(Radiographics. 2002;22:621-637.)
© RSNA, 2002
Primary Thoracic Sarcomas1
Gregory W. Gladish, MD,
Bradley M. Sabloff, MD,
Reginald F. Munden, DMD, MD,
Mylene T. Truong, MD,
Jeremy J. Erasmus, MD and
Marvin H. Chasen, MD
1 From the Department of Diagnostic Radiology, M.D. Anderson Cancer Center, 1515 Holcombe Blvd, Box 57, Houston, TX 77030. Presented as an education exhibit at the 2000 RSNA scientific assembly. Received April 18, 2001; revision requested July 9; final revision received January 30, 2002; accepted January 30. Address correspondence to G.W.G. (e-mail: ggladish@mdanderson.org).
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Abstract
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Primary sarcomas of the thorax are rare. The diagnosis is established only after sarcomalike primary lung malignancies and metastatic disease have been excluded. Primary sarcomas of the thorax are classified according to their histologic features and constitute a large group of tumors that occur in the lung, mediastinum, pleura, and chest wall. Angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and mesothelioma (sarcomatoid variant) are the most common primary intrathoracic sarcomas. Ewing sarcoma, primitive neuroectodermal tumor, chondrosarcoma, malignant fibrous histiocytoma, osteosarcoma, synovial sarcoma, and fibrosarcoma usually arise in the chest wall. Although primary thoracic sarcomas commonly manifest as large, heterogeneous masses, they have a wide spectrum of radiologic manifestations, including solitary pulmonary nodules, central endobronchial tumors, and intraluminal masses within the pulmonary arteries. The different histologic types of sarcomas are frequently indistinguishable at radiologic analysis. However, differences in clinical presentation and the location of the tumor, as well as morphologic features such as calcification within the mass and rib involvement, can be useful in suggesting the appropriate diagnosis. For example, a large rib mass in a child with fever and malaise indicates a Ewing sarcoma, a mass with a calcified matrix is likely a chondrosarcoma or osteosarcoma, and a pulmonary artery mass is likely a leiomyosarcoma.
© RSNA, 2002
Index Terms: Sarcoma, 47.32, 47.37, 50.32, 60.325 • Thorax, neoplasms, 47.32, 47.37, 50.32, 60.325
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LEARNING OBJECTIVES FOR TEST 4
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After reading this article and taking the test, the reader will be able to:
- Identify the more common appearances of thoracic sarcomas.
- List the features of the typical patients with the various thoracic sarcomas.
- Describe the combinations of clinical, anatomic, and radiologic features that can suggest particular thoracic sarcomas.
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Introduction
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Primary sarcomas of the thorax are rare. They occur in the lung, mediastinum, pleura, and chest wall. Angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and mesothelioma (sarcomatoid variant) are the most common primary intrathoracic sarcomas. The diagnosis is established only after sarcomalike primary lung malignancies (sarcomatoid carcinomas) and metastatic disease have been excluded. Ewing sarcoma, primitive neuroectodermal tumor, chondrosarcoma, malignant fibrous histiocytoma, osteosarcoma, synovial sarcoma, and fibrosarcoma usually arise in the chest wall. However, occasionally these manifest as intrathoracic masses with only limited or no chest wall involvement.
Thoracic sarcomas typically receive a thorough imaging evaluation predominantly focused on determining the extent of tumor involvement and the potential for resectability. In particular, although most thoracic sarcomas are initially evaluated with conventional radiography and computed tomography (CT), chest wall and mediastinal lesions often require magnetic resonance (MR) imaging to determine involvement of adjacent structures. This article reviews the most common radiologic manifestations of primary thoracic sarcomas (Tables 1, 2) and the features that can assist diagnosis and, on occasion, suggest a particular histologic origin.
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Sarcomas with a Predominantly Intrathoracic Location
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Angiosarcoma
Although primary intrathoracic angiosarcomas are rare, they are the most common primary cardiac sarcoma and the majority of primary right atrial masses are angiosarcomas (Fig 1) (1). Primary angiosarcomas also occur in the anterior mediastinum, typically without an obvious vascular origin (Fig 2), and, rarely, in the lung (2,3).
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Figure 1a. (1) Cardiac angiosarcoma in a 19-year-old man. (a) Contrast material-enhanced CT scan shows diffuse thickening of the right atrial wall with heterogeneous enhancement (arrows). Diffuse, heterogeneous enhancement is a typical feature of cardiac angiosarcomas. (b) Axial T2-weighted MR image shows a diffuse mass with high signal intensity in the right atrial wall (arrows). Right atrial origin is characteristic of cardiac angiosarcomas.
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Figure 1b. (1) Cardiac angiosarcoma in a 19-year-old man. (a) Contrast material-enhanced CT scan shows diffuse thickening of the right atrial wall with heterogeneous enhancement (arrows). Diffuse, heterogeneous enhancement is a typical feature of cardiac angiosarcomas. (b) Axial T2-weighted MR image shows a diffuse mass with high signal intensity in the right atrial wall (arrows). Right atrial origin is characteristic of cardiac angiosarcomas.
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Figure 2a. Mediastinal angiosarcoma in a 38-year-old man. (a) Posteroanterior chest radiograph shows a mediastinal contour abnormality (arrows). (b) Contrast-enhanced CT scan shows a large, homogeneous mediastinal mass (arrows) abutting the aorta (A) and superior vena cava (S). At exploratory thoracotomy, the mass did not arise from or involve the aorta and heart, and resection was performed. Location in the anterior mediastinum and absence of an obvious vascular origin are typical of angiosarcomas.
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Figure 2b. Mediastinal angiosarcoma in a 38-year-old man. (a) Posteroanterior chest radiograph shows a mediastinal contour abnormality (arrows). (b) Contrast-enhanced CT scan shows a large, homogeneous mediastinal mass (arrows) abutting the aorta (A) and superior vena cava (S). At exploratory thoracotomy, the mass did not arise from or involve the aorta and heart, and resection was performed. Location in the anterior mediastinum and absence of an obvious vascular origin are typical of angiosarcomas.
