1513 - Primary sarcomas of the heart and great vessels

Date 28 September 2012
Event ESMO Congress 2012
Session Publication Only
Topics Soft Tissue Sarcomas
Presenter Katarzyna Wiater
Authors K. Wiater1, H.M. Kosela2, M. Zdzienicki3, P. Rutkowski2
  • 1Soft Tissue/bone Sarcoma And Melanoma Department, MSC Memorial Cancer Centre and Institute Maria Sklodowska-Curie, 02-781 - Warsaw/PL
  • 2Soft Tissue/bone Sarcoma And Melanoma, MSC Memorial Cancer Centre and Institute Maria Sklodowska-Curie, 02-781 - Warsaw/PL
  • 3Department Of Soft Tissue/bone Sarcoma And Melanoma, MSC Memorial Cancer Centre and Institute Maria Sklodowska-Curie, 02-781 - Warsaw/PL

Abstract

Background

Primary sarcomas of the heart and great vessels are rare entities, accounting for less than 25 % of all primary cardiac tumors. They are characterized by an aggressive course and poor prognosis. The aim of the study was to analyze treatment and outcomes in the group of patients referred to sarcoma center.

Methods

We retrospectively analyzed 12 patients (8 male, 4 females) treated at our institution between 2003 – 2011. Median age at the time of diagnosis was 33 years (range: 27-73). The histological types included angiosarcoma (n = 4), pleomorphic sarcoma (n = 4), leiomyosarcoma (n = 3), desmoplastic small round cell tumour (n = 1). Primary tumors were located in right atrium (n = 6), left atrium (n = 5) and pulmonary artery (n = 1). The median tumour size was 50 mm (range 25-112). Four patients (33%) had a metastatic disease at diagnosis. Nine patients (75%) underwent curative intended surgery, including one patient who had a heart transplantation. Five patients (42%) received a perioperative anthracycline-based chemotherapy.

Results

The median overall survival (OS) was 19.5 months (range: 3-42). There was a significant improvement in overall survival for patients who underwent surgical resection versus those who did not (median OS 24.5 versus 6 months, respectively). Even better results were observed in patients receiving perioperative chemotherapy. The median OS for these patients was 32 months (range 27-42). Despite the curative intention of surgery, 90% of patients have subsequently developed local recurrence.

Conclusion

Complete surgical excision in combination with chemotherapy improves overall survival in patients with primary sarcomas of heart and great vessels. It underlines significance of close cooperation of cardiac surgery centers with tertiary cancer centers in treatment of patients with these rare entities.

Disclosure

All authors have declared no conflicts of interest.