Clin Investig (1992) 70:927-931
Glinical Investigator
C se Report with a Review of the Literature
© Springer-Verlag 1992
Pulmonary blastoma-
a rare tumour
V. Kliem 1, M. Biigge 2, K. Leimenstoll 3, and H. Maschek 4 Abteilung Nephrologie, Zentrum Innere Medizin und Dermatologie, Medizinische Hochschule Hannover 2 Innere und 3 Chirurgische Abteilung, Kreiskrankenhaus Rinteln 4 Pathologisches Institut, Zentrum Pathologie und Rechtsmedizin, Medizinische Hochschule Hannover
Summary. A 57-year-old man was found to have a tumour in the right lower lobe of the lungs, which was not classifiable by biopsy. The tumour could only be partially removed by surgical resection. The diagnosis of a pulmonary blastoma was made from the resected tissue. Clinically, rapid progress occured with invasion in the mediastinal space and the epigastrium. In spite of radiation therapy, the patient died about 21/2 months after surgery of respiratory insufficiency. Autopsy confirmed a pulmonary blastoma with extensive infiltration of the mediastinal space and upper abdomen as well as metastases in the regional lymph nodes, pleura, peritoneum, thyroid gland, heart and central nervous system. The present report of a pulmonary blastoma should draw attention to this extremely rare tumour. It should be included in the differential diagnosis, because the survival time can be increased if the correct diagnosis is made very early. Key words: Pulmonary blastoma
Malignant lung tumours - Epidemiology - Histogenesis - Diagnosis - Therapy
The pulmonary blastoma was described for the first time as an independent entity by Barret and Barnard in 1945 [3]. Because of its histological similarity to fetal lung tissue in the third gestational month [7], it was denoted as an embryoma [2]. In analogy to nephroblastoma, Spencer named the tumour pulmonary blastoma, because in his opinion the tumour developed from a pluripotential mesenchymal blastema [17]. There have only been 150 cases of a pulmonary blastoma reported to Abbreviations: AFP = alpha-fetoprotein; fl-HCG = beta-human chorionic gonadotropin; CA = cancer antigen; CEA = carcinoembryonic antigen; CHE=butyryl-cholinesterase; E S R = erythrocyte sedimentation rate; LDH =lactate dehydrogenase; NSE=neuron-specific enolase; TPA=tissue polypeptide antigen
the present day, including about 40 cases involving children [8, 16]. In more than 75% of these cases, the children are younger than 5 years old at the onset of the illness [16]. However, the pulmonary blastoma appears in every age group. The average age in adults is about 43 years. In men the tumour seems to develop somewhat later (49 years) and more often (2.6:1) than in women [6]. In contrast to nephroblastoma, late occurrences could be attributed to a different organogenesis - the lungs develop up to the 20th year of life [17]. Cases of a familiar or regional accumulation have not been reported.
Case report Anamnesis and findings A 57-year-old Caucasian man smoked about 25 cigarettes per day for more than 30 years. The patient had had nausea, loss of appetite, slight weight loss, pain in the epigastrium and in the thoracic and lumbal spinal column area as well as occasionally hemoptysis since May 1989. Chest roentgenography, gastroscopy, and abdominal sonography examinations were all without abnormalities. In late July 1989 at right basal lung tumour was suspected. The bronchoalveolar lavage yielded a strong granulomatous inflammation with cellular atypias (Papanicolaou's test III D), and in the pleural biopsy tumour tissue, which could not be classified, had been found. Surgery revealed a tumour in the right lobe of the lungs located on the spinal column and infiltrating the mediastinal space. Only 80% of the tumour (11 x 8 cm) could be removed. The patient was readmitted to our hospital on September 27, ]989, because of increasing dysphagia, dyspnea and weakness. On admission the patient was found to be pale and cachectic (181 cm, 54 kg). There was dyspnea at rest, and he was unable to get up without assistance. A thoracotomy scar was located in the right
928
impression and in part due to livid polypoid structures (histopathological infiltration by the pulmonary blastoma).
