malignant teratoid tumour of the lung: malignant hamartoma

Thzorax (I960), 15, 120.

MALIGNANT TERATOID TUMOUR OF THE LUNG:? MALIGNANT HAMARTOMA

BY

TORBEN SCHI0DT AND KAJ GOTLIEB JENSEN

From 0resundshospitalet, Copenhagen, Denm7at-rk

(RECEIVED FOR PUBLICATION NOVEMBER 24, 1959)

In a previous paper (Jensen and Schi0dt, 1958)describing the conditions of growth of 22 lunghamartomas we mentioned that in one case amalignant tumour had been ruled out, becausethe evidence of it having been developed from ahamartoma was doubtful. This case will now bereported and discussed.

CASE REPORTA man, aged 66, had attended regularly for radio-

graphic control of the lungs since 1944. Noabnormality had been detected. About the middle ofNovember, 1950, a walnut-sized, well-defined shadowwas observed in the left third intercostal space (Fig. 1).The patient neither coughed nor expectorated.Weight 104.5 kg., height 187 cm., B.S.R. 5 mm. /hour. Bronchoscopy and bronchography showed noabnormality in the bronchial tree. A radiographtaken just over one month later (Fig. 2) showed thatthe infiltration had grown about 1 cm. both acrossand lengthwise. The patient's general condition hadremained good. There was no loss of weight.Cancer being suspected, thoracotomy was done on

January 19, 1951. The left lung surface was foundto be free. A somewhat oblong, hard tumour waspalpated centrally in the upper lobe. The superiorsegments of the upper lobe were resected, and a softlymph node in the hilum, as well as a similar nodeat the origin of the segmental bronchus, were alsoremoved.On out-patient control two months later the patient

was feeling well. Radiological examination revealeda fluid level above the diaphragm, but no otherabnormality. Radiographs taken on May 18 showedunchanged conditions. On August 10 the patientlooked sallow and was feverish. He had lost weight,and x-ray examination disclosed a massive blurringof the lower field of the left lung. On August 22the outlines of more than one tumour mass wereapparent. There were no signs of metastases.On August 30 explorative thoracotomy was done.

A pleural exudate and a closely adhering lung werefound. In the paravertebral sulcus, on a level withthe third and fourth ribs, necrosing tumour tissue was

encountered; this extended so far into the thoracicwall and towards the vertebral column that radicaloperation was out of the question.On November 3, 1951, one year after the discovery

of the tumour, the patient died. Necropsy wasprohibited by his relatives.

Pathological examination of a preparation fromthe first operation showed a 3 x 10 x 15 cm. large pieceof lung tissue containing, close to the line of resection,a 1 x 1.5 x 3 cm. large, well-defined mass. The anteriorsegmental bronchus was occluded and seemed todisappear into the tumour; but it was easily shelledout from the lung tissue, having no demonstrablerelation to the bronchus. The surface and the cutsurface were lobulated, the consistency was firm,elastic, and cartilaginous. The remaining lung andbronchi presented no changes.On microscopy the tumour tissue presented a pol-

morphous histological picture. Most of the tissuehad a mesenchymal character, with gradual transi-tions between loose connective tissue, myxoedematoustissue, and rounded areas of a character resemblingfoetal cartilage, showing in several places pronouncednuclear polymorphism (Fig. 3). " Mature " cartilagewas not demonstrated. Here and there the connectivetissue had proliferated, having a sarcomatouscharacter. There was lymphocyte infiltration. Inaddition islands of squamous epithelium, here andthere with marked corneous pearl formation, werefound. The transition from epithelial tissue to stromawas in several places indistinct. Narrow clefts linedwith a low stratified squamous epithelium were alsofound (Fig. 4); but not the ramified clefts lined withcolumnar epithelium characteristic of hamartomas.Parts of the tumour were rich in vessels, some ofwhich were large and cavernous. There was no fat.The whole tumour was well defined, surrounded bycompressed but otherwise normal lung. The tumourwas nowhere seen to be in relation to bronchial struc-tures. Lymph nodes displayed sinusoid reticulosis,but no signs of malignancy.

