Primary Hepatic Carcinosarcoma Composed of Hepatocellular Carcinoma, Cholangiocarcinoma, Osteosarcoma and Rhabdomyosarcoma With Poor Prognosis.
- 作者列表："Liu LI","Ahn E","Studeman K","Campbell K","Lai J
BACKGROUND:Primary hepatic carcinosarcoma is a rare subtype of liver malignancy, with only a small number of cases described in the English literature. CASE REPORT:We report the case of a 72-year-old man with a history of hepatitis C, who presented with complaints of abdominal pain. The patient's alpha fetoprotein (AFP) level was highly elevated at 7,406 ng/ml. His albumin, total bilirubin, aspartate aminotransferase, alanine aminotransferase, and alkaline phosphatase levels were within normal ranges. Computer tomographic scans discovered a 12×9×8 cm mass in the left lobe of the liver, extending to the anterior gastric wall. A partial hepatectomy of segments 2 and 3 with en bloc distal gastrectomy and omentectomy, a Roux-en-Y gastrojejunostomy, and a cholecystectomy were performed. Pathology revealed the mass to be a hepatic carcinosarcoma composed of collision tumor of four malignant components: hepatocellular carcinoma, cholangiocarcinoma, osteosarcoma and rhabdomyosarcoma. One and half month post-surgery, the patient was found to have a mass confirmed by biopsy as hepatocellular carcinoma in the right lobe, nodules in his lung and bone, and his AFP level elevated to 51,027.6 ng/ml. He died after two months during hospice care. CONCLUSION:To the best of our knowledge, this is the first documented case of primary hepatic carcinosarcoma with collision tumor of four malignant entities (hepatocellular carcinoma, cholangiocarcinoma, osteosarcoma and rhabdomyosarcoma). The pathogenesis, diagnosis, treatment and prognosis of this disease are discussed.
背景: 原发性肝癌肉瘤是一种罕见的肝脏恶性肿瘤亚型，只有少数病例在英文文献中描述。 C ASE报告: 我们报告了一位 72 岁男性的c ase，他有肝炎C史，c症状腹痛。患者的甲胎蛋白 (AFP) 水平在 7,406 ng/ml时高度升高。他的白蛋白、总胆红素、天冬氨酸氨基转移酶、丙氨酸氨基转移酶和碱性磷酸酶水平均在正常范围内。计算机断层扫描发现肝左叶有一个 12 × 9 × 8厘米的肿块，延伸至胃前壁。行 2 段和 3 段的部分肝切除术，行整块远端胃切除术和大网膜切除术，胃空肠Roux-en-Y吻合术和胆囊切除术。病理证实为肝癌肉瘤，由肝细胞癌、胆管癌、骨肉瘤和横纹肌肉瘤四种恶性成分的碰撞瘤组成。术后 1 个半月，发现患者有一个肿块，经活检证实为右叶肝细胞癌，肺和骨有结节，他的AFP水平升高到 51,027.6 纳克/毫升。他在临终关怀期间两个月后死亡。 结论: 据我们所知，这是第一例原发性肝癌肉瘤与四种恶性实体 (肝细胞癌、胆管癌、骨肉瘤和横纹肌肉瘤) 碰撞肿瘤的记录病例。对本病的发病机制、诊断、治疗及预后进行了讨论。
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.