Open Access

An uncommon long-term survival case of primary cardiac leiomyosarcoma

  • Meriam Glaoui1,
  • Zineb Benbrahim2Email author,
  • Rhizlane Belbaraka3,
  • Sara Naciri4,
  • Hassan Errihani4 and
  • Axel Lescene2
World Journal of Surgical Oncology201412:338

https://doi.org/10.1186/1477-7819-12-338

Received: 20 January 2013

Accepted: 20 October 2014

Published: 10 November 2014

Abstract

Primary cardiac sarcoma is a rare aggressive entity. It constitutes the second most common type of primary cardiac neoplasms. Its management has largely been guided by small retrospective series with a median survival of 6 months. Here, we discuss a unique case of 8-year survival cardiac leiomyosarcoma managed by surgical and adjuvant therapy.

Keywords

Cardiac neoplasm Leiomyosarcoma Survival

Background

Primary cardiac sarcomas constitute a rare entity. At present, only a few hundred primary cardiac sarcomas have been reported, most of which are based on autopsy series. Despite this rarity, they are the second most common type of primary cardiac neoplasm and account for most of the malignant primary cardiac tumors. Primary cardiac sarcomas are aggressive tumors that generally do not produce symptoms until they are locally advanced. Their management has largely been guided by small retrospective series and non-cardiac sarcoma management principles. However, the prognosis of primary cardiac sarcomas in general remains poor according to the current medical literature. The median survival in patients with cardiac sarcomas has been reported to be about 6 months with a mean of 11 months. Here, we discuss a unique case of 8-year survival cardiac leiomyosarcoma managed by surgical and adjuvant therapy.

Case presentation

A 47-year-old man presented with acute pulmonary edema with severe progressive dyspnea and orthopnea. He had a 10 pack-year history of cigarette smoking, but no other medical or familial conditions. Chest radiograph showed a small right-sided pleural effusion. Electrocardiogram revealed sinus tachycardia without any conduction abnormalities. Transthoracic echocardiography and cardiac magnetic resonance imaging (CRMI) showed a tumor (4 × 5 cm) occupying the side wall of the right atrium budding into the lumen (Figure 1).
Figure 1

MRI T2 cuts, minor axis passing throughout the right atrium showing an irregular isosignal wall thickening of the side wall of the right atrium.

Through a median sternotomy, a radical excision of the neoplasm was performed; the atrial wall was reconstructed using a bovine pericardial patch. The post-operative course was unremarkable and the anatomical pathology analysis revealed fusiform cells with considerable nuclear pleomorphism and mitotic activity. The immunohistochemical study was positive to vimentin, desmin, actin, and HHF-35, and negative for PS-100, cytokeratin, and hormone receptors. The final diagnosis was cardiac leiomyosarcoma. Adjuvant chemotherapy with doxorubicin (60 mg/m2 day 1) and ifosfamide (3 g/m2 day 1 and 2) were realized and MRI after the four cycles did not show any sign of local or distant relapse.

Seven years after the first operation, local recurrence with a metastatic paravertebral soft tissue mass was revealed by magnetic resonance imaging. There was no lung metastasis. The patient received antalgic vertebral radiotherapy with systemic palliative chemotherapy consisting of oral cyclophosphamide. One year later, he died of progressive disease and multiorgan failure.

Conclusions

Primary leiomyosarcomas of the heart[1] are extremely rare, accounting for 0.019% of all malignant cardiac neoplasms in autopsy studies[2]. They typically occur in the fourth decade with no sex predilection. As all other cardiac sarcomas, primary leiomyosarcoma of the heart are usually asymptomatic until an advanced stage. Consequently, the diagnosis is often clinically delayed until hemodynamic consequences and embolisms. Cardiac MRI is the gold standard complementary exam to distinguish malignant lesions and assess the resectability of the tumors.

Due to the small series reporting this disease, the treatment guidelines are not well defined. Radical surgical interventions seem to offer the best outcome; however, a complete surgical resection is often difficult to achieve. The role of adjuvant chemotherapy and radiotherapy to prolong survival in this group of patients is not clear[35]. Another therapeutic option is orthotopic heart transplantation. In this case, the work up should include a total body positron emission tomography and CT scans to exclude distant metastases[6]. Thus, the adequate multimodal treatment strategy should be discussed in a pluridisciplinary meeting in order to improve survival. Prognosis is poor. Nevertheless, it seems that for patients who survived the initial surgery, the prospect for long-term survival is very promising. The present case has been discussed in view of rarity of long-term survival in cardiac leiomyosarcomas since our patient is the longest survivor reported thus far in the English language medical literature with good interim quality of life (Table 1).
Table 1

Cases of cardiac leiomyosarcoma reported in the literature with available data of treatment modalities and survival

Reference

Age/sex

Origin site

Tumor size

Grade

Margin status

Treatment received

Survival (months)

[7]

64/F

Left atrium

17

3

Positive

Curative surgery and adjuvant chemotherapy

9

[7]

54/M

Right atrium

6

3

Positive

Curative surgery and adjuvant chemotherapy

13

[5]

45/F

Left atrium

10

3

Positive

Palliative surgery

5 days

[8]

46/M

Left ventricle

2–3

Palliative chemotherapy

13

[8]

42/F

Left ventricle

3

Palliative chemotherapy

18

[9]

63/F

Right ventricle

4

  

Curative surgery

36

[10]

86/F

Left atrium

4

Curative surgery

15

[11]

74/M

Left atrium

Positive

Curative surgery and adjuvant radiotherapy

8

[12]

54/F

Right atrium

   

Curative surgery

Few hours

[13]

35/F

Right atrium

5.6

3

Positive

Surgery and palliative chemotherapy

10

Consent

Oral informed consent was obtained from the patient for publication of this case report and any accompanying images.

