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Radiographic, CT, and MRI findings in primary pulmonary angiosarcoma
Journal of Clinical Imaging 25 (2001) 337 – 340
Radiographic, CT, and MRI findings in primary pulmonary angiosarcoma C¸etin Atasoy*, Suat Fitoz, Hasan Yigˇit, Pinar Atasoy, Ilhan Erden, Serdar Akyar School of Medicine, Ankara University, Ibn-i Sina Hospital, 06100, Sihhiye- Ankara, Turkey Received 10 January 2001
Abstract We report a rare instance of primary pulmonary angiosarcoma presenting as a large solitary mass in the left upper lobe with mediastinal invasion. In particular, we emphasize the magnetic resonance (MR) imaging features, which included a markedly heterogeneous pattern consisting of hyperintense areas scattered throughout a background of intermediate signal intensity, rendering the lesion a cauliflower-like appearance especially on T2-weighted images. Being unreported so far in primary pulmonary angiosarcomas, these distinct MR imaging findings may be helpful in the differentiation of these neoplasms from lung cancers. D 2001 Elsevier Science Inc. All rights reserved. Keywords: Angiosarcoma; Lung; Neoplasm; CT; MRI
1. Introduction Angiosarcomas of the lung are very rare tumors and usually represent metastases from the heart, pulmonary arterial trunk, or extrathoracic organs [1]. Primary pulmonary origin of this tumor has been reported very infrequently [2– 10]. In this paper, we describe radiological findings in a patient with a solitary left upper lobe mass, which was diagnosed as angiosarcoma by histopathological and immunohistochemical examinations.
2. Case report A 50-year-old man presented with a 2-month history of chest pain, malaise, and hoarseness. Physical examination revealed decreased respiratory sounds in the left upper zone and left-sided Horner’s syndrome. Routine laboratory examinations were unremarkable. Chest X-ray showed a large, well-defined homogeneous opacity occupying the upper zone of the left hemithorax (Fig. 1). Computed tomography (CT) of the chest demonstrated a 10-cm * Corresponding author. Emek, 10. Cadde (Kirim Caddesi), Senyuva Sitesi, N2/21, 06510, Ankara, Turkey. Tel.: +90-312-2158259; fax: +90312-3107117. E-mail address: [email protected] (C¸. Atasoy).
diameter hypodense mass with soft tissue attenuation in the left upper lobe. The mass had slightly irregular, lobulated margins and showed inconspicuous peripheral contrast enhancement. The esophagus, left subclavian artery, and trachea appeared infiltrated by the lesion (Fig. 2), while the main pulmonary arteries were unaffected. The left paratracheal lymph nodes were enlarged. The mass was heterogeneous on T1-weighted magnetic resonance (MR) images with scattered areas of relatively high signal intensity and several hypointense punctate foci (Fig. 3). T2-weighted MR images better displayed the marked heterogeneity of the lesion caused by regions of high signal intensity interspersed in a background of intermediate intensity, resembling a cauliflower (Fig. 4). After the injection of gadolinium-DTPA, several linear structures probably representing vessels were observed entering the interior of the mass from the enhancing peripheral rim (Fig. 5). Histopathological examination of biopsy material showed vascular spaces lined by malignant endothelial cells, some containing vesicular nuclei, suggesting high-grade angiosarcoma (Fig. 6). Immunohistochemistry, using streptavidine – biotin peroxidase technique, showed positive staining for factor VIII-related antigen and CD31, confirming the diagnosis of angiosarcoma. Due to extensive mediastinal invasion, the tumor was considered inoperable, and chemotherapy was commenced. The patient is alive 9 months after the diagnosis.
0899-7071/01/$ – see front matter D 2001 Elsevier Science Inc. All rights reserved. PII: S 0 8 9 9 - 7 0 7 1 ( 0 1 ) 0 0 3 0 2 - 3
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C¸. Atasoy et al. / Journal of Clinical Imaging 25 (2001) 337–340
Fig. 3. Axial T1-weighted MR image shows internal heterogeneity of the lesion.
Fig. 1. Frontal chest radiograph shows a large well-defined opacity in the left upper lobe.
