Abstract：
This systematic review was performed to understand better the myriad presentations, various therapeutic options, response to therapy, and its clinical outcomes in hyperphosphatemic tumoral calcinosis (HTC). Full texts were selected according to strict inclusion criteria. All case reports of HTC wherein baseline phosphate was measured, treatment offered was mentioned, and information on follow-up and response to therapy that were available were included. A total of 43 of 188 eligible studies (N = 63 patients) met the inclusion criteria. A list of desired data was extracted and graded for methodological quality. A total of 63 individuals (Males = 33) were included from the 43 eligible case studies. The median age of the patients was 18 (IQR 8-32) years. The most frequently involved sites were the hip/gluteal region (34/63; 53.9%) followed by the elbow/forearm (26/63; 41.2%), and the shoulder (18/63; 28.5%). Three patients had conjunctival calcific deposits. The mean (SD) phosphate was 6.9 (1.1) mg/dL. Among the subjects, 36/63 (57.1%) underwent surgical excision with some form of medical therapy. Two patients underwent only surgical excision (2.1%). One patient was maintained on follow-up (1.6%) and 24/63 (38.1%) patients were treated with medical measures. The median (IQR) follow-up duration was 3 (1-9) years. Regression or reduction in lesion size was reported in 19/63 (30.2%) subjects; 20/63 (31.7%) showed progression, 24/63 (38.1%) had features of stable disease, and mortality was reported in 3 patients (4.7%). We report for the first time a detailed description of the clinical and therapeutic response of HTC. A combination of medical measures aimed at lowering serum phosphate appears to be the cornerstone of treatment, although clinical responses may vary.
摘要：
进行这项系统回顾是为了更好地理解无数的演讲，各种治疗选择，对治疗的反应，及其在高磷血症性肿瘤钙质沉着症（HTC）中的临床结果。根据严格的纳入标准选择全文。HTC的所有病例报告，其中测量了基线磷酸盐，提到了提供的治疗，纳入了现有的随访和治疗反应信息.188项符合条件的研究中，共有43项（N=63例）符合纳入标准。提取了所需数据列表，并对方法质量进行了分级。共有63个人（男性=33）从43个合格的案例研究中纳入。患者的中位年龄为18(IQR8-32)岁。最常见的部位是髋关节/臀区（34/63;53.9％），其次是肘部/前臂（26/63;41.2％），和肩膀（18/63；28.5％）。三名患者有结膜钙化沉积。平均(SD)磷酸盐为6.9(1.1)mg/dL。在主题中，36/63（57.1％）接受了某种形式的药物治疗的手术切除。两名患者仅接受手术切除（2.1％）。1例患者维持随访（1.6％），24/63（38.1％）患者接受医学治疗。中位随访时间（IQR）为3（1-9）年。在19/63（30.2%）受试者中报告了病变大小的消退或减少；20/63（31.7%）显示进展，24/63(38.1%)具有疾病稳定的特征，3例患者报告死亡(4.7%).我们首次报告了HTC的临床和治疗反应的详细描述。旨在降低血清磷酸盐的综合医疗措施似乎是治疗的基石，尽管临床反应可能有所不同。
