Volume 154, Issue 5 pp. 983-987

Detection of COL1A1PDGFB fusion transcripts and platelet-derived growth factor α and β receptors in giant cell fibroblastoma of the postsacrococcygeal region

A. Kashima

A. Kashima

First Department of Pathology, Department of Dermatology, Miyazaki Medical College, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan
Departments of *Neurosurgery and †Pathology, Nobeoka Prefectural Hospital, 2-1-10 Shinkoji, Nobeoka, Japan

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A. Yamashita

A. Yamashita

First Department of Pathology, Department of Dermatology, Miyazaki Medical College, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan
Departments of *Neurosurgery and †Pathology, Nobeoka Prefectural Hospital, 2-1-10 Shinkoji, Nobeoka, Japan

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S. Moriguchi

S. Moriguchi

First Department of Pathology, Department of Dermatology, Miyazaki Medical College, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan
Departments of *Neurosurgery and †Pathology, Nobeoka Prefectural Hospital, 2-1-10 Shinkoji, Nobeoka, Japan

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K. Marutsuka

K. Marutsuka

First Department of Pathology, Department of Dermatology, Miyazaki Medical College, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan
Departments of *Neurosurgery and †Pathology, Nobeoka Prefectural Hospital, 2-1-10 Shinkoji, Nobeoka, Japan

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S. Tsumori

S. Tsumori

First Department of Pathology, Department of Dermatology, Miyazaki Medical College, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan
Departments of *Neurosurgery and †Pathology, Nobeoka Prefectural Hospital, 2-1-10 Shinkoji, Nobeoka, Japan

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K. Yoshizato

K. Yoshizato

First Department of Pathology, Department of Dermatology, Miyazaki Medical College, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan
Departments of *Neurosurgery and †Pathology, Nobeoka Prefectural Hospital, 2-1-10 Shinkoji, Nobeoka, Japan

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A. Ishihara

A. Ishihara

First Department of Pathology, Department of Dermatology, Miyazaki Medical College, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan
Departments of *Neurosurgery and †Pathology, Nobeoka Prefectural Hospital, 2-1-10 Shinkoji, Nobeoka, Japan

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M. Setoyama

M. Setoyama

First Department of Pathology, Department of Dermatology, Miyazaki Medical College, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan
Departments of *Neurosurgery and †Pathology, Nobeoka Prefectural Hospital, 2-1-10 Shinkoji, Nobeoka, Japan

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Y. Asada

Y. Asada

First Department of Pathology, Department of Dermatology, Miyazaki Medical College, University of Miyazaki, 5200 Kihara, Kiyotake, Miyazaki 889-1692, Japan
Departments of *Neurosurgery and †Pathology, Nobeoka Prefectural Hospital, 2-1-10 Shinkoji, Nobeoka, Japan

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First published: 20 February 2006
Citations: 10
Yujiro Asada.
E-mail: [email protected]

Conflicts of interest
None declared.

Summary

We describe a 2-year-old girl with recurrent giant cell fibroblastoma (GCF) of the postsacrococcygeal region. Both the initial and recurrent tumours contained solid and angiectoid areas. The former was composed of loosely arranged wavy spindle cells and giant cells with a well-vascularized myxoid to collagenous stroma. The angiectoid spaces were often lined by multinucleated giant cells. Immunohistochemically, the tumour cells and small vessels in the tumour tissue were positive for platelet-derived growth factor (PDGF) α and β receptors. Molecular analysis revealed fusion of collagen type Iα1 exon 26 with PDGF-B chain exon 2 that induced unscheduled production of PDGF-BB. These findings suggest that PDGF and its receptors significantly contribute to the development of GCF in both an autocrine and a paracrine manner.

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