3. Discussion Angiosarcoma is a rare tumor, which constitutes less than 1% of all sarcomas [4]. The most frequently involved sites
Fig. 2. Contrast-enhanced CT section demonstrates a hypodense solid mass with obvious mediastinal invasion and peripheral enhancement (arrows).
are the skin and subcutaneous tissue, liver, breast, and heart [1,4]. Angiosarcomas occurring in the lung usually represent metastases from the heart, pulmonary arterial trunk, and extrathoracic organs [1]. Primary pulmonary origin is very uncommon, being reported only in a handful number of cases [2 – 10]. Predisposing factors include thoratrast, polyvinyl chloride, and phenylethylhydrazine for liver tumors, postmastectomy and postirradiation states for skin and chest wall lesions, and chronic empyema for pleural space angiosarcomas. Our patient had no history of radiotherapy and exposure to the former agents. Primary pulmonary angiosarcomas may occur either as multifocal lesions or as a solitary nodule. Patients usually present with chest pain, hemoptysis, dyspnea, cough, and
Fig. 4. Axial T2-weighted MR image shows to better advantage the cauliflower-like heterogeneity of the mass owing to regions of high signal intensity (arrows) in a background of intermediate signal intensity.
C¸. Atasoy et al. / Journal of Clinical Imaging 25 (2001) 337–340
Fig. 5. Contrast-enhanced axial T1-weighted image displays the enhancing periphery from which a linear enhancing structure is noted to enter the interior of the mass (arrow).
malaise [5]. When the involvement is multifocal, as has been described in many of the previous patients, chest X-rays show bilateral reticulonodular or alveolar infiltrates, with or without pleural effusion. This pattern of involvement suggests metastatic cancer and lymphangitic carcinoma [7].
339
The solitary form of primary pulmonary angiosarcoma has been described in a few patients. The size of the lesion may vary from a small nodule to a large mass invading the mediastinum or chest wall [4,5]. There may be accompanying pleural or pericardial effusions, and the surrounding lung may contain hemorrhage. Erosion of the adjacent bronchial structures may cause hemoptysis [7]. Ott et al. [5] reported the CT appearance of a solitary pulmonary angiosarcoma as a large, inhomogeneous solid mass involving all three lobes of the right lung with mediastinal invasion. In our patient, CT demonstrated a large mass with peripheral contrast enhancement and a slightly heterogeneous hypodense interior without appreciable opacification. Marked peripheral contrast enhancement and hypodense central parts may suggest inflammatory lesions including abscesses and infected hydatic cysts. However, absence of consolidation in the surrounding lung and fever and laboratory findings indicating inflammation may favor an alternative diagnosis. Demonstration of mediastinal and/or chest wall invasion and mediastinal lymph node enlargement may lead to a preliminary diagnosis of malignancy. Yet, differentiation from the more common bronchogenic carcinoma is practically impossible. To our knowledge, MR features of primary pulmonary angiosarcomas have not been described. Angiosarcomas of the heart were reported to have heterogeneous signal intensity on MR images. In cardiac angiosarcomas, Kim
Fig. 6. Microscopic section showing vascular spaces (asterisks) containing erythrocytes and lined by malignant endothelial cells, some of which had vesicular nuclei (arrows) (H&E 100).
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C¸. Atasoy et al. / Journal of Clinical Imaging 25 (2001) 337–340
et al. [11] described a cauliflower-like appearance consisting of focal nodular areas of increased signal intensity interspersed within areas of intermediate signal intensity on T1- and T2-weighted sequences. Likewise, the tumor in our patient exhibited a very heterogeneous pattern containing focal areas of high signal intensity in an overall background of intermediate intensity on both sequences yet more prominent on T2-weighted images. Such a high degree of heterogeneity is unusual in bronchogenic carcinomas and may be a useful hint in the distinction of angiosarcomas from lung cancers. Further case descriptions and patient series are needed to validate the potential utility of this finding. Despite the availability of sophisticated radiological methods and peculiar MR signal intensity characteristics, accurate differentiation of pulmonary angiosarcomas from other masses, especially lung cancer, is not possible without biopsy. The distinction between benign and malignant vascular lesions can be quite challenging even on histopathological grounds and requires immunohistochemical methods in most patients. Among the various immunohistochemical markers used for classification, factor VIIIrelated antigen and CD31 are considered specific for tumors derived from endothelium [7,9]. Pulmonary angiosarcomas are characterized by an insidious growth and a late stage at presentation, when extensive local invasion and hematogeneous metastases have already occurred [5]. They are usually inoperable at the time of diagnosis and respond poorly to chemotherapy and radiation. Thus, prognosis is dismal, with most patients surviving less than 1 year [7]. In conclusion, despite their rarity, primary pulmonary angiosarcomas should be considered in the differential diagnosis of aggressive lung masses. Radiologically, the most helpful sign in the characterization of these lesions
seems to be the marked heterogeneity on T2-weighted images with focal areas of high signal intensity interspersed on a background of intermediate intensity, giving a cauliflower-like appearance. Nevertheless, the falseproof diagnosis rests on histopathological and immunohistochemical examinations.
