Tan Teik Hin, Chen Ew-Jun, Chew Ming Tsuey, Chye Ping Ching, Wong Ming
Centre for Applied Physics and Radiation Technologies, School of Engineering and Technology, Sunway University, No. 5 Jalan Universiti, Bandar Sunway, 47500 Petaling Jaya, Selangor Darul Ehsan Malaysia.
Nuclear Medicine Centre, Sunway Medical Centre, No. 5 Jalan Lagoon Selatan, Bandar Sunway, 47500 Petaling Jaya, Selangor Darul Ehsan Malaysia.
Nucl Med Mol Imaging. 2021 Jun;55(3):130-135. doi: 10.1007/s13139-021-00695-6. Epub 2021 Apr 19.
Tumour-induced osteomalacia is a rare paraneoplastic syndrome that manifests as chronic hypophosphataemia, non-specific bone pain and muscle weakness. It is generally caused by phosphaturic mesenchymal tumour (PMT), which is uncommonly associated with synchronous tumours. However, diagnosis is often delayed for several years due to the rarity, indolent growing nature and non-specific symptoms of the disease, often resulting in an overlook by clinicians during assessments. The patient initially presented with hypophosphataemia and generalised skeletal pain with multiple atraumatic fractures. Blood tests revealed serum calcium levels at the upper limit and extremely low inorganic phosphate levels. Herein, we report a case where two synchronous PMTs from two different sites were detected by 'extended' whole-body Ga-68 DOTATATE PET-CT, leading to remission of the disease after complete surgical removal. Early detection and diagnosis of PMT neoplasm is crucial, as complete surgical resection of this tumour is the only definitive treatment currently known. Upon excision, this curable disease will result in complete resolution of symptoms and blood parameters, leading to remission of the disease which significantly improves the patient's quality of life. PMT often over-expresses somatostatin receptors (SSTR), predominantly subtype 2A, and Ga-68 DOTATATE PET-CT is a selective SSTR imaging that targets this characteristic over-expression in these tumours. The high diagnostic accuracy of Ga-68 DOTATATE PET-CT should be the primary imaging modality for full evaluation of this disease.
肿瘤诱导的骨软化症是一种罕见的副肿瘤综合征,表现为慢性低磷血症、非特异性骨痛和肌肉无力。它通常由磷尿性间叶肿瘤(PMT)引起,这种肿瘤很少与同步性肿瘤相关。然而,由于该疾病罕见、生长缓慢且症状不特异,诊断往往会延迟数年,常常导致临床医生在评估时忽视。该患者最初表现为低磷血症和全身骨骼疼痛伴多发非创伤性骨折。血液检查显示血清钙水平处于上限,无机磷水平极低。在此,我们报告一例通过“扩展”全身Ga - 68 DOTATATE PET - CT检测到两个不同部位的同步性PMT的病例,在完整手术切除后疾病得到缓解。PMT肿瘤的早期检测和诊断至关重要,因为目前已知完整手术切除该肿瘤是唯一的确定性治疗方法。切除后,这种可治愈的疾病将导致症状和血液参数完全缓解,使疾病得到缓解,显著提高患者的生活质量。PMT通常过度表达生长抑素受体(SSTR),主要是2A亚型,而Ga - 68 DOTATATE PET - CT是一种针对这些肿瘤中这种特征性过表达的选择性SSTR成像。Ga - 68 DOTATATE PET - CT的高诊断准确性应是全面评估该疾病的主要成像方式。