Histological examination of a biopsy specimenfrom the second operation on August 30 showedtumour tissue consisting of closely packed, dark, poly-morphous, here and there spindle-shaped nuclei and

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FIG. I.-Radiograph of the thorax taken in the middle of November,1950. A well-defined shadow is seen in the left third intercostalspace.

FIG. 2.-Radiograph of the thorax taken at the beginning of January,1951. The shadow in the left third intercostal space is seen tohave grown.

FIG. 3.-Photomicrograph (x about 145) of a preparation from the FIG. 4.-Photomicrograph (x about 72) of a preparation from thefirst operation, showing solid tissue with nuclear polymorphism first operation showing a long cleft bounded by epithelial-likeand indistinct transition to connective tissue, here and there tissue.reminiscent of foetal cartilage and in other places of epithelialformation. At the top right compressed pulmonary tissue is seen.

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TORBEN SCHIODT and KAJ GOTLIEB JENSEN

FIG. 5.-Photomicrograph (x about 280) of a biopsy specimen fromthe second operation showing a polymorphous, sarcoma-liketumour rich in cells.

scant cytoplasm. There were no epithelial cells, norwas the stroma present in the tumour tissue (Fig. 5).The lesion was diagnosed as a malignant tumour,rather of the type of pure sarcoma.

DISCUSSIONA developmental malformation, including a

hamartoma, may be the site of a malignanttumour. A well-known instance of this is themalignant teratoma of the testis. As for hamar-toma of the lung, it is suggested in the literaturethat the chance of malignant transformation isextremely small. Few cases of malignant lunghamartoma have been reported, and none of theseis quite certain.

Tapie (quoted by Hickey and Simpson, 1926) isstated to have published in 1892 a case of osteomain the left lower lobe; the tumour was lying inclose relation to an epithelial neoplasm. Thepatient, aged 28, also had multiple adeno-carcinomatous infiltrations in both lungs. Thereseems to be no definite evidence of developmentfrom a hamartoma. Greenspan (1933) describeda case of primary osteochondrosarcoma of the

lung, which is often termed " malignant hamar-toma" in the literature. However, the authorhimself found the chondroblastic tissue in thebronchus itself to be the most likely site of origin;epithelial structures were not demonstrated.Lowell and Tuhy (1949) described a chondro-sarcoma filling both branches of the pulmonaryartery and continuing into lung. The authorsfound that the structure and the site were againsta hamartoma as the point of origin. One of themost convincing cases of malignant hamartoma isthat published by Simon and Ballon (1947). Thiswas a histologically verified hamartoma of thelung, which here and there showed pronouncedpolymorphism of the mesenchymal tissue andinvasion of mediastinal fatty tissue. The authorsconcluded that, "although, from a clinical point ofview and in the absence of metastases, this tumourmay be regarded as benign, it is apparent froman objective point of view that malignancy cannotbe excluded." Kuyjer (1955), in a society report,described a case where a man, aged 18, had abenign, typical hamartoma of the lung removed.Six months later the patient was reoperated upon.The histological diagnosis was then spindle-cellsarcoma. The patient died three months later,and necropsy revealed round-cell sarcoma. Thecase seems convincing according to the description.but has not been substantiated by illustrations.Adams (1957) prefers the name of " chondro-adenoma " to that of hamartoma and designatesSimon and Ballon's case as one of " ossifyingadenoma." Adams himself reports a similarexample, which is not convincingly malignant.Cavin, Masters, and Moody (1958) report a caseof hamartoma with development of a leiomyo-sarcoma in a girl, aged 22 months. These authorscite a fatal case of "chondroma of the lung"described by Sherwood and Sherwood (1932). Wehave been unable to procure the original article.The difficulties of substantiation in the stated

cases from the literature may be classified intotwo categories: (1) either an obvious hamartomaof the lung was present, the malignancy of whichmay be called in doubt (Simon and Ballon), or (2)the patient had an unquestionable malignantteratoid tumour, of which the development froma hamartoma was doubtful (Tapie, Greenspan,Lowell and Tuhy).

