ACP Case Presentation: LEIOMYOSARCOMA OF THE INFERIOR VENA LEIOMYOSARCOMA OF THE INFERIOR VENA CAVA – DIAGNOSTIC AND THERAPEUTIC CAVA – DIAGNOSTIC AND THERAPEUTIC CHALLENGES 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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ACP Case Presentation:
LEIOMYOSARCOMA OF THE INFERIOR LEIOMYOSARCOMA OF THE INFERIOR VENA CAVA – DIAGNOSTIC AND VENA CAVA – DIAGNOSTIC AND
THERAPEUTIC CHALLENGESTHERAPEUTIC CHALLENGES
Prasanth Reddy MD; Department of Internal Medicine
Peter J. VanVeldhuizen MD; Division of Hematology/Oncology; Department of Internal
Medicine
University of Kansas – Kansas CitySeptember 10, 2004
Case PresentationCase Presentation• 68 year old female• Chief complaint – progressive dyspnea
over two years• Initial Evaluation prior to transfer –
“Normal”• Sleep study
– Nocturnal hypoxia• Echocardiogram
– Right atrial mass
Case PresentationCase Presentation• Past Medical History:
– Hypertension– Peripheral neuropathy of feet– Interstitial cystitis– Hysterectomy
Panel A - Large mass in the inferior vena cava. Panel B - Tumor mass within the right atrium.
A B
CT ChestCT Chest
MRA/MRIMRA/MRI
Panel A - Large mass centered at the mass centered at the confluence of the inferior vena cava and right confluence of the inferior vena cava and right atrium, extending into the right atrium.atrium, extending into the right atrium. Panel B - Mass extending beneath the ass extending beneath the diaphragm.diaphragm.
A B
Further Evaluation - Further Evaluation - NegativeNegative
CT HeadCT Abdomen/PelvisV/Q ScanDoppler US bilateral lower
extremities
PET/CTPET/CT
PathologyPathology
Panel A: H&E stain showing spindle cells. Panel B: Tumor is positive for desmin (brown) indicating smooth muscle lineage consistent with leiomyosarcoma.
H&E Desmin
A B
Operative FindingsOperative Findings• Origin
– Posterior aspect of the IVC
• Dimensions– 8 x 4 cm
• Procedure– Excision of mass and adherent IVC– Closure of Patent Foramen Ovale
(PFO)
Leiomyosarcoma of the inferior vena Leiomyosarcoma of the inferior vena cavacava
• Malignant tumor of vascular origin 1
• About 200 cases reported worldwide 2
• Metastatic disease – <50% of cases 3,4
– Liver, Lung, Lymph nodes, Bone
• Sixth decade 5
• Female predominance 5
1 Brewster DC, et al. Arch 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. Medicine (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.
• Various imaging modalities assist diagnosis– echocardiography, CT, MRI, PET, and PET/CT– make earlier diagnosis possible
• Aggressive surgical management combined with adjuvant therapy
Leiomyosarcoma of the inferior vena Leiomyosarcoma of the inferior vena cavacava
AcknowledgementsAcknowledgements• Gregory F. Muehlebach, MDGregory F. Muehlebach, MD; Division of
Cardiovascular Surgery, Department of Surgery, University of Kansas School of Medicine, Kansas City, KS.
• David G. Meyers, MD, MPHDavid G. Meyers, MD, MPH; Division of Cardiology, Department of Internal Medicine, University of Kansas School of Medicine, Kansas City, KS.
• James P. Birkbeck, MDJames P. Birkbeck, MD; Division of Cardiology, Department of Internal Medicine, University of Kansas School of Medicine, Kansas City, KS.
• Stephen K. Williamson, MDStephen K. Williamson, MD; Division of Hematology/Oncology, Department of Internal Medicine, University of Kansas School of Medicine, Kansas City, KS.
• Peter J. VanVeldhuizen, MDPeter J. VanVeldhuizen, MD; Division of Hematology/Oncology, Department of Internal Medicine, University of Kansas School of Medicine, Kansas City, KS.