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Діафізарний медулярний стеноз - злоякісний синдром кісток

ORPHA:85182· ICD-10 M89.8· Diaphyseal medullary stenosis-bone malignancy syndrome

Визначення(English summary)

Diaphyseal medullary stenosis with malignant fibrous histiocytoma is a very rare autosomal dominant bone dysplasia/cancer syndrome characterized clinically by bone infarctions, cortical growth abnormalities, pathological fractures, and development of bone sarcoma (malignant fibrous histiocytoma).

Успадкування
Autosomal dominant