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Angiosarcomas typically occur in middle-aged adults, and the clinical symptoms depend on their location. Cardiac tumors manifest as congestive heart failure or arrhythmia, mediastinal tumors manifest as the result of local mass effect, and primary pulmonary angiosarcomas are asymptomatic or manifest as hemoptysis. These tumors have been associated with radiation therapy and with occupational exposure to vinyl chloride and arsenic.
At radiography, extracardiac mediastinal sarcomas manifest as large masses. They are typically located in the anterior mediastinum. The attenuation at CT can be homogeneous but is more often heterogeneous due to areas of hemorrhage, necrosis, and cyst formation (4). At CT and MR imaging, cardiac angiosarcomas manifest as diffuse wall thickening or focal masses that typically involve the right atrium and enhance heterogeneously after intravenous administration of contrast material (5). Primary pulmonary angiosarcomas usually manifest as multiple bilateral nodules (6).
The prognosis is poor, with few patients surviving beyond 3 years (2). When possible, treatment includes resection and radiation therapy. Chemotherapy has been used to decrease the size of the tumor prior to surgery to facilitate resection, as well as in the treatment of metastatic disease.
Leiomyosarcoma
Primary leiomyosarcomas occur in the mediastinum, heart, and lung (5–10). Although they are reported to arise from mediastinal structures such as the esophagus or mediastinal vasculature, including the pulmonary artery (8) and superior vena cava (9), most mediastinal tumors do not involve these structures (10). Cardiac leiomyosarcomas differ from angiosarcomas in that they are less common and typically occur in the left atrium (6,7). Leiomyosarcoma is one of the more common histologic subtypes of sarcoma to occur in the lung (6,7).
Leiomyosarcomas usually occur in the sixth decade or later. There tends to be predominance in men, and this occurs particularly in patients with primary pulmonary neoplasms. However, pulmonary artery leiomyosarcomas occur with an equal frequency in men and women, usually a decade or so younger (mean age at diagnosis of 50 years). Leiomyosarcomas located in the mediastinum typically manifest clinically as a result of local mass effect (eg, pain, cough, superior vena cava syndrome). Patients with pulmonary artery sarcomas present with chest pain, dyspnea, or intractable congestive heart failure (8). Primary pulmonary leiomyosarcomas are often asymptomatic, but patients can present with hemoptysis.
At radiologic analysis, these neoplasms manifest in the lung as well-marginated smooth or lobular homogeneous nodules or large necrotic masses (Fig 3) (11). Mediastinal leiomyosarcomas are usually large neoplasms that are often heterogeneous in appearance due to necrosis or hemorrhage within the masses. Becauseleiomyosarcomas of the pulmonary artery frequently grow within the lumen, they can be difficult to distinguish from pulmonary artery thrombi. MR imaging can often be useful in establishing the diagnosis because leiomyosarcomas, unlike thrombi, enhance after intravenous administration of gadolinium contrast material (12) (Fig 4).
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Figure 3a. Pulmonary leiomyosarcoma in a 62-year-old woman. (a) Contrast-enhanced CT scan shows a large, heterogeneous lung mass that extends into the azygoesophageal recess and compresses and displaces the left atrium (LA). Note the origin in the lung, the large size, and the heterogeneous attenuation, common features of leiomyosarcoma. (b) Axial gadolinium-enhanced fat-saturated T1-weighted MR image shows that the mass is heterogeneous. Diffuse enhancement surrounds well-circumscribed areas of low signal intensity (arrows), which are consistent with cystic spaces. (c) Axial fast spin-echo T2-weighted MR image shows a fluid-fluid level (arrow), which is consistent with blood products within a cystic space. At surgery, the tumor was confined to the lung and visceral pleura.
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Figure 3b. Pulmonary leiomyosarcoma in a 62-year-old woman. (a) Contrast-enhanced CT scan shows a large, heterogeneous lung mass that extends into the azygoesophageal recess and compresses and displaces the left atrium (LA). Note the origin in the lung, the large size, and the heterogeneous attenuation, common features of leiomyosarcoma. (b) Axial gadolinium-enhanced fat-saturated T1-weighted MR image shows that the mass is heterogeneous. Diffuse enhancement surrounds well-circumscribed areas of low signal intensity (arrows), which are consistent with cystic spaces. (c) Axial fast spin-echo T2-weighted MR image shows a fluid-fluid level (arrow), which is consistent with blood products within a cystic space. At surgery, the tumor was confined to the lung and visceral pleura.
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Figure 3c. Pulmonary leiomyosarcoma in a 62-year-old woman. (a) Contrast-enhanced CT scan shows a large, heterogeneous lung mass that extends into the azygoesophageal recess and compresses and displaces the left atrium (LA). Note the origin in the lung, the large size, and the heterogeneous attenuation, common features of leiomyosarcoma. (b) Axial gadolinium-enhanced fat-saturated T1-weighted MR image shows that the mass is heterogeneous. Diffuse enhancement surrounds well-circumscribed areas of low signal intensity (arrows), which are consistent with cystic spaces. (c) Axial fast spin-echo T2-weighted MR image shows a fluid-fluid level (arrow), which is consistent with blood products within a cystic space. At surgery, the tumor was confined to the lung and visceral pleura.
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Figure 4a. Pulmonary artery leiomyosarcoma in a 41-year-old man. (a) Posteroanterior chest radiograph shows a sharply marginated, lobular left hilar mass (M). (b) Contrast-enhanced CT scan shows that the mass is homogeneous. It fills the left main pulmonary artery and extends into the left upper and lower lobe pulmonary arteries (arrows). (c, d) Axial T1-weighted (c) and gadolinium-enhanced T1-weighted (d) MR images show that the mass has high signal intensity with heterogeneous enhancement (arrows in d). Slow flow around the periphery of the mass causes high signal intensity (arrowheads in d). Because enhancement is useful in distinguishing a pulmonary artery mass from a thrombus, intravenous administration of gadolinium contrast material is recommended in cases in which the diagnosis is uncertain.
