Pulmonary Blastoma: Report of a Case By J. Cantillana Martinez, F. Chaves Pecero, C. Guti6rrez de la Pena, R. Martin Gomez, J. A. Guti(~rrez del Manzano, T. Flores Corral, and J. Loscertales Abril 9 This is a c a s e report of a p u l m o n a r y b l a s t o m a occurring in a 2 - y r - o l d girl. INDEX
WORDS:
Pulmonary blastoma.
As a result of its close histologic resemblance to fetal lung tissue, this tumor was originally called fetal embryorna by Barnard. ~ It is now universally called "pulmonary blastoma" following the proposal of Spencer, 2 who used this n a m e b e c a u s e o f its similarity to n e p h r o b l a s t o m a . Pulmonary blastoma is considered to be a primary lung tumor of doubtful origin, capable of producing metastases. On the basis of the 1973 review by Iverson et al. 3 in which 19 cases were reported, we think that at present no more than 25 cases have been reported, demonstrating the extreme rarity of the tumor. This is the cause of the difficulties in establishing a definitive histopathologic classification.4 6
CASE REPORT
dull to percussion and had diminished movements. The apex beat was located on the right in the inframammillary region. Routine studies of the blood and urine were normal. Chest radiographs d e m o n s t r a t e d a shift of the mediastinum toward the right (Fig. 1). Two densely rounded shadows were observed in the left hemithorax, one situated paramediastinal and the other in the lower part of the thoracic wall. There was also a pleural effusion (Fig. 2). Barium studies of the gastrointestinal tract were normal. The lung scintigram showed normal perfusion of the right lung and diminished perfusion of the left. Angiographic study revealed images compatible with polycystic disease of the left lung. The diagnosis of a congenital lung cyst was made and a left thoracotomy was carried out. A multilobed cystic swelling was noticed in the left lung with a collapse of lung parenchyma beneath it. The cystic swelling was excised (Fig. 3). The left lung expanded again, without any sign of macroscopic changes. Culture of the pleural effusion was negative. Histologic study of the specimen revealed that the tumor was composed of glandlike structures separated by mesenchymal tissue (Fig. 4). These glandlike structures were lined by one
A 2-yr-old girl, who had been diagnosed a year previously as suffering from bronchopneumonia of the left lung, was admitted on September 6, 1976 because of an irritant cough that occurred especially at night. This cough was sometimes associated with vomiting and with slight hemoptysis. There was no hyperthermia and no signs of respiratory distress. The physical examinations revealed good get, era] condition with no signs of illness, good state of nutrition, and normal color of skin and mucosa, There was a swelling of the left hemithorax, which was
From the Department of Surgery, Hospital Clinico Universitario, Sevilla, Spain. Address reprint requests to J. Cantillana Martinez, Department of Surgery, Hospital Clinico Universitario, S evilla, Spain, 9 1978 by Grune & Stratton, Inc. 0022 3468/78/1301-0021501.00/0
Fig. 1. Thoracic roentgenogram showing a shift of the mediastinum toward the right.
Fig. 2. Thoracic roentgenogram effusion (patient lying fiat).
Journal of Pediatric Surgery, Vol. 13, No. 1 (February),1978
showing a pleural
93
94
CASE REPORT
Fig. 3.
Removed tissue,
Fig. 5. Irregular masses of immature cartilage surrounded by undifferentiated mesenchymal cells. H.E. x 5.
layer that consisted of normal cuboidal and columnar cells, occasionally ciliated. The mesenchymal tissue was composed of cells with round or oval nuclei and scanty cytoplasm. In some areas the mesenchymal tissue presented a fibrosarcomatous degeneration (atypical spindle cells); in other areas masses of immature and atypical cartilage surrounded by undifferentiated mesenchymal cells were observed (Fig. 5). Areas of very low density of cells (mixoid) were seen, as were zones that presented a differentiation toward mature or immature smooth muscle. It was considered that this was a primary malignant neoplasm of the lung with both epithelial and mesenchymal components. REFERENCES
Fig, 4. Glandlike or tubular structures separated by mesenchymal cells, Most of the glands are lined by one layer of cuboidal and columnar cells; ciliated cells are occasionally seen, Note the poor demarcation between glands and ruesenchymal tissue, H,E, • 14.
1. Barnard WG: Embryoma of lung. Thorax 7:299, 1952 2. Spencer H: Pulmonary Blastoma. J Pathol Bacteriol 82:161, 1961 3. Iverson RE, Strafhley C J: Pulmonary Blastoma: Long-term survival of juvenile patient. Chest 63:436, 1973 4. Parker JC Jr, Payne WS, Wooler LB: Pulmonary blastoma (embryoma): Report of 2 cases. J Thorac Cardiovasc Surg 51:694, 1966 5. N a z a r a A, A m e r - M o k r i E, S a r r a m A, et al: Pulmonary blastoma. Chest 60:187, 1971 6. Kodaira Y, Akiyama H, Morikawa M, et ah Pulmonary blastoma in a child. J Pediatr Surg 11:239, 1976
WORDS:
Pulmonary blastoma.
