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Unusual localization of a primary pleomorphic malignant fibrous histiocytoma on the mitral valve: a case report and review of the literature
© Prifti et al. 2015
Received: 2 May 2015
Accepted: 7 October 2015
Published: 31 October 2015
It has been reported that cardiac malignant fibrous histiocytomas occur more frequently in the left side of the heart, especially in the left atrium, but rarely invade the mitral valve. We present a case with a giant malignant fibrous histiocytoma with an unusual localization involving almost the entire left atrium, mitral valve, and left superior pulmonary vein.
We describe the case of a 54-year-old woman from Kosovo admitted to our emergency department with dyspnea. A transthoracic echocardiography demonstrated a giant mass localized on her left atrium. Our patient underwent emergent total surgical removal of the mass. The mass extended between her left superior pulmonary vein, and extended to her left atrium and the posterior mitral valve leaflet. We formulated a surgical plan for total separation of the mass from the endocardium. Total removal was performed and her left side pulmonary veins were entirely freed from the mass. We then performed a mitral valve replacement. The differential diagnosis included other masses of the left atrium, including thrombi, vegetations, and cardiac tumors. Postsurgical histopathologic results showed a pleomorphic malignant fibrous histiocytoma. Six monthly follow-up cardiac and abdominal sonographic examinations revealed no tumor recidivism.
We reviewed 90 cases with malignant fibrous histiocytoma reported in the literature. Our case was especially unusual because of the primary location in the mitral valve, the pleomorphic variant, and the dimensions and extension. Complete surgical resection is mandatory to ameliorate symptoms and to obtain histologic information.
The first reported case of a cardiac malignant fibrous histiocytoma (MFH) was published by O’Brien et al.  in 1964 and the first case undergoing surgical removal in 1978 . In 2001, Okamoto et al.  analyzed 46 cases of reported MFH. Since then, 44 additional cases of MFH have been reported, resulting in a total of 90 cases of MFH. Cardiac MFH occur more frequently in the left side of the heart, especially in the left atrium [4–6], but rarely invade the mitral valve. The pleomorphic variant of MFH is rarely found and comprises a high cellularity with bizarre tumor cells with marked atypia and high mitotic index. Here, we report the case of a patient with a pleomorphic MFH invading her mitral valve that underwent successful surgical removal. To the best of our knowledge, this is the first reported case of a pleomorphic variant of such dimensions and tumor extension involving the mitral valve.
A 54-year-old woman from Kosovo was admitted to our hospital with dyspnea. On clinical examination, our patient’s blood pressure was 100/60 mmHg, and her pulse rate was 130 beats per minute with a regular rate and rhythm. Cardiac auscultation revealed a diastolic murmur. End-inspiratory crackles suggested pulmonary edema. Two-dimensional transthoracic echocardiography revealed a giant mass originating from her posterior mitral valve leaflet, occupying almost her entire left atrial cavity. Cerebral, thoracic, and abdominal computed tomography was also performed, showing no evidence of additional tumors.
Our patient’s postoperative course was uneventful. Six weeks after surgery, she started a six-course chemotherapy regimen of 1.5 mg/m2 of ifosfamide on days 1 to 3 and 80 mg/m2 of epidoxorubicin on day 1. The treatment was well tolerated with no unacceptable toxicities. Our patient was still alive with no signs of metastasis six months later.
Cases reported in the literature with primary cardiac malignant fibrous histiocytoma
60 (67 %)
Inferior vena cava
63 (70 %)
40 (45 %)
12 (13 %)
Clinical manifestations of primary MFH are dependent on their size and location. With small tumors, no clinical manifestations are evident. As the tumor grows, the most frequent symptoms are shortness of breath, palpitation, or chest discomfort. The MFH in our case is, to the best of our knowledge, the largest reported.
Histologically, MFH is a variously shaped and multilobulated mass, sessile or pedunculated. It is a tumor of the fibroblasts, with giant cells and atypical nuclear and cytoplasmic findings. The term MFH has now become synonymous of undifferentiated high-grade pleomorphic sarcoma, because the fibrohistiocytic differentiation is not characteristic of a specific tumor type. Sarcomas of myoblastic or fibroblastic origin, including leiomyosarcoma, fibrosarcoma, myxoid fibrosarcoma, and pleomorphic sarcoma (MFH), occur most often as endocardium-based pathologies. The differential diagnosis of cardiac sarcoma in these circumstances is cardiac myxoma, especially in the case of a left atrial intimal-type sarcoma. MFH is a pleomorphic sarcoma composed of fibroblasts, myofibroblasts, and histiocyte-like cells. The pleomorphic variant of the MFH is rarely reported and only had mitral valve involvement in two of the 90 cases we found in the literature [8, 9].
The treatment plan for MFH depends on the tumor status, size, histology, location, and metastatic spread. Complete surgical resection is mandatory to ameliorate symptoms and to obtain histologic information. In our case, once we had started surgery, we suspected that we had a malignant tumor. However, owing to the fact that the tumor had invaded her posterior mitral valve leaflet, causing severe mitral valve obstruction, we had to remove her mitral valve as this was the only measure to complete total tumoral mass removal and relieve the mitral valve stenosis.
Although the prognosis of MFH is poor and the possibility of local recurrence and metastasis is high, patients benefit from surgery. Multiple studies have reported a median survival of six months for right-sided tumors, whereas left heart tumors seem to have a better prognosis . The role of chemotherapy and radiotherapy in the treatment of primary cardiac sarcomas has not proven to be beneficial, and complete surgical excision is the only mode of therapy that has been shown to prolong survival.
Our case was especially unusual because of the primary location of an MFH in the mitral valve, the pleomorphic variant, and the dimensions and extension. Complete surgical resection, including mitral valve replacement, is mandatory to improve symptoms and to obtain histologic information.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We would like to thank Mrs Enida Hoxha/Prifti for her support regarding the linguistic editing of the manuscript.
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