Giant Intrapericardial Solitary Fibrous Tumor Uberto Bortolotti, MD, Francesco Calabrb, MD, Monica Loy, MD, Giuseppe Fasoli, MD, Giuseppe Altavilla, MD, a n d Domenico Marchese, MD Departments of Cardiovascular Surgery, Thoracic Surgery, Cardiology, and Pathology, University of Padova Medical School, Padova, Italy, and Servizio di Cardiologia, Ospedale Civile di Piove di Sacco, Italy
A 60-year-old man with a large pericardial effusion was found to have a giant intrapericardial solitary fibrous mesothelioma firmly attached to the ascending aorta and pulmonary trunk. Nine months after excision of the mass the patient is free from symptoms and signs of tumor
recurrence. Solitary fibrous mesothelioma is a rare benign tumor and its excision is curative; however, because of the lack of information on its long-term behavior, close noninvasive follow-up of this patient is necessary. (Ann Thorac Surg 1992;54:1219-20)
S
olitary fibrous mesotheliomas are rare tumors that most frequently arise from the pleura [1-31. Their location within the pericardial sac, as in the case herein described, is exceedingly rare. A 60-year-old man was occasionally found to have moderate cardiomegaly in 1989. In January 1991 he required hospitalization because of fatigue, anemia, chest discomfort, and increasing dyspnea of recent onset. On admission pulse rate was 104 beats/min and blood pressure was 170/100 mm Hg. Jugular venous distention and mild peripheral edema were present. The heart sounds were distant with no murmurs and the liver was palpable 2 cm below the costal border. The electrocardiogram showed sinus tachycardia with complexes of reduced amplitude and nonspecific ST changes, and a chest roentgenogram revealed marked cardiomegaly. A two-dimensional transthoracic echocardiogram showed the presence of abundant pericardial effusion, normal ventricular function, and a large intrapericardial mass anterior to the right ventricle (Fig 1A). Pericardiocentesis yielded approximately 900 mL of serohematic fluid with absence of neoplastic cells. Computed tomography of the chest confirmed that the mass was located within the pericardium anterior to the ascending aorta and pulmonary trunk (Fig 1B). The patient underwent operation on March 14, 1991. The chest was entered through a median sternotomy. After the pericardium was opened, a pear-shaped whitish mass was found firmly attached by means of a wide base of implantation to the anterior aspect of the ascending aorta and pulmonary trunk (Fig 2). With cardiopulmonary bypass on standby, by careful dissection the mass was excised with a large cuff of aortic adventitia. Meticulous hemostasis was required because of highly vascularized adhesions to the wall of both great vessels. The tumor measured approximately 14 x 7 cm and weighed 270 g. Section showed a white tissue with whorled appearance and scattered hemorrhages. At histologic examination cellular areas were rich in fusiform Accepted for publication March 19, 1992. Address reprint requests to Dr Bortolotti, Istituto di Chirurgia Cardiovascolare, Centro di Cardiochirurgia “V. Gallucci,” Via Giustiniani, 2, 35128 Padova, Italy.
0 1992 by The Society of Thoracic Surgeons
A
B Fig 1. (A) Transthoracic two-dimensional echocardiogram showing abundant pericardial effusion and the presence of an intrapericardial mass (circled area) anterior to the right ventricle. ( B ) Computed tomographic scan at the level of the origin of the great vessels. The presence of pericardial efusion and a large anterior intrapericardial tumor (T) in contact with both the ascending aorta (A) and pulmonary trunk (P) is confirmed. 0003-4975/92/$5.00
1220
CASE REPORT BORTOLOTTI ET AL BENIGN INTRAPERICARDIAL TUMOR
Fig 2. lntraoperative vim, after opening of the pericardium, of the mass, which almost completely covers the anterior surface of the heart. The patient's head is on the left.
fibroblastlike cells at times arranged in bundles, whereas in other areas collagen fibers predominated with few cells; no mitoses were observed (Fig 3). At electron microscopy tumor cells showed fusiform central nuclei, and the cytoplasm contained few organelles and bundles of microfilaments. Immunohistochemical study showed diffuse reactivity to vimentin and negative staining for keratin. The patient recovered uneventfully and is asymptomatic 9 months after operation. A two-dimensional echocardiogram and computed tomography of the chest showed absence of pericardial effusion and no signs of tumor recurrence.
