Abstract
A woman with Carney complex presented at the age of 22 years with abdominal pain and hirsutism. As a baby, she had undergone excision of a right eyelid lesion, and at age 14 years she had undergone removal of a left lower eyelid nodule that subsequently recurred. Investigation revealed an elevated level of serum testosterone and a 2-cm left ovarian tumor. A left salpingo-oophorectomy was performed. Postoperatively, she experienced relief from abdominal pain, the serum level of testosterone normalized, and the hirsutism ameliorated. The tumor featured sheets of eosinophilic cells with lipochrome pigment, myeloid metaplasia, stromal metaplasia, and markedly abnormal blood vessels. Immunocytochemically, the tumor cells were positive for vimentin, synaptophysin, inhibin-A, and calrenin. Because of the clinical setting in which the neoplasm occurred, it is likely that is occurrence was related to Carney complex.
Publication types
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Research Support, N.I.H., Intramural
MeSH terms
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Biomarkers, Tumor / metabolism
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Calbindin 2
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Carney Complex / pathology*
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Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / genetics
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Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / metabolism
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DNA Mutational Analysis
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Female
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Humans
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Inhibins / metabolism
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Leydig Cell Tumor / complications
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Leydig Cell Tumor / metabolism
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Leydig Cell Tumor / pathology*
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Ovarian Neoplasms / complications
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Ovarian Neoplasms / metabolism
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Ovarian Neoplasms / pathology*
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Ovariectomy
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S100 Calcium Binding Protein G / metabolism
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Stromal Cells / pathology
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Synaptophysin / metabolism
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Testosterone / blood
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Vimentin / metabolism
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Virilism / etiology
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Virilism / pathology*
Substances
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Biomarkers, Tumor
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Calbindin 2
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Cyclic AMP-Dependent Protein Kinase RIalpha Subunit
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PRKAR1A protein, human
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S100 Calcium Binding Protein G
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Synaptophysin
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Vimentin
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inhibin A
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Testosterone
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Inhibins