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DOI:10.2214/AJR.05.0970
AJR 2006; 187:S473-S475
© American Roentgen Ray Society

AJR Teaching File: Necrotic Mass Invading the Heart

Alvin C. Silva1

1 Department of Radiology, Mayo Clinic Scottsdale, 13400 E Shea Blvd., Scottsdale, AZ 85259

Received June 7, 2005; accepted after revision August 18, 2005.

 
Address correspondence to A. C. Silva (silva.alvin{at}mayo.edu).

Keywords: heart • leiomyosarcoma • metastasis • uterus


Clinical History
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Clinical History
Radiologic Description
Differential Diagnosis
Diagnosis
Commentary
Objective
Conclusion
References
 
An asymptomatic 43-year-old woman presents for routine surveillance following total abdominal hysterectomy with bilateral salpingo-oophorectomy 4 years earlier as a result of uterine leiomyosarcoma.


Radiologic Description
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Clinical History
Radiologic Description
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Contiguous axial CT images (Figs. 1A and 1B) show a necrotic mass invading the pericardium and extending into the left atrium via the left inferior pulmonary vein. T2-weighted MRI shows an intrapelvic mass and a proximal left thigh mass (Fig. 1C). A small-bowel follow-through depicts the typical coiled-spring appearance of an intussusception (Fig. 1D). Subsequent contrast-enhanced MRI shows the irregularly enhancing lead point (Fig. 1E) and a large metastasis involving the proximal left thigh (Fig. 1F).


Figure 1
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Fig. 1A —43-year-old woman with uterine leiomyosarcoma metastases. Contiguous contrast-enhanced axial CT images show large left pericardial mass (arrows) with transvenous extension (arrowheads, B).

 

Figure 2
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Fig. 1B —43-year-old woman with uterine leiomyosarcoma metastases. Contiguous contrast-enhanced axial CT images show large left pericardial mass (arrows) with transvenous extension (arrowheads, B).

 

Figure 3
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Fig. 1C —43-year-old woman with uterine leiomyosarcoma metastases. Coronal T2-weighted MR image shows hyperintense intrapelvic (long arrow) and left thigh (short arrow) metastases.

 

Figure 4
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Fig. 1D —43-year-old woman with uterine leiomyosarcoma metastases. Spot image from small-bowel follow-through examination shows classic appearance of intussusception (arrows) as sequela of intrapelvic metastasis.

 

Figure 5
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Fig. 1E —43-year-old woman with uterine leiomyosarcoma metastases. Contrast-enhanced axial T1-weighted MR images show heterogeneous hypervascular enhancement of metastatic lead point (arrow, E) and leiomyosarcoma metastasis in left thigh (arrow, F).

 

Figure 6
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Fig. 1F —43-year-old woman with uterine leiomyosarcoma metastases. Contrast-enhanced axial T1-weighted MR images show heterogeneous hypervascular enhancement of metastatic lead point (arrow, E) and leiomyosarcoma metastasis in left thigh (arrow, F).

 

Differential Diagnosis
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Clinical History
Radiologic Description
Differential Diagnosis
Diagnosis
Commentary
Objective
Conclusion
References
 
The differential diagnosis in a patient with a cardiac mass includes myxoma, leiomyosarcoma, papillary fibroelastoma, and angiosarcoma.


Diagnosis
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Clinical History
Radiologic Description
Differential Diagnosis
Diagnosis
Commentary
Objective
Conclusion
References
 
The diagnosis is leiomyosarcoma metastatic to the heart.


Commentary
Top
Clinical History
Radiologic Description
Differential Diagnosis
Diagnosis
Commentary
Objective
Conclusion
References
 
Cardiac metastases are more prevalent than primary neoplasms of the heart and generally portend a less favorable prognosis. In contradistinction, primary leiomyosarcomas of the heart are more common than secondary involvement, which is highly invasive and more often fatal.

In 1999, the patient had a hysterectomy as a result of leiomyosarcoma. Two years later, an intraperitoneal metastasis with resultant intussusception (Figs. 1D and 1E) developed, as did soft-tissue metastases (Fig. 1F). A subsequent metastasis to the pericardium had transvenous extension (Figs. 1A and 1B).

