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The Journal of Thoracic and Cardiovascular Surgery, Vol 91, 389-396, Copyright © 1986 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
PM McCarthy, JM Piehler, HV Schaff, JR Pluth, TA Orszulak, HJ Vidaillet Jr and JA Carney
We reviewed the Mayo Clinic records of 56 patients who underwent operation
for cardiac myxoma and 29 cases in which cardiac myxoma was found at
autopsy. Five patients had a "complex" of unusual findings including
multiple pigmented skin lesions (lentiginosis), myxoid fibroadenomas of the
breast, skin myxomas, and primary pigmented nodular adrenocortical disease
(a cause of Cushing's syndrome). Four of these five patients had multiple
cardiac myxomas. Three of the four patients who underwent surgical excision
of the cardiac myxomas had recurrent myxomas (the only recurrences in our
series), and one of these patients had a second recurrence. The occurrence
of multiple and recurrent myxomas in patients with the complex was
significantly (p less than 0.001) higher than in our 80 patients with
sporadic myxomas. The world literature was searched for cases of cardiac
myxomas with the unusual associations of the complex, and also for
familial, multiple, and recurrent myxomas. A group of patients were
identified who had unusual biologic behavior including early development of
myxomas, atypical myxoma locations, and a high risk for the development of
recurrent myxomas. For these patients, we recommend a thorough search for
multiple tumors at operation, close postoperative follow-up, and careful
screening of family members.
ARTICLES
The significance of multiple, recurrent, and "complex" cardiac myxomas
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M. Casey, C. Mah, A. D. Merliss, L. S. Kirschner, S. E. Taymans, A. E. Denio, B. Korf, A. D. Irvine, A. Hughes, J. A. Carney, et al. Identification of a Novel Genetic Locus for Familial Cardiac Myxomas and Carney Complex Circulation, December 8, 1998; 98(23): 2560 - 2566. [Abstract] [Full Text] [PDF]
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