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Superior vena cava leiomyosarcoma

MedGen UID:
236963
•Concept ID:
C1336531
•
Neoplastic Process
Synonyms: anterior vena cava leiomyosarcoma; leiomyosarcoma of anterior vena cava; Leiomyosarcoma of Superior Vena Cava; leiomyosarcoma of Superior vena cava; Leiomyosarcoma of the Superior Vena Cava; leiomyosarcoma of the Superior vena cava; Superior Vena Cava Leiomyosarcoma; superior vena cava leiomyosarcoma
 
Monarch Initiative: MONDO:0004208

Definition

An aggressive malignant smooth muscle neoplasm, arising from the superior vena cava. It is characterized by a proliferation of neoplastic spindle cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSuperior vena cava leiomyosarcoma

Recent clinical studies

Etiology

Vena cava leiomyosarcoma surgery results in a retrospective cohort of 41 patients from two centers.
Bertrand T, Faron M, Mercier O, Ngo C, Le Pechoux C, Levy A, Issard J, Henon C, Honoré C, Fadel E, Le Cesne A
J Surg Oncol 2024 Sep;130(3):552-561. Epub 2024 Jul 7 doi: 10.1002/jso.27765. PMID: 38973131
Caval Reconstruction with Undersized Ringed Graft after Resection of Inferior Vena Cava Leiomyosarcoma.
Pantoja JL, Patel RP, Baril DT, Quinones-Baldrich W, Lawrence PF, Woo K
Ann Vasc Surg 2020 May;65:25-32. Epub 2019 Nov 9 doi: 10.1016/j.avsg.2019.11.007. PMID: 31715248Free PMC Article
Diagnosis, treatment and prognosis of the leiomyosarcoma of the inferior vena cava. Three cases and summary of published reports.
Mönig SP, Gawenda M, Erasmi H, Zieren J, Pichlmaier H
Eur J Surg 1995 Apr;161(4):231-5. PMID: 7612763

Diagnosis

Imaging sarcomas of the great vessels and heart.
Bendel EC, Maleszewski JJ, Araoz PA
Semin Ultrasound CT MR 2011 Oct;32(5):377-404. doi: 10.1053/j.sult.2011.06.001. PMID: 21963161
Superior vena cava thrombosis secondary to hickman catheter and complete resolution after fibrinolytic therapy.
Morales M, Llanos M, Dorta J
Support Care Cancer 1997 Jan;5(1):67-9. doi: 10.1007/BF01681965. PMID: 9010993
Leiomyosarcoma of the superior vena cava.
Spaggiari L, Regnard JF, Nottin R, Dulmet EM, Rusca M, Bobbio P, Levasseur P
Ann Thorac Surg 1996 Jul;62(1):274-6. doi: 10.1016/0003-4975(96)00152-x. PMID: 8678660
Diagnosis, treatment and prognosis of the leiomyosarcoma of the inferior vena cava. Three cases and summary of published reports.
Mönig SP, Gawenda M, Erasmi H, Zieren J, Pichlmaier H
Eur J Surg 1995 Apr;161(4):231-5. PMID: 7612763
Radiation-induced leiomyosarcoma of the great vessels presenting as superior vena cava syndrome.
Weiss KS, Zidar BL, Wang S, Magovern GJ Sr, Raju RN, Lupetin AR, Shackney SE, Simon SR, Singh M, Pugh RP
Cancer 1987 Sep 15;60(6):1238-42. doi: 10.1002/1097-0142(19870915)60:6<1238::aid-cncr2820600613>3.0.co;2-v. PMID: 3304612

Therapy

Caval Reconstruction with Undersized Ringed Graft after Resection of Inferior Vena Cava Leiomyosarcoma.
Pantoja JL, Patel RP, Baril DT, Quinones-Baldrich W, Lawrence PF, Woo K
Ann Vasc Surg 2020 May;65:25-32. Epub 2019 Nov 9 doi: 10.1016/j.avsg.2019.11.007. PMID: 31715248Free PMC Article
Inferior vena caval resection with autogenous peritoneo-fascial patch graft caval repair: a new technique.
Chin PT, Gallagher PJ, Stephen MS
Aust N Z J Surg 1999 May;69(5):391-2. doi: 10.1046/j.1440-1622.1999.01579.x. PMID: 10353558
Superior vena cava thrombosis secondary to hickman catheter and complete resolution after fibrinolytic therapy.
Morales M, Llanos M, Dorta J
Support Care Cancer 1997 Jan;5(1):67-9. doi: 10.1007/BF01681965. PMID: 9010993
Primary leiomyosarcoma of the superior vena cava with massive thrombosis treated by local fibrinolysis.
Marini M, Tovar E, López-Fernández MF, Pombo F, Rodríguez E
Br J Radiol 1992 Dec;65(780):1131-2. doi: 10.1259/0007-1285-65-780-1131. PMID: 1286424
Superior vena cava syndrome associated with gynecologic malignancy.
Puleo JG, Clarke-Pearson DL, Smith EB, Barnard DE, Creasman WT
Gynecol Oncol 1986 Jan;23(1):59-64. doi: 10.1016/0090-8258(86)90116-2. PMID: 3943753

Prognosis

Vena cava leiomyosarcoma surgery results in a retrospective cohort of 41 patients from two centers.
Bertrand T, Faron M, Mercier O, Ngo C, Le Pechoux C, Levy A, Issard J, Henon C, Honoré C, Fadel E, Le Cesne A
J Surg Oncol 2024 Sep;130(3):552-561. Epub 2024 Jul 7 doi: 10.1002/jso.27765. PMID: 38973131
Right atrial and SVC infiltrating mass-the entity of infiltrating lipoma.
Shah OA, Badran A, Kaarne M, Velissaris T
J Cardiothorac Surg 2019 Dec 2;14(1):210. doi: 10.1186/s13019-019-1015-7. PMID: 31791367Free PMC Article
Cardiac Metastasis of Nonvisceral Soft-tissue Leiomyosarcoma.
Martinez C, Rana JS, Solomon MD
Rev Cardiovasc Med 2017;18(2):78-81. doi: 10.3909/ricm0859. PMID: 29038416
Liver and Vena Cava En Bloc Resection for an Invasive Leiomyosarcoma Causing Budd-Chiari Syndrome, Under Veno-Venous Bypass and Liver Hypothermic Perfusion : Liver Hypothermic Perfusion and Veno-Venous Bypass for Inferior Vena Cava Leiomyosarcoma.
Ravaioli M, Serenari M, Cescon M, Savini C, Cucchetti A, Ercolani G, Del Gaudio M, Casati A, Pinna AD
Ann Surg Oncol 2017 Feb;24(2):556-557. Epub 2016 Jul 18 doi: 10.1245/s10434-016-5285-1. PMID: 27431416
Massive cardiac chondroma presenting with heart failure and superior vena cava obstruction in a teenage boy.
Sebire NJ, Ramsay AD, Malone M, Sheppard MN, Roebuck D, Elliott MJ
Fetal Pediatr Pathol 2004 Sep-Dec;23(5-6):325-31. doi: 10.1080/15227950490952460. PMID: 16137169

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