Malignant transformation of polyostotic fibrous dysplasia. 1979

C B Johnson, and E F Gilbert, and L I Gottlieb

We have described an unusual case of polyostotic fibrous dysplasia in a 25-year-old white woman who had malignant transformation into a mesenchymal tumor with widespread metastases and features of rhabdomyosarcoma. Masculinization also occurred with high plasma testosterone levels. A mechanism for the development of masculinizing features is discussed.

UI MeSH Term Description Entries
D001859 Bone Neoplasms Tumors or cancer located in bone tissue or specific BONES. Bone Cancer,Cancer of Bone,Cancer of the Bone,Neoplasms, Bone,Bone Neoplasm,Neoplasm, Bone
D002471 Cell Transformation, Neoplastic Cell changes manifested by escape from control mechanisms, increased growth potential, alterations in the cell surface, karyotypic abnormalities, morphological and biochemical deviations from the norm, and other attributes conferring the ability to invade, metastasize, and kill. Neoplastic Transformation, Cell,Neoplastic Cell Transformation,Transformation, Neoplastic Cell,Tumorigenic Transformation,Cell Neoplastic Transformation,Cell Neoplastic Transformations,Cell Transformations, Neoplastic,Neoplastic Cell Transformations,Neoplastic Transformations, Cell,Transformation, Cell Neoplastic,Transformation, Tumorigenic,Transformations, Cell Neoplastic,Transformations, Neoplastic Cell,Transformations, Tumorigenic,Tumorigenic Transformations
D005260 Female Females
D005357 Fibrous Dysplasia of Bone A disease of bone marked by thinning of the cortex by fibrous tissue containing bony spicules, producing pain, disability, and gradually increasing deformity. Only one bone may be involved (FIBROUS DYSPLASIA, MONOSTOTIC) or several (FIBROUS DYSPLASIA, POLYOSTOTIC). Osteitis Fibrosa Disseminata,Fibrocartilaginous Dysplasia of Bone,Fibrocystic Dysplasia of Bone,Jaffe-Lichtenstein Disease,Bone Fibrocartilaginous Dysplasia,Bone Fibrocartilaginous Dysplasias,Bone Fibrocystic Dysplasia,Bone Fibrocystic Dysplasias,Bone Fibrous Dysplasia,Bone Fibrous Dysplasias,Jaffe Lichtenstein Disease
D005359 Fibrous Dysplasia, Polyostotic FIBROUS DYSPLASIA OF BONE affecting several bones. When melanotic pigmentation (CAFE-AU-LAIT SPOTS) and multiple endocrine hyperfunction are additionally associated it is referred to as Albright syndrome. Albright's Syndrome,McCune-Albright Syndrome,Albright Syndrome,Albright's Disease,Albright's Disease of Bone,Albright's Syndrome with Precocious Puberty,Albright-Mccune-Sternberg Syndrome,Albright-Sternberg Syndrome,Fibrous Dysplasia with Pigmentary Skin Changes and Precocious Puberty,Polyostotic Fibrous Dysplasia,Dysplasia, Polyostotic Fibrous,Dysplasias, Polyostotic Fibrous,Fibrous Dysplasias, Polyostotic,McCune Albright Syndrome,Polyostotic Fibrous Dysplasias,Syndrome, Albright,Syndrome, Albright's,Syndrome, McCune-Albright
D006621 Hip Joint The joint that is formed by the articulation of the head of FEMUR and the ACETABULUM of the PELVIS. Acetabulofemoral Joint,Acetabulofemoral Joints,Hip Joints,Joint, Acetabulofemoral,Joint, Hip,Joints, Acetabulofemoral,Joints, Hip
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D012208 Rhabdomyosarcoma A malignant solid tumor arising from mesenchymal tissues which normally differentiate to form striated muscle. It can occur in a wide variety of sites. It is divided into four distinct types: pleomorphic, predominantly in male adults; alveolar (RHABDOMYOSARCOMA, ALVEOLAR), mainly in adolescents and young adults; embryonal (RHABDOMYOSARCOMA, EMBRYONAL), predominantly in infants and children; and botryoidal, also in young children. It is one of the most frequently occurring soft tissue sarcomas and the most common in children under 15. (From Dorland, 27th ed; Holland et al., Cancer Medicine, 3d ed, p2186; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, pp1647-9) Rhabdomyosarcomas
D014770 Virilism Development of male secondary SEX CHARACTERISTICS in the FEMALE. It is due to the effects of androgenic metabolites of precursors from endogenous or exogenous sources, such as ADRENAL GLANDS or therapeutic drugs. Androgenization,Virilization

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