References [1] Patel AM, Ryu JH. Angiosarcoma in the lung. Chest 1993;103: 1531 – 5. [2] Tralka GA, Katz S. Hemangioendothelioma of the lung. Am Rev Respir Dis 1981;87:107 – 15. [3] Spragg RG, Wolf PL, Haghighi P, Abraham JL, Astarita RW. Angiosarcoma of the lung with fatal pulmonary hemorrhage. Am J Med 1983;74:1072 – 6. [4] Palvio DHB, Paulsen SM, Henneberg EW. Primary angiosarcoma of the lung presenting as intractable hemoptysis. Thorac Cardiovasc Surg 1987;35:105 – 7. [5] Ott RA, Eugene J, Kollin J, Kanas RJ, Conston DE, Chi JCS. Primary pulmonary angiosarcoma associated with multiple synchronous neoplasms. J Surg Oncol 1987;35:269 – 76. [6] Segal SL, Lenchner GS, Cichelli AV, Promisloff RA, Hofman WI, Baiocchi GA. Angiosarcoma presenting as diffuse alveolar hemorrhage. Chest 1988;94:214 – 6. [7] Yousem SA. Angiosarcoma presenting in the lung. Arch Pathol Lab Med 1986;110:112 – 5. [8] Miller DL, Allen MS. Rare pulmonary neoplasms. Mayo Clin Proc 1993;68:492 – 8. [9] Sheppard MN, Hansell DM, Du Bois RM, Nicholson AG. Primary epitheloid angiosarcoma of the lung presenting as pulmonary hemorrhage. Hum Pathol 1997;28(3):383 – 5. [10] Keel SB, Bacha E., Mark EJ, Nielsen P, Rosenberg AE. Primary pulmonary sarcoma: a clinicopathologic study of 26 cases. Mod Pathol 1999;12(12):1124 – 31. [11] Kim EE, Wallace S, Abello R, Coan JD, Ewer MS, Salem PA, Ali MK. Malignant cardiac fibrous histiocytomas and angiosarcomas: MR features. J Comput Assist Tomogr 1989;13:627 – 32.
Radiographic, CT, and MRI findings in primary pulmonary angiosarcoma C¸etin Atasoy*, Suat Fitoz, Hasan Yigˇit, Pinar Atasoy, Ilhan Erden, Serdar Akyar School of Medicine, Ankara University, Ibn-i Sina Hospital, 06100, Sihhiye- Ankara, Turkey Received 10 January 2001
Abstract We report a rare instance of primary pulmonary angiosarcoma presenting as a large solitary mass in the left upper lobe with mediastinal invasion. In particular, we emphasize the magnetic resonance (MR) imaging features, which included a markedly heterogeneous pattern consisting of hyperintense areas scattered throughout a background of intermediate signal intensity, rendering the lesion a cauliflower-like appearance especially on T2-weighted images. Being unreported so far in primary pulmonary angiosarcomas, these distinct MR imaging findings may be helpful in the differentiation of these neoplasms from lung cancers. D 2001 Elsevier Science Inc. All rights reserved. Keywords: Angiosarcoma; Lung; Neoplasm; CT; MRI
1. Introduction Angiosarcomas of the lung are very rare tumors and usually represent metastases from the heart, pulmonary arterial trunk, or extrathoracic organs [1]. Primary pulmonary origin of this tumor has been reported very infrequently [2– 10]. In this paper, we describe radiological findings in a patient with a solitary left upper lobe mass, which was diagnosed as angiosarcoma by histopathological and immunohistochemical examinations.
2. Case report A 50-year-old man presented with a 2-month history of chest pain, malaise, and hoarseness. Physical examination revealed decreased respiratory sounds in the left upper zone and left-sided Horner’s syndrome. Routine laboratory examinations were unremarkable. Chest X-ray showed a large, well-defined homogeneous opacity occupying the upper zone of the left hemithorax (Fig. 1). Computed tomography (CT) of the chest demonstrated a 10-cm * Corresponding author. Emek, 10. Cadde (Kirim Caddesi), Senyuva Sitesi, N2/21, 06510, Ankara, Turkey. Tel.: +90-312-2158259; fax: +90312-3107117. E-mail address: [email protected] (C¸. Atasoy).