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Figure 4b. Pulmonary artery leiomyosarcoma in a 41-year-old man. (a) Posteroanterior chest radiograph shows a sharply marginated, lobular left hilar mass (M). (b) Contrast-enhanced CT scan shows that the mass is homogeneous. It fills the left main pulmonary artery and extends into the left upper and lower lobe pulmonary arteries (arrows). (c, d) Axial T1-weighted (c) and gadolinium-enhanced T1-weighted (d) MR images show that the mass has high signal intensity with heterogeneous enhancement (arrows in d). Slow flow around the periphery of the mass causes high signal intensity (arrowheads in d). Because enhancement is useful in distinguishing a pulmonary artery mass from a thrombus, intravenous administration of gadolinium contrast material is recommended in cases in which the diagnosis is uncertain.
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Figure 4c. Pulmonary artery leiomyosarcoma in a 41-year-old man. (a) Posteroanterior chest radiograph shows a sharply marginated, lobular left hilar mass (M). (b) Contrast-enhanced CT scan shows that the mass is homogeneous. It fills the left main pulmonary artery and extends into the left upper and lower lobe pulmonary arteries (arrows). (c, d) Axial T1-weighted (c) and gadolinium-enhanced T1-weighted (d) MR images show that the mass has high signal intensity with heterogeneous enhancement (arrows in d). Slow flow around the periphery of the mass causes high signal intensity (arrowheads in d). Because enhancement is useful in distinguishing a pulmonary artery mass from a thrombus, intravenous administration of gadolinium contrast material is recommended in cases in which the diagnosis is uncertain.
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Figure 4d. Pulmonary artery leiomyosarcoma in a 41-year-old man. (a) Posteroanterior chest radiograph shows a sharply marginated, lobular left hilar mass (M). (b) Contrast-enhanced CT scan shows that the mass is homogeneous. It fills the left main pulmonary artery and extends into the left upper and lower lobe pulmonary arteries (arrows). (c, d) Axial T1-weighted (c) and gadolinium-enhanced T1-weighted (d) MR images show that the mass has high signal intensity with heterogeneous enhancement (arrows in d). Slow flow around the periphery of the mass causes high signal intensity (arrowheads in d). Because enhancement is useful in distinguishing a pulmonary artery mass from a thrombus, intravenous administration of gadolinium contrast material is recommended in cases in which the diagnosis is uncertain.
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Although the frequency of metastatic disease is not related to tumor size, the prognosis of mediastinal leiomyosarcomas is poorer with larger tumors because of an associated higher frequency of local invasion and recurrence. Pulmonary artery lesions have a particularly poor prognosis, with few patients alive at 6–12 months. Treatment consists, when possible, of local excision, radiation, and chemotherapy.
Rhabdomyosarcoma
Rhabdomyosarcomas are rare thoracic neoplasms that occur within the lung, bronchi, mediastinum, heart, and chest wall. They have a wide age range of presentation but typically occur in childhood or in the fifth to seventh decades of life with a male predominance (13). In children, rhabdomyosarcoma is the most common cardiac sarcoma and the sarcoma that most frequently originates from the cardiac valves (5). Chest wall rhabdomyosarcomas in children differ from Ewing sarcoma and primitive neuroectodermal tumors in that rib involvement is uncommon (14). In adults, rhabdomyosarcomas tend to occur in the chest wall or arise from the diaphragm. Patients with pulmonary and bronchial rhabdomyosarcomas can present with cough, dyspnea, hemoptysis, and pneumothorax, whereas those with chest wall masses typically present with pain. The presentation of cardiac tumors varies with the chamber of origin and involved structures and includes arrhythmias and left- and right-sided heart failure.
At radiologic analysis, rhabdomyosarcomas manifest as masses that are often large and of variable attenuation due to necrosis and cystic components within the masses (Fig 5). Invasion and growth into adjacent vasculature and bronchi have been reported. Cardiac tumors typically manifest at CT as low-attenuation masses within a cardiac chamber. The signal intensity at MR imaging is variable; the tumors may be isointense to muscle or have heterogeneous signal intensity if there are cystic or necrotic components within the mass (5). Chest wall masses are typically large and can be homogeneous or heterogeneous. Masses can be confined to the muscle of origin, although focal invasion is common.
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Figure 5a. Rhabdomyosarcoma in a 42-year-old man. (a) Posteroanterior chest radiograph shows a well-circumscribed mass in the right hemithorax that arises from the mediastinum. Note the small right pleural effusion and the surgical clips from incisional biopsy. (b) Contrast-enhanced CT scan shows that the mass is large and compresses the superior vena cava (arrow). Note the heterogeneous attenuation within the mass, which is consistent with necrosis.
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Figure 5b. Rhabdomyosarcoma in a 42-year-old man. (a) Posteroanterior chest radiograph shows a well-circumscribed mass in the right hemithorax that arises from the mediastinum. Note the small right pleural effusion and the surgical clips from incisional biopsy. (b) Contrast-enhanced CT scan shows that the mass is large and compresses the superior vena cava (arrow). Note the heterogeneous attenuation within the mass, which is consistent with necrosis.
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The prognosis is related to the histologic subtype, with patients with alveolar rhabdomyosarcomas having a poorer prognosis than those with the embryonal and pleomorphic subtypes (15). The prognosis is also determined by the extent and degree of differentiation of the tumor; well-differentiated tumors have the best prognosis, and survival may be greater than 80% if the disease is localized.
Rhabdomyosarcomas are usually treated with chemotherapy; radiation therapy and surgery are used in some patients, depending on tumor location and the extent of local invasion.
Sarcomatoid Mesothelioma
Sarcomatoid mesothelioma, a pleural malignancy associated with exposure to asbestos, is a histologic subtype of mesothelioma that consists predominantly of spindle cells. Origin from the pleura is typically unilateral, and patients are usually in the sixth or seventh decade of life. The strong male predominance of 85%–90% is related to the occupational exposure to asbestos. Patients often present with chest or shoulder pain. Dyspnea, cough, and fatigue are typically late clinical manifestations. Sarcomatoid mesotheliomas can secrete hormones; gynecomastia and clinical manifestations of inappropriate antidiuretic hormone secretion have been reported.
