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1997 ; 12
(1
): 89-95
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Oncogenic osteomalacia caused by a phosphaturic mesenchymal tumor of the oral
cavity: a case report
#MMPMID9159046
Yang IM
; Park YK
; Hyun YJ
; Kim DY
; Woo JT
; Kim SW
; Kim JW
; Kim YS
; Choi YK
Korean J Intern Med
1997[Jan]; 12
(1
): 89-95
PMID9159046
show ga
We report a case of oncogenic osteomalacia associated with a phosphaturic
mesenchymal tumor in a 31-year-old woman. She was presented with severe
generalized bone and muscle pain and was restricted to bed. She lost 20 cm in
height over the 8 years since she had first noticed a pain in her thigh. A
walnut-sized, hard, soft tissue tumor was found very easily beside her lower
molar teeth Radiologic examination revealed a remarkable decrease in bone density
and multiple pathologic fractures of spine, femur and phalangeal bones. Severe
hypophosphatemia, hyperphosphaturia, low plasma 1,25-dihydroxyvitamin D3 level
and high plasma PTH level were disclosed at presentation. Histomorphometric
examination revealed an extensive area of unmineralized osteoid and little
mineralizing activity. A pharmacologic dose of 1 alpha-hydroxyvitamin D3 or or
1,25-dihydroxyvitamin D3 slightly increased the serum phosphate level and renal
tubular reabsorption of phosphate, and slightly decreased plasma PTH level
without any symptomatic improvement. Histologic examination of the tumor revealed
a mixed connective tissue tumor that consisted of central woven bones and
surrounding primitive spindle cells with prominent vascularities. After removal
of the tumor, all biochemical, hormonal and radiologic abnormalities disappeared
with remarkable symptomatic improvement.