diameter hypodense mass with soft tissue attenuation in the left upper lobe. The mass had slightly irregular, lobulated margins and showed inconspicuous peripheral contrast enhancement. The esophagus, left subclavian artery, and trachea appeared infiltrated by the lesion (Fig. 2), while the main pulmonary arteries were unaffected. The left paratracheal lymph nodes were enlarged. The mass was heterogeneous on T1-weighted magnetic resonance (MR) images with scattered areas of relatively high signal intensity and several hypointense punctate foci (Fig. 3). T2-weighted MR images better displayed the marked heterogeneity of the lesion caused by regions of high signal intensity interspersed in a background of intermediate intensity, resembling a cauliflower (Fig. 4). After the injection of gadolinium-DTPA, several linear structures probably representing vessels were observed entering the interior of the mass from the enhancing peripheral rim (Fig. 5). Histopathological examination of biopsy material showed vascular spaces lined by malignant endothelial cells, some containing vesicular nuclei, suggesting high-grade angiosarcoma (Fig. 6). Immunohistochemistry, using streptavidine – biotin peroxidase technique, showed positive staining for factor VIII-related antigen and CD31, confirming the diagnosis of angiosarcoma. Due to extensive mediastinal invasion, the tumor was considered inoperable, and chemotherapy was commenced. The patient is alive 9 months after the diagnosis.
0899-7071/01/$ – see front matter D 2001 Elsevier Science Inc. All rights reserved. PII: S 0 8 9 9 - 7 0 7 1 ( 0 1 ) 0 0 3 0 2 - 3
338
C¸. Atasoy et al. / Journal of Clinical Imaging 25 (2001) 337–340
Fig. 3. Axial T1-weighted MR image shows internal heterogeneity of the lesion.
Fig. 1. Frontal chest radiograph shows a large well-defined opacity in the left upper lobe.
3. Discussion Angiosarcoma is a rare tumor, which constitutes less than 1% of all sarcomas [4]. The most frequently involved sites
Fig. 2. Contrast-enhanced CT section demonstrates a hypodense solid mass with obvious mediastinal invasion and peripheral enhancement (arrows).
are the skin and subcutaneous tissue, liver, breast, and heart [1,4]. Angiosarcomas occurring in the lung usually represent metastases from the heart, pulmonary arterial trunk, and extrathoracic organs [1]. Primary pulmonary origin is very uncommon, being reported only in a handful number of cases [2 – 10]. Predisposing factors include thoratrast, polyvinyl chloride, and phenylethylhydrazine for liver tumors, postmastectomy and postirradiation states for skin and chest wall lesions, and chronic empyema for pleural space angiosarcomas. Our patient had no history of radiotherapy and exposure to the former agents. Primary pulmonary angiosarcomas may occur either as multifocal lesions or as a solitary nodule. Patients usually present with chest pain, hemoptysis, dyspnea, cough, and
Fig. 4. Axial T2-weighted MR image shows to better advantage the cauliflower-like heterogeneity of the mass owing to regions of high signal intensity (arrows) in a background of intermediate signal intensity.
C¸. Atasoy et al. / Journal of Clinical Imaging 25 (2001) 337–340
Fig. 5. Contrast-enhanced axial T1-weighted image displays the enhancing periphery from which a linear enhancing structure is noted to enter the interior of the mass (arrow).
malaise [5]. When the involvement is multifocal, as has been described in many of the previous patients, chest X-rays show bilateral reticulonodular or alveolar infiltrates, with or without pleural effusion. This pattern of involvement suggests metastatic cancer and lymphangitic carcinoma [7].
339
The solitary form of primary pulmonary angiosarcoma has been described in a few patients. The size of the lesion may vary from a small nodule to a large mass invading the mediastinum or chest wall [4,5]. There may be accompanying pleural or pericardial effusions, and the surrounding lung may contain hemorrhage. Erosion of the adjacent bronchial structures may cause hemoptysis [7]. Ott et al. [5] reported the CT appearance of a solitary pulmonary angiosarcoma as a large, inhomogeneous solid mass involving all three lobes of the right lung with mediastinal invasion. In our patient, CT demonstrated a large mass with peripheral contrast enhancement and a slightly heterogeneous hypodense interior without appreciable opacification. Marked peripheral contrast enhancement and hypodense central parts may suggest inflammatory lesions including abscesses and infected hydatic cysts. However, absence of consolidation in the surrounding lung and fever and laboratory findings indicating inflammation may favor an alternative diagnosis. Demonstration of mediastinal and/or chest wall invasion and mediastinal lymph node enlargement may lead to a preliminary diagnosis of malignancy. Yet, differentiation from the more common bronchogenic carcinoma is practically impossible. To our knowledge, MR features of primary pulmonary angiosarcomas have not been described. Angiosarcomas of the heart were reported to have heterogeneous signal intensity on MR images. In cardiac angiosarcomas, Kim
Fig. 6. Microscopic section showing vascular spaces (asterisks) containing erythrocytes and lined by malignant endothelial cells, some of which had vesicular nuclei (arrows) (H&E 100).
