Vol. 68, No. 3
243
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ADEMAKINWA M. ADEKUNLE, M.D., LUTHER B. ADAIR, M.D., Instructor in Radiology, Resident in Radiology, GERARD VERLY, M.D., HARRY C. PRESS, JR., M.D., Associate Professor of Pathology, Chairman & Associate Professor of Radiolo2v, Departments of Radiology and Pathology, Howard University Hospital, Washington, D.C.
CLINICAL BACKGROUND
This was the first hospital admission for this 35 year old black engineer who complained of progressive dyspnea on exertion for five to six years before admission. The remainder of his past history and physical examination was non-contributory. Laboratory data obtained upon admission was normal. Admitting chest radiographs are shown below in figure 1 A and B. No old films were available. What is your diagnosis? 1) Bronchogenic cyst; 2) Bronchogenic carcinoma; 3) Metastatic tumor; 4) Neurogenic tumor; 5) Bronchopulmonary sequestration; 6) Hamartoma.
genital anomaly of budding and branching of the tracheoesophageal tree. It may develop in the mediastinum or pulmonary parenchyma. The cyst is usually thin walled, has epithelial lining in its wall and contains a mucoid material. When infected it may communicate
ROENTGENOGRAPHIC FINDINGS
On the postero-anterior film (Fig. 1 left) a well circumscribed homogeneous soft tissue density is seen through the cardiac silhouette along the left paraspinal line. There is no air-fluid level or obvious calcification in the mass. There is no extrapleural sign indicating that it is not in the mediastinum. The lateral margin of this density extends beyond the left cardiac border and there is an absence of the silhouette sign which suggests that the lesion is not in juxta-position to the heart. The lateral (Fig. 1 right) confirms the posterior location of the mass. No bone erosion is noted. Except for some hilar calcification the chest radiograph is otherwise normal. Tomograms were not performed. DIFFERENTIAL DIAGNOSIS
Bronchogenic Cyst. A bronchogenic cyst is a con-
Fig. 1. A-P view of chest showing well circumscribed tissue density along the left paraspinal line (left). Lateral view (right). with the tracheobronchial tree and hence contain air with or without fluid. In the absence of earlier radiographs it is impossible in this case to exclude bronchogenic cyst. Bronchogenic Carcinoma. This disease is rare below the age of 35 but does occur. The first roentgenographic sign of bronchogenic carcinoma is not the lesion itself but the consequences of obstruction of the airway in which the lesion is situated. The most common sign is atelectasis which is usually segmental but may be lobar or even involve a whole lung. Since no air passes through the obstruction, airbronchograms are usually absent in atelectasis caused by endobronchial lesions. The fact that this lesion is
244
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION
well circumscribed without any evidence of obstructive pathology in the surrounding lung parenchyma makes it unlikely to be a carcinoma. In addition, the patient's age and absence of hilar lymph node enlargement make it less so. Metastatic Tumor. Hematogenous pulmonary metastases are usually multiple. They may be uniform in size or vary widely. However, sometimes metastatic tumors may be solitary and large in size. These may arise from any organ, however, the more likely primary sites will include colon, kidney, testicle, breast, or malignant melanoma and bone sarcoma. Sometimes a solitary metastasis from osteogenic sarcoma or chondrosarcoma will undergo calcification or ossification. From this patient's general work-up no primary neoplasm was found. Neurogenic Tumor. Neurofibroma (neurilemmoma) and ganglioneuroma are the two commonest neurogenic tumors found in the chest. Neurogenic tumors are exceedingly rare in.the lungs although not uncommon in the mediastinum. When neurofibroma occurs in the lung periphery, it is usually asymptomatic. It may reach a large size before being discovered. If calcification is present within the tumor, it is likely benign but if there is no calcification malig-
MAY, 1976
Generally a neurofibroma is a round mass while a ganglioneuroma is elongated with the vertical diameter greater than the transverse diameter. In this case, except for the absence of bony involvement, a neurofibroma would have been difficult to exclude. Bronchopulmonary Sequestration. This is a congenital pulmonary malformation in which a portion of the lung is detached from the rest of the normal lung tissue and receives its blood supply from a separate systemic artery. Almost invariably it has a predilection for the lower lobes especially the posterior basal segment in close proximity to the diaphragm. When uncomplicated, it is a homogeneous mass of fluid density, sharply circumscribed. The location of this lesion makes it very unlikely to be a bronchopulmonary sequestration.
