Ginical Practice

Pulmonary Blastoma
Summary: A case of rapidly growing solitary tumour ofthe right upper lobe of the lung in a 72-year-old man is presented. It shows the typical histologi cal appearance ofa pulmonary blastoma. Only 13 cases were published pre viously, none from Canada. It is a matter of opinion whether pulmonary blastoma should be classified as a separate entity or as a distinct form of
negative for tumour cells was examined. At bronchoscopy (June 24, 1968) the right Welland, Ont. upper lobe bronchus was patent and blood was seen issuing from it. Bron The term pulmonary blastoma for chial smears were negative for tumour cells. Results of laboratory examinations a peculiar carcinosarcomatous tumour of the lungs was first em were essentially unremarkable. A rightployed by Spencer in 1962.! An side scalene node biopsy showed anthraneedle earlier case described by Barnard2 cosis and a showed biopsy of the right upper lung has been diagnosed as pulmonary and anthracosis. Twopulmonary fibrosis pleural Since then more reports recovered a few mesothelial aspirations embryoma. cells only. have been published; Minken, The patient developed excessive hemop Craver and Adams5 reviewed 12 tysis and was not in a condition for sur cases, including one of their own gical exploration. He was treated with and another case was reported by intravenous nitrogen mustard. The Barson, Jones and Lodge,4 bringing hemoptysis was checked but the size of
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showed evidence of anthracosis. Liver and adrenals were free of secondary tumour, as were all other organs. The brain was not examined; there had been no clinical evidence of cerebral involve
ment.

carcinosarcoma.

C.R.C.P.[C]

DANZIGER, M.D., D.T.M.,

sputum
on

was

the 15 occasions it

the total up to 13 cases. The case we describe below is the first in stance of this rare condition to be reported from Canada.

the tumour increased. The last film

The patient, a 72-year-old man, was admitted on three occasions in 1968 to the Niagara Peninsula Sanatorium in St. Catharines. He complained of dys pnea and blood-streaked sputum. A radiograph ofthe chest (June 7, 1968) showed in the right upper lung field a round, sharply circumscribed mass 4.0 by 4.5 cm., with clearly defined borders. On admission he was dyspneic and cya nosed and had edema ofthe legs. Physi cal examination showed emphysema; the blood pressure was 150/88 mm.Hg. the Tiny lymph nodes were palpable inThe of the right scalenus muscle. region
H. Danziger, m.d.. d.t.m., c.R.c.p.[c], Pathologist, Welland County General Hospital. Welland. Ontario.

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Dan/igcr. Pathologist.

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9.0 by 9.5 cm.; the lateral border was still sharply outlined but the medial border could not be defined from the mediastinal structures. The patient be came increasingly anemie and died on December 7, 1968. Autopsy was per formed about six hours after death. The essential autopsy findings were limited to the lungs. The pleural cavities were free of fluid. The right lung weighed 1548 g. and the left lung 610 g. The lower lobes showed confluent broncho pneumonia, more pronounced on the right side. The tumour ofthe right upper lobe measured 10 cm. in diameter and was round, whitish, of soft consistence and sharply circumscribed. The cut sur face had a variegated appearance, whitish with red mottling and irregular areas of necrosis, making the tumour rather friable. The main upper right lobe bronchus led into the tumour. The hilar lymph nodes were not enlarged and

(November 22, 1968) showed a tumour

Sections ofthe tumour were stained with H & E and iron hematoxylin-van Gieson; mucin stains were used on some of the sections. Two distinct intermixed components were evident. The stroma was sarcomatous in type; the nuclei were large, spindle-shaped and often hyperchromic. Numerous giant nuclei, up to four or five times the size of their neighbours, were present, with mitotic figures, some of abnormal type. The arrange ment ofthe stroma was in fascicles, often intertwined. It was very vascular, with many thin-walled blood vessels of simple type; the lining ofswollen endothelial cells was absent in places, so that the blood cells appeared in direct contact with the cellular or stromal elements of the tumour. Present in the sarcomatous stroma were epithelial cells of carcinomatous type. Often these lined duct-like spaces, arranged in one to four layers, were cuboidal or low columnar in type; no ciliated cells were seen. The nuclei were round or ovoid and hyperchromatic. Mitotic figures were not so numer ous as in the sarcomatous stroma; some multinucleated giant cells were present. Frequently the carcinomatous compo nent was arranged in sheets or ill-defined lobules without duct formation. In only one section was differentiation into

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stroma

and mitotic

figure. (Original

146 C.M.A. JOURNAL/JAN.31.1970/VOL. 102

squamous cells apparent. The latter were arranged in small groups showing a pinkish staining cytoplasm. The nuclei were less hyperchromatic than in the surrounding irregular aggregations of carcinomatous cells. There was widespread, often hemorrhagic necrosis throughout the tumour. Mucin stains were negative. To some sections surrounding lung tissue was attached which was compressed but not infiltrated by tumour. The tumour had not spread to the hilar lymph nodes. The sections of the lower lobes showed bronchopneumonia. The author's first impression was that the tumour represented a carcinosarcoma, and consultations were sought, including referral to the Canadian Tumour Reference Centre (C.T.R. No. 12823). Four consultants made a diagnosis of pulmonary blastoma as described by Spencer, while two preferred the diagnosis of carcinosarcoma, though one of the latter conceded that it might very well be a pulmonary blastoma. Opinion on the malignancy of the tumour was unanimous.