Although metastatic cardiac disease is 16-50 times more common than primary cardiac malignancy [1], uterine leiomyosarcoma metastases to the heart are extremely rare [2, 3] because these highly aggressive tumors have a proclivity for local invasion. However, the incidence of their metastasizing to the heart appears to be increasing [3], likely because of prolonged cancer survival. Thus, the onset of new cardiac symptoms in affected patients should raise the index of suspicion for cardiac involvement.

The most common cardiac or pericardiac metastases are due to breast cancer, lung cancer, melanoma, or lymphoma [4]. Such metastases generally do not occur as isolated events but instead are more likely to occur in patients with widespread disease. Since 1996, the incidence of metastases to the heart has increased to more than 10%, compared with a previously reported range of 0.2-6% [5]. The increase is presumably attributable to the increased longevity of patients treated with improved therapeutic regimens.

Secondary malignancies can involve the heart and pericardium by direct invasion, by hematogenous or lymphatic dissemination, or by transvenous propagation. Direct invasion occurs from the contiguous spread of tumors in the chest cavity, most commonly from the lung, breast, or esophagus, or from lymphoma or thymoma. However, when a patient has widespread disease, it may be difficult to differentiate a tumor with direct involvement from metastatic mediastinal or hilar adenopathy with retrograde extension.

Hematogenous dissemination to the heart also occurs in conjunction with hematogenous metastases to the rest of the body, with melanoma having the greatest propensity for involvement. Classic primary tumors that exploit the transvenous route include renal and adrenal malignancies (by way of the inferior vena cava [IVC]), hepatic malignancies (via the IVC), thyroid malignancies (via the superior vena cava [SVC]), and bronchogenic malignancies (via the pulmonary veins and SVC) [5-7].

The other considerations in the differential diagnosis are unlikely for several reasons. Although myxomas are the most common benign primary tumor in adults [8], these masses are predominantly intracardiac, without an extracardiac component, which is in contradistinction to this patient. Angiosarcomas, the most common primary cardiac malignancy, typically involve the right atrium, arising from the free wall and sparing the atrial septum [9]. Finally, papillary fibroelastomas are avascular tumors that typically involve the aortic valve. As such, they must be differentiated from endocarditis, and they are generally not detectable with CT [8].


Objective
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Clinical History
Radiologic Description
Differential Diagnosis
Diagnosis
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Objective
Conclusion
References
 
The educational objective of this teaching article is to describe the imaging features in a case of cardiac metastases from uterine leiomyosarcoma.


Conclusion
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Clinical History
Radiologic Description
Differential Diagnosis
Diagnosis
Commentary
Objective
Conclusion
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Cardiac metastases are more common than primary cardiac tumors, are generally a late manifestation of the primary malignancy, and are usually indicative of a fatal outcome.


References
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Clinical History
Radiologic Description
Differential Diagnosis
Diagnosis
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References
 

  1. Greenwald EF, Breen JL, Gregori CA. Cardiac metastases associated with gynecologic malignancies. Gynecol Oncol1980; 10:75 -83[CrossRef][Medline]
  2. Rose PG, Piver MS, Tsukada Y, Lau T. Patterns of metastasis in uterine sarcoma: an autopsy study. Cancer1989; 63:935 -938[CrossRef][Medline]
  3. Cordioli E, Pizzi C, Bugiardini R. Left ventricular metastasis from uterine leiomyosarcoma. Cardiologia 1999;44 : 1001-1003[Medline]
  4. Hoy F, Boucher R, Brody A. Uterine leiomyosarcoma with cardiac metastases. Can J Surg 1988;31 : 418-420[Medline]
  5. Butany J, Nair V, Naseemuddin A, Nair GM, Catton C, Yau T. Cardiac tumours: diagnosis and management. Lancet Oncol2005; 6:219 -228[Medline]
  6. Chiles C, Woodard PK, Gutierrez FR, Link KM. Metastatic involvement of the heart and pericardium: CT and MR imaging. RadioGraphics 2001;21 : 439-449[Abstract/Free Full Text]
  7. Gilkeson RC, Chiles C. MR evaluation of cardiac and pericardial malignancy. Magn Reson Imaging Clin N Am2003; 11:173 -186, viii[CrossRef][Medline]
  8. Araoz PA, Mulvagh SL, Tazelaar HD, Julsrud PR, Breen JF. CT and MR imaging of benign primary cardiac neoplasms with echocardiographic correlation. RadioGraphics 2000;20 : 1303-1319[Abstract/Free Full Text]
  9. Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas: a review and a case report. Cancer 1986;57 : 852-859[CrossRef][Medline]

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