At radiologic analysis, sarcomatoid mesotheliomas typically manifest as unilateral, lobular pleural masses that encase the hemithorax. Most patients have an ipsilateral pleural effusion that can be small to large in volume. Rarely, sarcomatoid mesotheliomas manifest as large, solitary masses (Fig 6). This manifestation is less common in the epithelioid subtype of mesothelioma (16). The mass can directly involve the adjacent lung or chest wall. Mediastinal adenopathy and, less commonly, pulmonary metastases occur as late manifestations (17). CT and MR imaging are optimal in assessing the extent and degree of invasion of adjacent structures. At MR imaging, the tumor is usually isointense to slightly hyperintense (compared with muscle) on T1-weighted images and hyperintense on T2-weighted images (17).
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Figure 6a. Sarcomatoid mesothelioma in a 37-year-old man. (a) Posteroanterior chest radiograph shows a focal extraparenchymal mass (arrows). Note the bullous changes and scarring in the upper lobes. (b, c) Axial T1-weighted (b) and gadolinium-enhanced T1-weighted (c) MR images show that the mass has intermediate signal intensity (similar to that of muscle) and heterogeneous enhancement. Note the chest wall invasion (arrowheads).
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Figure 6b. Sarcomatoid mesothelioma in a 37-year-old man. (a) Posteroanterior chest radiograph shows a focal extraparenchymal mass (arrows). Note the bullous changes and scarring in the upper lobes. (b, c) Axial T1-weighted (b) and gadolinium-enhanced T1-weighted (c) MR images show that the mass has intermediate signal intensity (similar to that of muscle) and heterogeneous enhancement. Note the chest wall invasion (arrowheads).
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Figure 6c. Sarcomatoid mesothelioma in a 37-year-old man. (a) Posteroanterior chest radiograph shows a focal extraparenchymal mass (arrows). Note the bullous changes and scarring in the upper lobes. (b, c) Axial T1-weighted (b) and gadolinium-enhanced T1-weighted (c) MR images show that the mass has intermediate signal intensity (similar to that of muscle) and heterogeneous enhancement. Note the chest wall invasion (arrowheads).
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Patients with sarcomatoid mesothelioma usually have a poor prognosis, with 2-year survival less than 25%, 5-year survival close to zero, and median survival under 12 months. Treatment is typically palliative; curative extrapleural pneumonectomy is typically reserved for patients with the more favorable epithelioid histologic subtype (17).
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Sarcomas with a Predominantly Chest Wall Location
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Ewing Sarcoma
Ewing sarcomas are associated with a chromosome 22 translocation and are composed of small round cells. Cytoplasmic glycogen is detected at periodic acid–Schiff staining, a feature used to differentiate Ewing sarcoma from primitive neuroectodermal tumors. Fifteen percent of all Ewing sarcomas arise primarily in the chest wall, usually from a rib or, less frequently, the scapula (18). Ewing sarcomas have a male predominance of 1.6:1, typically occur in children or young adults, and are the most common tumor of the chest wall in this age group (14,18,19). Most patients (90%) present with a painful chest wall mass. Fever and malaise are common associated systemic manifestations (18).
The characteristic radiologic manifestation is a chest wall mass associated with bone destruction. However, Ewing sarcomas occasionally manifest as a large mass with only a small component of bone involvement (Fig 7). At CT, the attenuation of the mass is usually homogeneous when small and heterogeneous when large (14). Ewing sarcomas are rarely extraosseous in origin, usually in older patients (mean age 20 years), and manifest as well-circumscribed, noncalcified masses in the thorax, more frequently in a paravertebral location (20,21). Involvement of bone marrow, the hallmark of an osseous origin, is typically absent at CT or MR imaging (21) in these extraosseous tumors.
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Figure 7a. Ewing sarcoma in a 9-year-old boy. (a) Posteroanterior chest radiograph shows a large mass within the left hemithorax that extends into the right hemithorax. (b) Nonenhanced CT scan shows that the mass is large and heterogeneous. There is destruction and inward displacement of a left rib (long arrow). Rib destruction is typical but may not be extensive. Note the local invasion into the subcutaneous tissues (short arrow). (c-e) Axial fast spin-echo T2-weighted (c), coronal T1-weighted (d), and coronal gadolinium-enhanced T1-weighted (e) MR images show focal areas of high signal intensity and heterogeneous enhancement, findings consistent with necrosis and hemorrhage. Note the marked displacement of mediastinal structures into the right hemithorax. LV = left ventricle.
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Figure 7b. Ewing sarcoma in a 9-year-old boy. (a) Posteroanterior chest radiograph shows a large mass within the left hemithorax that extends into the right hemithorax. (b) Nonenhanced CT scan shows that the mass is large and heterogeneous. There is destruction and inward displacement of a left rib (long arrow). Rib destruction is typical but may not be extensive. Note the local invasion into the subcutaneous tissues (short arrow). (c-e) Axial fast spin-echo T2-weighted (c), coronal T1-weighted (d), and coronal gadolinium-enhanced T1-weighted (e) MR images show focal areas of high signal intensity and heterogeneous enhancement, findings consistent with necrosis and hemorrhage. Note the marked displacement of mediastinal structures into the right hemithorax. LV = left ventricle.
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Figure 7c. Ewing sarcoma in a 9-year-old boy. (a) Posteroanterior chest radiograph shows a large mass within the left hemithorax that extends into the right hemithorax. (b) Nonenhanced CT scan shows that the mass is large and heterogeneous. There is destruction and inward displacement of a left rib (long arrow). Rib destruction is typical but may not be extensive. Note the local invasion into the subcutaneous tissues (short arrow). (c-e) Axial fast spin-echo T2-weighted (c), coronal T1-weighted (d), and coronal gadolinium-enhanced T1-weighted (e) MR images show focal areas of high signal intensity and heterogeneous enhancement, findings consistent with necrosis and hemorrhage. Note the marked displacement of mediastinal structures into the right hemithorax. LV = left ventricle.