In the case described above our problem is inthe latter category. At first operation a solid,macroscopically cartilaginous, well-defined tumourwas found in the parenchyma of the lung,unrelated to the bronchus. Histologically thetumour consisted chiefly of mesenchymal struc-tures, among which were large areas having the

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MALIGNANT TERATOID TUMOUR OF THE LUNG

appearance of foetal cartilage (Fig. 3). Inaddition, there were definite epithelial cells, hereand there lining narrow clefts (Fig. 4). It was, inother words, a teratoid tumour, but composedexclusively of germ layers and constituentsrelating to pulmonary tissue. This accords withAlbrecht's (1904) definition of a hamartoma, butthe overall picture was not typical of such,columnar epithelium, fat, and mature cartilagebeing absent. On the other hand, cavernous vesselsand, as is characteristic of hamartomas, an abrupttransition peripherally to compressed but other-wise normal pulmonary tissue was found. Thissomewhat unusual picture was, perhaps, due to thepresence of unequivocally malignant features inthe mesenchymal tissue already present in the firstspecimen. During the further course the tumourdeveloped into a definite sarcoma (Fig. 5) as inKuyjer's case.

In the case under review it was not proved, butseemed highly probable, that the tumour haddeveloped from a hamartoma. Metastases from amalignant teratoma-in practice from a testis-canbe left out of account. The testes were normal onclinical examination, and on the first operation thetumour was considered to be benign. Teratoidtumours in free lung parenchyma are rare(Liebow, 1952; Collier, Dowling, Plott, andSchneider, 1959), unlike in the mediastinum,where benign teratomas are relatively frequent. Inour case the tumour had no connexion withapparently normal mediastinum. The fact that a

previous routine radiological examination of thepatient's lungs had not revealed infiltration doesnot preclude a diagnosis of hamartoma (Jensenand Schi0dt, 1958). The growth rate of the tumourreported here was considerably faster than hasbeen observed for any one of 22 benignhamartomas of the lung.

SUMMARYA case is reported of a teratoid, well-defined

tumour with epithelial and in places malignantmesenchymal components, localized in free lungparenchyma. The patient was a man, aged 66.The possibility of a diagnosis of malignanthamartoma is discussed. The tumour recurredas a pure sarcoma after operation, and the patientdied after one year.

REFERENCESAdams, M. J. T. (1957). Thorax, 12, 268.Albrecht, E. (1904). V'erh. dtsch. path. Ges., 7, 153.Cavin, E., Masters, J. H., and Moody, J. (1958). J. thorac. Surg., 35,

816.Collier, F. C., Dowling, E. A., Plott, D., and Schneider, H. (1959).

A.M.A. Arch. Path., 68, 138.Greenspan, E. B. (1933). Amer. J. Cancer, 18, 603.Hickey, P. M., and Simpson, W. M. (1926). Acta radiol. (Stockh.),

5, 475.Jensen, K. G., and Schiodt, T. (1958). Thorax, 13, 233.Kuyjer, P. J. (1955). Ned. T. Geneesk., 99, 1884.Liebow, A. A. (1952). Atlas of Tumor Pathology, sect. 5, fasc. 17:

Tumors of the Lower Respiratory Tract, p. 141. Armed ForcesInstitute of Pathology, Washington, D.C.

Lowell, L. M., and Tuhy, J. E. (1949). J. thorac. Surg., 18, 476.Sherwood, K. K., and Sherwood, H. H. (1932). J.-Lancet, 52, 395.

(Cited by Cavin et al.)Simon, M. A., and Ballon, H. C. (1947). J. thorac. Surg., 16, 379.Tapie (1892). Midi med. (Toulouse), 1, 385. (Cited by Hickey and

Simpson.)

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