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Intraosseous pilomatricoma: a possible rare skeletal manifestation of Gardner syndrome

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Abstract

Here we report a case of intraosseous pilomatricoma in a patient with Gardner syndrome. A 17-year-old boy with a family history of Gardner syndrome and multiple cutaneous epidermoid cysts presented with pain in the region below the knee of the left leg. Plain radiographs displayed a well-circumscribed eccentric lesion of mixed radiolucent and radiopaque density within the diaphysis of the left proximal tibia. By magnetic resonance (MR) imaging, the lesion appeared as a low intensity lesion in T1-weighted images and a heterogeneous high intensity lesion with a low-signal radiation pattern on T2-weighted images. Gadolinium enhancement was observed partially within the lesion and intensely in the lesion rim. After curettage, histological examination revealed a massive accumulation of keratinous material with epithelial nests that displayed both epidermal and trichilemmal keratinization with basaloid and shadow cells and with metaplastic ossification. These features confirmed a pathological diagnosis of intraosseous counterpart of pilomatricoma. In the context of this patient’s skin lesions, intraosseous pilomatricoma may be a rare skeletal manifestation of Gardner syndrome.

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Authors and Affiliations

  1. Department of Pathology and Laboratory Medicine, National Center of Neurology and Psychiatry, Kohnodai Hospital, Kohnodai 1-7-1, Chiba, Ichikawa, 272-8516, Japan

    Tsuyoshi Ishida

  2. Department of Orthopaedic Surgery, Teikyo University School of Medicine, Tokyo, Japan

    Satoshi Abe & Yuji Miki

  3. Department of Surgical Pathology, Teikyo University School of Medicine, Tokyo, Japan

    Tetsuo Imamura

  4. Department of Molecular Bone and Cartilage Pathology, Hard Tissue Genome Research Center, Tokyo Medical and Dental University, Tokyo, Japan

    Tsuyoshi Ishida

Authors
  1. Tsuyoshi Ishida
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  2. Satoshi Abe
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  3. Yuji Miki
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  4. Tetsuo Imamura
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Correspondence to Tsuyoshi Ishida.

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Ishida, T., Abe, S., Miki, Y. et al. Intraosseous pilomatricoma: a possible rare skeletal manifestation of Gardner syndrome. Skeletal Radiol 36, 693–698 (2007). https://doi.org/10.1007/s00256-006-0264-4

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  • DOI: https://doi.org/10.1007/s00256-006-0264-4

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