The Anterior Mediastinal Mass: X-ray Differential Diagnosis James E. Smith, MD, Harry C. Press, Jr., MD, and Oswald Warner, MD Washington, D.C.
Hodgkin's lymphoma? 2. Metastatic cervical carcinoma to the percardium? 3. Aneurysm of the aortic sinus of Valsalva? 4. Pleuropericardial cyst (mesothelial cyst)?
Differential Diagnosis The pencil-sharp lateral and tapering superior margin of the mass
AL-
Figure 1. AP (left) and lateral (right) views of the chest showing adjacent to the right cardiac shadow.
Clinical History A 40-year-old, well-nourished, well-developed, black female had had a radical hysterectomy and irradiation for carcinoma of the cervix. Some months later, she developed gastrointestinal (GI) tract symptoms and was admitted -for work-up. An admission chest x-ray revealed a mass adjacent to the right heart border. The patient's cardiac status was normal. Mediastinoscopy was performed and the diagnosis was obtained.
Requests for reprints should be addressed to Dr. Harry C. Press, Jr. Chairman, Department of Radiology, Howard University Hospital, Washington, D.C. 20060.
120
a
soft tissue
establishes its position in the radiographic "anterior" mediastinum1 (the anatomical "middle" mediastinum2 ). Obliteration of a portion of the cardiac silhouette by the mass indicates that it is arising from, attached to, or abutting the right cardiac border at, or just below, the level of the root of the great vessels.
mass
Radiographic Findings A single, 3 cm, soft tissue density mass having smooth, pencil-sharp margins is seen adjacent to the right cardiac shadow. Tapering (draping) of the superior margin of the mass is noted. The medial margin of the mass cannot be seen. A portion of the right cardiac silhouette adjacent to the mass has been obliterated (Figure 1). The mass does not contain fat, air, calcium, bone densities, or cavitary areas. Metallic clips are projected over the mass. The mass did not change in shape or configuration with recumbent or decubitus positioning.
What's Your Diagnosis? 1. Nodular sclerosing type
of
Nodular Sclerosing Hodgkin's Disease Mediastinal lymph node enlargement from lymphoma is one of the most common causes of mediastinal masses (24 percent of one group of masses, in one survey). The majority of these were Hodgkin's disease. Hodgkin's disease may present as a single, well-defined mass with a smooth contour. In fact, nodular sclerosing Hodgkin's not uncommonly presents in this way.3 Usually these lesions are multiple, lobulated, and less well d e fined. Nodular sclerosing Hodgkin's disease is seldom associated with hilar or paratracheal lymph node enlargement (in contradistinction to other varieties of Hodgkin's).3
JOURNAL OF THE NATIONAL MEDICAL
ASSOCIATION,
VOL. 69, NO. 2, 1977
However, anterior mediastinal involvement with Hodgkin's disease is usually located more superiorly and closer to the sternum than is this mass. In addition, the absence of radiographically obvious cervical lymph node enlargement (which is often associated with nodular sclerosing Hodgkin's) makes a diagnosis of this disease less likely.
Aneurysm of the Aortic Sinus of Valsalva Aneurysm of the aortic sinuses almost invariably involves either the posterior or right anterior sinus.3 Before rupturing (the former into the right atrium, the latter into the right ventricle) this aneurysm may form a mediastinal mass in the region of the root of the aorta. 3 When such an aneurysm extends downward, it may simulate the appearance described for pericardial diverticulum (pleuropericardial cyst).4 The absence of broad lobulated borders, calcification, or right heart failure (from pressure produced by the aneurysm) makes this an unlikely diagnosis. In addition, it would be highly unlikely for a surgeon to place clips on an aneurysm.
