Ann Thorac Surg 2005;79:351-353
© 2005 The Society of Thoracic Surgeons
Case report
Malignant Fibrous Histiocytoma of the Heart Complicated By Factor XI Deficiency in a Jehovah's Witness Patient
Toru Uezu, MDa,*,
Yukio Kuniyoshi, MD, PhDa,
Kazufumi Miyagi, MDa,
Katsuya Arakaki, MDa,
Satoshi Yamashiro, MD, PhDa,
Katsuhito Mabuni, MDa,
Kageharu Koja, MD, PhDa
a Second Department of Surgery, Faculty of Medicine, University of the Ryukyus, Okinawa, Japan
Accepted for publication August 13, 2003.
* Address reprint requests to Dr Uezu, Second Department of Surgery, Faculty of Medicine, University of the Ryukyus, 207 Uehara, Nishihara-cho, Okinawa 903-0215, Japan
tuezu{at}med.u-ryukyu.ac.jp
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Abstract
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We describe a 16-year-old girl with malignant fibrous histiocytoma (MFH) of the heart complicated by factor XI deficiency. The preoperative diagnosis was left atrial myxoma. We decided to perform the operation owing to a normal bleeding time. Operative findings suggested a malignant tumor. The patient was a Jehovah's Witness, and extensive excision was not performed because blood transfusion was not allowed. We resected as much of the tumor and left atrial appendage as possible. The pathologic diagnosis was MFH. Excessive bleeding was not observed during the operation. Bleeding time helps to determine whether a surgical procedure is indicated in patients with factor XI deficiency.
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Introduction
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Malignant fibrous histiocytoma (MFH) of the heart is a rare condition with an unfavorable prognosis [1–3]. Although there is no known cure, surgical excision is thought to be the most effective treatment [1]. Preoperative coagulation testing is particularly important for patients who reject blood and blood-product transfusion, such as Jehovah's Witnesses. The bleeding tendency associated with factor XI deficiency is variable, but it is typically provoked by surgery [4]. We describe the surgical management of an MFH originating in the left atrium of a patient who was a Jehovah's Witness. The case was complicated by factor XI deficiency, which is associated with bleeding during surgical procedures.
A 16-year-old girl, a Jehovah's Witness, presented with a 3-year history of headache, vertigo, and light-headedness. Echocardiography showed a left atrial tumor. Laboratory tests revealed prolonged activated partial thromboplastin time (114.4 seconds; reference range, 30.6–49.7 seconds), but the thrombocyte count (250,000/mm3), prothrombin time (international normalized ratio, 1.02), thrombotest (74%; reference range, 70%–130%), and bleeding time (3 minutes) were normal. A slightly increased fibrinogen level was seen (523 mg/dL; reference range, 200–400 mg/dL). We measured intrinsic coagulation factors (XII, XI, IX, VIII). Factor XI had low activity (8%; reference range, 70%–120%), but the others were within normal limits. Factor XI deficiency (activity 8%; reference range, 70%–120%) and left atrial myxoma were diagnosed based on the echocardiography, magnetic resonance imaging (Fig 1), and computed tomography (CT) findings. Preoperative cardiac catheterization revealed pulmonary hypertension (40/24 mm Hg). The operation was performed using median sternotomy and cardiopulmonary bypass (CPB) after injection of 300 IU/kg of heparin sodium. The right femoral artery was exposed before median sternotomy to ensure that we would not encounter the bleeding tendency associated with factor XI deficiency during the operation. After cardiac arrest, the tumor was identified by a right atrial incision and transseptal approach. The tumor surface was irregular and lobulated, and we suspected malignancy. We resected as much of the tumor and left atrial appendage as possible (Fig 2); however, resection was not extensive because transfusion of blood or blood products was not allowed. We thought that bleeding tendency due to wider resection and prolonged CPB would have been more critical than extirpation of the tumor during the operation. Furthermore, no drugs to treat factor XI deficiency are available in Japan. Excessive bleeding was not observed during the operation, which we performed without transfusion of blood or blood-coagulating factors. Table 1 shows the results of perioperative coagulation testing. The total amount of blood loss was 643 mL. The patient received 24,000 IU epoetin alpha subcutaneously before and after the operation. Preoperative hemoglobin level was 10.2 g/dL, and postoperative minimum hemoglobin level was 7.4 g/dL. Pathologic analysis of the tumor confirmed the diagnosis of MFH (Fig 3). Microscopic examination of the resected tumor showed spindle cell proliferation arranged in storiorm and fascicular patterns; scattered multinucleated giant cells, coagulation necrosis, and occasional mitotic figures were also seen. Immunohistochemically, reaction to smooth muscle actin resulted in focally positive fiberlike cells. A few cells showed a focally positive reaction to S-100 protein. Immunostains to desmin, keratin, or epithelial membrane antigen produced negative results, as did immunostains to 
-1-antichymotrypsin and myoglobin. Based on these findings, the tumor was diagnosed as a MFH. After fully informing the patient's parents of the prognosis and the effects of adjuvant therapy, we acceded to their request that no postoperative chemotherapy or radiation be administered.
