Asian Cardiovasc Thorac Ann 2007;15:e72-e74
© 2007 Asia Publishing EXchange Ltd
Papillary Fibroelastoma of the Left Ventricle: Report of Two Cases
Haruaki Hino, MD,
Takeshi Miyairi, MD,
Tadashi Kitamura, MD,
Sumio Miura, MD,
Ikutaro Kigawa, MD,
Sachito Fukuda, MD
The Division of Cardiovascular Surgery Mitsui Memorial Hospital Tokyo, Japan
For reprint information contact: Haruaki Hino, MD, Tel: 81 33 862 9111, Fax: 81 33 862 5100, Email: haruakihino{at}mitsuihosp.or.jp, The Division of Cardiovascular Surgery, Mitsui Memorial Hospital, Kanda-Izumicho 1, Chiyoda-ku, Tokyo 101-8643, Japan.
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ABSTRACT
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Papillary fibroelastoma is a relatively rare cardiac tumor. We report two cases of papillary fibroelastoma. The first case involved a 45-year-old woman who presented with rheumatic valves and three tumors developing from the papillary muscle and left ventricle. The second case involved a 68-year-old man who was asymptomatic and whose tumor was detected incidentally on echocardiogram. Both cases were treated surgically. An additional 71 cases of papillary fibroelastoma reported in the medical literature in Japan are reviewed.
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INTRODUCTION
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Papillary fibroelastoma is a relatively rare cardiac tumor. Recent advances in general health screening in Japan have facilitated detection of cases of papillary fibroelastoma on echogram during routine health examinations. We report two cases of papillary fibroelastoma, which were treated surgically. An additional 71 cases of papillary fibroelastoma reported in the medical literature in Japan are reviewed.
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CASE REPORTS
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CASE 1
A 45-year-old woman presented with a history of rheumatic valve disease since the age of 25. She had been taking medication for mitral valve stenosis, aortic valve stenosis, and tricuspid valve regurgitation since she was 28 years of age. The patient suffered from dyspnea on exertion, which was thought to originate from her rheumatic valve disease. During the course of therapy, an echocardiogram was performed almost annually and a cardiac mass was detected in the left ventricle for the first time when she was 39 years old.1 The high echoic mass was growing gradually and mitral valve stenosis was progressing. Papillary fibroelastoma was not included in the differential diagnosis. The mitral valve area was 0.9 cm2 and the aortic valve pressure gradient was 77 mm Hg as measured by echocardiogram. She suffered from increasing dyspnea on exertion prior to aortic and mitral valve replacement (SJM 19/27, St Jude Medical, St Paul, MN, USA) and tricuspid annuloplasty (CE 34, Carpentier Edwards; Edwards Lifesciences, Irvine, CA, USA), performed in 1996. Her postoperative course was uneventful.
CASE 2
A 68-year-old man underwent medical examination by echocardiogram, which incidentally revealed a hairy high echoic mass (7 x 9 mm) in the left ventricular outflow tract. He was admitted to our hospital for treatment of the ventricular mass. He had no history of infarction of other organs, and examination by brain computed tomography and abdominal echogram revealed no evidence of thromboembolism. Due to concerns about the risk of potential development of thromboembolism, surgery was performed in 2004. The heart was arrested under cardiopulmonary bypass and the ascending aorta was opened. A white mass, with fine transparent hair, was easily located under the right coronary cusp (Figure 1A, B
). The mass was carefully shaved off the endocardium and the patient was weaned from cardiopulmonary bypass without difficulty. The patient’s postoperative course was uneventful and he was discharged 12 days after surgery.
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Figure 1. Papillary fibroelastoma (A) Intraoperative picture, distant view; (B) Intraoperative picture, close view; RCC = right coronary cusp; (C) Macroscopic findings; (D) Microscopic findings.
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Both cases were followed up in the outpatient clinic and no recurrence of the tumor was detected at 10 years postoperatively in Case 1, and at 6 months postoperatively in Case 2. Macroscopically, the masses in both cases were characterized by the formation of papillary white hairy fibers, resembling a sea anemone in appearance (Figure 1C
). Microscopic examination demonstrated linings of endocardial cells with a central core of elastic fibers, characteristic of papillary fibroelastoma (Figure 1D).2,3
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DISCUSSION
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Papillary fibroelastoma is a relatively rare type of benign primary cardiac tumor (incidence 7%–10%), and is the third-most common benign cardiac tumor after myxoma and lipoma.4 The precise incidence of papillary fibroelastoma in general health screening in Japan could not be ascertained. Several years ago this type of tumor was only demonstrated at autopsy. However recently we have had opportunity to detect it on echogram during routine health examinations. Cardiac tumors can be detected on transthoracic and transesophageal echo, which in some cases is useful for differentiating them from other masses such as myxoma or thrombus. Echocardiographically, papillary fibroelastoma is characterized as a pedunculated mass with surface hair-like figures and internally as a low echo-density lesion. However, it is not possible to diagnose papillary fibroelastoma prior to surgery by echocardiogram alone in all cases. When this kind of tumor is suspected or confirmed, surgical intervention is indicated even if the patient is not symptomatic as it can induce severe complications such as cerebral and myocardial infarction, regardless of its size. However, papillary fibroelastomas are rarely identified preoperatively.
A search of the medical literature revealed 73 cases of papillary fibroelastoma, which have been reported and surgically removed in Japan. Almost half of the cases (36/73, 49%) did not present with clinical symptoms. We speculate that routine health examination could detect many such cardiac tumors before clinical symptoms develop. Of the symptoms reported, the majority of patients presented with paralysis and/or transient ischemic attack (TIA): a thrombus can attach to the tumor surface or the fragile tumor itself may destabilize, causing a brain embolism.5 In rare cases, sudden death can occur from the first attack.6 Among the 73 reported cases in Japan (including our two), the papillary fibroelastoma developed from the aortic and mitral valve cusp in 41 cases (58%), and from the left ventricle in 19 cases (26%). This shows that the tumor often originates from the left side of the heart. Tumors are often solitary, but multiple tumors were reported in 10 cases (14%) involving another portion of the heart.7 Therefore, it is important to carefully examine the valves and endocardium during surgery to ensure that multiple tumors are not missed. While tumor size is generally within 1 cm, a 5.7 cm tumor in the right atrium was reported in Japan in 1995.8
Surgical therapy is indicated when this type of tumor is strongly suspected on echogram. The surgical procedure of choice is simple resection of the tumor. In 24 (36%) cases where the mitral and aortic valves attached to the tumor could not be preserved, valve replacement or plasty was performed. 7 Papillary fibroelastoma is attached to the surface of the endocardium pathologically, so macroscopic resection of the tumor without further resection is thought to be adequate. At this time no recurrent cases after surgery have been reported.
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REFERENCES
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- McAllister HA Jr, Fenoglio JJ Jr. Papillary fibroelastoma. In: Tumors of the Cardiovascular System. Washington, DC: Armed Forces Institute of Pathology.1978;20–5.
- Sato Y, Yokoyama H, Satokawa H, Takase S, Maruyama Y. A report of a surgical case of papillary fibroelastoma in the left ventricular outflow tract. Ann Thorac Cardiovasc Surg 2003;9:270–3.[Medline]
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ABSTRACT
INTRODUCTION