Pericardial Cyst (Pleuropericardial Cyst, Mesothelial Cyst, Pericardial "Diverticulum ") This is a congenital cyst of the pericardium that results from an aberration in the formation of the coelomic cavities. These cysts seldom communicate with the pericardial cavity. The great majority of these cysts are smooth in contour and round or oval on an AP radiographic projection. Lacking in our case is the "tear-drop" configuration on the lateral projection which is seen with a pericardial cyst. This is due to insertion of the cyst in the interlobar septum between the middle and lower lobes. Also, this mass is too high and too far posteriorly located since the most common location, by far, for a pericardial cyst is the right cardiophrenic angle,3 near the anterior costophrenic sulcus. In addition, because of their fluid content, pericardial cysts change shape when the body position is altered.
Metastatic Tumor to Pericardium from Cervix This is the correct diagnosis. No extension into adjacent lung or pleura
was seen on mediastinoscopy. Most often, distant metastases from the cervix find their way to para-aortic nodes, liver, or lung5 (although metastases to bone are more often detected clinically). However, one survey indicates that ten percent of carcinomas of the cervix metastasize to the pericardium, heart, or great vessels,6 and are found at autopsy. Malignancies of distant organs that may involve the pericardium, heart, or great vessels when they metastasize, include ovary (29 percent),6 breast (23 percent), pancreas (ten percent), and stomach (nine percent). These metastases are invariably associated with metastases elsewhere. Tumors that may involve the pericardium, heart, or great vessels by direct extension (esophagus, thymoma, and thyroid) or that involve the mediastinum as a usual or common phenomenon during the course of their natural histories (ie, leukemias, lymphomas, most varieties of Hodgkin's disease, and myeloma) are excluded. Also excluded is carcinoma of the lung, which not uncommonly spreads to the mediastium hematogenously, by lymphatics, or by direct extension. Primary tumors of the pericardium which may resemble this mass include teratomas, teratocarcinomas, and hemangioendotheliom'as, all of which usually involve younger patients.7-10 Teratomas are usually symptomatic (respiratory distress, cardiac compromise, superior vena caval obstruction) due to compression of adjacent structures. 9, 1 0 Hemangioendotheliomas are always associated with pericardial effusions. 1 1 Pericardial mesotheliosarcomas are nearly always associated with pericardial effusion. 1 2-1 5
Other "Anterior" Mediastinal Tumors Thymomas and germinal cell tumors (dermoid cysts, malignant teratomas, seminomas, and choriocarci-
nomas) comprise 30 percent of all mediastinal tumors and are usually situated closer to the sterum near the origin of the great vessels.3 Seminomas and choriocarcinomas invariably occur in young males. Thymomas and teratomas may calcify. Mesenchymal tumors (lipomas, fibromas, hemangiomas, and lymphangiomas) are usually situated closer to the sternum. 3 Lymphangiomas are usually lower in the mediastinum. Hygroma (childhood lymphangioma) is usually more superi-
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 2, 1977
orly located and commonly extends into the neck. Thyroid masses are usually more anterior and superiorly situated. Lymph node hyperphasia usually presents as a solitary mass, situated more anteriorly in the mediastinum when it occurs there.3 Infectious mononucleosis rarely may present as a mediastinal mass and is more anteriorly situated in the mediastinum.3 Mediastinal masses from leukemias, non-Hodgkin's lymphomas, most varieties of Hodgkin's disease, granulomatous mediastinitis (tuberculosis, histoplasmosis, sarcoidosis, etc), and metastases (lung, upper GI tract, kidney) invariably are associated with paratracheal, tracheobronchial, or bronchopulmonary node involvement and limit the differential diagnosis to disease causing lymph node enlargement. A right phrenic