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The Mafucci-Kast syndrome

Dyschondroplasia with hemangiomas and frontal lobe astrocytoma

  • Original Papers Reported by the Control European Bone Tumor Association
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Summary

The authors describe radiologic and pathoanatomic findings in the very rare Mafucci-Kast syndrome. In a 39-year-old Libyan patient multiple enchondromas of the fifth ray of the left hand were found with signs of malignant transformation. Furthermore, there were angiomas of the skin, of the soft meninges, and the bone. In addition, there was a low-grade malignant astrocytoma of the frontal lobe of the brain. Chromosome analysis revealed a normal male chromosome set. The tendency to develop malignant tumors which is repeatedly emphasized in the literature was also shown in the present case. The reason for this, especially for the high spontaneous rate of malignant transformation of multiple enchondromas, is unknown. The occurrence of angiomas and multiple enchondromas in cartilaginous performed bone suggests the presence of mesodermodysplasia.

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Authors and Affiliations

  1. Institute of Pathology (Director: Prof. P. Gedigk, MD), University of Bonn, P.O. Box 2120, D-5300, Bonn 1, Federal Republic of Germany

    H. Cremer

  2. Institute of Neuropathology (Director: Prof. G. Kersting, MD, University of Bonn, P.O. Box 2120, D-5300, Bonn 1, Federal Republic of Germany

    F. Gullotta

  3. Dept. of Orthopedics and Accident Surgery (Head Physician: R. Dederich, MD), St. Petrus Hospital, D-5300, Bonn, Federal Republic of Germany

    L. Wolf

Authors
  1. H. Cremer
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  2. F. Gullotta
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  3. L. Wolf
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Cremer, H., Gullotta, F. & Wolf, L. The Mafucci-Kast syndrome. J Cancer Res Clin Oncol 101, 231–237 (1981). https://doi.org/10.1007/BF00413318

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  • DOI: https://doi.org/10.1007/BF00413318

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