Successful resection of giant hemangiopericytoma originating from the left atrium
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《血管的通路杂志》
Department of Cardiovascular Surgery, Hyogo Brain and Heart Center at Himeji, 520 Saisho-ko, Himeji, 670-0981, Japan
Abstract
We report a case of a giant hemangiopericytoma in the left atrium showing severe dyspnea. Preoperative echocardiogram showed the giant tumor herniating into the left ventricle during cardiac diastole. Emergency surgery was performed due to failure of hemodynamics. The tumor was identified to originate from the posterior wall of the left atrium and was successfully excised with the left atrial wall. Histological investigations revealed existence of malignant hemangiopericytoma.
Key Words: Tumor; Left atrium
1. Introduction
To date seven cases of cardiac hemangiopericytoma have been reported [1,2]. Cardiac hemangiopericytoma in the left atrium is very rare and was identified in two cases only [1]. We report a malignant hemangiopericytoma in the left atrium showing severe dyspnea.
2. Case report
A 57-year-old female was admitted to our hospital due to progressive dyspnea.
Initial findings of physical examination included a heart rate of 106 beats/min, a blood pressure of 100/78 mmHg, and a respiratory rate of 30 times/min. Examination of chest X-ray revealed severe pulmonary congestion and bilateral pleural effusion. Tansthoracic echocardiography showed a huge mass occupying a space of the left atrium (Fig. 1). The tumor was herniating into the left ventricle leading to occlusion of the mitral valve orifice during the diastolic phase. The tumor had been attached to the posterior wall of the left atrium. Emergency operation was carried out before cardiac collapse occurred.
Cardiopulmonary bypass with ascending aortic perfusion and bicaval venous return was started. After cardiac arrest with cardioplegia, the left atrium was opened through left atriotomy. The tumor was elastic, soft and had a broad-based attachment to the posterior wall of the left atrium between the bilateral pulmonary veins (Fig. 2a). The tumor was excised 5 mm apart from the attachment with the posterior wall of the left atrium. The mitral valve was intact. It was confirmed that no tumor was left in the left atrium and ventricle. The tumor was yellowish, oval, lobular, and 5 cmx3.5 cmx3.5 cm in size.
Her postoperative course was uneventful. Microscopic investigation showed the antler-like form of the sinusoidal vascular architecture surrounded with mesenchymal cells showing heteromorphic mitotic figures (Fig. 2b). Histological examinations revealed the existence of a malignant hemangiopericytoma. Positive findings for vimentin were obtained by an immunohistological study using immunoperoxidase staining. She had no additional treatment such as chemotherapy or radiation. She has been well for 7 months after operation.
3. Discussion
Hemangiopericytoma belongs to a subclass of angiosarcoma, originating from vascular perithelial cells. It is often observed in soft tissues of body surface [3]. Cardiac hemangiopericytoma is a very rare disease. To the best of our knowledge, seven cases have been reported before [1]. In five cases the tumor originated from the right side of the heart and the pericardium. In three cases, including this case, the tumor originated from the left atrium. Various symptoms might be observed depending on location and size of tumor. Arrhythmia or dysphagia might be an initial symptom but symptoms related to superior vena cava obstruction and pericarditis could be observed first or at a later stage [2].
Although hemangiopericytoma is generally considered to be a low-grade malignancy, it could have malignant potential resulting in local recurrence or metastasis [4]. From a viewpoint of cell division frequency and heteromorphism degree, it has been proposed that histologic findings of one mitotic figure per 10 high-powered fields with mild heteromorphism or one mitotic figure per 20 high-powered fields with moderate heteromorphism are associated with malignant potential [3].
The management for hemangiopericytomas is not yet standardized because numbers of case are limited [1,2]. Surgical resection might be done first of all if it is possible. Radiation therapy is generally ineffective to reduce tumor volume. Trial of chemotherapy is controversial but it has been reported that chemotherapy, using doxorubicin hydrochloride, resulted in good outcome in some cases [1,5,6]. No evidence of local recurrence and metastasis was obtained in this patient after the operation. Additional therapies including radiation and chemotherapy might be considered in our case. Careful observation for local recurrence of the tumor has been performed. Further accumulation of cases of cardiac hemangiopericytoma is necessary to understand pathological mechanisms of the disease and to determine the priority of the possible therapies.
References
Ohtani M, Ohnishi K, Imagawa H, Kato M, Yoshioka Y, Kumagai K, Houki T. Cardiac hemangiopericytoma growing in the left atrium. Ann Thorac Surg 1994; 58:1544–1546.
Galvin IF, Bowe P, Mellon K, Gibbons JRP, Maghout M, Hanukah H. Pericardial hemangiopericytoma as a cause of dysphagia. Ann Thorac Surg 1988; 45:94–96.
McMaster MJ, Soule EH, Ivins JC. Hemangiopericytoma; a clinicopathologic study and long-term follow-up of 60 patients. Cancer 1975; 36:2232–2244.
Backwinkel KD, Diddams JA. Hemangiopericytoma; report of a case and comprehensive review of the literature. Cancer 1970; 25:896.
Morris DM, Vuthiganon C, Chang P, Wiernik PH, Elias EG. Adriamycin in the management of malignant hemangiopericytoma. Am Surg 1981; 47:441–446.