nerve neurilemmoma probably could not be differentiated without biopsy (although lack of concomitant lesions in the posterior mediastinum might lead us away from this diagnosis, since they much more commonly occur there). Literature Cited 1. Felson B: Chest Roentgenology. Philadelphia, WB Saunders, 1973, p 419 2. Gray H: Gray's Anatomy, ed 283. Philadelphia, Lea and Febiger, 1973, p 1145 3. Fraser R, Pare J: Diagnosis of Diseases of the Chest, vol 2. Philadelphia, WB Saunders, 1970, pp 1180-1205 4. Meschan I: Analysis of Roentgen Signs in General Radiology, vol 2. Philadelphia, WB Saunders, 1973, pp 1127, 1129 5. Podworski H: Distant metastases of carcinoma of the cervix, uteri in the autopsy material of the Institute of Oncology in Gliwice. Patol Pol 23(1): 19-25, 1972 6. Laplume SO, Salvia C: Neoplasias Secundarias de Corazon, Pericardio Y Grandes Vasos Estudio Sobre 102 Casos" Dia Med 17:2196-2200, 1970 7. Robbins SL: Pathologic Basis of Disease. Philadelphia, WB Saunders, 1974, p 685 8. H ansen H E, S iogren M B, Westermark P: Malignant hemangioendothelioma of the pericardium: A case report. Scand J Respir Dis 51(3): 223-230, 1970 9. Pernot C: Primary tumors of the heart and intrapericardial neoplasms in six children. J Radiol Electrol Med Nucl 53(2):115-123, 1972 10. Zaorski JR, Evangalist FA, Sakakibara S, et al: Intrapericardial teratoma: A case report Milit Med 136:582-583, 1971 11. Coloignier JC, Leborgne P: Relapsing pericardial effusion in an infant due to benign hemangioendothelioma of the pericardium. Sem Hop Paris 47:2995-3003, 1971 12. Steinberg I: Angiocardiography in mesothelioma of the pericardium. Am J Roentgenol Radium Ther Nucl Med 114(4):
817-821, 1972
13. Dorra M, Berque A, Gouffier E, et al: Association of a malignant mesothelioma of the pericardium and of cardiac amyloidosis. Nouv Presse Med 1: 1972 14. Bjork L, Culhed I, Hallen A: Pericardial mesothelioma presented as constrictive pericarditis. Scand J Thorac Cardiovasc Surg 2(3):227-232, 1968 1 5. Norayanon PS, Chandrasekar 5, Mahavan M: I ntrathoracic mesotheliomas associated with tuberculosis. Indian J Med Sci 26(7): 432-436, 1972
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Hodgkin's lymphoma? 2. Metastatic cervical carcinoma to the percardium? 3. Aneurysm of the aortic sinus of Valsalva? 4. Pleuropericardial cyst (mesothelial cyst)?
Differential Diagnosis The pencil-sharp lateral and tapering superior margin of the mass
AL-
Figure 1. AP (left) and lateral (right) views of the chest showing adjacent to the right cardiac shadow.
Clinical History A 40-year-old, well-nourished, well-developed, black female had had a radical hysterectomy and irradiation for carcinoma of the cervix. Some months later, she developed gastrointestinal (GI) tract symptoms and was admitted -for work-up. An admission chest x-ray revealed a mass adjacent to the right heart border. The patient's cardiac status was normal. Mediastinoscopy was performed and the diagnosis was obtained.
Requests for reprints should be addressed to Dr. Harry C. Press, Jr. Chairman, Department of Radiology, Howard University Hospital, Washington, D.C. 20060.
120
a
soft tissue
establishes its position in the radiographic "anterior" mediastinum1 (the anatomical "middle" mediastinum2 ). Obliteration of a portion of the cardiac silhouette by the mass indicates that it is arising from, attached to, or abutting the right cardiac border at, or just below, the level of the root of the great vessels.
mass
Radiographic Findings A single, 3 cm, soft tissue density mass having smooth, pencil-sharp margins is seen adjacent to the right cardiac shadow. Tapering (draping) of the superior margin of the mass is noted. The medial margin of the mass cannot be seen. A portion of the right cardiac silhouette adjacent to the mass has been obliterated (Figure 1). The mass does not contain fat, air, calcium, bone densities, or cavitary areas. Metallic clips are projected over the mass. The mass did not change in shape or configuration with recumbent or decubitus positioning.
