Recurrent malignant variant of phosphaturic mesenchymal tumor with oncogenic osteomalacia

Citation
A. Ogose et al., Recurrent malignant variant of phosphaturic mesenchymal tumor with oncogenic osteomalacia, SKELETAL RA, 30(2), 2001, pp. 99-103
Citations number
16
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Radiology ,Nuclear Medicine & Imaging
Journal title
SKELETAL RADIOLOGY
ISSN journal
0364-2348 → ACNP
Volume
30
Issue
2
Year of publication
2001
Pages
99 - 103
Database
ISI
SICI code
0364-2348(200102)30:2<99:RMVOPM>2.0.ZU;2-S
Abstract
Phosphaturic mesenchymal tumor is a rare neoplasm which causes osteomalacia or rickets. The tumor typically follows a benign clinical course. Even in the rare malignant cases, local recurrence and distant metastasis are uncom mon. We report on an example of a malignant phosphaturic mesenchymal tumor which recurred several times over 16 years concurrently causing hypophospha temia, bone pain, and osteomalacia. Following each surgery, symptoms and hy pophosphatemia improved. The patient died of disease 17 years after the fir st surgery. Histologically, the initial tumor was composed of small spindle with clusters of giant cells, prominent blood vessels, poorly formed carti laginous areas, and crystalline material. Cytological atypia was minimal. F ollowing multiple recurrences, the tumor demonstrated areas of high-grade s arcoma exhibiting marked pleomorphism, numerous mitotic figures, and p53 ov erexpression. This case illustrates the potential lethality of incompletely removed phosphaturic mesenchymal tumors.