Comment Solitary fibrous tumors, or the so-called localized fibrous mesotheliomas, are well-known neoplasms of the serosal
Fig 3. Histologic section of the mass (solitary fibrous mesothelioma) showing moderate cellularity consisting of fibroblastlike cells in less collagen matrix. (Hematoxylin and eosin; x160 before 26% reduction.)
Ann Thorac Surg 1992;54:1219-20
cavities that were first recognized as a distinct entity by Klemperer and Rabin in 1931 [4.]. The vast majority of these tumors arise from the pleura [l-31, and their growth into the pericardium is extremely uncommon. In reviewing the literature we have found only 2 cases of intrapericardial mesothelioma. El-Naggar and associates [5], reporting in 1989 14 patients with localized fibrous tumors of the serosal cavities, described a patient with a large pericardial mass that was surgically excised; local recurrence was observed after 120 months with no further recurrence after reoperation. Roggli and co-workers [6], in their review on the pathology of human mesothelioma, showed a figure of another such case without giving clinical details. Although controversy still exists on the histogenesis of this neoplasm, immunohistochemical and ultrastructural studies have suggested that it most likely originates from pluripotential mesenchymal cells located in the subserosal connective tissue [5, 7, 81. Localized mesotheliomas are considered to be benign neoplasms, and their excision is curative in most cases [2, 51. However, in up to 12% of cases the tumor may be responsible for death because of its extensive intrathoracic growth, unresectable recurrences, or metastatic diffusion [I, 21. The patient herein described had a giant intrapericardial solitary fibrous mesothelioma that was successfully excised. Solitary fibrous mesotheliomas of the pericardium are exceptionally rare tumors, and due to the lack of experience with such neoplasms and their often unpredictable behavior long-term prognosis in this patient remains uncertain. Even if radical excision has been obtained and no signs or regrowth are evident after 9 months, continuous noninvasive follow-up is mandatory because tumor recurrence also has been observed in the rare intrapericardial location [5].
References 1. Dalton WT, Zolliker AS, McCaughey WTF, Jacques J, Kannerstein M. Localized primary tumors of the pleura. An analysis of 40 cases. Cancer 1979;44:1465-75. 2. Briselli M, Mark EJ, Dickersin GR. Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature. Cancer 1981;47:267&89. 3. England DM, Hochholzer L, McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura. A clinicopathological review of 223 cases. Am J Surg Pathol 1989;13:640-58. 4. Klemperer P, Rabin CB. Primary neoplasms of the pleura: a report of five cases. Arch Pathol 1931;11:385-412. 5. El-Naggar AK, Ro JY, Ayala AG, Ward R, Ordonez NG. Localized fibrous tumor of the serosal cavities. Immunohistochemical, electron-microscopic, and flow-cytometric DNA study. Am J Clin Pathol 1989;92:561-5. 6. Roggli VL, Kolbeck J, Sanfilippo F, Shelburne JD. Pathology of human mesothelioma. Etiologic and diagnostic considerations. In: Rosen PP, Fechner RE, eds. Pathology annual, part 2, Vol 22. NorwalWSan Mateo: Appleton & Lange, 1987:91. 7. Dervan PA, Tobin B, OConnor MO. Solitary (localized) fibrous mesothelioma: evidence against mesothelial cell origin. Histopathology 1986;10:867-75. 8. Steinetz C , Clarke R, Jacobs GH, Abdul-Karim FW, Petrelli M, Tomashefski JF Jr. Localized fibrous tumor of the pleura: correlation of histopathological, imrnunohistochemical and ultrastructural features. Pathol Res Pract 1990;186:344-57.