What's Your Diagnosis? 1. Nodular sclerosing type
of
Nodular Sclerosing Hodgkin's Disease Mediastinal lymph node enlargement from lymphoma is one of the most common causes of mediastinal masses (24 percent of one group of masses, in one survey). The majority of these were Hodgkin's disease. Hodgkin's disease may present as a single, well-defined mass with a smooth contour. In fact, nodular sclerosing Hodgkin's not uncommonly presents in this way.3 Usually these lesions are multiple, lobulated, and less well d e fined. Nodular sclerosing Hodgkin's disease is seldom associated with hilar or paratracheal lymph node enlargement (in contradistinction to other varieties of Hodgkin's).3
JOURNAL OF THE NATIONAL MEDICAL
ASSOCIATION,
VOL. 69, NO. 2, 1977
However, anterior mediastinal involvement with Hodgkin's disease is usually located more superiorly and closer to the sternum than is this mass. In addition, the absence of radiographically obvious cervical lymph node enlargement (which is often associated with nodular sclerosing Hodgkin's) makes a diagnosis of this disease less likely.
Aneurysm of the Aortic Sinus of Valsalva Aneurysm of the aortic sinuses almost invariably involves either the posterior or right anterior sinus.3 Before rupturing (the former into the right atrium, the latter into the right ventricle) this aneurysm may form a mediastinal mass in the region of the root of the aorta. 3 When such an aneurysm extends downward, it may simulate the appearance described for pericardial diverticulum (pleuropericardial cyst).4 The absence of broad lobulated borders, calcification, or right heart failure (from pressure produced by the aneurysm) makes this an unlikely diagnosis. In addition, it would be highly unlikely for a surgeon to place clips on an aneurysm.
Pericardial Cyst (Pleuropericardial Cyst, Mesothelial Cyst, Pericardial "Diverticulum ") This is a congenital cyst of the pericardium that results from an aberration in the formation of the coelomic cavities. These cysts seldom communicate with the pericardial cavity. The great majority of these cysts are smooth in contour and round or oval on an AP radiographic projection. Lacking in our case is the "tear-drop" configuration on the lateral projection which is seen with a pericardial cyst. This is due to insertion of the cyst in the interlobar septum between the middle and lower lobes. Also, this mass is too high and too far posteriorly located since the most common location, by far, for a pericardial cyst is the right cardiophrenic angle,3 near the anterior costophrenic sulcus. In addition, because of their fluid content, pericardial cysts change shape when the body position is altered.
Metastatic Tumor to Pericardium from Cervix This is the correct diagnosis. No extension into adjacent lung or pleura
was seen on mediastinoscopy. Most often, distant metastases from the cervix find their way to para-aortic nodes, liver, or lung5 (although metastases to bone are more often detected clinically). However, one survey indicates that ten percent of carcinomas of the cervix metastasize to the pericardium, heart, or great vessels,6 and are found at autopsy. Malignancies of distant organs that may involve the pericardium, heart, or great vessels when they metastasize, include ovary (29 percent),6 breast (23 percent), pancreas (ten percent), and stomach (nine percent). These metastases are invariably associated with metastases elsewhere. Tumors that may involve the pericardium, heart, or great vessels by direct extension (esophagus, thymoma, and thyroid) or that involve the mediastinum as a usual or common phenomenon during the course of their natural histories (ie, leukemias, lymphomas, most varieties of Hodgkin's disease, and myeloma) are excluded. Also excluded is carcinoma of the lung, which not uncommonly spreads to the mediastium hematogenously, by lymphatics, or by direct extension. Primary tumors of the pericardium which may resemble this mass include teratomas, teratocarcinomas, and hemangioendotheliom'as, all of which usually involve younger patients.7-10 Teratomas are usually symptomatic (respiratory distress, cardiac compromise, superior vena caval obstruction) due to compression of adjacent structures. 9, 1 0 Hemangioendotheliomas are always associated with pericardial effusions. 1 1 Pericardial mesotheliosarcomas are nearly always associated with pericardial effusion. 1 2-1 5
