Jellyfish in the Heart
A 41-year-old man with a partially cystic cardiac mass was referred to our institutions. About 17 years previously, he had undergone the excision of a lipoma in the left popliteal region. The patient had been well until 3 to 4 months earlier, when dyspnea on exertion developed.
Two-dimensional echocardiography (Figure 1A and 1B), computed tomography (Figure 2A and 2B), and magnetic resonance imaging (Figure 3A and 3B and Movies I–3 in the online-only Data Supplement) displayed a patchy lobulated epicardial mass (transversal diameter, 7 cm; thickness, 4 cm) near the left cardiophrenic angle, partially occupying the left ventricular chamber.
Cardiac catheterization showed dislocation of the left anterior descending artery by the mass without a significant coronary stenosis.
A review of his medical history revealed that the tumor resected in the popliteal region was actually a myxoid liposarcoma. Whole-body positron emission tomography revealed only a mild myocardial uptake, and a gated myocardial perfusion single-photon emission computed tomography (Figure 4 and Movies IV and V in the online-only Data Supplement) showed a perfusion defect in the myocardial area occupied by the mass. Surgical resection of the mass was judged technically feasible, so the patient underwent surgery (Figure 5A and 5B).
First, the left phrenic nerve was dissected from the pericardium via an intrapleuric approach, and then dissection of the mass was started with an incision of the pericardium around the mass. The epicardial fat above the left descending anterior coronary artery was dissected, and the mass was separated from the epicardial surface of the heart. Extracorporeal circulation was then started. A cleavage plane was found, and the mass was resected up to the base of the posterior papillary muscle, which was partially reimplanted. The hole in the left ventricular wall was closed by a Dacron patch (2.5 × 3 cm). The histopathological analysis of the tumor, which macroscopically resembled a jellyfish (Figure 5B), revealed a low-grade myxoid liposarcoma (Figure 6).
The postoperative course was uneventful, and the patient was discharged after 12 days.
The patient presented 8 months later with disease progression (heart, mediastinum, pleura), so chemotherapy with trabectedin was begun and is still ongoing 1 year after surgical treatment.
Liposarcoma is the most common soft-tissue sarcoma; the myxoid subtype is the most prevalent and the least aggressive. These tumors arise more commonly in the lower extremities and have an indolent course, with high disease-free survival rates when appropriate surgical resection with or without radiotherapy is performed.1 Histological grade remains the most important prognostic factor. Myxoid tumors tend to metastasize to extrapulmonary and unusual sites, with an overall rate of 14.5% at 5 years and 15.3% at 10 years1; the time to distant metastases may vary from 3 to 73 months.2 Definitive differentiation between metastatic disease and metachronous primary disease is often elusive. Primary cardiac sarcoma has a worse prognosis compared with secondary tumors, with a median overall survival of 6 versus 93 months in a series3; median survival is significantly better for patients suitable for surgery,3,4 which remains the best therapeutic option, even if most patients eventually die of widespread disease. Few anecdotal reports have been published on cardiac myxoid liposarcomas, so the role of adjuvant therapy (radiotherapy or chemotherapy) still needs to be defined. Chemotherapy is indicated in extensive disease. Anthracyclines and ifosfamide are the agents of choice, but recently the novel agent trabectedin has proved to have special antitumor activity in myxoid liposarcoma.
The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.112.116848/-/DC1.
- © 2013 American Heart Association, Inc.
- Fiore M,
- Grosso F,
- Lo Vullo S,
- Pennacchioli E,
- Stacchiotti S,
- Ferrari A,
- Collini P,
- Lozza L,
- Mariani L,
- Casali PG,
- Gronchi A