Fig. 3. Photomicrograph of the hamartoma showing islands of cartilage, epithelial lines spaces and adipose tissue, left. H.&E. X10. Higher power view of the hamartoma, right.
Fig. 2. Line drawing illustrating a neurofibroma, left and
a
ganglioneuroma, right.
nancy cannot be excluded. Neurofibromas are found most commonly in cases of Von Recklinghausen's disease where the tumors are multiple. Some of them occur in the region of the spinal foramen and extend into the spinal canal, producing the so-called dumbbell type of tumor. This often results in pressure erosions on the vertebral body and pedicles. Oblique views and tomograms are sometimes necessary to rule out bony involvement. There are a few roentgenographic changes which aid in differentiating these two neurogenic tumors. Neurofibroma tends to have a narrow mediastinal base as seen on frontal projection, whereas ganglioneuroma has a broad mediastinal base. The angle between the tumor and the mediastinum tends to be acute in neurofibroma but obtuse in ganglioneuroma. Figure 2 is a line drawing illustrating the radiographic findings of neurofibroma and ganglioneuroma, left and right, respectively.
H.&E. X40. Hamartoma. Roentgenographically, a hamartoma usually appears as a sharply circumscribed and well demarcated pulmonary parenchymal nodule without surrounding infiltration. The frequency in hamartomas occurring in calcification, has a broad range, between 3% to 75% of cases. 16 When calcification is present it is usually scattered throughout the lesion, a pattern which had been described as "pop-corn" calcification. Calcification can be best seen by laminograms. However, calcification is not pathognomonic of hamartomas as it may occur in histoplasmosis, coccidiodomycosis and other granulomatous lesions. Metastatic osteogenic sarcoma, cystadeno-carcinoma of the ovary, hematomas and adenomas may present with calcifications.
HOSPITAL COURSE The patient had a thoracotomy with resection of the left lower lobe. The post-operative course was uneventful. Pathologic Findings. A segment of lung submitted as left lower lobe, measured 12.0 x 15.0 x 6.6 cm. and weighed 441 gms. The pleural surfaces appeared
Vol. 68, No. 3
Roentgenogram
smooth and glistening. Cut sections revealed a well demarcated, firm, nodular mass, consisting of cartilaginous tissue, measuring 5.0 cm. in greatest dimension. Microscopic examination revealed a well circumscribed growth consisting of irregular islands of cartilage, separated by cleft-like spaces (Fig. 3 left). The cartilaginous tissue was often irregularly circular, enclosing compressed spaces lined by a single layer of cuboidal, occasionally ciliated cells. In areas, papillary formations composed of connective tissue covered by flattened cells, projected into the epithelium-lined spaces. These spaces were separated from the cartilage by varying amounts of adipose tissue, with slight infiltrates of lymphocytes (Fig. 3 right). The surrounding lung parenchyma showed a mild degree of atelectasis. A diagnosis of pulmonary hamartoma was made. DISCUSSION
Hamartomas were first described in 1904 by Albrecht as tumor-like malformations composed of an abnormal mixture of normal constituents of the organ in which they are found.3', Less than 1% of all tumors of the lungs are benign.3 Hamartomas are by far the commonest of them, accounting for about 8-10% on the average. Jones and Cleve reviewed the series of 12 separate reports on isolated circumscribed lesions of the lung. Of the 714 total number of cases, 57 (8%) were hamartomas.5 Davis et al. reviewed more than 1200 solitary circumscribed nodules less than 6 cm in diameter and found 8.5% were hamartomas.5 The incidence of hamartomas in the general population is 0.25%3 Multiple hamartomas are rare. They are slowly grow-
245
ing lesions and their importance is in their differentiation from a malignant tumor. The average age recorded in most large series is 45-50 years but the age span is from birth to 76 years.5 Males are affected 2 to 4 times more frequently than females.6 No significant predilection for a given lobe has been noted.7 Although hamartomas are usually asymptomatic, as they enlarge they cause bronchial obstruction with subsequent atelectasis and pneumonitis. Endobronchial hamartomas account for 3 to 4% of pulmonary hamartomas.f Indobronchial hamartomas, of course, may present as obstructive lesions causing distal atelectasis indistinguishable from that produced by bronchogenic carcinoma. LITERATURE CITED
1. WEINBERGE, M. et al. The Adult Form of Pulmonary Hamartoma. Ann. Thoracic Surg., 15:6772, 1973. 2. LESTER, W. P. and H. J. JOHN. The Essentials of Roentgen Interpretation. Harper and Row Publishers, 1972. 3. SHAH, J. P., et al. Hamartomas of the Lung. Surg. Gyn. Obst., 136:406-408, 1973. 4. FRASER, R. G. and J. A. P. PARE. Diagnosis of Diseases of the Chest. W. B. Saunders, Philadelphia, 1970. 5. POIRER, T. J., et al. Pulmonary Chondromatous Hamartomas. Report of Seventeen Cases and Review of Literature. Chest, 59:50-55, 1971. 6. KONTRAS, P. et al. Hamartoma of the Lung. J. Thoracic & Cardiovasc. Surg., 61:768-776, 1971. 7. D'ALTORIA, R. A. Case of the Fall Season. Seminars in Roentgenology. 10:253-254, 1975.
