J Korean Med Sci.  2006 Oct;21(5):940-943. 10.3346/jkms.2006.21.5.940.

Primary Cardiac Sarcoma in Pregnancy: A Case Report

• Cho GJ
• Kim HJ
• Kang JS

• KMID: 1781920
• DOI: http://doi.org/10.3346/jkms.2006.21.5.940

Abstract

Primary cardiac sarcoma is a rare disease in adults. It is also associated with poor prognoses, due to diagnostic delay, therapeutic difficulty, and high metastatic potential. The coincidence of pregnancy and a primary cardiac intimal sarcoma is extremely rare. We report a pregnant woman at 27(+5) weeks gestation who was admitted to the hospital with acute-onset dyspnea. A mass was found on the left atrium by transthoracic echocardiography. Subsequently, the intracardiac mass was removed, and mitral valve replacement and modified DeVega tricuspid annuloplasty were performed. The patient was diagnosed with a undifferentiated sarcoma, and gave birth to a 1,230 g living baby boy by Caesarean section from preterm contraction at 29(+5) weeks gestation. The patient then received systemic chemotherapy. However, 10 months after the initial clinical onset, the patient suddenly died. Surgery is the standard treatment for cardiac tumors, and their removal should always be attempted, even in pregnant women. Although the overall survival rates of the patients are rather poor, palliative cardiac surgery allows the prolonging of pregnancy, until an acceptable fetal viability level is reached.

Keyword

Heart Neoplasm; Sarcoma; Pregnancy

MeSH Terms

Sarcoma/*surgery
Pregnancy Complications, Neoplastic/*surgery
Pregnancy
Humans
Heart Neoplasms/*surgery
Female
Cardiopulmonary Bypass
Adult

Figure

• 

  Fig. 1 Transthoracic echocardiography shows a mass spreading from the anterior of the left atrium to the mitral leaflet and mitral annulus.

• 

  Fig. 2 The mass originating from the atrioventricular wall was found to have spread all the way to the anterior leaflet and annulus of the mitral valve.

• 

  Fig. 3 Photomicrograph of the undifferentiated cardiac intimal sarcoma. (A) The tumor is a highly cellular, showing areas of coagulative necrosis without any organoid pattern (H&E, ×40). (B) The high power view shows tumor cells with oval to spindle shaped, hyperchromatic or vesicular nuclei and frequent mitoses (H&E, ×400).

• 

  Fig. 4 Computed tomography image shows a huge solid mass with cystic degeneration occupying the pelvic cavity.

• 

  Fig. 5 The metastatic ovarian tumor is composed of spindle shaped, malignant cells identical to cardiac tumor (H&E, ×400).

Reference

1. Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996. 77:107.

2. Ceresoli G, Passoni P, Benussi S, Alfieri O, Dell'Antonio G, Bolognesi A. Primary cardiac sarcoma in pregnancy: a case report and review of the literature. Am J Clin Oncol. 1999. 22:460–465.

3. Simon BC, Funck R, Drude L, Bohle RM, Reichart B, Maisch B. Malignant angiosarcoma of the right atrium in pregnancy. Herz. 1994. 19:166–170.

4. Salamat SM, Landy HJ, O'Sullivan MJ. Pulmonary artery sarcoma in a pregnant woman: report of a case. Obstet Gynecol. 1994. 84:668–669.

5. Laya MB, Mailliard JA, Bewtra C, Levin HS. Malignant fibrous histiocytoma of the heart. A case report and a review of the literature. Cancer. 1987. 59:1026–1031.

6. Putnam JB Jr, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA. Primary cardiac sarcomas. Ann Thorac Surg. 1991. 51:906–910.

7. Parry AJ, Westaby S. Cardiopulmonary bypass during pregnancy. Ann Thorac Surg. 1996. 61:1865–1869.

8. Pomini F, Mercogliano D, Cavalletti C, Caruso A, Pomini P. Cardiopulmonary bypass in pregnancy. Ann Thorac Surg. 1996. 61:259–268.

9. Jahangiri M, Clarke J, Prefumo F, Pumphrey C, Ward D. Cardiac surgery during pregnancy: pulsatile or nonpulsatile perfusion? J Thorac Cardiovasc Surg. 2003. 126:894–895.