Cacoub P, Piette J C, Wechsler B, Ziza J M, Blétry O, Bahnini A, Kieffer E, Godeau P
Department of Internal Medicine, Groupe Hospitalier La Pitié-Salpétrière, Paris, France.
Medicine (Baltimore). 1991 Sep;70(5):293-306. doi: 10.1097/00005792-199109000-00002.
Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor originating in the smooth muscle of the media. Although rare, it is the most common malignancy in the IVC. One hundred and six cases have been reported thus far in the world literature, usually as isolated case reports. Clinical, radiologic, and therapeutic management and follow-up, including 7 additional cases, have been reviewed and summarized. Clinical manifestations are dependent upon the location of the tumor. The main symptom was a palpable mass for a tumor in segment I, abdominal pain for segment II, the presence of Budd-Chiari syndrome for segment III. Segment II was the most frequent site of leiomyosarcoma of the IVC, alone (n = 41) or with other segments (n = 39). Before laparotomy, clinical recognition was difficult or impossible. Recently, however, newer imaging modalities including ultrasound and CT scan have permitted earlier diagnosis. Metastases, when diagnosed, were either present at diagnosis (n = 20) or appeared as the disease progressed (n = 18). Metastatic disease frequently involved the liver, lung, lymph nodes, or bone. The small number of patients alive without metastases (16/113) must be analyzed all the more carefully because these patients were followed for less than 2 years. When prolonged follow-up is possible, the number of patients alive without neoplastic disease is significantly reduced. We found the prognosis of patients with LMS of the IVC to be poor. Diagnosis was made at autopsy for 27 patients. Among the 86 patients with follow-up information, 59 died within a mean of 16 months, and 26 were alive 25 months after the diagnosis. The main prognostic factor is topography, particularly the highest level of extension of the tumor. The upper-segment tumors have the poorest prognosis. The best therapeutic management is difficult to recommend because most of the cases in the literature did not include a sufficient follow-up. Given the very small number of patients completely free of neoplastic disease after sufficient follow-up, it seems unlikely that leiomyosarcoma of the IVC can now be cured. Patients who received a combination of surgery, radiotherapy and chemotherapy remained free of disease for longer periods. The unanswered question is: what is the best timing for each of these treatments? We recommend diagnosis of leiomyosarcoma of the IVC through biopsy guided by ultrasonography or computed tomographic scan. Therapeutic management should include large doses of chemotherapy preoperatively with or without radiotherapy to reduce tumor size.(ABSTRACT TRUNCATED AT 250 WORDS)
下腔静脉平滑肌肉瘤是一种起源于中膜平滑肌的罕见恶性肿瘤。尽管罕见，但它是下腔静脉最常见的恶性肿瘤。迄今为止，世界文献中已报道了106例，通常为孤立病例报告。本文回顾并总结了包括另外7例病例在内的临床、放射学、治疗管理及随访情况。临床表现取决于肿瘤的位置。I段肿瘤的主要症状是可触及肿块，II段为腹痛，III段为布加综合征。II段是下腔静脉平滑肌肉瘤最常见的部位，单独出现（n = 41）或与其他段合并出现（n = 39）。在剖腹手术前，临床诊断困难甚至无法诊断。然而，最近包括超声和CT扫描在内的新型影像学检查手段使得早期诊断成为可能。转移瘤在诊断时即已存在（n = 20）或随病情进展出现（n = 18）。转移瘤常累及肝脏、肺、淋巴结或骨骼。少数无转移存活的患者（16/113）必须更仔细地分析，因为这些患者的随访时间不足2年。如果能够延长随访时间，无肿瘤疾病存活的患者数量会显著减少。我们发现下腔静脉平滑肌肉瘤患者的预后较差。27例患者在尸检时确诊。在86例有随访信息的患者中，59例在平均16个月内死亡，26例在诊断后25个月时仍存活。主要预后因素是肿瘤位置，尤其是肿瘤延伸的最高水平。上段肿瘤预后最差。由于文献中的大多数病例随访不足，难以推荐最佳治疗方案。鉴于经过充分随访后完全无肿瘤疾病的患者数量极少，下腔静脉平滑肌肉瘤目前似乎无法治愈。接受手术、放疗和化疗联合治疗的患者无病生存期更长。尚未解决的问题是：这些治疗的最佳时机是什么？我们建议通过超声或计算机断层扫描引导下的活检诊断下腔静脉平滑肌肉瘤。治疗方案应包括术前大剂量化疗，可联合或不联合放疗以缩小肿瘤大小。
