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

• Social History:– Remote tobacco use

• Family History:– Coronary artery disease– Gastric cancer– Stroke– Diabetes Mellitus

Physical ExamPhysical 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

Differential diagnosisDifferential diagnosis• Thrombus• Myxoma• Sarcoma• Metastatic disease

Transesophageal Transesophageal echocardiogramechocardiogram

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.

Clinical FindingsClinical 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-Apr;55(2):213-6.

Clinical FindingsClinical 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 Sep;70(5):293-306.

PathologyPathology• 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 Apr;54(4):256-60.

Diagnostic ModalitiesDiagnostic 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 Nov;45(4):448-51.

ManagementManagement• Not adequately described 8

– Limited international experience

– “Optimal management unknown”

8 Hines OJ, et al. Cancer. 1999 Mar 1;85(5):1077-83.

ManagementManagement• 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

4 Griffin AS, et al. J Surg Oncol. 1987 Jan;34(1):53-60.8 Hines OJ, et al. Cancer. 1999 Mar 1;85(5):1077-83. 9 Hollenbeck ST, et al. J Am Coll Surg. 2003 Oct;197(4):575-9.

PrognosisPrognosis• 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 Oct;197(4):575-9.

PrognosisPrognosis• 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 Sep;70(5):293-306.10 Bendayan P, et al. Ann Chir. 1991;45(2):149-54.

• 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

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.