• Benign tumor of bone or soft tissue associated with rickets and osteomalacia (Am J Surg Pathol 1989;13:588)
• Most cases of tumor associated oncogenic osteomalacia are due to phosphaturic mesenchymal tumor, which produces fibroblast growth factor-2, a protein that inhibits renal tubular phosphate reabsorption (Am J Surg Pathol 2004;28:1) or dentin matrix protein 1 (Mod Pathol 2004;17:573)

• Extremely rare, median age 53 years, range 9-80 years, slight female predominance

• Low serum phosphate, renal phosphate wasting, low 1,25-dihydroxy Vitamin D3

• 22 year old man with tumor of craniofacial sinuses (Clin Cases Miner Bone Metab 2011;8:45)
• 42 year old woman with tumor of ethmoid sinus (Indian J Pathol Microbiol 2010;53:384)
• 42 year old woman with mandible tumor (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;108:925)
• 45 year old man with mixed connective tissue varian (Intern Med 2006;45:1157)

• Complete excision causes dramatic reversal of signs and symptoms

Tumor of ethmoid sinus

• 2-14 cm, arises in soft tissue and bone

• Hypocellular tumor of bland spindle cells with small nuclei, indistinct nucleoli
• Has hemangiopericytoma-like vasculature, osteoclast-like giant cells, distinctive "grungy" calcified matrix, fat, microcysts, hemorrhage, incomplete rim of membranous ossification, metaplastic bone
• Infiltrative
• No/rare mitotic activity, no atypia
• Malignant cases: rare cases with nuclear atypia, 5+ mitotic figures/10 HPF, high cellularity, resembles MFH

Tumor of craniofacial sinuses

Dentin matrix protein 1

Tumor of ethmoid sinus

Mandible tumor

• Fibroblast growth factor-23, dentin matrix protein 1

• Giant cell tumor
• Hemangiopericytoma
• Osteosarcoma

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