Other "Anterior" Mediastinal Tumors Thymomas and germinal cell tumors (dermoid cysts, malignant teratomas, seminomas, and choriocarci-
nomas) comprise 30 percent of all mediastinal tumors and are usually situated closer to the sterum near the origin of the great vessels.3 Seminomas and choriocarcinomas invariably occur in young males. Thymomas and teratomas may calcify. Mesenchymal tumors (lipomas, fibromas, hemangiomas, and lymphangiomas) are usually situated closer to the sternum. 3 Lymphangiomas are usually lower in the mediastinum. Hygroma (childhood lymphangioma) is usually more superi-
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 2, 1977
orly located and commonly extends into the neck. Thyroid masses are usually more anterior and superiorly situated. Lymph node hyperphasia usually presents as a solitary mass, situated more anteriorly in the mediastinum when it occurs there.3 Infectious mononucleosis rarely may present as a mediastinal mass and is more anteriorly situated in the mediastinum.3 Mediastinal masses from leukemias, non-Hodgkin's lymphomas, most varieties of Hodgkin's disease, granulomatous mediastinitis (tuberculosis, histoplasmosis, sarcoidosis, etc), and metastases (lung, upper GI tract, kidney) invariably are associated with paratracheal, tracheobronchial, or bronchopulmonary node involvement and limit the differential diagnosis to disease causing lymph node enlargement. A right phrenic nerve neurilemmoma probably could not be differentiated without biopsy (although lack of concomitant lesions in the posterior mediastinum might lead us away from this diagnosis, since they much more commonly occur there). Literature Cited 1. Felson B: Chest Roentgenology. Philadelphia, WB Saunders, 1973, p 419 2. Gray H: Gray's Anatomy, ed 283. Philadelphia, Lea and Febiger, 1973, p 1145 3. Fraser R, Pare J: Diagnosis of Diseases of the Chest, vol 2. Philadelphia, WB Saunders, 1970, pp 1180-1205 4. Meschan I: Analysis of Roentgen Signs in General Radiology, vol 2. Philadelphia, WB Saunders, 1973, pp 1127, 1129 5. Podworski H: Distant metastases of carcinoma of the cervix, uteri in the autopsy material of the Institute of Oncology in Gliwice. Patol Pol 23(1): 19-25, 1972 6. Laplume SO, Salvia C: Neoplasias Secundarias de Corazon, Pericardio Y Grandes Vasos Estudio Sobre 102 Casos" Dia Med 17:2196-2200, 1970 7. Robbins SL: Pathologic Basis of Disease. Philadelphia, WB Saunders, 1974, p 685 8. H ansen H E, S iogren M B, Westermark P: Malignant hemangioendothelioma of the pericardium: A case report. Scand J Respir Dis 51(3): 223-230, 1970 9. Pernot C: Primary tumors of the heart and intrapericardial neoplasms in six children. J Radiol Electrol Med Nucl 53(2):115-123, 1972 10. Zaorski JR, Evangalist FA, Sakakibara S, et al: Intrapericardial teratoma: A case report Milit Med 136:582-583, 1971 11. Coloignier JC, Leborgne P: Relapsing pericardial effusion in an infant due to benign hemangioendothelioma of the pericardium. Sem Hop Paris 47:2995-3003, 1971 12. Steinberg I: Angiocardiography in mesothelioma of the pericardium. Am J Roentgenol Radium Ther Nucl Med 114(4):
817-821, 1972
13. Dorra M, Berque A, Gouffier E, et al: Association of a malignant mesothelioma of the pericardium and of cardiac amyloidosis. Nouv Presse Med 1: 1972 14. Bjork L, Culhed I, Hallen A: Pericardial mesothelioma presented as constrictive pericarditis. Scand J Thorac Cardiovasc Surg 2(3):227-232, 1968 1 5. Norayanon PS, Chandrasekar 5, Mahavan M: I ntrathoracic mesotheliomas associated with tuberculosis. Indian J Med Sci 26(7): 432-436, 1972
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