A nonphosphaturic mesenchymal tumor mixed connective tissue variant of the sacrum.
Tumor-induced or oncogenic osteomalacia is a rare paraneoplastic syndrome characterized by overproduction of fibroblast growth factor-23 as a phosphaturic agent and renal phosphate wasting. A range of predominantly mesenchymal neoplasms have been associated with tumor-induced osteomalacia and classified as phosphaturic mesenchymal tumor mixed connective tissues. However, phosphaturic mesenchymal tumor mixed connective tissues could be nonphosphaturic in the first stage of the disease, either because the tumors are resected early in the clinical course or because the patient's osteomalacia was attributed to another cause. This article presents a case of a 42-year-old woman with a 2-year history of low back and right leg pain. Laboratory examinations including serum and urine calcium and phosphorous were within normal values. Imaging of the lumbar spine and pelvis showed an osteolytic lesion occupying the right sacral wing. Histology was unclear. Reverse-transcription polymerase chain reaction analysis for fibroblast growth factor-23 was positive and confirmed the diagnosis of phosphaturic mesenchymal tumor mixed connective tissues. Preoperative selective arterial embolization and complete intralesional excision, bone grafting, and instrumented fusion from L4 to L5 to the iliac wings bilaterally was performed. Postoperative recovery was uneventful. Neurological deficits were not observed. A lumbopelvic corset was applied for 3 months. At 12 months, the patient was asymptomatic. Serum and urine values of calcium and phosphorous were normal throughout the follow-up evaluation.
First Department of Orthopedics, Athens University Medical School, Athens, Greece.
Andreas F
AF
Mavrogenis
Vasileios I
VI
Sakellariou
Konstantinos
K
Soultanis
Helen
H
Mahera
Demetrios S
DS
Korres
Panayiotis J
PJ
Papagelopoulos
D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D005260:Female; D000089703:Fibroblast Growth Factor-23; D005346:Fibroblast Growth Factors / Q000378:metabolism; D006801:Humans; D007015:Hypophosphatemia, Familial; D008159:Lumbar Vertebrae / Q000000981:diagnostic imaging / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008637:Mesenchymoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D010014:Osteolysis / Q000000981:diagnostic imaging; D010018:Osteomalacia / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D010384:Pelvic Bones / Q000000981:diagnostic imaging / Q000473:pathology; D011859:Radiography; D012447:Sacrum / Q000473:pathology* / Q000601:surgery; D013123:Spinal Fusion; D013125:Spinal Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D016896:Treatment Outcome
D014408:Biomarkers, Tumor; D005346:Fibroblast Growth Factors; D000089703:Fibroblast Growth Factor-23