Remote Leptomeningeal Dissemination in Olfactory Neuroblastoma Mimicking Multiple Parasagittal Meningiomas: Diagnostic and Therapeutic Challenge.
- 作者列表："Martinez-Perez R","Hardesty DA","Palmer J","Zachariah M","Otto BA","Carrau RL","Prevedello DM
BACKGROUND:Olfactory neuroblastoma (ON) is a highly aggressive and locally recurrent neoplasm. Distant systemic metastases are not uncommon, but remote leptomeningeal dissemination is extremely rare. CASE DESCRIPTION:We report 2 cases of ON previously treated with endoscopic endonasal radical surgical resection and radiotherapy. After a relatively long period of disease-free survival, multiple leptomeningeal lesions were seen around the sagittal sinus giving a radiologic appearance of parasagittal meningiomas. Both patients underwent surgery and multimodal treatment with radiotherapy and chemotherapy for the disseminated disease. Pathologic examination confirmed the parasagittal lesions as metastatic ON. CONCLUSIONS:A high suspicion of secondary disease should be maintained in patients with previous history of ON and parasagittal leptomeningeal enhancing lesion, particularly when multiple lesions are detected around the sagittal sinus. Radical resection and multimodal treatment are warranted to improve long-term outcome. Understanding the venous drainage route as a potential pathway for remote seeding from the primary site of disease has therapeutic implications. We postulate that en-bloc tumor resection and proximal sagittal sinus ligation might reduce potential for leptomeningeal metastasis.
背景: 嗅神经母细胞瘤 (ON) 是一种高度侵袭性和局部复发的肿瘤。远处全身转移并不少见，但远端软脑膜播散极为罕见。 病例描述: 我们报告 2 例先前接受内镜下鼻内根治性手术切除和放疗的ON。经过相对较长时间的无病生存后，矢状窦周围可见多发性软脑膜病变，影像学表现为矢状窦旁脑膜瘤。两例患者均接受手术和多模式治疗，并配合放化疗治疗播散性疾病。病理检查证实上矢状窦旁病变为转移性。 结论: 既往有ON和矢状窦旁软脑膜强化病变病史的患者，尤其是在矢状窦周围发现多发病变时，应高度怀疑继发病变。根治性切除和多模式治疗是改善长期预后的必要条件。了解静脉引流途径作为从疾病原发部位远程播种的潜在途径具有治疗意义。我们假设肿瘤整块切除和近端矢状窦结扎可能会降低软脑膜转移的可能性。
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.