title: Spectrum of cutaneous and soft tissue lesions in two Carney complex patients-adnexal induction versus authentic adnexal neoplasms.
abstract: Two unusual Carney complex patients are described. In one of them, several cutaneous biopsies revealed myxoid lesions that were rather more close to authentic adnexal neoplasms with myxoid stroma than to a "myxoma with an epithelial component." These included lesions resembling trichofolliculoma, infundibular cyst, and trichodiscoma. Additionally, 1 soft tissue myxoma was unique in the sense that it greatly resembled a cardiac myxoma, begging the question whether this could represent an embolus from the patient's cardiac myxoma. Given the large size and complexity and heterogeneity of the cutaneous lesions, the authors suggest that these may represent authentic cutaneous neoplasms accompanied by myxoid stroma and not adnexal elements induced by the stroma. However, the latter mechanism is well recognized and demonstrated by our second patient in whom adnexal-type elements in the cutaneous lesion were clearly induced by the myxoid stroma but were unusually complex by manifesting panfollicular and also sebaceous differentiation.
authors.affiliation: Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague, Pilsen, Czech Republic.
authors.forename: Denisa
authors.initials: D
authors.lastname: Kacerovska
authors.forename: Luis
authors.initials: L
authors.lastname: Requena
authors.forename: Michal
authors.initials: M
authors.lastname: Michal
authors.forename: Petr
authors.initials: P
authors.lastname: Grossmann
authors.forename: Inka
authors.initials: I
authors.lastname: Treskova
authors.forename: Patrik
authors.initials: P
authors.lastname: Roucka
authors.forename: Dmitry V
authors.initials: DV
authors.lastname: Kazakov
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D056733:Carney Complex / Q000235:genetics / Q000473:pathology*; D002454:Cell Differentiation; D054756:Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / Q000235:genetics; D004252:DNA Mutational Analysis; D005260:Female; D006801:Humans; D008297:Male; D009154:Mutation; D018294:Neoplasms, Adnexal and Skin Appendage / Q000235:genetics / Q000473:pathology*; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000235:genetics / Q000473:pathology*; D017154:Stromal Cells / Q000473:pathology
chemical_list: D054756:Cyclic AMP-Dependent Protein Kinase RIalpha Subunit; C517834:PRKAR1A protein, human