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Figure 7d. Ewing sarcoma in a 9-year-old boy. (a) Posteroanterior chest radiograph shows a large mass within the left hemithorax that extends into the right hemithorax. (b) Nonenhanced CT scan shows that the mass is large and heterogeneous. There is destruction and inward displacement of a left rib (long arrow). Rib destruction is typical but may not be extensive. Note the local invasion into the subcutaneous tissues (short arrow). (c-e) Axial fast spin-echo T2-weighted (c), coronal T1-weighted (d), and coronal gadolinium-enhanced T1-weighted (e) MR images show focal areas of high signal intensity and heterogeneous enhancement, findings consistent with necrosis and hemorrhage. Note the marked displacement of mediastinal structures into the right hemithorax. LV = left ventricle.
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Figure 7e. Ewing sarcoma in a 9-year-old boy. (a) Posteroanterior chest radiograph shows a large mass within the left hemithorax that extends into the right hemithorax. (b) Nonenhanced CT scan shows that the mass is large and heterogeneous. There is destruction and inward displacement of a left rib (long arrow). Rib destruction is typical but may not be extensive. Note the local invasion into the subcutaneous tissues (short arrow). (c-e) Axial fast spin-echo T2-weighted (c), coronal T1-weighted (d), and coronal gadolinium-enhanced T1-weighted (e) MR images show focal areas of high signal intensity and heterogeneous enhancement, findings consistent with necrosis and hemorrhage. Note the marked displacement of mediastinal structures into the right hemithorax. LV = left ventricle.
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Treatment is initially chemotherapy, usually followed by resection with or without radiation therapy. Overall 5- and 10-year survival rates are about 50%. The prognosis is affected by the development of metastases, which occurs in about 75% of patients; the 5-year survival with metastases is less than 30%, whereas survival without metastases approaches 100% (18).
Primitive Neuroectodermal Tumor
Primitive neuroectodermal tumors, also known as Askin tumors, are associated with a chromosome 22 translocation and are composed of small round cells with neural differentiation (22–24). These tumors have also been reported to occur after radiation therapy for Hodgkin lymphoma. They characteristically arise in the soft tissues of the thorax, but rarely occur primarily within the lung, often with pleural involvement (25). Patients are usually children or young adults, although primitive neuroectodermal tumors can occur at any age. Chest wall pain is the most frequent presentation and can be accompanied by a pleural effusion and dyspnea (26).
At radiologic analysis, primitive neuroectodermal tumors typically manifest as chest wall masses (Fig 8) that can be associated with adjacent rib destruction, pleural thickening or pleural effusion, and focal invasion of lung (26,27). CT usually reveals a soft-tissue mass with heterogeneous attenuation (14). The mass typically has high signal intensity on T1-weighted images and intermediate or high signal intensity on T2-weighted images (27,28).
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Figure 8a. Primitive neuroectodermal tumor in a 44-year-old man. (a) Posteroanterior chest radiograph shows a large, right-sided chest mass. (b) Nonenhanced CT scan shows that the mass is heterogeneous. Note the absence of rib destruction. Primitive neuroectodermal tumors are typically located in the chest wall and demonstrate rib destruction less commonly than does Ewing sarcoma.
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Figure 8b. Primitive neuroectodermal tumor in a 44-year-old man. (a) Posteroanterior chest radiograph shows a large, right-sided chest mass. (b) Nonenhanced CT scan shows that the mass is heterogeneous. Note the absence of rib destruction. Primitive neuroectodermal tumors are typically located in the chest wall and demonstrate rib destruction less commonly than does Ewing sarcoma.
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Treatment consists of resection, augmented by chemotherapy and radiation therapy. The overall survival is less than 40% at 2 years. Poor prognostic factors include metastases at diagnosis and inability to completely resect the mass.
Chondrosarcoma
Chondrosarcomas are the most common primary malignancy of the chest wall (29,30) but occasionally arise within the lungs and bronchi (31). Chondrosarcomas are typically located anteriorly within the chest wall, arising from the sternum or costochondral arches (Fig 9) (32). They may also arise from the scapula. Occasionally, tumors arising from the ribs manifest as intrathoracic masses with only minimal osseous involvement.
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Figure 9a. Chest wall chondrosarcoma in a 62-year-old man. (a) Nonenhanced CT scan shows a large mass that arises from the costochondral junction. The mass extends into the subcutaneous tissue and compresses and displaces the heart. Note the focal punctate and linear calcifications. Anterior location, origin from the costochondral arches, and chondroid calcification are characteristic of chondrosarcomas of the chest wall. (b, c) Axial gadolinium-enhanced T1-weighted (b) and fast spin-echo T2-weighted (c) MR images show that the mass is heterogeneous and enhances. Focal regions of high signal intensity on the T2-weighted image (c) are suggestive of necrosis. Chondroid calcification, a feature that can be useful in suggesting the diagnosis, is suboptimally visualized at MR imaging. A = aorta.
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Figure 9b. Chest wall chondrosarcoma in a 62-year-old man. (a) Nonenhanced CT scan shows a large mass that arises from the costochondral junction. The mass extends into the subcutaneous tissue and compresses and displaces the heart. Note the focal punctate and linear calcifications. Anterior location, origin from the costochondral arches, and chondroid calcification are characteristic of chondrosarcomas of the chest wall. (b, c) Axial gadolinium-enhanced T1-weighted (b) and fast spin-echo T2-weighted (c) MR images show that the mass is heterogeneous and enhances. Focal regions of high signal intensity on the T2-weighted image (c) are suggestive of necrosis. Chondroid calcification, a feature that can be useful in suggesting the diagnosis, is suboptimally visualized at MR imaging. A = aorta.