Therapy and course
Fig. 1. Chest roentgenogram: large tumour in the right lower lobe of the lung that could not be demarcated from the mediastinal space and the diaphragm
As the patient could only drink in small sips, he was nourished parenterally as well as by a duodenal tube. Systemic chemotherapy could not be initiated because of the patient's poor condition. However, high voltage irradiation was directed at the lower right half of the lungs and the mediastinal space because of the rapidly progressing tumour, which had meanwhile been classified as a pulmonary blastoma. In spite of this treatment, the patient's condition deteriorated, and the dyspnea increased. Pulmonary congestion and inflow congestion were clinically and radiologically apparent. Lymphangiosis carcinomatosa and/or pneumonic infiltrations were suspected. The patient died on October 20, 1989, of respiratory insufficiency.
Autopsy basal thorax, percussion showed dullness over the right basal lung area. The erythrocyte sedimentation rate (ESR) was elevated to 55/95 mm. Normochromic, normocytemic anaemia was present with a haemoglobin content of 9.8 g/dl. The butyryl-cholinesterase level (CHE) had decreased to 2.02 kU/1, and on S-electrophoresis the albumin had diminished to 50% in the presence of normal total protein. The c~1globulin was elevated to 6%, ~2-globulin to 13% and gamma-globulin to 22%. The lactate dehydrogenase activity (LDH) was markedly increased to 1164 U/l, the tumour markers carcinoembryonic antigen (CEA), alpha-fetoprotein (AFP), neuronspecific enolase (NSE) and cancer antigens CA 19/ 9 and CA 125 were all unremarkable. The tissue polypeptide antigen (TPA) was elevated to 296 U/1. Chest roentgenography study showed a diffuse mass covering the entire lower right lobe of the lungs that could not be demarcated from the diaphragm, the mediastinal space and the right periphery of the heart. Infiltrations or metastases were not apparent (Fig. 1). On sonography the liver appeared to be blotchy but without metastases. In the midabdomen dorsal of the left lobe of the liver was a large, nonhomogenous mass, about 11 cm diameter with necrotic areas, which appeared to impress on the vena cava. Endoscopy showed a distinct narrowing of the lumen in the esophagus between 35 and 40 cm aboral. The stenosis was in part due to external
The autopsy revealed a tumour in the right lower lobe of the lungs which was almost the size of a child's head. The remaining right lung was dystelectatic by the compression of the turnout. The left lung proved to have bronchopneumonia. The pulmonary blastoma had infiltrated the right diaphragm, pericardium and pleura. It had also extensively invaded the mediastinal space and had surrounded the vena cava. A fistula with secondary mediastinitis arose as a result of the invasion of the esophagus. Metastases were found in the regional lymph nodes, epicardial in the left ventricle, in the thyroid gland, in the abdominal wall, as carcinomatous peritonitis as well as in the right frontal lobe of the central nervous system and in the left cerebellar medulla. Concerning histopathology, the tumour showed a trilineal differentiation with mature epithelial elements of both trabeculate and glandular construction (Fig. 2a). Furthermore, mesenchymal tissue with a sarcomatous component with spindle cells arranged in fascicles could be found (Fig. 2 c). There was also immature mesenchyme abundant in myxomatous ground substance (Fig. 2d). Figure 2b shows the blastomatous component consisting of small undifferentiated cells often arranged around vessels. In the survey the extent of the necrosis within the tumour was striking. The immunohistochemical characterization yielded a focal positivity for cytokeratin in the epithelial areas and in individual cells of the blastomatous component,
929
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~eq
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930
while the mesenchymal elements reacted negatively. In this area the neuronal marker S-100 appeared sporadically. The reactions to vimentin, desmin, chromogranin, CEA as well as fl-human chorionic gonadotropin were negative in all forms of differentiation. Discussion
