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Perivascular epithelioid cell tumor of the rib

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Abstract

We present a rare case of perivascular epithelioid cell tumor (PEComa) in the right 6th rib of a 28-year-old man. A plain computed tomography scan showed a round osteolytic lesion in the right 6th rib. The resected tissue contained a globular-shaped, soft tumor. Histologically, the tumor was rich in vasculature and exclusively composed of perivascular epithelioid cells with clear cytoplasm. Immunohistochemically, the tumor expressed diffusely a melanocyte marker, human melanoma black-45, and focally a myogenic marker, α-smooth muscle actin, but not an epithelial marker, AE1/AE3. Fontana–Masson-positive melanin pigments were present and c-kit receptor tyrosine kinase (CD117), involved in the development of melanocytes but not myogenic cells, was expressed in tumor cells. These findings indicate that the tumor is PEComa with some differentiation into melanocytes. Notably, owing to the unique location of the occurrence, the tumor occupied bone marrow tissues of the rib, resulting that the tumor has the potential for hematogenous metastasis. In spite of the lack of cells with severe atypia, necrosis, and numerous mitoses, tumor cells invaded into surrounding tissues and overexpressed cyclin D1. To the best of our knowledge, this is the first case report of PEComa arising from the rib with the signs of malignant potential.

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Acknowledgments

We thank Mr. T. Yamamoto and Ms. R. Nishikawa of Hyogo College of Medicine for their technical assistance.

Conflict of interest statement

We declare that we have no conflict of interest.

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Authors and Affiliations

  1. Department of Pathology, Hyogo College of Medicine, 1-1 Mukogawa, Nishinomiya, Hyogo, 663-8501, Japan

    Ikuko Torii, Ayuko Sato, Takashi Nishigami & Tohru Tsujimura

  2. General Thoracic Surgery, Hyogo College of Medicine, Nishinomiya, Hyogo, 663-8501, Japan

    Nobuyuki Kondo, Teruhisa Takuwa, Seiji Matsumoto, Yoshitomo Okumura, Fumihiro Tanaka & Seiki Hasegawa

Authors
  1. Ikuko Torii
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  2. Nobuyuki Kondo
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  3. Teruhisa Takuwa
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  4. Seiji Matsumoto
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  5. Yoshitomo Okumura
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  6. Ayuko Sato
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  8. Takashi Nishigami
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  10. Tohru Tsujimura
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Correspondence to Tohru Tsujimura.

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Torii, I., Kondo, N., Takuwa, T. et al. Perivascular epithelioid cell tumor of the rib. Virchows Arch 452, 697–702 (2008). https://doi.org/10.1007/s00428-008-0612-y

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  • DOI: https://doi.org/10.1007/s00428-008-0612-y

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