A 60-year-old man with a large pericardial effusion was found to have a giant intrapericardial solitary fibrous mesothelioma firmly attached to the ascending aorta and pulmonary trunk. Nine months after excision of the mass the patient is free from symptoms and signs of tumor
recurrence. Solitary fibrous mesothelioma is a rare benign tumor and its excision is curative; however, because of the lack of information on its long-term behavior, close noninvasive follow-up of this patient is necessary. (Ann Thorac Surg 1992;54:1219-20)
S
olitary fibrous mesotheliomas are rare tumors that most frequently arise from the pleura [1-31. Their location within the pericardial sac, as in the case herein described, is exceedingly rare. A 60-year-old man was occasionally found to have moderate cardiomegaly in 1989. In January 1991 he required hospitalization because of fatigue, anemia, chest discomfort, and increasing dyspnea of recent onset. On admission pulse rate was 104 beats/min and blood pressure was 170/100 mm Hg. Jugular venous distention and mild peripheral edema were present. The heart sounds were distant with no murmurs and the liver was palpable 2 cm below the costal border. The electrocardiogram showed sinus tachycardia with complexes of reduced amplitude and nonspecific ST changes, and a chest roentgenogram revealed marked cardiomegaly. A two-dimensional transthoracic echocardiogram showed the presence of abundant pericardial effusion, normal ventricular function, and a large intrapericardial mass anterior to the right ventricle (Fig 1A). Pericardiocentesis yielded approximately 900 mL of serohematic fluid with absence of neoplastic cells. Computed tomography of the chest confirmed that the mass was located within the pericardium anterior to the ascending aorta and pulmonary trunk (Fig 1B). The patient underwent operation on March 14, 1991. The chest was entered through a median sternotomy. After the pericardium was opened, a pear-shaped whitish mass was found firmly attached by means of a wide base of implantation to the anterior aspect of the ascending aorta and pulmonary trunk (Fig 2). With cardiopulmonary bypass on standby, by careful dissection the mass was excised with a large cuff of aortic adventitia. Meticulous hemostasis was required because of highly vascularized adhesions to the wall of both great vessels. The tumor measured approximately 14 x 7 cm and weighed 270 g. Section showed a white tissue with whorled appearance and scattered hemorrhages. At histologic examination cellular areas were rich in fusiform Accepted for publication March 19, 1992. Address reprint requests to Dr Bortolotti, Istituto di Chirurgia Cardiovascolare, Centro di Cardiochirurgia “V. Gallucci,” Via Giustiniani, 2, 35128 Padova, Italy.
0 1992 by The Society of Thoracic Surgeons
A
B Fig 1. (A) Transthoracic two-dimensional echocardiogram showing abundant pericardial effusion and the presence of an intrapericardial mass (circled area) anterior to the right ventricle. ( B ) Computed tomographic scan at the level of the origin of the great vessels. The presence of pericardial efusion and a large anterior intrapericardial tumor (T) in contact with both the ascending aorta (A) and pulmonary trunk (P) is confirmed. 0003-4975/92/$5.00
1220
CASE REPORT BORTOLOTTI ET AL BENIGN INTRAPERICARDIAL TUMOR
Fig 2. lntraoperative vim, after opening of the pericardium, of the mass, which almost completely covers the anterior surface of the heart. The patient's head is on the left.
fibroblastlike cells at times arranged in bundles, whereas in other areas collagen fibers predominated with few cells; no mitoses were observed (Fig 3). At electron microscopy tumor cells showed fusiform central nuclei, and the cytoplasm contained few organelles and bundles of microfilaments. Immunohistochemical study showed diffuse reactivity to vimentin and negative staining for keratin. The patient recovered uneventfully and is asymptomatic 9 months after operation. A two-dimensional echocardiogram and computed tomography of the chest showed absence of pericardial effusion and no signs of tumor recurrence.
