Clinical Profile and Treatment Outcomes of Tumour-Induced Osteomalacia - A Single-Centre Experience.

Prashiddha Dhakal, Alpesh Goyal, Viveka P Jyotsna, Ashutosh K Arya, Devasenathipathy Kandasamy, Shipra Agarwal, Madhvi Tripathi, Mani Kalaivani, Rajesh Khadgawat, Nikhil Tandon
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引用次数: 0

Abstract

Introduction: Tumour-induced osteomalacia (TIO) is rare. At our referral centre, we see a substantial number of TIO. Therefore, we planned to study their profile and treatment outcomes to provide insight in management.

Methods: This ambispective study evaluated 43 patients with TIO treated at our centre (2014-2024). Patients were grouped into (a) Localised TIO (n = 31; histopathology suggesting phosphaturic mesenchymal tumour or disease remission and (b) Unlocalised TIO (n = 12; occult tumour and negative genetic testing for hereditory hypophosphatemic disorders).

Results: The mean age of participants was 40.9 ± 13.4 years. The median diagnostic delay was 3 years from symptom onset. Bone pain, muscle weakness, fractures, teeth loss, and palpable lump were presenting features. Two had intact fibroblast growth factor 23 (iFGF23) in normal range. No significant clinical bias existed between tubular maximum reabsorption of phosphate/glomerular filtration rate (TmP/GFR) calculated using second void urine and 24-h urine samples. The most common location was lower limbs (41.9%), followed by head and neck (32.3%). Tracer avid lesions on Ga-68-DOTANOC-positron emission tomography/computed tomography (PET/CT) were noted in 30/43 (69.8%) patients. Of the 13 patients negative on somatostatin receptor-based imaging, only one showed tracer avid lesion on F-18-fluorodeoxyglucose-PET/CT scan. Disease remission was documented in 19/24 (79.2%) patients undergoing surgical excision and 1/5 (20%) treated with radiofrequency ablation. After successful intervention, plasma iFGF23 levels normalised by Day-3, tubular reabsorption of phosphate and TmP/GFR by Day-4, and serum phosphate by Day-7. No patient with remission experienced relapse at a median follow-up of 4.25 years.

Conclusion: Ga-68-DOTANOC-PET/CT picked up maximum lesions, F-18-FDG-PET/CT picked up one additional lesion. Surgery was curative in most. Post successful intervention, iFGF23 normalised in all by the third day.

Abstract Image

Abstract Image

Abstract Image

肿瘤诱导的骨软化症的临床概况和治疗结果-单中心经验。
肿瘤诱导的骨软化症(TIO)是罕见的。在我们的转介中心,我们看到了相当数量的TIO。因此,我们计划研究他们的概况和治疗结果,为管理提供见解。方法:本研究对我院2014-2024年收治的43例TIO患者进行了两方面的评估。患者被分为(a)局部TIO (n = 31,组织病理学提示磷化间质肿瘤或疾病缓解)和(b)非局部TIO (n = 12,隐匿性肿瘤和遗传性低磷血症基因检测阴性)。结果:参与者平均年龄40.9±13.4岁。中位诊断延迟为症状出现后3年。骨痛、肌肉无力、骨折、牙齿脱落和可触及的肿块是主要特征。2例成纤维细胞生长因子23 (iFGF23)在正常范围内。用第二次空尿计算的肾小管最大磷酸盐重吸收/肾小球滤过率(TmP/GFR)与24小时尿样之间不存在显著的临床偏差。最常见的部位是下肢(41.9%),其次是头颈部(32.3%)。43例患者中有30例(69.8%)在ga -68- dotanoc -正电子发射断层扫描/计算机断层扫描(PET/CT)上发现示踪性病变。在13例生长抑素受体成像阴性的患者中,只有1例在f -18-氟脱氧葡萄糖- pet /CT扫描中显示示踪性病变。24例患者中有19例(79.2%)接受手术切除,5例患者中有1例(20%)接受射频消融治疗。干预成功后,血浆iFGF23水平在第3天恢复正常,磷酸小管重吸收和TmP/GFR在第4天恢复正常,血清磷酸在第7天恢复正常。在中位随访4.25年期间,没有缓解的患者复发。结论:Ga-68-DOTANOC-PET/CT检出最大病灶,F-18-FDG-PET/CT检出1个病灶。手术治疗大多数病例。干预成功后,iFGF23在第三天全部恢复正常。
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来源期刊
Indian Journal of Endocrinology and Metabolism
Indian Journal of Endocrinology and Metabolism Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
2.10
自引率
0.00%
发文量
75
期刊介绍: The Indian Journal of Endocrinology and Metabolism (IJEM) aims to function as the global face of Indian endocrinology research. It aims to act as a bridge between global and national advances in this field. The journal publishes thought-provoking editorials, comprehensive reviews, cutting-edge original research, focused brief communications and insightful letters to editor. The journal encourages authors to submit articles addressing aspects of science related to Endocrinology and Metabolism in particular Diabetology. Articles related to Clinical and Tropical endocrinology are especially encouraged. Sub-topic based Supplements are published regularly. This allows the journal to highlight issues relevant to Endocrine practitioners working in India as well as other countries. IJEM is free access in the true sense of the word, (it charges neither authors nor readers) and this enhances its global appeal.
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