The clinical picture of the pulmonary blastoma is similar to that of other lung tumours - uncharacteristic. The early symptoms are cough, expectoration, dyspnea or thoracic pain (each appears in 20% of all cases) as reported in the present case. Haemoptysis appears in about 30% of all patients [6]. Pathognomonic laboratory parameters, i.e. tumour markers, are not known. Usually, as also in this case, the tumour is only discovered by radiography. It generally lies peripheral-subpleural and is rather distinctly demarcated from the surrounding pulmonary parenchyma [1, 16]. Because of the varied differentiations of the tumour tissue, an exact histopathologic classification from transthoracic or transbronchial biopsies is successful in only a few cases. In the present case, in spite of bronchial lavage and a transthoracic pleural biopsy, the diagnosis could only postoperatively by made from the resected tissue. The histogenesis of the pulmonary blastoma is unclear. Some authors suppose that a pluripotential cell of a single germ layer may give rise to both the epithelial and mesenchymal elements [17, 20]. However, an origin from two germ layers is also discussed [5]. Other authors consider the tumour to be a subgroup of carcinosarcomas [12, 15]. Even if the immunohistochemical examinations finally do not shed light on the histogenesis of the pulmonary blastoma, it should continue to be viewed as an independent entity with specific histopathologic characteristics. With regard to the therapy, it is important to define the tumour as a pulmonary blastoma in contrast to other malignant lung tumours. A negative reaction to CEA may be helpful for diagnosis because this reaction is normally positive for pulmonary adenocarcinomas [4]. The therapy of choice is surgical resection. Because of the uncharacteristic symptoms and diagnostic difficulties, as for this patient, in most cases radical surgery is no longer possible. Although the tumour is surrounded by a pseudocapsule, it grows invasively: Intraoperatively 25% of tumors reveal intrabronchial growth, 25% infiltration of the pleura and 10% infiltration of the mediastinal space [6]. In nearly half of all cases, local recur-
rence occurs postoperatively [6, 16]. The pulmonary blastoma often metastasizes to the regional lymph nodes. Haematogenous metastasis is rare, involving most often the liver, central nervous system and bone. Moreover, metastases in the lungs, kidneys, pancreas, spleen, gastrointestinal tract and adrenal glands have been described [6]. Local recurrence and metastasis generally occur within the first postoperative year. But for all that, suc, cessful surgical interventions even after the appearance of local recurrence or metastasis have been reported [9, 10]. Further therapeutic measures are chemotherapy and irradiation, either in combination with the primary operation or concerning a recurrence. The best type of therapy is difficult to determine because of the small number of cases. There are some positive reports about combining surgery with cytostatic therapy and about the application of chemotherapy with recurrences [13, 14, 18]. In particular, the cytostatics cyclophosphamide, vincristine, doxorubicin, and dactinomycin are used because of their success with the histogenetically related nephroblastoma and embryonic rhabdomyosarcoma [11-14, 18, 19]. Due to the late diagnosis of pulmonary blastoma, the 5-year survival rate is only 16% [6]. The present report of a pulmonary blastoma should remind us of this extremely rare tumour. The typical clinical course of this tumour, including all diagnostic difficulties, has been described. Because of the uncharacteristic clinical symptoms and the rapid progress of the illness, invasive diagnostic procedures including an early thoracotomy should be made at the slightest suspicion. Whenever histopathology indicates that mesenchymal and ePithelial structures resemble fetal lung tissue, a pulmonary blastoma should be suspected. Therapeutically, immediate surgical resection is necessary. In view of the small number of cases, no controlled therapy protocols can be expected in the near future. Therefore, according to the experience available, a postoperative systemic chemotherapy - possibly even in combination with irradiation - should be taken into consideration depending on the clinical condition of the patient. References 1. Bfihr R, StoRe M, Kienzle H-F (1987) Das pulmonale Biastom. Ein kasuistischer Beitrag. Chirurg 58:358 360 2. Barnard WG (1952) Embryoma of lung. Thorax 7:299-301 3. Barret NR, Bamard WG (1945) Some unusual thoracic tu-" mors. Br J Surg 32:447-457 4. Brockmann M, Hecker A, Mfiller K-M (1990) Das pulmohale Blastom. Immunhistochemische Charakterisierung he-
