Tumorlokalisation und Therapie der onkogenen Osteomalazie

TY - JOUR

T1 - Tumorlokalisation und Therapie der onkogenen Osteomalazie

AU - Beil, Frank Timo

AU - Stürznickel, Julian

AU - Rolvien, Tim

AU - Amling, Michael

AU - Oheim, Ralf

N1 - © 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.

PY - 2022/4

Y1 - 2022/4

N2 - Tumor-induced osteomalacia (TIO) or oncogenic osteomalacia (OOM) is a rare paraneoplastic renal phosphate wasting syndrome. The disease is mostly triggered by small, benign mesenchymal tumors that express somatostatin receptors (SSTR) and produce excessive levels of fibroblast growth factor 23 (FGF 23) or other phosphatonins. These reduce the phosphate back resorption in the proximal tubules of the kidneys, thereby causing hypophosphatemia and lead to an absolute or relatively low calcitriol serum concentration. The main symptoms include muscle weakness, bone pain and recurrent insufficiency fractures secondary to sometimes pronounced osteomalacia. The suspected diagnosis can only be confirmed by determination of the phosphate level. It can often take years before the tumor is successfully localized. The necessary tumor localization is often the most difficult step in the treatment before the OOM can be curatively treated by open surgical resection of the tumor. In recent years new approaches for faster tumor localization and treatment of the tumor have been developed. Positron emission tomography (PET) in co-registration with computed tomography (68Ga-DOTA-TATE PET/CT) is currently the most sensitive imaging methodology for tumor detection. The application of the monoclonal FGF 23 antibody burosumab represents a promising new option in the treatment of inoperable adult OOM.

AB - Tumor-induced osteomalacia (TIO) or oncogenic osteomalacia (OOM) is a rare paraneoplastic renal phosphate wasting syndrome. The disease is mostly triggered by small, benign mesenchymal tumors that express somatostatin receptors (SSTR) and produce excessive levels of fibroblast growth factor 23 (FGF 23) or other phosphatonins. These reduce the phosphate back resorption in the proximal tubules of the kidneys, thereby causing hypophosphatemia and lead to an absolute or relatively low calcitriol serum concentration. The main symptoms include muscle weakness, bone pain and recurrent insufficiency fractures secondary to sometimes pronounced osteomalacia. The suspected diagnosis can only be confirmed by determination of the phosphate level. It can often take years before the tumor is successfully localized. The necessary tumor localization is often the most difficult step in the treatment before the OOM can be curatively treated by open surgical resection of the tumor. In recent years new approaches for faster tumor localization and treatment of the tumor have been developed. Positron emission tomography (PET) in co-registration with computed tomography (68Ga-DOTA-TATE PET/CT) is currently the most sensitive imaging methodology for tumor detection. The application of the monoclonal FGF 23 antibody burosumab represents a promising new option in the treatment of inoperable adult OOM.

U2 - 10.1007/s00393-022-01160-1

DO - 10.1007/s00393-022-01160-1

M3 - SCORING: Review

C2 - 35103802

VL - 81

SP - 182

EP - 188

JO - Z RHEUMATOL

JF - Z RHEUMATOL

SN - 0340-1855

IS - 3

ER -