ACP Case Presentation: LEIOMYOSARCOMA OF THE Second rate VENA CAVA .


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Case Presentation. 68 year old femaleChief protestation
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ACP Case Presentation: LEIOMYOSARCOMA OF THE INFERIOR VENA CAVA – DIAGNOSTIC AND THERAPEUTIC CHALLENGES Prasanth Reddy MD; Department of Internal Medicine Peter J. VanVeldhuizen MD; Division of Hematology/Oncology; Department of Internal Medicine University of Kansas – Kansas City September 10, 2004

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Case Presentation 68 year old female Chief protest – dynamic dyspnea more than two years Initial Evaluation before exchange – "Typical" Sleep examine Nocturnal hypoxia Echocardiogram Right atrial mass

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Case Presentation Past Medical History: Hypertension Peripheral neuropathy of feet Interstitial cystitis Hysterectomy Social History: Remote tobacco utilize Family History: Coronary supply route malady Gastric malignancy Stroke Diabetes Mellitus

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Physical Exam Vital signs Temperature 37 8 Blood weight 104/76 Pulse 100 Respirations 18 Oxygen immersion 90% on room air Not tachypneic or cyanotic Lungs – ordinary Cardiovascular – typical Extremities – follow pedal edema

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Differential determination Thrombus Myxoma Sarcoma Metastatic infection

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Transesophageal echocardiogram

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A B CT Chest Panel A - Large mass in the second rate vena cava. Board B - Tumor mass inside the right chamber.

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A B MRA/MRI Panel A - Large mass focused at the intersection of the substandard vena cava and right chamber, reaching out into the right chamber. Board B - M ass reaching out underneath the stomach.

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Further Evaluation - Negative CT Head CT Abdomen/Pelvis V/Q Scan Doppler US respective lower limits

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PET/CT

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A B H&E Desmin Pathology Panel A: H&E recolor indicating shaft cells. Board B: Tumor is sure for desmin (chestnut) showing smooth muscle ancestry predictable with leiomyosarcoma.

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Operative Findings Origin Posterior part of the IVC Dimensions 8 x 4 cm Procedure Excision of mass and follower IVC Closure of Patent Foramen Ovale (PFO)

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Leiomyosarcoma of the substandard vena cava Malignant tumor of vascular inception 1 About 200 cases reported overall 2 Metastatic malady – <50% of cases 3,4 Liver, Lung, Lymph hubs, Bone Sixth decade 5 Female prevalence 5 1 Brewster DC, et al. Curve Surg. 1976 Oct;111(10):1081-5. 2 Lee SW, et al. Korean J Gastroenterol. 2003 Sep;42(3):249-54. 3 Cacoub P, et al. Drug (Baltimore). 1991 Sep;70(5):293-306. 4 Griffin AS, et al. J Surg Oncol. 1987 Jan;34(1):53-60. 5 Hemant D, et al. Australas Radiol. 2001 Nov;45(4):448-51.

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Clinical Findings Non-particular clinical discoveries 6 Dyspnea Malaise Weight misfortune Abdominal or back agony Symptoms may go before conclusion by quite a while 6 Gowda RM, et al. Angiology. 2004 Mar-Apr;55(2):213-6.

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Clinical Findings Manifestations subordinate upon the area of the tumor 3 Segment I - Palpable mass Segment II - Abdominal torment Segment III - Variants of Budd-Chiari disorder 3 Cacoub P, et al. Solution (Baltimore). 1991 Sep;70(5):293-306.

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Pathology Biopsy required for conclusion Histopathology 7 Spindle tumor cells Positive for markers of smooth muscle movement Desmin Vimentin Muscle actin Alpha-smooth muscle actin 7 Nikaido T, et al. Pathol Int. 2004 Apr;54(4):256-60.

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Diagnostic Modalities Imaging modalities 5 Echocardiography CT MRI PET/CT First reported utilization of PET/CT that helped with the finding of leiomyosarcoma of the second rate vena cava 5 Hemant D, et al. Australas Radiol. 2001 Nov;45(4):448-51.

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Management Not sufficiently depicted 8 Limited worldwide experience "Ideal administration obscure" 8 Hines OJ, et al. Growth. 1999 Mar 1;85(5):1077-83.

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Management Aggressive surgical treatment is suggested 4 Slow development design Relatively low metastatic potential Complete resection 9 practical connected with enhanced survival Chemotherapy and radiation treatment may serve as extras 8 4 Griffin AS, et al. J Surg Oncol. 1987 Jan;34(1):53-60. 8 Hines OJ, et al. Malignancy. 1999 Mar 1;85(5):1077-83. 9 Hollenbeck ST, et al. J Am Coll Surg. 2003 Oct;197(4):575-9.

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Prognosis Case arrangement from Memorial Sloan-Kettering 9 25 patients Complete resection 3-year survival rate - 76% 5-year survival rate - 33% Incomplete resection - No 3-year survivors 9 Hollenbeck ST, et al. J Am Coll Surg. 2003 Oct;197(4):575-9.

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Prognosis Main prognostic component – "geology" 3 Highest level of expansion of the tumor Upper-portion tumors - poorest anticipation Overall guess 10 Poor Mean survival of around 2 years 3 Cacoub P, et al. Solution (Baltimore). 1991 Sep;70(5):293-306. 10 Bendayan P, et al. Ann Chir. 1991;45(2):149-54.

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Leiomyosarcoma of the second rate vena cava Rare harmful tumor smooth muscle cells of the media Diagnosis testing non-particular objections - dyspnea, discomfort, weight reduction, and stomach or back torment Various imaging modalities help determination echocardiography, CT, MRI, PET, and PET/CT make prior finding conceivable Aggressive surgical administration joined with adjuvant treatment

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Acknowledgments Gregory F. Muehlebach, MD ; Division of Cardiovascular Surgery, Department of Surgery, University of Kansas School of Medicine, Kansas City, KS. David G. Meyers, MD, MPH ; Division of Cardiology, Department of Internal Medicine, University of Kansas School of Medicine, Kansas City, KS. James P. Birkbeck, MD ; Division of Cardiology, Department of Internal Medicine, University of Kansas School of Medicine, Kansas City, KS. Stephen K. Williamson, MD ; Division of Hematology/Oncology, Department of Internal Medicine, University of Kansas School of Medicine, Kansas City, KS. Dwindle J. VanVeldhuizen, MD ; Division of Hematology/Oncology, Department of Internal Medicine, University of Kansas School of Medicine, Kansas City, KS.

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