931 teromorpher Tumorkomponenten. Pneumologie 44:605 606 5. Dienemann D, Hartmann C-A, Minck C (1989) Pulmonary blastomas. Immunhistochemical investigations of three cases. Pathol Res Pract 184:306-311 6. Francis D, Jacobsen M (1983) Pulmonary blastoma. Curr Top Pathol 73 : 265-294 7. Fung CH, Lo JW, Yonan TN, Milloy F J, Hakami MM, Changus GW (1977) Pulmonary blastoma, an ultrastructural study with a brief review of literature and a discussion of the pathogenesis. Cancer 39:153-163 8. Jimenez JF (i 987) Pulmonary blastoma in childhood. J Surg Oncol 34:87 93 9. Kennedy A, Prior AL (1976) Pulmonary blastoma: a report of two cases and a review of the literature. Thorax 31:776781 I0. Kern WH, Stiles QR (1976) Pulmonary blastoma. J Thorac Cardiovasc Surg 11:239-241 11. Livingston RB, Einhorn LH, Bodey GP, Burgess MA, Freireich EJ, Gottlieb JA (1975) COMB (cyclophosphamide, oncovin, methyl-CCNU, and bleomycin): a four-drug combination in solid tumors. Cancer 36:327-332 12. McCann MP, Fu Y, Kay S (1976) Pulmonary blastoma: a light and electron microscopic study. Cancer 38 : 789-797 13. Medberry CA, Bibro MC, Phares JC, Veach SR, Martin JE, Pasquale DN (1984) Pulmonary blastoma. Case report and literature review of chemotherapy experience. Cancer 53:2413 2416 14. Ozkaynak MF, Ortega JA, Laug W, Gilsanz V, Isaacs H
(1990) Role of chemotherapy in pediatric chemotherapy. Med Pediatr Oncol 18:53 56 15. Roth JA, Elguezabal A (1978) Pulmonary blastoma involving into a carcinosarcoma. A case study. Am J Pathol 2:407-413 16. Selle B, D6rffel W, Wit J (1987) Das pulmonale Blastom im Kindesalter - Eine Kasuistik und Ubersicht iiber die Literatur. Z Kinderchir 42:373-377 17. Spencer H (1961) Pulmonary blastomas. J Pathol Bacterioi 82:161 165 18. Valderrama E, Saluja G, Shende A, Lanzkowsky P, Berkman J (1978) Pulmonary blastoma: report of two cases in children. Am J Surg Pathol 2:415-422 19. Weinblatt ME, Siegel SE, Isaacs H (1982) Pulmonary blastoma associated with cystic lung disease. Cancer 49 : 669-671 20. Yousem SA, Wick MR, Randhawa P, Manivel JC (1990) Pulmonary blastoma. An immunohistochemical analysis with comparison with fetal lung in its pseudoglandular stage. Am J Clin Pathol 93:167-175 Received: April 29, 1992 Accepted: May 27, 1992 Dr. med. V. Kliem Medizinische Hochschule Hannover Abteilung Nephrologie Konstanty-Gutschow-Strasse 8 W-3000 Hannover 61, FRG
Glinical Investigator
C se Report with a Review of the Literature
© Springer-Verlag 1992
Pulmonary blastoma-
a rare tumour
V. Kliem 1, M. Biigge 2, K. Leimenstoll 3, and H. Maschek 4 Abteilung Nephrologie, Zentrum Innere Medizin und Dermatologie, Medizinische Hochschule Hannover 2 Innere und 3 Chirurgische Abteilung, Kreiskrankenhaus Rinteln 4 Pathologisches Institut, Zentrum Pathologie und Rechtsmedizin, Medizinische Hochschule Hannover
Summary. A 57-year-old man was found to have a tumour in the right lower lobe of the lungs, which was not classifiable by biopsy. The tumour could only be partially removed by surgical resection. The diagnosis of a pulmonary blastoma was made from the resected tissue. Clinically, rapid progress occured with invasion in the mediastinal space and the epigastrium. In spite of radiation therapy, the patient died about 21/2 months after surgery of respiratory insufficiency. Autopsy confirmed a pulmonary blastoma with extensive infiltration of the mediastinal space and upper abdomen as well as metastases in the regional lymph nodes, pleura, peritoneum, thyroid gland, heart and central nervous system. The present report of a pulmonary blastoma should draw attention to this extremely rare tumour. It should be included in the differential diagnosis, because the survival time can be increased if the correct diagnosis is made very early. Key words: Pulmonary blastoma
Malignant lung tumours - Epidemiology - Histogenesis - Diagnosis - Therapy
The pulmonary blastoma was described for the first time as an independent entity by Barret and Barnard in 1945 [3]. Because of its histological similarity to fetal lung tissue in the third gestational month [7], it was denoted as an embryoma [2]. In analogy to nephroblastoma, Spencer named the tumour pulmonary blastoma, because in his opinion the tumour developed from a pluripotential mesenchymal blastema [17]. There have only been 150 cases of a pulmonary blastoma reported to Abbreviations: AFP = alpha-fetoprotein; fl-HCG = beta-human chorionic gonadotropin; CA = cancer antigen; CEA = carcinoembryonic antigen; CHE=butyryl-cholinesterase; E S R = erythrocyte sedimentation rate; LDH =lactate dehydrogenase; NSE=neuron-specific enolase; TPA=tissue polypeptide antigen
the present day, including about 40 cases involving children [8, 16]. In more than 75% of these cases, the children are younger than 5 years old at the onset of the illness [16]. However, the pulmonary blastoma appears in every age group. The average age in adults is about 43 years. In men the tumour seems to develop somewhat later (49 years) and more often (2.6:1) than in women [6]. In contrast to nephroblastoma, late occurrences could be attributed to a different organogenesis - the lungs develop up to the 20th year of life [17]. Cases of a familiar or regional accumulation have not been reported.