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Figure 9c. Chest wall chondrosarcoma in a 62-year-old man. (a) Nonenhanced CT scan shows a large mass that arises from the costochondral junction. The mass extends into the subcutaneous tissue and compresses and displaces the heart. Note the focal punctate and linear calcifications. Anterior location, origin from the costochondral arches, and chondroid calcification are characteristic of chondrosarcomas of the chest wall. (b, c) Axial gadolinium-enhanced T1-weighted (b) and fast spin-echo T2-weighted (c) MR images show that the mass is heterogeneous and enhances. Focal regions of high signal intensity on the T2-weighted image (c) are suggestive of necrosis. Chondroid calcification, a feature that can be useful in suggesting the diagnosis, is suboptimally visualized at MR imaging. A = aorta.
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Chondrosarcomas have a wide age range of presentation but typically occur in patients 30–60 years of age. There is a male predominance of 1.3:1. Most chest wall chondrosarcomas (80%) manifest clinically as masses that can be painless or painful (18). When arising in the lungs, these tumors tend to manifest as cough and chest pain.
At radiologic analysis, chondrosarcomas usually manifest as large chest wall masses with bone destruction and soft-tissue involvement. Scattered areas of calcification can occur in the chondroid matrix and are optimally detected with CT (29). The masses are of intermediate signal intensity on T1-weighted MR images and are heterogeneous on T2-weighted images, typically with scattered areas of high signal intensity (30). Within the lung and airways, chondrosarcomas manifest as lobular lung masses and polypoid endotracheal or endobronchial masses, respectively (31,33).
Therapy consists of resection of the mass along with any synchronous metastatic lesions. Surgical therapy is occasionally accompanied by chemotherapy or radiation therapy. Five-year survival rates are greater than 60% (>80% in patients without metastases). Poor prognostic factors include incomplete resection, metastases, and local recurrence, as well as age over 50 years (18).
Malignant Fibrous Histiocytoma
Malignant fibrous histiocytoma is the most common soft-tissue sarcoma in adults, although thoracic origin is infrequent. This tumor can be associated with previous bone lesions, including Paget disease and bone infarcts, and is the most common sarcoma to develop after irradiation (15). In the thorax, malignant fibrous histiocytoma usually arises from the chest wall musculature (Fig 10) and rarely occurs in the lung, mediastinum, or pleura (34,35). Most malignant fibrous histiocytomas occur in older adults, with an equal prevalence in men and women. Patients are typically asymptomatic, but those with intrathoracic tumors can present with cough, dyspnea, or hemoptysis.
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Figure 10a. Malignant fibrous histiocytoma of the chest wall in a 76-year-old woman. (a) Posteroanterior chest radiograph shows a large extraparenchymal mass in the right upper hemithorax (arrowheads) with associated rib fractures and rib destruction (arrows). Note the left pleural effusion. (b-d) Axial T1-weighted (b), gadolinium-enhanced T1-weighted (c), and fat-saturated T2-weighted (d) MR images show that the mass has heterogeneous signal intensity and marked enhancement. Focal areas of low signal intensity on the T1-weighted images (arrow in b and c) have high signal intensity on the T2-weighted image (arrow in d) and are consistent with necrosis and fluid. Origin within the chest wall musculature helps distinguish this tumor from osteosarcoma or chondrosarcoma.
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Figure 10b. Malignant fibrous histiocytoma of the chest wall in a 76-year-old woman. (a) Posteroanterior chest radiograph shows a large extraparenchymal mass in the right upper hemithorax (arrowheads) with associated rib fractures and rib destruction (arrows). Note the left pleural effusion. (b-d) Axial T1-weighted (b), gadolinium-enhanced T1-weighted (c), and fat-saturated T2-weighted (d) MR images show that the mass has heterogeneous signal intensity and marked enhancement. Focal areas of low signal intensity on the T1-weighted images (arrow in b and c) have high signal intensity on the T2-weighted image (arrow in d) and are consistent with necrosis and fluid. Origin within the chest wall musculature helps distinguish this tumor from osteosarcoma or chondrosarcoma.
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Figure 10c. Malignant fibrous histiocytoma of the chest wall in a 76-year-old woman. (a) Posteroanterior chest radiograph shows a large extraparenchymal mass in the right upper hemithorax (arrowheads) with associated rib fractures and rib destruction (arrows). Note the left pleural effusion. (b-d) Axial T1-weighted (b), gadolinium-enhanced T1-weighted (c), and fat-saturated T2-weighted (d) MR images show that the mass has heterogeneous signal intensity and marked enhancement. Focal areas of low signal intensity on the T1-weighted images (arrow in b and c) have high signal intensity on the T2-weighted image (arrow in d) and are consistent with necrosis and fluid. Origin within the chest wall musculature helps distinguish this tumor from osteosarcoma or chondrosarcoma.
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Figure 10d. Malignant fibrous histiocytoma of the chest wall in a 76-year-old woman. (a) Posteroanterior chest radiograph shows a large extraparenchymal mass in the right upper hemithorax (arrowheads) with associated rib fractures and rib destruction (arrows). Note the left pleural effusion. (b-d) Axial T1-weighted (b), gadolinium-enhanced T1-weighted (c), and fat-saturated T2-weighted (d) MR images show that the mass has heterogeneous signal intensity and marked enhancement. Focal areas of low signal intensity on the T1-weighted images (arrow in b and c) have high signal intensity on the T2-weighted image (arrow in d) and are consistent with necrosis and fluid. Origin within the chest wall musculature helps distinguish this tumor from osteosarcoma or chondrosarcoma.
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At radiologic analysis, malignant fibrous histiocytoma usually manifests as a well-marginated, smooth or lobular, soft-tissue chest wall mass. CT usually reveals heterogeneous attenuation and enhancement (36). Calcification is uncommon prior to therapy.
The prognosis is related to the size and depth of the lesion, with superficial or chest wall lesions having a better prognosis than lesions within the lung or mediastinum. There are four distinct histologic types: pleomorphic, myxoid, giant cell, and inflammatory. Of these, the myxoid type is associated with a better prognosis (15). Treatment is usually with resection and radiation therapy for local control, supplemented by chemotherapy for prevention of recurrence and treatment of micrometastatic disease. Preoperative chemotherapy may be used to improve the resectability of the tumor.
