Surgical treatment of recurrent spinal phosphaturic mesenchymal tumor-induced osteomalacia: A case report.
手术治疗复发性脊髓磷间质瘤引起的骨软化症 1 例。
- 作者列表："Liu S","Zhou X","Song A","Huo Z","Wang Y","Liu Y
RATIONALE:Tumor-induced osteomalacia (TIO) is a highly unusual disease with enormous difficulties in clinical diagnosis and curative managements. The objective of this study is to report a very rare case who underwent surgical treatment of recurrent spinal phosphaturic mesenchymal tumor. The management of these unique cases has yet to be further elucidated. PATIENT CONCERNS:A 52-year-old man presented with a 3-year history of back pain and 1-year history of continuous and progressive systemic bone pain. The patient, who had been diagnosed of TIO for 3 years, received surgical treatment of extended resection of spinal phosphaturic mesenchymal tumor at L5. Somatostatin receptor tomography revealed the expression of somatostatin in the spine increased significantly, with high suspicion of recurrent phosphaturic mesenchymal tumor. DIAGNOSIS:Magnetic resonance imaging of spine and positron emission tomography-computed tomography showed the mass in L5, which was highly indicative of the recurrent pathogenic tumor. Postoperative pathology confirmed the diagnosis of phosphaturic mesenchymal tumor in the spinal region. INTERVENTIONS:The patient underwent posterior L5 tumor resection, bone cement reconstruction, L4-S1 spinal canal decompression, and L3-S2 internal fixation. OUTCOMES:The patient's symptoms improved significantly after the surgery, and we noticed that his hypophosphatemia was successfully corrected after the 2nd operation. Follow-up at 1 month after surgery revealed no recurrence, and the serum phosphorus level of the patient turned to be normal postoperatively. There were no complications associated with the operation during the follow-up period. LESSONS:Taken together, the lesion's clinical features, imaging results, and pathologic characteristics are unique. Combined efforts of specialists from orthopedics, endocrinology, nuclear medicine, radiology, pathology, and medical oncology led to the successful diagnosis and management of this patient. TIO, although rare, should be part of the differential diagnosis when the patient has a history of hypophosphatemia and systemic multiple bone pain. We recommend surgical treatment of the phosphaturic mesenchymal tumor in the spinal region. Osteoplasty by bone cement may be a treatment option for patients with TIO who cannot undergo appropriate surgery or decline open surgery.
肿瘤引起的骨软化症 (TIO) 是一种高度罕见的疾病，在临床诊断和治疗管理方面存在巨大困难。本研究的目的是报告一例非常罕见的谁接受了手术治疗的复发性脊髓磷酸间叶性肿瘤。这些独特病例的管理尚未进一步阐明。 患者顾虑: 1 例 52 岁男性，3 年腰背痛病史，1 年持续性、进行性全身骨痛病史。该患者已被诊断为TIO 3 年，在l5 接受了扩大切除脊髓磷间质瘤的手术治疗。生长抑素受体断层扫描发现生长抑素在脊柱中的表达明显增加，高度怀疑复发的磷尿间质瘤。 诊断: 脊柱磁共振成像和正电子发射断层扫描-计算机断层扫描显示L5 肿块，高度提示复发性致病性肿瘤。术后病理证实为脊髓区磷间质瘤。 干预措施: 患者行后路L5 肿瘤切除，骨水泥重建，L4-S1 椎管减压，L3-S2 内固定。 结果: 患者手术后症状明显改善，我们注意到他的低磷血症在第2 手术后被成功纠正。术后 1 个月随访，无复发，术后血磷恢复正常。随访期间未出现与手术相关的并发症。 教训: 综合起来，病变的临床特征、影像学结果和病理特征是独一无二的。骨科、内分泌学、核医学、放射学、病理学和医学肿瘤学专家的共同努力导致了该患者的成功诊断和管理。TIO虽然罕见，但当患者有低磷血症和全身多发性骨痛病史时，应作为鉴别诊断疾病的一部分。我们推荐手术治疗脊髓区的磷性间叶性肿瘤。对于不能进行适当手术或拒绝开放手术的TIO患者，骨水泥骨成形术可能是一种治疗选择。
METHODS:OBJECTIVE:Large inoperable sacral chordomas show unsatisfactory local control rates even when treated with high dose proton therapy (PT). The aim of this study is assessing feasibility and reporting early results of patients treated with PT and concomitant hyperthermia (HT). METHODS: :Patients had histologically proven unresectable sacral chordomas and received 70 Gy (relative biological effectiveness) in 2.5 Gy fractions with concomitant weekly HT. Toxicity was assessed according to CTCAE_v4. A volumetric tumor response analysis was performed. RESULTS: :Five patients were treated with the combined approach. Median baseline tumor volume was 735 cc (range, 369-1142). All patients completed PT and received a median of 5 HT sessions (range, 2-6). Median follow-up was 18 months (range, 9-26). The volumetric analysis showed an objective response of all tumors (median shrinkage 46%; range, 9-72). All patients experienced acute Grade 2-3 local pain. One patient presented with a late Grade 3 iliac fracture. CONCLUSION:Combining PT and HT in large inoperable sacral chordomas is feasible and causes acceptable toxicity. Volumetric analysis shows promising early results, warranting confirmation in the framework of a prospective trial. ADVANCES IN KNOWLEDGE: :This is an encouraging first report of the feasibility and early results of concomitant HT and PT in treating inoperable sacral chordoma.
