Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0598934 (tumor growth)
 58,965 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 1992 The International Registry of Inferior Vena Cava (IVC) Leiomyosarcomas was established to study the pathogenesis and natural history of the tumor and to support the most rational treatment. We collected 218 patients through a literature review and personal communications. We corresponded with several Authors to obtain up-to-date follow-up and any other data lacking at the initial review. The series was analyzed to identify predictive factors for clinical outcome. Tumors arose from the IVC lower segment in 80 patients, from the middle in 94 and from the upper in 41. A radical tumor resection was undertaken in 134 (61.5%) patients, 26 (11.9%) had a palliative resection and 58 (26.6%) were inoperable. An increased risk of death was associated with upper IVC segment involvement (p < 0.001), lower limb edema (p < 0.001), Budd-Chiari's syndrome (p < 0.001), intraluminal tumor growth (p < 0.001) and IVC occlusion (p < 0.001). Radical tumor resection was associated with better 5- and 10-year survival rates (49.4% and 29.5%). Tumors which arose from the middle segment fared better (56.7% and 47.3%) than those of the lower segment (37.8% and 14.2%) (p < 0.002). No palpable abdominal mass and abdominal pain were associated, in patients radically operated, with a better outcome and longer survival (p < 0.03 and p > 0.04 respectively). Despite the high rate of recurrence, radical tumor resection is the only long-term cure.
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PMID:International registry of inferior vena cava leiomyosarcoma: analysis of a world series on 218 patients. 892 Jul 90

Leiomyosarcomas of the inferior Vena Cava (IVC) are rare soft tissue sarcomas accounting for only 0.5% of all soft tissue sarcomas in adults with fewer than 300 cases reported. Extraluminal tumor growth along the adventitia of the IVC seems to be the common presentation. Intraluminal tumor growth is rare. The origin of the tumor is divided into three levels in relation to the hepatic and renal veins. The presentations and surgical modalities vary accordingly. Retroperitoneal tumors are often not diagnosed until the disease is at an advanced stage with large tumor growth and involvement of surrounding structures. This is partly because of the nonspecific clinical presentation as well as absence of early symptoms. Most patients present with abdominal or flank pain. Symptoms vary according to the dimensions of the tumor, growth pattern and localization of the tumor. Radical en bloc resection of the affected venous segment remains the only therapeutic option associated with prolonged survival. The goals of surgical management of these tumors include the achievement of local tumor control, maintenance of caval flow, and the prevention of recurrence. The involvement of renal or hepatic veins determines the strategy for vascular reconstruction. Reconstruction of the IVC is not always required, because gradual occlusion of the IVC allows the development of venous collaterals. However, when pararenal leiomyosarcoma of the IVC is present, reconstruction of the IVC and the renal vein is necessary to prevent transient or permanent renal dysfunction. Recent study has shown that radical surgery combined with adjuvant multimodal therapy has improved the cumulative survival rate. We report a case of IVC leiomyosarcoma in a young healthy woman along with details of its diagnostic workup and discussion of the surgical options and reconstruction of caval continuity.
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PMID:Leiomyosarcoma of the Inferior Vena Cava - Radical Resection, Vascular Reconstruction and Challenges: A Case Report and Review of Relevant Literature. 2914 40