Declarations

Authors’ Affiliations

(1)
Department of Medical Oncology, clinique le littoral
(2)
Gustave Roussy Institute
(3)
Department of medical oncology, Mohamed VI University hospital, Faculty of medicine of Marrakesh, Cadi ayad University
(4)
Department of Medical Oncology, National institute of oncology, hay irfane

References

  1. Butany J, Nair V, Nassmuddin A, Nair GM, Catton C: Cardiac tumors: diagnosis and treatment. Lancet. 2005, 6: 219-228. 10.1016/S1470-2045(05)70093-0.View ArticlePubMedGoogle Scholar
  2. McAllister HA, Fenoglio JJ: 2nd series, Fascicle 15. Tumors of the cardiovascular system. Atlas of tumor pathology. 1978, Washington, DC: Armed Forces Institute of PathologyGoogle Scholar
  3. Burke AP, Cowan D, Virmani R: Primary sarcomas of the heart. Cancer. 1992, 69 (2): 387-395. 10.1002/1097-0142(19920115)69:2<387::AID-CNCR2820690219>3.0.CO;2-N.View ArticlePubMedGoogle Scholar
  4. Boey S, Tribouilloy C, Lesbre JP, Stankowiak C, Copin MC, Haffreingue E, Espriet G: Leiomyosarcome du l’oreillette gauche opere’. Arch Mal Coeur. 1994, 87: 291-294.PubMedGoogle Scholar
  5. Zhang PJ, Brooks JS, Goldblum JR, Yoder B, Seethala R, Pawel B, Gorman JH, Gorman RC, Huang JH, Acker M, Narula N: Primary cardiac sarcomas: a clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol. 2008, 39: 1385-1395. 10.1016/j.humpath.2008.01.019.PubMed CentralView ArticlePubMedGoogle Scholar
  6. Winther C, Immermans-Wielenga V, Daugaard S, Mortensen SA, Sander K, Andersen CB: Primary cardiac tumors: a clinicopathologic evaluation of four cases. Cardiovasc Pathol. 2011, 20: 63-67. 10.1016/j.carpath.2009.11.004.View ArticlePubMedGoogle Scholar
  7. Kim CH, Dancer JY, Coffey D, Zhai QJ, Reardon M, Ayala AG, Ro JY: Clinicopathologic study of 24 patients with primary cardiac sarcomas: a 10-year single institution experience. Hum Pathol. 2008, 39: 933-938. 10.1016/j.humpath.2007.12.018.View ArticlePubMedGoogle Scholar
  8. Mayer F, Aebert H, Rudert M, Königsrainer A, Horger M, Kanz L, Bamberg M, Ziemer G, Hartmann JT: Primary malignant sarcomas of the heart and great vessels in adult patients-a single-center experience. Oncologist. 2007, 12: 1134-1142. 10.1634/theoncologist.12-9-1134.View ArticlePubMedGoogle Scholar
  9. Kim JD, Kim JS, Hwang TS, Shin JK, Song MG: Surgical management of recurrent leiomyosarcoma in heart Korean. J Thorac Cardiovasc Surg. 2014, 47 (1): 35-38.Google Scholar
  10. Andersen RE, Kristensen BW, Gill S: Cardiac leiomyosarcoma, a case report. Int J Clin Exp Pathol. 2013, 6 (6): 1197-1199.PubMed CentralPubMedGoogle Scholar
  11. Nakanishi H, Furukawa K, Noguchi R, Furutachi A, Itoh M, Kamohara K, Okazaki Y, Yamasaki F, Morita S: Primary cardiac leiomyosarcoma originating from the left atrium. Kyobu Geka. 2012, 65 (12): 1057-1061.PubMedGoogle Scholar
  12. Lotze U, Reponova J, Muth G, Oltmanns G, Reich HC, Etzrodt G, Kaiser WA, Mutschke O, Ortmann M, Stippel D, Wahlers T: Leiomyosarcoma of the inferior vena cava extending into the right atrium. A rare differential diagnosis of a right atrial tumor with fatal outcome. Herz. 2012, 37 (5): 573-578. 10.1007/s00059-011-3580-y.View ArticlePubMedGoogle Scholar
  13. Jellis C, Doyle J, Sutherland T, Gutman J, MacIsaac A: Cardiac epithelioid leiomyosarcoma. Clin Cardiol. 2010, 33 (6): E6-E9. 10.1002/clc.20734.View ArticlePubMedGoogle Scholar

Copyright

© Glaoui et al.; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited.

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