METHODS:BACKGROUND:National guidelines recommend screening and treatment for cancer-related bone disease and continued monitoring of bone-modifying agents. It is unclear whether a standardized screening tool is utilized to identify eligible patients and ensure appropriate supportive care is implemented. The purpose of this study was to evaluate current prescribing practices and optimize management of bone-modifying agents. METHODS:A retrospective chart review was performed to identify patients who received hormone deprivation therapy or had bone metastases through Hematology/Oncology or Urology clinics from 1 November 2016 to 31 October 2017. The primary endpoints of this study were the incidence of completed baseline dual-energy X-ray absorptiometry (DEXA) scan for patients on hormone deprivation therapy and percent of patients started on a bone-modifying agent for the prevention of skeletal-related events secondary to bone metastasis. Secondary endpoints included percent of patients with dental examinations prior to initiation, adequate calcium and vitamin D supplementation, incidence of osteonecrosis of the jaw or flu-like symptoms and education, and percent of bisphosphonate doses appropriately adjusted based on renal function. RESULTS:A total of 375 patients were assessed for baseline DEXA scans and bone-modifying therapy. Of the 226 patients on hormone deprivation therapy, 111 (49%) patients were appropriately screened with a DEXA scan prior to initiation of hormone deprivation therapy. Among the 149 patients with bone metastases, only 94 (63.1%) patients were started on a bone-modifying agent. CONCLUSIONS:Opportunities have been identified to optimize management of patients with cancer-related bone disease. Implementation of standardized tools may increase the rate of appropriate screening and initiation of bone-modifying therapy when warranted.
METHODS:PURPOSE:Low skeletal muscle mass has been associated with poor prognosis in patients with advanced lung cancer. However, little is known about the relationship between skeletal muscle mass and overall survival in patients with bone metastases from lung cancer. The objective of the present study was to evaluate the prognostic value of low trunk muscle mass in predicting overall survival in these patients. METHODS:The data from 198 patients who were diagnosed with bone metastases from lung cancer from April 2009 to May 2017 were retrospectively reviewed. The areas of the psoas and paravertebral muscles were measured at the level of the third lumbar vertebra on computed tomography scans taken at the time nearest to the diagnosis of bone metastasis. Muscle area was evaluated for male and female cohorts separately using different cutoff points. Cox proportional hazards analysis was performed to evaluate the factors independently associated with overall survival. RESULTS:The overall survival of patients in the lowest quartile for psoas muscle area or paravertebral muscle area was significantly shorter than that of patients above the 25th percentile for muscle area (p < 0.001). Multivariate analyses showed that paravertebral muscle mass (hazard ratio, 1.73; 95% confidence interval, 1.17-2.56; p = 0.006), epidermal growth factor receptor-targeted therapy, and performance status were independent prognostic factors. CONCLUSIONS:Low paravertebral muscle mass was associated with shorter survival, independently of known prognostic factors.