Απεικονιστικά ευρήματα στο μαστό ασθενών με το σύμπλεγμα carney: μελέτη με μαστογραφία, υπερηχοτομογραφία και μαγνητική μαστογραφία

Abstract

Carney complex is a familial multiple -neoplasia and lentiginosis syndrome first reported on in 1985 by Carney and colleagues. Carney complex is characterized by spotty pigmentation of the skin and mucosae (lentigines and blue nevi); myxomatous tumors of the skin, heart, breast, and other organs; and a variety of endocrine tumors which include primary pigmented nodular adrenocortical disease, growth hormone- secreting pituitary adenoma, testicular neoplasms (large-cell calcifying Sertoli and Leydig cell tumors), psammomatous melanotic schwannomas, and thyroid follicular tumors (adenoma and papillary and follicular carcinoma). The syndrome is autosomal dominant, and the genetic locus for it has been mapped to the short arm of chromosome 2 (2pl6) and to the short arm of chromosome 17 (17q 22-24). The disease is familial in approximately half of the patients; in the remaining patients, the syndrome appears sporadically. Breast masses in Carney Complex may represent myxoid fibroadenomas or ductal adenomas. The former have been found in at least one-fifth of the patients with reported sporadic and familial cases of the complex, with the latter have been described in five patients, which include two in the same family. The myxomatous lesion is characterized by the accumulation of myxoid material in the stroma that involves single or small groups of nodules. Large aggregates of these lesions result in the formation of a myxoid fibroadenoma. Ductal adenomas are solid tumors with tubular features and are composed of distinct epithelial and myoepithelial cells with a modest amount of fibrous tissue. Myxoid fibroadenomas and ductal adenomas are benign tumors that can manifest at a young age and are autosomal dominant. We suggest that MR imaging be performed when a solid, benign appearing mass in a patient with Carney Complex is found on a screening mammogram. If additional lesions with similar features exist and no other signs of malignancy are present, the patient can be followed up clinically. Because ductal adenomas are unusual benign tumors, incidental discovery of such a lesion should prompt one to exclude Carney complex.