Adnan Zaina, Nikomarov David, Weiler-Sagie Michal, Roguin Maor Noga
Endocrinology and Metabolism Department, Zvulon Medical Center, Clalit Medical Health Care Services, Haifa, Israel.
Orthopedic Surgery Department, Nuclear Medicine Department, Rambam Health Care Campus, Haifa, Israel.
Endocrinol Diabetes Metab Case Rep. 2019 May 3;2019. doi: 10.1530/EDM-18-01396.
Phosphaturic mesenchymal tumor (PMT) represents a rare cause of osteomalacia. The clinical signs and symptoms are vague and these lead to diagnosis delay. In the presence of hypophosphatemia and relatively high urine phosphate excretion, this entity should be taken into consideration in the deferential diagnosis of osteomalacia. In the present article, we report 81-year-old man presented to our clinic for evaluation due to osteopenia. His laboratory results disclosed hypophosphatemia, relatively increased urine phosphate excretion and increased level of intact fibroblast growth factor 23 (FGF23). A 68Gallium DOTATATE PET/CT revealed pathological uptake in the upper aspect of the left shoulder adjacent to the coracoid process. For suspected PMT a wide resection of the tumor was performed and pathological findings were consistent for PMT. Laboratory tests were normalized postoperatively. Reviewing the literature, we had identified 33 reported cases of PMTs among elderly patients age ≥70 years. Unlike previously reported data, where tumors predominantly localized in the lower extremities and pelvis, our search disclosed a high rate of tumor localization (10 cases - 33.3%) in the head with equal number of tumors (14 cases - 42.4%) localized in the head and upper extremity as well as in pelvis and lower extremity. The present case describes unique tumor localization in an elderly patient and our literature search demonstrated for the first time a high rate of tumor localization in the head among this group of patients. Learning points: PMTs represent a rare entity that should be considered in the differential diagnosis of elderly patients presented with persistent hypophosphatemia. Unlike previously reported data, head and neck tumor localization is frequent among elderly patients. 68Gallium-conjugated somatostatin peptide analogs, such as 68Ga-DOTATATE PET/CT demonstrated the greatest sensitivity and specificity for tumor localization in patients with phosphaturic mesenchymal tumors (PMTs). Wide tumor resection using intraoperative ultrasound is of major importance in order to ensure long-term cure.
磷尿性间叶肿瘤(PMT)是骨软化症的一种罕见病因。其临床症状和体征不明确,容易导致诊断延误。在存在低磷血症和相对较高的尿磷排泄情况下,在骨软化症的鉴别诊断中应考虑到该疾病。在本文中,我们报告了一名81岁男性因骨质减少前来我院就诊。他的实验室检查结果显示低磷血症、尿磷排泄相对增加以及完整的成纤维细胞生长因子23(FGF23)水平升高。68镓-奥曲肽PET/CT显示左肩部靠近喙突处有异常摄取。对于疑似PMT患者,进行了肿瘤广泛切除,病理结果与PMT一致。术后实验室检查恢复正常。查阅文献,我们在年龄≥70岁的老年患者中确定了33例已报告的PMT病例。与先前报告的数据不同,先前报告的肿瘤主要位于下肢和骨盆,而我们的检索发现头部肿瘤定位率较高(10例 - 33.3%),头部和上肢的肿瘤数量与骨盆和下肢的肿瘤数量相等(14例 - 42.4%)。本病例描述了老年患者中独特的肿瘤定位情况,我们的文献检索首次证明了该组患者中头部肿瘤定位率较高。学习要点:PMT是一种罕见疾病,在出现持续性低磷血症的老年患者鉴别诊断中应予以考虑。与先前报告的数据不同,老年患者中头颈部肿瘤定位较为常见。68镓标记的生长抑素肽类似物,如68Ga-奥曲肽PET/CT对磷尿性间叶肿瘤(PMT)患者的肿瘤定位显示出最高的敏感性和特异性。术中使用超声进行肿瘤广泛切除对于确保长期治愈至关重要。