As a result of its close histologic resemblance to fetal lung tissue, this tumor was originally called fetal embryorna by Barnard. ~ It is now universally called "pulmonary blastoma" following the proposal of Spencer, 2 who used this n a m e b e c a u s e o f its similarity to n e p h r o b l a s t o m a . Pulmonary blastoma is considered to be a primary lung tumor of doubtful origin, capable of producing metastases. On the basis of the 1973 review by Iverson et al. 3 in which 19 cases were reported, we think that at present no more than 25 cases have been reported, demonstrating the extreme rarity of the tumor. This is the cause of the difficulties in establishing a definitive histopathologic classification.4 6
CASE REPORT
dull to percussion and had diminished movements. The apex beat was located on the right in the inframammillary region. Routine studies of the blood and urine were normal. Chest radiographs d e m o n s t r a t e d a shift of the mediastinum toward the right (Fig. 1). Two densely rounded shadows were observed in the left hemithorax, one situated paramediastinal and the other in the lower part of the thoracic wall. There was also a pleural effusion (Fig. 2). Barium studies of the gastrointestinal tract were normal. The lung scintigram showed normal perfusion of the right lung and diminished perfusion of the left. Angiographic study revealed images compatible with polycystic disease of the left lung. The diagnosis of a congenital lung cyst was made and a left thoracotomy was carried out. A multilobed cystic swelling was noticed in the left lung with a collapse of lung parenchyma beneath it. The cystic swelling was excised (Fig. 3). The left lung expanded again, without any sign of macroscopic changes. Culture of the pleural effusion was negative. Histologic study of the specimen revealed that the tumor was composed of glandlike structures separated by mesenchymal tissue (Fig. 4). These glandlike structures were lined by one
A 2-yr-old girl, who had been diagnosed a year previously as suffering from bronchopneumonia of the left lung, was admitted on September 6, 1976 because of an irritant cough that occurred especially at night. This cough was sometimes associated with vomiting and with slight hemoptysis. There was no hyperthermia and no signs of respiratory distress. The physical examinations revealed good get, era] condition with no signs of illness, good state of nutrition, and normal color of skin and mucosa, There was a swelling of the left hemithorax, which was
From the Department of Surgery, Hospital Clinico Universitario, Sevilla, Spain. Address reprint requests to J. Cantillana Martinez, Department of Surgery, Hospital Clinico Universitario, S evilla, Spain, 9 1978 by Grune & Stratton, Inc. 0022 3468/78/1301-0021501.00/0
Fig. 1. Thoracic roentgenogram showing a shift of the mediastinum toward the right.
Fig. 2. Thoracic roentgenogram effusion (patient lying fiat).
Journal of Pediatric Surgery, Vol. 13, No. 1 (February),1978
showing a pleural
93
94
CASE REPORT
Fig. 3.
Removed tissue,
Fig. 5. Irregular masses of immature cartilage surrounded by undifferentiated mesenchymal cells. H.E. x 5.
layer that consisted of normal cuboidal and columnar cells, occasionally ciliated. The mesenchymal tissue was composed of cells with round or oval nuclei and scanty cytoplasm. In some areas the mesenchymal tissue presented a fibrosarcomatous degeneration (atypical spindle cells); in other areas masses of immature and atypical cartilage surrounded by undifferentiated mesenchymal cells were observed (Fig. 5). Areas of very low density of cells (mixoid) were seen, as were zones that presented a differentiation toward mature or immature smooth muscle. It was considered that this was a primary malignant neoplasm of the lung with both epithelial and mesenchymal components. REFERENCES
Fig, 4. Glandlike or tubular structures separated by mesenchymal cells, Most of the glands are lined by one layer of cuboidal and columnar cells; ciliated cells are occasionally seen, Note the poor demarcation between glands and ruesenchymal tissue, H,E, • 14.
1. Barnard WG: Embryoma of lung. Thorax 7:299, 1952 2. Spencer H: Pulmonary Blastoma. J Pathol Bacteriol 82:161, 1961 3. Iverson RE, Strafhley C J: Pulmonary Blastoma: Long-term survival of juvenile patient. Chest 63:436, 1973 4. Parker JC Jr, Payne WS, Wooler LB: Pulmonary blastoma (embryoma): Report of 2 cases. J Thorac Cardiovasc Surg 51:694, 1966 5. N a z a r a A, A m e r - M o k r i E, S a r r a m A, et al: Pulmonary blastoma. Chest 60:187, 1971 6. Kodaira Y, Akiyama H, Morikawa M, et ah Pulmonary blastoma in a child. J Pediatr Surg 11:239, 1976