(Sullivan, from page 251) from disadvantaged backgrounds; 4. Initiate and enact, possibly under the Health Manpower Education Initiative Award Program, a new capability for providing institutional aid to those medical schools which demonstrate the capacity and ability to respond to the national need to train more black and other ethnic minority students. These institutions should be recognized as national resources.
This Subcommittee has a responsibility to make a substantial contribution through legislative innovation. The National Conference on the Status of Health in the Black Community sponsored in 1971 by the National Medical Association, the Howard and Meharry Colleges of Medicine, and the Congressional Black Caucus, recommended that: "priority funding (should) be given to any new schools of the health sciences that have as their primary commitment focusing on and
increasing the supply of Black minority students." None of the legislative proposals in the bills before you are concerned with a commitment such as that which is specified above. For this reason, we call for legislative authorization of and appropriation for a new program which would provide such institutional support. I know that the members of this distinguished Subcommittee are concerned about this situation. I believe that you are wrestling honestly with this and other problems which will have a tremendous impact on the black people of this nation. I suggest to you that of all your concerns none should challenge you more than this. We stand ready to work with you in these efforts.
243
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.-..
.....
...
The~~~~~~~~~~~~~~~~~~...........Soitr
......
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ADEMAKINWA M. ADEKUNLE, M.D., LUTHER B. ADAIR, M.D., Instructor in Radiology, Resident in Radiology, GERARD VERLY, M.D., HARRY C. PRESS, JR., M.D., Associate Professor of Pathology, Chairman & Associate Professor of Radiolo2v, Departments of Radiology and Pathology, Howard University Hospital, Washington, D.C.
CLINICAL BACKGROUND
This was the first hospital admission for this 35 year old black engineer who complained of progressive dyspnea on exertion for five to six years before admission. The remainder of his past history and physical examination was non-contributory. Laboratory data obtained upon admission was normal. Admitting chest radiographs are shown below in figure 1 A and B. No old films were available. What is your diagnosis? 1) Bronchogenic cyst; 2) Bronchogenic carcinoma; 3) Metastatic tumor; 4) Neurogenic tumor; 5) Bronchopulmonary sequestration; 6) Hamartoma.