340
C¸. Atasoy et al. / Journal of Clinical Imaging 25 (2001) 337–340
et al. [11] described a cauliflower-like appearance consisting of focal nodular areas of increased signal intensity interspersed within areas of intermediate signal intensity on T1- and T2-weighted sequences. Likewise, the tumor in our patient exhibited a very heterogeneous pattern containing focal areas of high signal intensity in an overall background of intermediate intensity on both sequences yet more prominent on T2-weighted images. Such a high degree of heterogeneity is unusual in bronchogenic carcinomas and may be a useful hint in the distinction of angiosarcomas from lung cancers. Further case descriptions and patient series are needed to validate the potential utility of this finding. Despite the availability of sophisticated radiological methods and peculiar MR signal intensity characteristics, accurate differentiation of pulmonary angiosarcomas from other masses, especially lung cancer, is not possible without biopsy. The distinction between benign and malignant vascular lesions can be quite challenging even on histopathological grounds and requires immunohistochemical methods in most patients. Among the various immunohistochemical markers used for classification, factor VIIIrelated antigen and CD31 are considered specific for tumors derived from endothelium [7,9]. Pulmonary angiosarcomas are characterized by an insidious growth and a late stage at presentation, when extensive local invasion and hematogeneous metastases have already occurred [5]. They are usually inoperable at the time of diagnosis and respond poorly to chemotherapy and radiation. Thus, prognosis is dismal, with most patients surviving less than 1 year [7]. In conclusion, despite their rarity, primary pulmonary angiosarcomas should be considered in the differential diagnosis of aggressive lung masses. Radiologically, the most helpful sign in the characterization of these lesions
seems to be the marked heterogeneity on T2-weighted images with focal areas of high signal intensity interspersed on a background of intermediate intensity, giving a cauliflower-like appearance. Nevertheless, the falseproof diagnosis rests on histopathological and immunohistochemical examinations.
References [1] Patel AM, Ryu JH. Angiosarcoma in the lung. Chest 1993;103: 1531 – 5. [2] Tralka GA, Katz S. Hemangioendothelioma of the lung. Am Rev Respir Dis 1981;87:107 – 15. [3] Spragg RG, Wolf PL, Haghighi P, Abraham JL, Astarita RW. Angiosarcoma of the lung with fatal pulmonary hemorrhage. Am J Med 1983;74:1072 – 6. [4] Palvio DHB, Paulsen SM, Henneberg EW. Primary angiosarcoma of the lung presenting as intractable hemoptysis. Thorac Cardiovasc Surg 1987;35:105 – 7. [5] Ott RA, Eugene J, Kollin J, Kanas RJ, Conston DE, Chi JCS. Primary pulmonary angiosarcoma associated with multiple synchronous neoplasms. J Surg Oncol 1987;35:269 – 76. [6] Segal SL, Lenchner GS, Cichelli AV, Promisloff RA, Hofman WI, Baiocchi GA. Angiosarcoma presenting as diffuse alveolar hemorrhage. Chest 1988;94:214 – 6. [7] Yousem SA. Angiosarcoma presenting in the lung. Arch Pathol Lab Med 1986;110:112 – 5. [8] Miller DL, Allen MS. Rare pulmonary neoplasms. Mayo Clin Proc 1993;68:492 – 8. [9] Sheppard MN, Hansell DM, Du Bois RM, Nicholson AG. Primary epitheloid angiosarcoma of the lung presenting as pulmonary hemorrhage. Hum Pathol 1997;28(3):383 – 5. [10] Keel SB, Bacha E., Mark EJ, Nielsen P, Rosenberg AE. Primary pulmonary sarcoma: a clinicopathologic study of 26 cases. Mod Pathol 1999;12(12):1124 – 31. [11] Kim EE, Wallace S, Abello R, Coan JD, Ewer MS, Salem PA, Ali MK. Malignant cardiac fibrous histiocytomas and angiosarcomas: MR features. J Comput Assist Tomogr 1989;13:627 – 32.