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Diaphyseal medullary stenosis (sclerosis) with bone malignancy (malignant fibrous histiocytoma): Hardcastle syndrome

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Abstract

Hardcastle syndrome is a rare, autosomally dominant inherited skeletal dysplasia, characterized by diaphyseal sclerosis, medullary stenosis, pathological fractures, bony infarction, and malignant transformation. A 19-year-old pro-band is presented and discussed, adding a fourth family to the world literature. Radiographic screening of family members is suggested from puberty onward. Thallium scanning is proposed as a more tumor-sensitive screening agent in affected individuals.

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Authors and Affiliations

  1. Department of Radiology, Mount Sinai Hospital and Mount Sinai School of Medicine, City University of New York, One Gustave L. Levy Place, Box 1234, 10029-6574, New York, NY, USA

    K. I. Norton & J. M. Wagreich

  2. Division of Pediatric Hematology-Oncology, Mount Sinai Hospital, New York, New York, USA

    L. Granowetter

  3. Department of Pediatrics, Mount Sinai Hospital, New York, New York, USA

    K. I. Norton & J. A. Martignetti

Authors
  1. K. I. Norton
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  2. J. M. Wagreich
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  3. L. Granowetter
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  4. J. A. Martignetti
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Norton, K.I., Wagreich, J.M., Granowetter, L. et al. Diaphyseal medullary stenosis (sclerosis) with bone malignancy (malignant fibrous histiocytoma): Hardcastle syndrome. Pediatr Radiol 26, 675–677 (1996). https://doi.org/10.1007/BF01356833

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  • DOI: https://doi.org/10.1007/BF01356833

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