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A rare case of Maffucci's syndrome combined with tuberculum sellae enchondroma, pituitary adenoma and thyroid adenoma

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Summary

Maffucci's syndrome is a rare, congenital mesodermal dysplasia combined with dyschondroplasia and haemangiomatosis, and there are only about 150 reported cases. This syndrome is often combined with other neoplasms. Our case was associated with goitre, enchondroma of the tuberculum sellae and pituitary adenoma; the latter brought about disturbance of vision. Including ours, four cases of Maffucci's syndrome associated with pituitary adenoma are found in the literature. In the case of Maffucci's syndrome, it is necessary to examine associated disease as well as associated pituitary adenoma and skull base enchondroma, similar to the correlation of von Recklinghausen's disease with acoustic neurinoma.

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Author notes

  1. K. Miki MD, K. Kawamoto, Y. Kawamura & H. Matsumura

    Present address: Department of Neurosurgery, Kansai Medical University, Osaka, Japan

  2. Y. Asada & A. Hamada

    Present address: Department of Orthopaedic Surgery, Kansai Medical University, Osaka, Japan

Authors and Affiliations

  1. Department of Neurosurgery, Shingu City Hospital, 451, Shingu, Shingu City, 647, Wakayama, Japan

    K. Miki MD

Authors
  1. K. Miki MD
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  2. K. Kawamoto
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  3. Y. Kawamura
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  4. H. Matsumura
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  5. Y. Asada
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  6. A. Hamada
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Miki, K., Kawamoto, K., Kawamura, Y. et al. A rare case of Maffucci's syndrome combined with tuberculum sellae enchondroma, pituitary adenoma and thyroid adenoma. Acta neurochir 87, 79–85 (1987). https://doi.org/10.1007/BF02076022

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  • DOI: https://doi.org/10.1007/BF02076022

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