Wong PP, Yagoda A. Chemotherapy of malignant hemangiopericytoma. Cancer 1978; 41:1256–1260.(Nobuchika Ozaki, Nobuhiko)
Abstract
We report a case of a giant hemangiopericytoma in the left atrium showing severe dyspnea. Preoperative echocardiogram showed the giant tumor herniating into the left ventricle during cardiac diastole. Emergency surgery was performed due to failure of hemodynamics. The tumor was identified to originate from the posterior wall of the left atrium and was successfully excised with the left atrial wall. Histological investigations revealed existence of malignant hemangiopericytoma.
Key Words: Tumor; Left atrium
1. Introduction
To date seven cases of cardiac hemangiopericytoma have been reported [1,2]. Cardiac hemangiopericytoma in the left atrium is very rare and was identified in two cases only [1]. We report a malignant hemangiopericytoma in the left atrium showing severe dyspnea.
2. Case report
A 57-year-old female was admitted to our hospital due to progressive dyspnea.
Initial findings of physical examination included a heart rate of 106 beats/min, a blood pressure of 100/78 mmHg, and a respiratory rate of 30 times/min. Examination of chest X-ray revealed severe pulmonary congestion and bilateral pleural effusion. Tansthoracic echocardiography showed a huge mass occupying a space of the left atrium (Fig. 1). The tumor was herniating into the left ventricle leading to occlusion of the mitral valve orifice during the diastolic phase. The tumor had been attached to the posterior wall of the left atrium. Emergency operation was carried out before cardiac collapse occurred.
Cardiopulmonary bypass with ascending aortic perfusion and bicaval venous return was started. After cardiac arrest with cardioplegia, the left atrium was opened through left atriotomy. The tumor was elastic, soft and had a broad-based attachment to the posterior wall of the left atrium between the bilateral pulmonary veins (Fig. 2a). The tumor was excised 5 mm apart from the attachment with the posterior wall of the left atrium. The mitral valve was intact. It was confirmed that no tumor was left in the left atrium and ventricle. The tumor was yellowish, oval, lobular, and 5 cmx3.5 cmx3.5 cm in size.
Her postoperative course was uneventful. Microscopic investigation showed the antler-like form of the sinusoidal vascular architecture surrounded with mesenchymal cells showing heteromorphic mitotic figures (Fig. 2b). Histological examinations revealed the existence of a malignant hemangiopericytoma. Positive findings for vimentin were obtained by an immunohistological study using immunoperoxidase staining. She had no additional treatment such as chemotherapy or radiation. She has been well for 7 months after operation.
3. Discussion
Hemangiopericytoma belongs to a subclass of angiosarcoma, originating from vascular perithelial cells. It is often observed in soft tissues of body surface [3]. Cardiac hemangiopericytoma is a very rare disease. To the best of our knowledge, seven cases have been reported before [1]. In five cases the tumor originated from the right side of the heart and the pericardium. In three cases, including this case, the tumor originated from the left atrium. Various symptoms might be observed depending on location and size of tumor. Arrhythmia or dysphagia might be an initial symptom but symptoms related to superior vena cava obstruction and pericarditis could be observed first or at a later stage [2].
Although hemangiopericytoma is generally considered to be a low-grade malignancy, it could have malignant potential resulting in local recurrence or metastasis [4]. From a viewpoint of cell division frequency and heteromorphism degree, it has been proposed that histologic findings of one mitotic figure per 10 high-powered fields with mild heteromorphism or one mitotic figure per 20 high-powered fields with moderate heteromorphism are associated with malignant potential [3].
The management for hemangiopericytomas is not yet standardized because numbers of case are limited [1,2]. Surgical resection might be done first of all if it is possible. Radiation therapy is generally ineffective to reduce tumor volume. Trial of chemotherapy is controversial but it has been reported that chemotherapy, using doxorubicin hydrochloride, resulted in good outcome in some cases [1,5,6]. No evidence of local recurrence and metastasis was obtained in this patient after the operation. Additional therapies including radiation and chemotherapy might be considered in our case. Careful observation for local recurrence of the tumor has been performed. Further accumulation of cases of cardiac hemangiopericytoma is necessary to understand pathological mechanisms of the disease and to determine the priority of the possible therapies.
References
Ohtani M, Ohnishi K, Imagawa H, Kato M, Yoshioka Y, Kumagai K, Houki T. Cardiac hemangiopericytoma growing in the left atrium. Ann Thorac Surg 1994; 58:1544–1546.
Galvin IF, Bowe P, Mellon K, Gibbons JRP, Maghout M, Hanukah H. Pericardial hemangiopericytoma as a cause of dysphagia. Ann Thorac Surg 1988; 45:94–96.
McMaster MJ, Soule EH, Ivins JC. Hemangiopericytoma; a clinicopathologic study and long-term follow-up of 60 patients. Cancer 1975; 36:2232–2244.
Backwinkel KD, Diddams JA. Hemangiopericytoma; report of a case and comprehensive review of the literature. Cancer 1970; 25:896.
Morris DM, Vuthiganon C, Chang P, Wiernik PH, Elias EG. Adriamycin in the management of malignant hemangiopericytoma. Am Surg 1981; 47:441–446.
Wong PP, Yagoda A. Chemotherapy of malignant hemangiopericytoma. Cancer 1978; 41:1256–1260.(Nobuchika Ozaki, Nobuhiko)