Primary sarcoma of the heart: case report and literature review

Abstract

Background: Primary cardiac tumors are extremely rare. Most primary cardiac tumors are benign and around one quarter is malign. Sarcomas are accounting for 95% of these malign tumors and they show different histologies. The prognosis is poor with a mean survival of 3 months to 1 year, even with complete radical resection. We report the cases of two patients with primary cardiac sarcoma treated with surgery and radiation and/or chemotherapy. In addition we retrospectively collected data of patients with primary cardiac sarcoma treated between 2005 and 2019 with minimum follow-up of 12 months. Clinical characteristics, treatment modalities and outcomes were collected and analyzed. Finally a literature review was done.

Case presentation: The first patient presented with cerebellar infarction. When she developed a recurrence analysis showed a suspicious myocardial lesion for which irradical surgery (R2) was performed. Histopathology showed an intimal sarcoma of the left atrium. Postoperative radiotherapy was applied without complications. Three months after treatment multiple metastases were diagnosed and she died 13 months after initial diagnosis. The second patient presented with pericardial effusion. A tumor was found located in the right atrium and radical surgery was performed. Histopathology showed an angiosarcoma, without signs of metastases. Adjuvant radiotherapy was added because of close margins and based on high risk of recurrence and metastases it was decided to add chemotherapy. One year after finishing treatment, evaluation showed local recurrence together with pulmonary metastases.

Conclusions: Surgery combined with postoperative radiotherapy is feasible in patients with resectable cardiac sarcoma. Distant metastases occur frequently. In patients with an irresectable sarcoma of the heart primary radiotherapy should be considered.

Keywords: Angiosarcoma; Cardiac sarcoma; Intimal sarcoma; Radiotherapy; Sarcoma of the heart.

Fig. 1

a. CT shows a mass located at the ventral and cranial side of the left atrium expanding into the dorsal site, in sagittal view. b. Transversal view. c. CT made 3 weeks after surgery shows remaining tumor. d. Radiotheraphy planning in transversal view. The red colored line reperesents the planning traget volume

Fig. 2

a. CT shows an inhomogenous mass located in the right atrium, expanding towards the pericardium and the anterior Mediastrium, with ingrowth into the superior vena cava. b. Scan made 3 weeks after surgery. c Radiotheraphy planning in transversal view. The red colored line represents the planning target volume. d. No evidence of disease 1 year after diagnosis. E. One year after finishing treatment CT shows local recurrence and mestastasis