ACP Case Presentation: LEIOMYOSARCOMA OF THE INFERIOR VENA CAVA DIAGNOSTIC AND THERAPEUTIC CHALLENGE

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ACP Case PresentationLEIOMYOSARCOMA 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 complaint progressive dyspnea over two
  years
• Initial Evaluation prior to transfer Normal
• Sleep study
• 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 use
• Family History
• Coronary artery disease
• Gastric cancer
• Stroke
• Diabetes Mellitus

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Physical Exam

• Vital signs
• Temperature 378
• Blood pressure 104/76
• Pulse 100
• Respirations 18
• Oxygen saturation 90 on room air
• Not tachypneic or cyanotic
• Lungs normal
• Cardiovascular normal
• Extremities trace pedal edema

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Differential diagnosis

• Thrombus
• Myxoma
• Sarcoma
• Metastatic disease

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Transesophageal echocardiogram
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CT Chest
Panel A - Large mass in the inferior vena cava.
Panel B - Tumor mass within the right atrium.
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MRA/MRI
Panel A - Large mass centered at the confluence
of the inferior vena cava and right atrium,
extending into the right atrium. Panel B - Mass
extending beneath the diaphragm.
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Further Evaluation - Negative

• CT Head
• CT Abdomen/Pelvis
• V/Q Scan
• Doppler US bilateral lower extremities

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PET/CT
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Pathology
Panel A HE stain showing spindle cells. Panel
B Tumor is positive for desmin (brown)
indicating smooth muscle lineage consistent with
leiomyosarcoma.
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Operative 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)

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Leiomyosarcoma of the inferior vena cava

• Malignant tumor of vascular origin 1
• About 200 cases reported worldwide 2
• Metastatic disease lt50 of cases 3,4
• Liver, Lung, Lymph nodes, Bone
• Sixth decade 5
• Female predominance 5
• 1 Brewster DC, et al. Arch Surg. 1976
  Oct111(10)1081-5.
• 2 Lee SW, et al. Korean J Gastroenterol. 2003
  Sep42(3)249-54.
• 3 Cacoub P, et al. Medicine (Baltimore). 1991
  Sep70(5)293-306.
• 4 Griffin AS, et al. J Surg Oncol. 1987
  Jan34(1)53-60.
• 5 Hemant D, et al. Australas Radiol. 2001
  Nov45(4)448-51.

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Clinical Findings

• Non-specific clinical findings 6
• Dyspnea
• Malaise
• Weight loss
• Abdominal or back pain
• Symptoms may precede diagnosis by several years 6
• 6 Gowda RM, et al. Angiology. 2004
  Mar-Apr55(2)213-6.

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Clinical Findings

• Manifestations dependent upon the location of the
  tumor 3
• Segment I - Palpable mass
• Segment II - Abdominal pain
• Segment III - Variants of Budd-Chiari syndrome
• 3 Cacoub P, et al. Medicine (Baltimore). 1991
  Sep70(5)293-306.

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Pathology

• Biopsy required for diagnosis
• Histopathology 7
• Spindle tumor cells
• Positive for markers of smooth muscle activity
• Desmin
• Vimentin
• Muscle actin
• Alpha-smooth muscle actin
• 7 Nikaido T, et al. Pathol Int. 2004
  Apr54(4)256-60.

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Diagnostic Modalities

• Imaging modalities 5
• Echocardiography
• CT
• MRI
• PET
• PET/CT
• First reported use of PET/CT that assisted in the
  diagnosis of leiomyosarcoma of the inferior vena
  cava
• 5 Hemant D, et al. Australas Radiol. 2001
  Nov45(4)448-51.

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Management

• Not adequately described 8
• Limited international experience
• Optimal management unknown
• 8 Hines OJ, et al. Cancer. 1999 Mar
  185(5)1077-83.

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Management

• Aggressive surgical treatment is recommended 4
• Slow growth pattern
• Relatively low metastatic potential
• Complete resection 9
• feasible
• associated with improved survival
• Chemotherapy and radiation therapy may serve as
  adjuncts 8

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Prognosis

• Case series 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
  Oct197(4)575-9.

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Prognosis

• Main prognostic factor topography 3
• Highest level of extension of the tumor
• Upper-segment tumors - poorest prognosis
• Overall prognosis 10
• Poor
• Mean survival of around 2 years
• 3 Cacoub P, et al. Medicine (Baltimore). 1991
  Sep70(5)293-306.
• 10 Bendayan P, et al. Ann Chir. 199145(2)149-54.

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Leiomyosarcoma of the inferior vena cava

• Rare malignant tumor
• smooth muscle cells of the media
• Diagnosis challenging
• non-specific complaints - dyspnea, malaise,
  weight loss, and abdominal or back pain
• Various imaging modalities assist diagnosis
• echocardiography, CT, MRI, PET, and PET/CT
• make earlier diagnosis possible
• Aggressive surgical management combined with
  adjuvant therapy

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Acknowledgements

• 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.
• Peter J. VanVeldhuizen, MD Division of
  Hematology/Oncology, Department of Internal
  Medicine, University of Kansas School of
  Medicine, Kansas City, KS.