Abadi Younes, Mileva Magdalena, Léger Marc-André, Sidiras Paschalis, Artigas Carlos, Flamen Patrick, Karfis Ioannis
Department of Nuclear Medicine, Institut Jules Bordet, Université Libre de Bruxelles, Brussels, Belgium.
Department of Rheumatology and Physical Medicine, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium.
EJNMMI Rep. 2024 Sep 18;8(1):30. doi: 10.1186/s41824-024-00219-3.
Tumor-induced osteomalacia (TIO) is a paraneoplastic syndrome caused by abnormally high levels of fibroblast growth factor 23 (FGF-23), most commonly produced and secreted by small phosphaturic mesenchymal tumors (PMT). These tumors can show various anatomic locations throughout soft tissue and bone. The presence of the tumor itself rarely causes symptoms. Nonspecific symptoms such as muscle weakness and musculoskeletal pain are related to the developing hypophosphatemia and osteomalacia as a secondary effect of the increased circulating levels of FGF-23. Therefore, as the initial presentation can mimic a wide range of metabolic or inflammatory diseases, proper diagnosis is often delayed. Localization of the tumor is crucial, as its complete surgical resection is the only curative treatment. Whole-body functional imaging targeting the overexpression of somatostatin receptors (SSTR) on the surface of the PMT cells, is a highly specific and sensitive imaging method to detect the primary tumor site. Here, we discuss a case of TIO in a patient initially presenting with symptoms of inflammatory spondyloarthritis. SSTR positron emission imaging using 68Ga-DOTATATE was central in diagnosing and localizing the primary tumor.
肿瘤诱导的骨软化症（TIO）是一种副肿瘤综合征，由成纤维细胞生长因子23（FGF-23）水平异常升高引起，最常见于由小的磷尿性间叶肿瘤（PMT）产生和分泌。这些肿瘤可出现在整个软组织和骨骼的不同解剖位置。肿瘤本身很少引起症状。诸如肌肉无力和肌肉骨骼疼痛等非特异性症状与FGF-23循环水平升高的继发效应——低磷血症和骨软化症的发展有关。因此，由于初始表现可模仿多种代谢或炎症性疾病，正确诊断往往延迟。肿瘤的定位至关重要，因为其完整的手术切除是唯一的治愈性治疗方法。针对PMT细胞表面生长抑素受体（SSTR）过表达的全身功能成像，是检测原发肿瘤部位的一种高度特异性和敏感性的成像方法。在此，我们讨论一例最初表现为炎性脊柱关节炎症状的TIO患者。使用68Ga-DOTATATE的SSTR正电子发射成像在诊断和定位原发肿瘤方面起了关键作用。
