title: Phosphaturic mesenchymal tumor: a report of 6 patients treated at a single institution and comparison with reported series.
abstract: Osteogenic osteomalacia (OO)-associated phosphaturic mesenchymal tumors (PMTs) might represent a single histopathologic paraneoplastic entity. These tumors are largely misunderstood, ignored, or unknown by pathologists and clinicians. To elucidate the characteristics of OO-associated PMTs, we retrospectively analyzed the clinicopathologic features of PMTs from 6 patients, with either known OO or features suggestive of PMT-mixed connective tissue variant, who were studied and managed at a single center during the period from 1993 to 2011. Histologically, the tumor showed proliferation of spindle cells with focal areas of matrix production that showed distinct calcification, ossification, and osteoid-like matrix. Two patients had no evidence of disease and normal biochemical values; the other 2 patients each had multiple surgeries for multiple recurrences. In conclusion, PMT is histologically a benign lesion, with the malignant and metastatic variant being extremely rare. Infiltration of surrounding tissue is a frequent feature that is best managed with complete surgical removal of all involved tissue, which dramatically resolves the tumor-associated osteomalacia.
authors.affiliation: Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, USA.
authors.forename: Hanadi A
authors.initials: HA
authors.lastname: Fatani
authors.forename: Mohamad
authors.initials: M
authors.lastname: Sunbuli
authors.forename: Stephen Y
authors.initials: SY
authors.lastname: Lai
authors.forename: Diana
authors.initials: D
authors.lastname: Bell
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001859:Bone Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007015:Hypophosphatemia, Familial; D008297:Male; D008637:Mesenchymoma / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D018204:Neoplasms, Connective and Soft Tissue / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D010018:Osteomalacia / Q000150:complications* / Q000473:pathology / Q000601:surgery; D011859:Radiography; D012189:Retrospective Studies; D016896:Treatment Outcome