Osteosarcoma
Primary osteosarcomas of the thorax are rare. They can occur de novo, arise in a preexisting bone lesion, including Paget disease, or be related to radiation exposure or chemotherapy. They are usually osseous in origin, although isolated cases of primary mediastinal, cardiac, and pulmonary osteosarcomas have been reported (37,38). Osseous osteosarcomas generally occur in the second and third decades, whereas the extraosseous tumors usually occur in patients in the fifth decade of life or later with a slight predominance in men (39). Most patients present with a painful mass, although occasionally chest pain alone or a painless mass occurs.
The tumors typically arise from a rib, the scapula, or the clavicle. At radiologic analysis, they manifest as bone destruction with an associated soft-tissue mass (Fig 11). CT and MR imaging usually show a large mass that is typically heterogeneous in appearance because of necrosis, hemorrhage, and ossification. Although all osteosarcomas contain osteoid matrix at histologic analysis, ossification may not be radiologically visible in some patients (38) (Fig 12).
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Figure 11. Osteosarcoma in a 43-year-old man. Contrast-enhanced CT scan shows a left-sided chest wall mass with an osteoid matrix (arrow), a finding characteristic of chest wall osteosarcoma.
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Primary osteosarcomas are usually treated with preoperative chemotherapy followed by resection. Postoperative chemotherapy is also used if the tumor exhibits a response to preoperative therapy. Radiation therapy is occasionally used for local control if adequate resection cannot be performed. Survival is poor, with a 5-year survival rate of about 15% compared with 60%–70% in extremity osteosarcomas treated with resection and chemotherapy. Metastases develop in about 70% of patients and are associated with a poor prognosis (0% 5-year survival). Patients who do not develop metastases have a 5-year survival of over 50% (18). Resection of synchronous and delayed pulmonary metastases results in improved survival (40).
Synovial Sarcoma
Primary thoracic synovial sarcomas typically occur as chest wall masses, although they can (rarely) arise in the lung and pleura (Fig 13). Because they arise from bursae and tendon sheaths, they typically manifest as masses in the soft tissues in a juxtaarticular location. They usually occur in adults, and the mean age at diagnosis is 38 years (41). Patients with chest wall synovial sarcomas typically present with chest pain; cough and dyspnea can occur with lung masses.
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Figure 13a. Pulmonary synovial sarcoma in a 63-year-old man with pneumonia. (a) Posteroanterior chest radiograph shows a mass in the right cardiophrenic angle (arrows) and heterogeneous areas of increased opacity in the middle and right lower lobes, findings consistent with pneumonia. (b) Contrast-enhanced CT scan shows that the mass is heterogeneous. Areas of low attenuation within the mass are consistent with extensive necrosis. Note the small right pleural effusion.
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Figure 13b. Pulmonary synovial sarcoma in a 63-year-old man with pneumonia. (a) Posteroanterior chest radiograph shows a mass in the right cardiophrenic angle (arrows) and heterogeneous areas of increased opacity in the middle and right lower lobes, findings consistent with pneumonia. (b) Contrast-enhanced CT scan shows that the mass is heterogeneous. Areas of low attenuation within the mass are consistent with extensive necrosis. Note the small right pleural effusion.
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At radiologic analysis, synovial sarcomas typically manifest as heterogeneous masses that occasionally contain calcium (42). At MR imaging, these tumors demonstrate heterogeneous signal intensity on T1- and T2-weighted images and may have fluid-fluid levels due to hemorrhage and necrosis within cystic components of the tumor (43).
Treatment for peripheral lesions is excision and radiation therapy. The 5-year survival in these patients is approximately 50% (15). There are few data about survival in pulmonary synovial sarcomas. Metastases are common with tumors greater than 10 cm in diameter.
Fibrosarcoma
Fibrosarcomas arise in the chest wall, mediastinum, heart, and lungs. In the thorax, fibrosarcomas usually occur in the soft tissues of the chest wall (Fig 14) in adults, although the age range of presentation is wide. Although originally thought to be one of the more common pulmonary sarcomas, they are now considered uncommon in this location on the basis of immunohistochemical and ultrastructural analysis (6). Intrathoracic fibrosarcomas tend to occur as endobronchial masses in the main or lobar bronchi in children and young adults; in the lung, they tend to occur as solitary or multiple nodules or masses in adults (7). The endobronchial lesions usually manifest as cough, hemoptysis, or chest pain, whereas the pulmonary nodules typically are asymptomatic and are usually detected incidentally. The left atrium is the most common cardiac location, and patients typically present with congestive heart failure (5).
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Figure 14. Fibrosarcoma of the breast in a 55-year-old woman. Contrast-enhanced CT scan shows a focal, well-circumscribed mass in the left breast (arrow). The central area of low attenuation is suggestive of necrosis; the presence of necrosis was confirmed at resection.
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At radiologic analysis, fibrosarcomas in the chest wall and heart usually manifest as masses that are often heterogeneous in attenuation and signal intensity on CT and MR images, respectively, due to internal necrosis and hemorrhage (5). In the lung, fibrosarcomas manifest as well-marginated, smooth or lobular nodules or masses. At CT, these lesions are often nonspecific homogeneous nodules without calcification or ossification (7). Endobronchial tumors can manifest as atelectasis or postobstructive pneumonitis.
The endobronchial lesions are treated with local excision, and long-term survival is common. Treatment of chest wall and mediastinal lesions includes resection, with preoperative chemotherapy used to improve resectability. Postoperative radiation therapy is used if surgical margins are positive or if complete resection is not possible (15). The response to therapy is often poor, with larger masses tending to recur locally whereas smaller lesions have a high likelihood of metastases (7).
Neurofibrosarcoma
Thoracic neurofibrosarcomas (also called malignant peripheral nerve sheath tumors or malignant schwannomas) usually arise in preexisting neurofibromas of the intercostal nerves or spinal nerve roots or in the brachial plexus (Fig 15). Occasionally, neurofibrosarcomas have been associated with radiation therapy. About half of these tumors are associated with type 1 neurofibromatosis and have been reported to occur in up to 29% of these patients (44). The tumors mostly occur in adults and present as an enlarging, painful mass.