genital anomaly of budding and branching of the tracheoesophageal tree. It may develop in the mediastinum or pulmonary parenchyma. The cyst is usually thin walled, has epithelial lining in its wall and contains a mucoid material. When infected it may communicate
ROENTGENOGRAPHIC FINDINGS
On the postero-anterior film (Fig. 1 left) a well circumscribed homogeneous soft tissue density is seen through the cardiac silhouette along the left paraspinal line. There is no air-fluid level or obvious calcification in the mass. There is no extrapleural sign indicating that it is not in the mediastinum. The lateral margin of this density extends beyond the left cardiac border and there is an absence of the silhouette sign which suggests that the lesion is not in juxta-position to the heart. The lateral (Fig. 1 right) confirms the posterior location of the mass. No bone erosion is noted. Except for some hilar calcification the chest radiograph is otherwise normal. Tomograms were not performed. DIFFERENTIAL DIAGNOSIS
Bronchogenic Cyst. A bronchogenic cyst is a con-
Fig. 1. A-P view of chest showing well circumscribed tissue density along the left paraspinal line (left). Lateral view (right). with the tracheobronchial tree and hence contain air with or without fluid. In the absence of earlier radiographs it is impossible in this case to exclude bronchogenic cyst. Bronchogenic Carcinoma. This disease is rare below the age of 35 but does occur. The first roentgenographic sign of bronchogenic carcinoma is not the lesion itself but the consequences of obstruction of the airway in which the lesion is situated. The most common sign is atelectasis which is usually segmental but may be lobar or even involve a whole lung. Since no air passes through the obstruction, airbronchograms are usually absent in atelectasis caused by endobronchial lesions. The fact that this lesion is
244
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION
well circumscribed without any evidence of obstructive pathology in the surrounding lung parenchyma makes it unlikely to be a carcinoma. In addition, the patient's age and absence of hilar lymph node enlargement make it less so. Metastatic Tumor. Hematogenous pulmonary metastases are usually multiple. They may be uniform in size or vary widely. However, sometimes metastatic tumors may be solitary and large in size. These may arise from any organ, however, the more likely primary sites will include colon, kidney, testicle, breast, or malignant melanoma and bone sarcoma. Sometimes a solitary metastasis from osteogenic sarcoma or chondrosarcoma will undergo calcification or ossification. From this patient's general work-up no primary neoplasm was found. Neurogenic Tumor. Neurofibroma (neurilemmoma) and ganglioneuroma are the two commonest neurogenic tumors found in the chest. Neurogenic tumors are exceedingly rare in.the lungs although not uncommon in the mediastinum. When neurofibroma occurs in the lung periphery, it is usually asymptomatic. It may reach a large size before being discovered. If calcification is present within the tumor, it is likely benign but if there is no calcification malig-
MAY, 1976
Generally a neurofibroma is a round mass while a ganglioneuroma is elongated with the vertical diameter greater than the transverse diameter. In this case, except for the absence of bony involvement, a neurofibroma would have been difficult to exclude. Bronchopulmonary Sequestration. This is a congenital pulmonary malformation in which a portion of the lung is detached from the rest of the normal lung tissue and receives its blood supply from a separate systemic artery. Almost invariably it has a predilection for the lower lobes especially the posterior basal segment in close proximity to the diaphragm. When uncomplicated, it is a homogeneous mass of fluid density, sharply circumscribed. The location of this lesion makes it very unlikely to be a bronchopulmonary sequestration.
Fig. 3. Photomicrograph of the hamartoma showing islands of cartilage, epithelial lines spaces and adipose tissue, left. H.&E. X10. Higher power view of the hamartoma, right.
Fig. 2. Line drawing illustrating a neurofibroma, left and
a
ganglioneuroma, right.
nancy cannot be excluded. Neurofibromas are found most commonly in cases of Von Recklinghausen's disease where the tumors are multiple. Some of them occur in the region of the spinal foramen and extend into the spinal canal, producing the so-called dumbbell type of tumor. This often results in pressure erosions on the vertebral body and pedicles. Oblique views and tomograms are sometimes necessary to rule out bony involvement. There are a few roentgenographic changes which aid in differentiating these two neurogenic tumors. Neurofibroma tends to have a narrow mediastinal base as seen on frontal projection, whereas ganglioneuroma has a broad mediastinal base. The angle between the tumor and the mediastinum tends to be acute in neurofibroma but obtuse in ganglioneuroma. Figure 2 is a line drawing illustrating the radiographic findings of neurofibroma and ganglioneuroma, left and right, respectively.
H.&E. X40. Hamartoma. Roentgenographically, a hamartoma usually appears as a sharply circumscribed and well demarcated pulmonary parenchymal nodule without surrounding infiltration. The frequency in hamartomas occurring in calcification, has a broad range, between 3% to 75% of cases. 16 When calcification is present it is usually scattered throughout the lesion, a pattern which had been described as "pop-corn" calcification. Calcification can be best seen by laminograms. However, calcification is not pathognomonic of hamartomas as it may occur in histoplasmosis, coccidiodomycosis and other granulomatous lesions. Metastatic osteogenic sarcoma, cystadeno-carcinoma of the ovary, hematomas and adenomas may present with calcifications.
