularity, surrounded by acid mucin). The peculiar combination of an atrial myxoma and a peripheral schwannoma in a young man is highly suggestive of Carney’s Complex, a hereditary syndrome characterized by unusual neoplasms and cutaneous and endocrine manifestations. Up to 10% of all cardiac myxomas are thought to be a part of this complex and have a greater potential for tumor recurrence.
Vol. 55, No. 13, 2010 ISSN 0735-1097/10/$36.00 doi:10.1016/j.jacc.2009.07.077
IMAGES IN CARDIOLOGY
Carney’s Complex Amit K. Pursnani, MD, Natalie K. Levy, MD, Maryellen Benito, BS, Susie N. Hong, MD, Monvadi B. Srichai, MD, Herman Yee, MD, Itzhak Kronzon, MD New York, New York
From the Division of Cardiology, New York University, New York, New York. Manuscript received June 22, 2009, accepted July 2, 2009.
A
22-year-old man with a palpable pelvic mass was admitted with left-sided weakness. Head computed tomography (CT) revealed right posterocerebral infarction (A). Pelvic CT unveiled an extensive retroperitoneal soft tissue mass (B). Echocardiography demonstrated a left atrial mass (C), which was hypointense on T1-weighted and hyperintense on T2-weighted magnetic resonance imaging (D). Biopsy analysis of the retroperitoneal mass revealed spindle-shaped cells with immunostaining consistent with a schwannoma (E). The atrial mass was surgically removed and had histologic features of an atrial myxoma (F, ovoid cells, low cellularity, surrounded by acid mucin). The peculiar combination of an atrial myxoma and a peripheral schwannoma in a young man is highly suggestive of Carney’s Complex, a hereditary syndrome characterized by unusual neoplasms and cutaneous and endocrine manifestations. Up to 10% of all cardiac myxomas are thought to be a part of this complex and have a greater potential for tumor recurrence.