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Figure 15a. Neurofibrosarcoma in a 33-year-old woman with type 1 neurofibromatosis. (a) Nonenhanced CT scan shows a large, homogeneous mass in the left axilla (arrows). Note the absence of rib destruction. (b, c) Coronal T1-weighted (b) and fat-saturated T2-weighted (c) MR images show that the mass (M) is well circumscribed with low signal intensity on the T1-weighted image (b) and high signal intensity on the T2-weighted image (c). Note the small neurofibroma with similar signal intensity in the lower lateral aspect of the left hemithorax (arrow). The patient presented with pain in the region of the left axillary mass; because the characteristic appearance of a neurofibroma on T2-weighted images (the target sign) was absent, biopsy was performed.
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Figure 15b. Neurofibrosarcoma in a 33-year-old woman with type 1 neurofibromatosis. (a) Nonenhanced CT scan shows a large, homogeneous mass in the left axilla (arrows). Note the absence of rib destruction. (b, c) Coronal T1-weighted (b) and fat-saturated T2-weighted (c) MR images show that the mass (M) is well circumscribed with low signal intensity on the T1-weighted image (b) and high signal intensity on the T2-weighted image (c). Note the small neurofibroma with similar signal intensity in the lower lateral aspect of the left hemithorax (arrow). The patient presented with pain in the region of the left axillary mass; because the characteristic appearance of a neurofibroma on T2-weighted images (the target sign) was absent, biopsy was performed.
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Figure 15c. Neurofibrosarcoma in a 33-year-old woman with type 1 neurofibromatosis. (a) Nonenhanced CT scan shows a large, homogeneous mass in the left axilla (arrows). Note the absence of rib destruction. (b, c) Coronal T1-weighted (b) and fat-saturated T2-weighted (c) MR images show that the mass (M) is well circumscribed with low signal intensity on the T1-weighted image (b) and high signal intensity on the T2-weighted image (c). Note the small neurofibroma with similar signal intensity in the lower lateral aspect of the left hemithorax (arrow). The patient presented with pain in the region of the left axillary mass; because the characteristic appearance of a neurofibroma on T2-weighted images (the target sign) was absent, biopsy was performed.
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Neurofibrosarcomas are usually of variable signal intensity on T1- and T2-weighted MR images. Imaging features suggestive of malignant degeneration of a benign neurogenic tumor to neurofibrosarcoma include a sudden increase in size or development of heterogeneous attenuation at CT due to internal necrosis and hemorrhage. The development of heterogeneous signal intensity on T2-weighted MR images with loss of the peripherally hyperintense and centrally hypointense appearance (target sign) that is characteristic of neurofibromas is indicative of malignant transformation (36,45).
Neurofibrosarcomas are treated with resection and radiation therapy, if possible. The overall 5-year survival rate is approximately 50% in patients with resectable tumors.
Undifferentiated or Spindle Cell Sarcomas
High-grade or poorly differentiated sarcomas are sarcomas that cannot be specifically classified histologically. These are termed undifferentiated sarcomas or spindle cell sarcomas.
At radiologic analysis, undifferentiated sarcomas tend to be large and can contain calcifications when there is a large component of myxoid matrix (15) (Fig 16). The masses typically have heterogeneous attenuation at CT and heterogeneous signal intensity on T1- and T2-weighted MR images.
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Figure 16a. Diaphragmatic undifferentiated sarcoma in a 48-year-old man. (a) Posteroanterior chest radiograph shows a left pleural effusion. Note the metastatic pulmonary nodule (short arrow) and the adenopathy in the aortopulmonary window (long arrow). (b) Contrast-enhanced CT scan shows a large, heterogeneous mass with focal punctate calcifications (arrows). The resected specimen had a large component of myxoid matrix, which can result in intratumoral calcification.
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Figure 16b. Diaphragmatic undifferentiated sarcoma in a 48-year-old man. (a) Posteroanterior chest radiograph shows a left pleural effusion. Note the metastatic pulmonary nodule (short arrow) and the adenopathy in the aortopulmonary window (long arrow). (b) Contrast-enhanced CT scan shows a large, heterogeneous mass with focal punctate calcifications (arrows). The resected specimen had a large component of myxoid matrix, which can result in intratumoral calcification.
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Treatment consists of resection and radiation therapy, usually with chemotherapy to treat micrometastatic disease that is presumed to be present. Chemotherapy can also be used preoperatively to facilitate resection of otherwise unresectable tumors. Although these tumors have a high stage based on their histologic features, the 5-year survival rate is approximately 50%. Adenopathy or metastases are poor prognostic indicators, with a 5-year survival rate of less than 20%.
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Conclusions
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Primary sarcomas of the thorax are rare and constitute a large group of tumors classified according to their histologic differentiation. Although they commonly manifest as large, heterogeneous masses, they have a wide spectrum of radiologic manifestations including solitary pulmonary nodules, central endobronchial masses, and intraluminal masses within the pulmonary arteries. The different histologic types of sarcomas are frequently indistinguishable at radiologic analysis. However, differences in clinical presentation, the location of the tumor, morphologic features such as calcification within the mass or rib involvement, and occasionally imaging characteristics can be useful in suggesting the appropriate diagnosis.
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125 - 138.
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P. Cascales, A. A Gomez-Caro, B. Ferri-Niguez, M. J. Roca, J. Torres, and P. Parrilla
Poorly Differentiated Primary Monophasic Synovial Sarcoma of the Lung
Asian Cardiovasc Thorac Ann,
December 1, 2006;
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[Abstract]
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A. A. Frazier, T. J. Franks, R. D. Pugatch, and J. R. Galvin
From the archives of the AFIP: Pleuropulmonary synovial sarcoma.
RadioGraphics,
May 1, 2006;
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Abstract
LEARNING OBJECTIVES FOR TEST...