Comment Solitary fibrous tumors, or the so-called localized fibrous mesotheliomas, are well-known neoplasms of the serosal
Fig 3. Histologic section of the mass (solitary fibrous mesothelioma) showing moderate cellularity consisting of fibroblastlike cells in less collagen matrix. (Hematoxylin and eosin; x160 before 26% reduction.)
Ann Thorac Surg 1992;54:1219-20
cavities that were first recognized as a distinct entity by Klemperer and Rabin in 1931 [4.]. The vast majority of these tumors arise from the pleura [l-31, and their growth into the pericardium is extremely uncommon. In reviewing the literature we have found only 2 cases of intrapericardial mesothelioma. El-Naggar and associates [5], reporting in 1989 14 patients with localized fibrous tumors of the serosal cavities, described a patient with a large pericardial mass that was surgically excised; local recurrence was observed after 120 months with no further recurrence after reoperation. Roggli and co-workers [6], in their review on the pathology of human mesothelioma, showed a figure of another such case without giving clinical details. Although controversy still exists on the histogenesis of this neoplasm, immunohistochemical and ultrastructural studies have suggested that it most likely originates from pluripotential mesenchymal cells located in the subserosal connective tissue [5, 7, 81. Localized mesotheliomas are considered to be benign neoplasms, and their excision is curative in most cases [2, 51. However, in up to 12% of cases the tumor may be responsible for death because of its extensive intrathoracic growth, unresectable recurrences, or metastatic diffusion [I, 21. The patient herein described had a giant intrapericardial solitary fibrous mesothelioma that was successfully excised. Solitary fibrous mesotheliomas of the pericardium are exceptionally rare tumors, and due to the lack of experience with such neoplasms and their often unpredictable behavior long-term prognosis in this patient remains uncertain. Even if radical excision has been obtained and no signs or regrowth are evident after 9 months, continuous noninvasive follow-up is mandatory because tumor recurrence also has been observed in the rare intrapericardial location [5].
References 1. Dalton WT, Zolliker AS, McCaughey WTF, Jacques J, Kannerstein M. Localized primary tumors of the pleura. An analysis of 40 cases. Cancer 1979;44:1465-75. 2. Briselli M, Mark EJ, Dickersin GR. Solitary fibrous tumors of the pleura: eight new cases and review of 360 cases in the literature. Cancer 1981;47:267&89. 3. England DM, Hochholzer L, McCarthy MJ. Localized benign and malignant fibrous tumors of the pleura. A clinicopathological review of 223 cases. Am J Surg Pathol 1989;13:640-58. 4. Klemperer P, Rabin CB. Primary neoplasms of the pleura: a report of five cases. Arch Pathol 1931;11:385-412. 5. El-Naggar AK, Ro JY, Ayala AG, Ward R, Ordonez NG. Localized fibrous tumor of the serosal cavities. Immunohistochemical, electron-microscopic, and flow-cytometric DNA study. Am J Clin Pathol 1989;92:561-5. 6. Roggli VL, Kolbeck J, Sanfilippo F, Shelburne JD. Pathology of human mesothelioma. Etiologic and diagnostic considerations. In: Rosen PP, Fechner RE, eds. Pathology annual, part 2, Vol 22. NorwalWSan Mateo: Appleton & Lange, 1987:91. 7. Dervan PA, Tobin B, OConnor MO. Solitary (localized) fibrous mesothelioma: evidence against mesothelial cell origin. Histopathology 1986;10:867-75. 8. Steinetz C , Clarke R, Jacobs GH, Abdul-Karim FW, Petrelli M, Tomashefski JF Jr. Localized fibrous tumor of the pleura: correlation of histopathological, imrnunohistochemical and ultrastructural features. Pathol Res Pract 1990;186:344-57.
