Epstein-Barr Virus-Associated Smooth Muscle Tumor of the Spine After Bone Marrow Transplant: Case Report and Review of Literature.
- 作者列表："Calafiore R","Mouchtouris N","Flomenberg N","Harrop JS
BACKGROUND:Epstein-Barr virus-associated smooth muscle tumors (SMTs) are rare neoplasms that have been found to develop in immunocompromised patients. Three distinct groups of affected patients have been described: (1) human immunodeficiency virus-infected patients, (2) post-transplant patients, and (3) patients with congenital immunodeficiency. The tumors can develop anywhere in the body, with 17 reported cases occurring in the spinal canal, all in patients with human immunodeficiency virus infection. CASE DESCRIPTION:We report the first case of Epstein-Barr virus-associated SMT affecting the spinal canal in a post-bone marrow transplant adult patient. Interestingly, unlike other reported cases, the patient described here had not been receiving immunosuppressive therapy in the 2 years prior to diagnosis of the tumor. CONCLUSIONS:Despite the growing number of case reports, this diagnosis presents a challenge, as the pathophysiology and optimal treatment regimens are not well understood. Results of a literature review of Epstein-Barr virus-associated SMT of the spine as well as a discussion of the presentation, management, and prognosis of this condition is presented here.
背景: Epstein-Barr病毒相关的平滑肌肿瘤 (SMTs) 是一种罕见的肿瘤，已被发现在免疫功能低下的患者中发展。已经描述了三组不同的受累患者 :( 1) 人类免疫缺陷病毒毒感染患者，(2) 移植后患者和 (3) 先天性免疫缺陷患者。肿瘤可以开发在身体的任何部位，17 个案件发生在椎管内，所有患者的人体免疫机能丧失病毒感染. 病例描述: 我们报道了第一例骨髓移植后成人患者中Epstein-Barr病毒相关SMT影响椎管的病例。有趣的是，与其他报道的病例不同，这里描述的患者在诊断肿瘤前 2 年内没有接受过免疫抑制治疗。 结论: 尽管病例报告越来越多，但由于病理生理学和最佳治疗方案还不是很清楚，这一诊断提出了挑战。本文介绍了Epstein-Barr病毒相关脊柱SMT的文献综述结果以及对该疾病的表现、处理和预后的讨论。
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.