Case report Anamnesis and findings A 57-year-old Caucasian man smoked about 25 cigarettes per day for more than 30 years. The patient had had nausea, loss of appetite, slight weight loss, pain in the epigastrium and in the thoracic and lumbal spinal column area as well as occasionally hemoptysis since May 1989. Chest roentgenography, gastroscopy, and abdominal sonography examinations were all without abnormalities. In late July 1989 at right basal lung tumour was suspected. The bronchoalveolar lavage yielded a strong granulomatous inflammation with cellular atypias (Papanicolaou's test III D), and in the pleural biopsy tumour tissue, which could not be classified, had been found. Surgery revealed a tumour in the right lobe of the lungs located on the spinal column and infiltrating the mediastinal space. Only 80% of the tumour (11 x 8 cm) could be removed. The patient was readmitted to our hospital on September 27, ]989, because of increasing dysphagia, dyspnea and weakness. On admission the patient was found to be pale and cachectic (181 cm, 54 kg). There was dyspnea at rest, and he was unable to get up without assistance. A thoracotomy scar was located in the right
928
impression and in part due to livid polypoid structures (histopathological infiltration by the pulmonary blastoma).
Therapy and course
Fig. 1. Chest roentgenogram: large tumour in the right lower lobe of the lung that could not be demarcated from the mediastinal space and the diaphragm
As the patient could only drink in small sips, he was nourished parenterally as well as by a duodenal tube. Systemic chemotherapy could not be initiated because of the patient's poor condition. However, high voltage irradiation was directed at the lower right half of the lungs and the mediastinal space because of the rapidly progressing tumour, which had meanwhile been classified as a pulmonary blastoma. In spite of this treatment, the patient's condition deteriorated, and the dyspnea increased. Pulmonary congestion and inflow congestion were clinically and radiologically apparent. Lymphangiosis carcinomatosa and/or pneumonic infiltrations were suspected. The patient died on October 20, 1989, of respiratory insufficiency.
Autopsy basal thorax, percussion showed dullness over the right basal lung area. The erythrocyte sedimentation rate (ESR) was elevated to 55/95 mm. Normochromic, normocytemic anaemia was present with a haemoglobin content of 9.8 g/dl. The butyryl-cholinesterase level (CHE) had decreased to 2.02 kU/1, and on S-electrophoresis the albumin had diminished to 50% in the presence of normal total protein. The c~1globulin was elevated to 6%, ~2-globulin to 13% and gamma-globulin to 22%. The lactate dehydrogenase activity (LDH) was markedly increased to 1164 U/l, the tumour markers carcinoembryonic antigen (CEA), alpha-fetoprotein (AFP), neuronspecific enolase (NSE) and cancer antigens CA 19/ 9 and CA 125 were all unremarkable. The tissue polypeptide antigen (TPA) was elevated to 296 U/1. Chest roentgenography study showed a diffuse mass covering the entire lower right lobe of the lungs that could not be demarcated from the diaphragm, the mediastinal space and the right periphery of the heart. Infiltrations or metastases were not apparent (Fig. 1). On sonography the liver appeared to be blotchy but without metastases. In the midabdomen dorsal of the left lobe of the liver was a large, nonhomogenous mass, about 11 cm diameter with necrotic areas, which appeared to impress on the vena cava. Endoscopy showed a distinct narrowing of the lumen in the esophagus between 35 and 40 cm aboral. The stenosis was in part due to external
