Mazabraud syndrome
Mazabraud syndrome is a rare primary bone dysplasia characterized by the association of fibrous dysplasia with intramuscular myxomas. Fibrous dysplasia (usually polyostotic, sometimes monostotic) occurs during the growth period and can be asymptomatic or can present with pain, skeletal deformities or fractures while intramuscular myxoma, associated with polyostotic fibrous dysplasia is usually multifocal, typically occurring in the vicinity of skeletal lesions, and presents in adulthood as a painless soft-tissue mass (most commonly in the thigh). Although it is a benign condition, local recurrences of myxomas after incomplete excision and malignant transformation of a fibrous dysplastic lesion into osteogenic sarcoma have been reported.
MONDO:0019708
obsolete primary bone dysplasia with disorganized development of skeletal components (obsolete MONDO:0019708)
excluded_subClassOf
MONDO:0003847
hereditary disease
is_a
MONDO:0006424
soft tissue neoplasm
is_a
Myxoma with fibrous dysplasia
exact