HOSPITAL COURSE The patient had a thoracotomy with resection of the left lower lobe. The post-operative course was uneventful. Pathologic Findings. A segment of lung submitted as left lower lobe, measured 12.0 x 15.0 x 6.6 cm. and weighed 441 gms. The pleural surfaces appeared
Vol. 68, No. 3
Roentgenogram
smooth and glistening. Cut sections revealed a well demarcated, firm, nodular mass, consisting of cartilaginous tissue, measuring 5.0 cm. in greatest dimension. Microscopic examination revealed a well circumscribed growth consisting of irregular islands of cartilage, separated by cleft-like spaces (Fig. 3 left). The cartilaginous tissue was often irregularly circular, enclosing compressed spaces lined by a single layer of cuboidal, occasionally ciliated cells. In areas, papillary formations composed of connective tissue covered by flattened cells, projected into the epithelium-lined spaces. These spaces were separated from the cartilage by varying amounts of adipose tissue, with slight infiltrates of lymphocytes (Fig. 3 right). The surrounding lung parenchyma showed a mild degree of atelectasis. A diagnosis of pulmonary hamartoma was made. DISCUSSION
Hamartomas were first described in 1904 by Albrecht as tumor-like malformations composed of an abnormal mixture of normal constituents of the organ in which they are found.3', Less than 1% of all tumors of the lungs are benign.3 Hamartomas are by far the commonest of them, accounting for about 8-10% on the average. Jones and Cleve reviewed the series of 12 separate reports on isolated circumscribed lesions of the lung. Of the 714 total number of cases, 57 (8%) were hamartomas.5 Davis et al. reviewed more than 1200 solitary circumscribed nodules less than 6 cm in diameter and found 8.5% were hamartomas.5 The incidence of hamartomas in the general population is 0.25%3 Multiple hamartomas are rare. They are slowly grow-
245
ing lesions and their importance is in their differentiation from a malignant tumor. The average age recorded in most large series is 45-50 years but the age span is from birth to 76 years.5 Males are affected 2 to 4 times more frequently than females.6 No significant predilection for a given lobe has been noted.7 Although hamartomas are usually asymptomatic, as they enlarge they cause bronchial obstruction with subsequent atelectasis and pneumonitis. Endobronchial hamartomas account for 3 to 4% of pulmonary hamartomas.f Indobronchial hamartomas, of course, may present as obstructive lesions causing distal atelectasis indistinguishable from that produced by bronchogenic carcinoma. LITERATURE CITED
1. WEINBERGE, M. et al. The Adult Form of Pulmonary Hamartoma. Ann. Thoracic Surg., 15:6772, 1973. 2. LESTER, W. P. and H. J. JOHN. The Essentials of Roentgen Interpretation. Harper and Row Publishers, 1972. 3. SHAH, J. P., et al. Hamartomas of the Lung. Surg. Gyn. Obst., 136:406-408, 1973. 4. FRASER, R. G. and J. A. P. PARE. Diagnosis of Diseases of the Chest. W. B. Saunders, Philadelphia, 1970. 5. POIRER, T. J., et al. Pulmonary Chondromatous Hamartomas. Report of Seventeen Cases and Review of Literature. Chest, 59:50-55, 1971. 6. KONTRAS, P. et al. Hamartoma of the Lung. J. Thoracic & Cardiovasc. Surg., 61:768-776, 1971. 7. D'ALTORIA, R. A. Case of the Fall Season. Seminars in Roentgenology. 10:253-254, 1975.
(Sullivan, from page 251) from disadvantaged backgrounds; 4. Initiate and enact, possibly under the Health Manpower Education Initiative Award Program, a new capability for providing institutional aid to those medical schools which demonstrate the capacity and ability to respond to the national need to train more black and other ethnic minority students. These institutions should be recognized as national resources.
This Subcommittee has a responsibility to make a substantial contribution through legislative innovation. The National Conference on the Status of Health in the Black Community sponsored in 1971 by the National Medical Association, the Howard and Meharry Colleges of Medicine, and the Congressional Black Caucus, recommended that: "priority funding (should) be given to any new schools of the health sciences that have as their primary commitment focusing on and
increasing the supply of Black minority students." None of the legislative proposals in the bills before you are concerned with a commitment such as that which is specified above. For this reason, we call for legislative authorization of and appropriation for a new program which would provide such institutional support. I know that the members of this distinguished Subcommittee are concerned about this situation. I believe that you are wrestling honestly with this and other problems which will have a tremendous impact on the black people of this nation. I suggest to you that of all your concerns none should challenge you more than this. We stand ready to work with you in these efforts.