The autopsy revealed a tumour in the right lower lobe of the lungs which was almost the size of a child's head. The remaining right lung was dystelectatic by the compression of the turnout. The left lung proved to have bronchopneumonia. The pulmonary blastoma had infiltrated the right diaphragm, pericardium and pleura. It had also extensively invaded the mediastinal space and had surrounded the vena cava. A fistula with secondary mediastinitis arose as a result of the invasion of the esophagus. Metastases were found in the regional lymph nodes, epicardial in the left ventricle, in the thyroid gland, in the abdominal wall, as carcinomatous peritonitis as well as in the right frontal lobe of the central nervous system and in the left cerebellar medulla. Concerning histopathology, the tumour showed a trilineal differentiation with mature epithelial elements of both trabeculate and glandular construction (Fig. 2a). Furthermore, mesenchymal tissue with a sarcomatous component with spindle cells arranged in fascicles could be found (Fig. 2 c). There was also immature mesenchyme abundant in myxomatous ground substance (Fig. 2d). Figure 2b shows the blastomatous component consisting of small undifferentiated cells often arranged around vessels. In the survey the extent of the necrosis within the tumour was striking. The immunohistochemical characterization yielded a focal positivity for cytokeratin in the epithelial areas and in individual cells of the blastomatous component,
929
8~
g~
~g
0
,.~,©
© ©
o
~eq
Rs~
930
while the mesenchymal elements reacted negatively. In this area the neuronal marker S-100 appeared sporadically. The reactions to vimentin, desmin, chromogranin, CEA as well as fl-human chorionic gonadotropin were negative in all forms of differentiation. Discussion
The clinical picture of the pulmonary blastoma is similar to that of other lung tumours - uncharacteristic. The early symptoms are cough, expectoration, dyspnea or thoracic pain (each appears in 20% of all cases) as reported in the present case. Haemoptysis appears in about 30% of all patients [6]. Pathognomonic laboratory parameters, i.e. tumour markers, are not known. Usually, as also in this case, the tumour is only discovered by radiography. It generally lies peripheral-subpleural and is rather distinctly demarcated from the surrounding pulmonary parenchyma [1, 16]. Because of the varied differentiations of the tumour tissue, an exact histopathologic classification from transthoracic or transbronchial biopsies is successful in only a few cases. In the present case, in spite of bronchial lavage and a transthoracic pleural biopsy, the diagnosis could only postoperatively by made from the resected tissue. The histogenesis of the pulmonary blastoma is unclear. Some authors suppose that a pluripotential cell of a single germ layer may give rise to both the epithelial and mesenchymal elements [17, 20]. However, an origin from two germ layers is also discussed [5]. Other authors consider the tumour to be a subgroup of carcinosarcomas [12, 15]. Even if the immunohistochemical examinations finally do not shed light on the histogenesis of the pulmonary blastoma, it should continue to be viewed as an independent entity with specific histopathologic characteristics. With regard to the therapy, it is important to define the tumour as a pulmonary blastoma in contrast to other malignant lung tumours. A negative reaction to CEA may be helpful for diagnosis because this reaction is normally positive for pulmonary adenocarcinomas [4]. The therapy of choice is surgical resection. Because of the uncharacteristic symptoms and diagnostic difficulties, as for this patient, in most cases radical surgery is no longer possible. Although the tumour is surrounded by a pseudocapsule, it grows invasively: Intraoperatively 25% of tumors reveal intrabronchial growth, 25% infiltration of the pleura and 10% infiltration of the mediastinal space [6]. In nearly half of all cases, local recur-
rence occurs postoperatively [6, 16]. The pulmonary blastoma often metastasizes to the regional lymph nodes. Haematogenous metastasis is rare, involving most often the liver, central nervous system and bone. Moreover, metastases in the lungs, kidneys, pancreas, spleen, gastrointestinal tract and adrenal glands have been described [6]. Local recurrence and metastasis generally occur within the first postoperative year. But for all that, suc, cessful surgical interventions even after the appearance of local recurrence or metastasis have been reported [9, 10]. Further therapeutic measures are chemotherapy and irradiation, either in combination with the primary operation or concerning a recurrence. The best type of therapy is difficult to determine