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Fig 3. Microscopic appearance of left atrial tumor showing a storiform pattern of spindle and foamy cells. (Original magnification x100.)
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The postoperative course was uneventful, and the patient was discharged 3 weeks after surgery. Three months later, echocardiography showed that the left atrial tumor had recurred. Five months after the operation CT showed bilateral metastatic adrenal tumors. The patient did not undergo reoperation because of severe anemia; her hemoglobin level was 7.7 g/dL and her hematocrit was 25.5%. She died 7 months after the operation. Autopsy disclosed an extensive tumor obstructing the left atrium, but the metastatic adrenal tumors were necrotic and had not invaded the surrounding tissue.
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Comment
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Primary malignant fibrous histiocytoma of the heart accounts for only 1.6% to 1.8% of primary cardiac tumors [1, 2]. Malignant fibrous histiocytoma is the second most common type of primary malignant cardiac tumor next to angiosarcoma [2, 3]. To date, 47 cases of MFH have been reported from several institutions [1–3, 5, 6]. These patients were between 14 and 77 years old, and the mean age of the female patients was 50.1 [6]. Our patient is the second-youngest cardiac MFH patient described. As with other primary malignant cardiac tumors, radiation and chemotherapy have no effect on MFH. Surgical resection is the most efficacious treatment [1], but local recurrence and metastasis are common and the mean time from diagnosis to death is 9 to 16 months [1, 2]. In our case, the tumor recurred 3 months after surgery. We believe extensive left atrial resection in the initial operation and reoperation to address recurrence would have prolonged the patient's life because the tumor did not invade surrounding tissue. However, we thought that the patient could not tolerate extensive resection or reoperation because of her religious convictions, coupled with factor XI deficiency. We thought that bleeding tendency due to extensive resection and prolonged CPB would have been critical because in Japan, we have no methods or drugs to manage factor XI deficiency except for fresh frozen plasma. Blood transfusion for Jehovah's Witnesses is controversial, and we were constrained to accommodate her family's wishes. There are a few reports of cardiac surgical procedures for factor XI–deficient patients in which fresh frozen plasma prevented excessive bleeding [4]. Preoperative evaluation of the bleeding tendency is important for patients with a coagulation abnormality such as factor XI deficiency, especially those who refuse blood transfusion. Our patient did not have a history of prolonged bleeding, and her bleeding time was normal. We decided to perform the operation after completing several preoperative determinations of bleeding time and ensuring that surgical incisions made in the groin for CPB would not provoke the bleeding tendency. Bleeding time helps to determine whether a surgical procedure is indicated in patients with factor XI deficiency.
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References
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- Korbmacher B, Doering C, Schulte HD, et al. Malignant fibrous histiocytoma of the heart: case report of a rare left-atrial tumor. Thorac Cardiovasc Surg. 1992;40:303–307[Medline]
- Bear PA, Moodie DS. Malignant primary cardiac tumors: the Cleveland Clinic experience, 1956 to 1986. Chest. 1987;92:860–862[Abstract/Free Full Text]
- Murphy MC, Sweeney MS, Putnam JB Jr, et al. Surgical treatment of cardiac tumors: a 25-year experience. Ann Thorac Surg. 1990;49:612–618[Abstract]
- Bolton-Maggs PHB. Factor XI deficiency and its management. Haemophilia. 2000;6(Suppl 1):100–109
- Ovcak Z, Masera A, Lamovec J. Malignant fibrous histiocytoma of the heart. Arch Pathol Lab Med. 1992;116:872–874[Medline]
- Okamoto K, Kato S, Katsuki S, et al. Malignant fibrous histiocytoma of the heart: case report and review of 46 cases in the literature. Intern Med. 2001;40:1222–1226[Medline]
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Abstract
Introduction
