Dominant Inheritance of the Complex of Myxomas, Spotty Pigmentation, and Endocrine Overactivity
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INVESTIGATION OF THE FAMILY
The index patient, her father, and her maternal grandmother had been examined at our institution. Six of her seven siblings and all six nephews and nieces were examined in Kansas City. The investigation included a thorough physical examination with anthropometry and study of the skin in natural and ultraviolet light. In each case, a chest roentgenogram and a two-dimensional echo cardiogram were obtained. Each person older than 6 years of age had a complete blood cell count, automated serum
Case 1.
The index patient (III.3), a 34-year-old woman, had come to our medical center in 1984 because of persistent headaches. She had had pigmented spots on her face and lips since birth. At age 14 years, she had undergone removal of a lesion from her right upper eyelid; microscopically, it was a “papilloma having a core of fibrovascular tissue.” Recurrence of the eyelid lesion 3 years later necessitated another excision. At age 16 years, she had had pigmented lesions excised from her cheeks, upper
DISCUSSION
In this family, lentigines were present in the index patient, in three of her seven siblings, in their mother, and in a niece (the daughter of an affected sister). Cutaneous myxomas were present in the index patient, in two of her brothers, and probably in their mother. In addition, the index patient had two cardiac myxomas, multiple myxoid mammary fibroadenomas, and the Cushing syndrome, and an affected brother had acromegaly caused by a growth hormone-secreting tumor of the pituitary gland.
ACKNOWLEDGMENT
We thank the following colleagues who provided clinical information, echocardiograms, or histologic slides for the study: J. L. Booth, L. R. Christie, J. D. Lott, G. A. Oliver, C. Siqueira, A. Starr, and G. Starr.
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Cushing's Disease
2022, The PituitaryCardiac Tumors: JACC CardioOncology State-of-the-Art Review
2020, JACC: CardioOncologySoft-Tissue Tumors of the Head and Neck
2020, Gnepp's Diagnostic Surgical Pathology of the Head and Neck, Third EditionSurgery for Tumors of the Heart
2018, Seminars in Thoracic and Cardiovascular Surgery
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Department of Cardiology, St. Luke's Hospital, Kansas City, Missouri.