because of the small number of cases. There are some positive reports about combining surgery with cytostatic therapy and about the application of chemotherapy with recurrences [13, 14, 18]. In particular, the cytostatics cyclophosphamide, vincristine, doxorubicin, and dactinomycin are used because of their success with the histogenetically related nephroblastoma and embryonic rhabdomyosarcoma [11-14, 18, 19]. Due to the late diagnosis of pulmonary blastoma, the 5-year survival rate is only 16% [6]. The present report of a pulmonary blastoma should remind us of this extremely rare tumour. The typical clinical course of this tumour, including all diagnostic difficulties, has been described. Because of the uncharacteristic clinical symptoms and the rapid progress of the illness, invasive diagnostic procedures including an early thoracotomy should be made at the slightest suspicion. Whenever histopathology indicates that mesenchymal and ePithelial structures resemble fetal lung tissue, a pulmonary blastoma should be suspected. Therapeutically, immediate surgical resection is necessary. In view of the small number of cases, no controlled therapy protocols can be expected in the near future. Therefore, according to the experience available, a postoperative systemic chemotherapy - possibly even in combination with irradiation - should be taken into consideration depending on the clinical condition of the patient. References 1. Bfihr R, StoRe M, Kienzle H-F (1987) Das pulmonale Biastom. Ein kasuistischer Beitrag. Chirurg 58:358 360 2. Barnard WG (1952) Embryoma of lung. Thorax 7:299-301 3. Barret NR, Bamard WG (1945) Some unusual thoracic tu-" mors. Br J Surg 32:447-457 4. Brockmann M, Hecker A, Mfiller K-M (1990) Das pulmohale Blastom. Immunhistochemische Charakterisierung he-
931 teromorpher Tumorkomponenten. Pneumologie 44:605 606 5. Dienemann D, Hartmann C-A, Minck C (1989) Pulmonary blastomas. Immunhistochemical investigations of three cases. Pathol Res Pract 184:306-311 6. Francis D, Jacobsen M (1983) Pulmonary blastoma. Curr Top Pathol 73 : 265-294 7. Fung CH, Lo JW, Yonan TN, Milloy F J, Hakami MM, Changus GW (1977) Pulmonary blastoma, an ultrastructural study with a brief review of literature and a discussion of the pathogenesis. Cancer 39:153-163 8. Jimenez JF (i 987) Pulmonary blastoma in childhood. J Surg Oncol 34:87 93 9. Kennedy A, Prior AL (1976) Pulmonary blastoma: a report of two cases and a review of the literature. Thorax 31:776781 I0. Kern WH, Stiles QR (1976) Pulmonary blastoma. J Thorac Cardiovasc Surg 11:239-241 11. Livingston RB, Einhorn LH, Bodey GP, Burgess MA, Freireich EJ, Gottlieb JA (1975) COMB (cyclophosphamide, oncovin, methyl-CCNU, and bleomycin): a four-drug combination in solid tumors. Cancer 36:327-332 12. McCann MP, Fu Y, Kay S (1976) Pulmonary blastoma: a light and electron microscopic study. Cancer 38 : 789-797 13. Medberry CA, Bibro MC, Phares JC, Veach SR, Martin JE, Pasquale DN (1984) Pulmonary blastoma. Case report and literature review of chemotherapy experience. Cancer 53:2413 2416 14. Ozkaynak MF, Ortega JA, Laug W, Gilsanz V, Isaacs H
(1990) Role of chemotherapy in pediatric chemotherapy. Med Pediatr Oncol 18:53 56 15. Roth JA, Elguezabal A (1978) Pulmonary blastoma involving into a carcinosarcoma. A case study. Am J Pathol 2:407-413 16. Selle B, D6rffel W, Wit J (1987) Das pulmonale Blastom im Kindesalter - Eine Kasuistik und Ubersicht iiber die Literatur. Z Kinderchir 42:373-377 17. Spencer H (1961) Pulmonary blastomas. J Pathol Bacterioi 82:161 165 18. Valderrama E, Saluja G, Shende A, Lanzkowsky P, Berkman J (1978) Pulmonary blastoma: report of two cases in children. Am J Surg Pathol 2:415-422 19. Weinblatt ME, Siegel SE, Isaacs H (1982) Pulmonary blastoma associated with cystic lung disease. Cancer 49 : 669-671 20. Yousem SA, Wick MR, Randhawa P, Manivel JC (1990) Pulmonary blastoma. An immunohistochemical analysis with comparison with fetal lung in its pseudoglandular stage. Am J Clin Pathol 93:167-175 Received: April 29, 1992 Accepted: May 27, 1992 Dr. med. V. Kliem Medizinische Hochschule Hannover Abteilung Nephrologie Konstanty-Gutschow-Strasse 8 W-3000 Hannover 61, FRG