Discussion Histologically this tumour resembles those in other cases of pulmonary blastoma reported in the literature. By the end of 1968, 13 cases had been published. These are summarized in detail in the papers by Minken, Craver and Adams3 and Barson, Jones and Lodge.4 The appearance is very similar to the illustrations in Spencer's book on the pathology of the lung' and in other papers. According to Spencer this tumour is the pulmonary counterpart of the renal nephroblastoma and "its existence can only be explained by the assumption that the peripheral part of the lung is derived from mesoblastic tissue (Waddell's theory)." According to Waddell,' the peripheral part of the lungs is formed from pulmonary mesenchyme ca-

pable of differentiation into stroma and epithelial tissue. This would explain the histogenesis of these tumours. However, Waddell's theory is not generally accepted, for many pathologists and embryologists maintain that the lung parenchyma arises from two germ layers. It is difficult to explain the occurrence of a rapidly growing malignant tumour in an old man on the basis of Waddell's theory. These doubts are thoroughly discussed in the paper by Barson, Jones and Lodge.4 The main objection is the wide range in age at which patients with this condition were seen first. Out of 12 cases, eight came under observation at the age of 40 or older. According to Willis,6 adult mesenchymal tissue may contain undifferentiated cells which can revert to the embryonic state and may then produce epithelial and mesenchymal elements. Barson and his colleagues4 regard pulmonary blastoma as a true carcinosarcoma which has a fortuitous resemblance to fetal lung. Certain features of pulmonary blastoma are different from true carcinosarcoma. Some cases have had a very benign clinical course and have survived surgical excision; in others the outcome was as bad as in most bronchogenic carcinomas. Especially noteworthy is the case of Bauermeister et al.7; the time of observation extended for 24 years until a right lower lobectomy was performed and the patient was cured. In most cases the localized nature of the tumour and the frequent absence of metastases are stressed, but in four cases of the 12 collected by Barson and his colleagues metastases had occurred, some of which were in the brain.

In most cases sputum cytology was negative or showed atypical cells only. The present case is peculiar in so far as the tumour remained localized without metastases, despite rapid growth and severe malignant histological characteristics, more pronounced than in some of the published cases. (As the brain was not examined, cerebral metastases cannot be entirely excluded.)
The author wishes to thank Dr. W. M. Gregor for supplying the clinical data, and the various consultants, including Dr. A. C. Ritchie, Toronto, and Dr. A. A. Liebow, San Diego, California, for their helpful co-operation.

Resume Cas de blastome pulmonaire L'auteur pr.sente un cas de tumeur solitaire, .t croissance rapide, du lobe sup&ieur droit du poumon, chez un homme de 72 ans. Cette tumeur pr.sentait, au point de vue histologique, les caract&ristiques d'un blastome pulmonaire. La litt.rature n'a publi. jusqu'ici que 13 cas semblables, dont aucun n'est survenu au Canada. Quant Li la question de savoir s'il faut consid.rer le blastome pulmonaire comme une entit& s.par.e ou comme une forme distincte de carcinosarcome, ce n'est qu'une question d'opinion personnelle. References
1. SPENCER, H.: Pathology of the. lung, excluding pulmonary tuberculosis, Pergamon Press Ltd., Oxford, 1962, p. 769.
2. BARNARD, W. G.: Thorax, 7: 299, 1952.
3. MINKEN, S. L., CRAVER, W. L. and ADAMS, J. T.:

Arch. Path. (Chicago), 86: 442, 1968.

4. BARSON, A. J., JONES, A. W. and LODGE, K. v.:

J. C/in. Path., 21: 480, 1968. 5. WADDELL, W. R.: Arch. Path. (Chicago), 47: 227,

1949. 6. WILLIs, R. A.: Pathology of tumours, 3rd ed., Butterworth & Co. (Publishers) Ltd., London, 1960. 46: 322, 1966.

7. BAUERMEISTER, D. E. et at.: Amer. .1. C/in. Path.,

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FIG. 2 - Duct-like structures in sarcomatous stroma. (Original magnification x 100.)

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FIG 3- Sarcomatous and carcmomatous areas intermixed. (Original magnification x 100.)

FIG. 4 - squamous metaplasia in carcinomatous area. (Original magnification x 250.)

C.M.A. JOURNAL/JAN.31, 1970/VOL. 102 147