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Relative frequency and site of predilection of intrathoracic tumors
RELATIVE
FREQUENCY
AND SITE OF PREDILECTION
OF INTRATHORACIC
TUMORS*
BRIAN BLADES, M.D. ST. LOUIS, MISSOURI RIMARY intrathoracic tumors are common. They may originate in the Iung, in the mediastinum or from the various eIements of the chest waII. By far the most important and frequent intrathoracic neopIasms are tumors of the Iung, which are more properIy termed bronchiogenie tumors because it has been estabIished that practicaIIy a11 Iung tumors are of bronchia origin and rareIy arise from the Iung parenchyma. Second in frequency are the various tumors of the mediastinum, and, third, the reIativeIy rare neopIasms which begin in the chest waI1 and invade the thoracic cavity.
P
BRONCHIOGENIC TUMORS Bronchiogenic tumors have unti1 recentIy been considered a rare form of disease. During the past ten years, however, it has become more and more apparent that the incidence of endobronchia1 neopIasms is increasing and that bronchiogenic carcinoma is one of the most common forms of cancer. At the present time, carcinoma of the Iung is of about the same frequency as carcinoma of the coIon, and constitutes between 5 and IO per cent of a11 cancers. KoIetsky’ in a study of 7,685 consecutive cases studied at autopsy found bronchiogenie carcinoma second onIy to carcinoma of the stomach. Kikuth,2 Hirsche and HaIpert,3 Matz,4 and many others, at postmortem observations, have found carcinoma of the Iung either second or third in frequency, being preceded onIy by carcinoma of the stomach and carcinoma of the large bowe1. Another important observation has been made in various series of autopsy studies, * From the Chest Service of Barnes
nameIy, that more recent autopsy reports demonstrate a progressive increase in the frequency of puImonary tumors. For exampIe, WeIIer5 at the University of Michigan found carcinoma of the lung occurred in o. I per cent of the first 1,000 autopsies performed at that institution. In the second 1,000 autopsies, the incidence was 0.5 per cent, and in 450 cases of the third 1,000 cases studied, the incidence was 0.8 per cent. HoIzeP reports that in autopsy studies conducted between 1895 and 1904, onIy 1.04 per cent of a11 carcinomas discovered at postmortem were bronchiogenic in origin. Corresponding data from 1915 to 1924 showed an increase to 6.9 per cent. Ochsner and DeBakey’ have further emphasized the steady increase of bronchiogenie carcinoma by a review of the death rates per IOO,OOOpopuIation in the United States who have died of maIignant tumors of various kinds and are recorded by the mortaIity statistics of the Bureau of Census. These data show that from rgzo to 1936 there was no appreciabIe increase in the incidence of carcinoma of the stomach, uterus or gaI1 bIadder, whereas there was a marked increase in the incidence of carcinoma of the Iung. The same authors have recentIy reported that in their experience carcinoma of the Iung has increased approximateIy five times in the past seven years. This statement was based on a study of necropsy materia1 at the Charity Hospita1 in New OrIeans. Brines and Kenning8 in a study of 936 patients with various types of tumors found carcinoma of the Iung fourth in frequency. At the Barnes HospitaI Chest Service, sixty-two proven cases of bronchiogenic carcinoma were seen in a
Hospital and the Department of Surgery, of Medicine, St. Louis, Missouri.
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three-year period from 1933 to 1936. During the foIIowing three years, I I 7 bronchiogenie carcinoma cases were admitted for treatment. It becomes apparent, therefore, that the amazing increase in the incidence of bronchiogenic carcinoma cannot be attributed to improvements in diagnosis or increased awareness of the condition, but must be considered actua1 as demonstrated both by autopsy materia1 and cIinica1 observations. It is now generaIIy agreed that practicaIIy a11 puImonary tumors arise from the bronchi. There has, however, been much confusion in cIassification of Iung tumors because of the uncertainty of exact ceIIuIar origin and muItipIicity of names which have been appIied to the various Iesions based on their microscopic appearances. This controversy does not, however, incIude true squamous ceI1 cancer of the bronchus. Like squamous carcinoma in other Iocations, this variety of cancer occurs more frequentIy between the ages of forty and sixty, and is predominantIy a disease of maIes. Other bronchiogenic carcinomas have been designated as round ceI1 carcinomas, oat ceI1 carcinomas, adenocarcinomas, depending upon the various arrangements of the ceIIs. It is beyond the province of this paper to discuss in detai1 various controversia1 conceptions regarding cIass&ation of puImonary tumors. However, it is necessary to mention briefly a few of the recent conceptions of puImonary tumor formation to avoid confusion in the subsequent discussion of the IocaIization of these tumors. EndobronchiaI tumors which are sometimes cIassified as benign are rare as compared to true bronchiogenic carcinomas. The most common of this group is the so-caIIed bronchia adenoma. These tumors may arise in any portion of the bronchia tree, but are more commonIy found in the The anatomic origin of Iarger bronchi. bronchia adenomas may, therefore, be considered identica1 to that of bronchiogenie carcinomas. Unlike squamous ceI1 carcinoma of the bronchus, these tumors
Tumors
OCTOBER,194.1
occur frequentIy in femaIes and may appear at any age. Brunn and GoIdmang report an incidence of 64 per cent in femaIes in fortytwo coIIected cases. The tumors may be in the shape of a poIyp attached to the bronchia mucosa by a smaI1 pedicIe, but more often the intramura1 type is present where onIy a part of the tumor presents endobronchiaIIy. Not uncommonIy, these tumors assume a dumbbe11 pattern, with the greatest portion of the neopIasm extending outside of the bronchus into the parenchyma of the Iung. Brunn and GoIdmang and others beIieve that the bronchia adenoma has such a distinct difference in appearance and growth potentia1 that it deserves to be cIassified in an entireIy different category from true bronchiogenic carcinoma. There is, however, a sharp difference of opinion on this point, and others, notabIy Womack and Graham,‘O beIieve that many endobronchia1 tumors, incIuding bronchia adenomas, have a common origin, and are the resuIt of the faiIure of embryonic bronchia buds to deveIop in a norma way. When the entodermic eIements in the embryonic bud predominate, the tumor may be considered a bronchia adenoma, aIveoIar carcinoma, adenocarcinoma, etc. Mesodermic domination wiI1 resuIt in what has been caIIed by others chondroma, sarcoma, etc. Their conception is that these tumors shouId be considered potentiaIIy maIignant, aIthough they may be benign when first recognized. FrequentIy, these tumors resembIe the mixed tumors of the salivary gIands and it is not unusua1 to find in them cartiIage and even bone. When maIignant they manifest a markedIy invasive quaIity but often without microscopic evidence of great activity of growth. HaIpert’l expIains the formation of endobronchia1 tumors on the basis of what he terms reserve ceIIs.These are undifferentiated entoderma1 ceIIs capabIe of deveIoping into ciIiated epitheIium, cyIindrica1 ceIIs, gobIet ceIIs, or may differentiate into any kind of epitheIium that an entoderma1 ceI1 is capabIe of producing. Another common classification of bronchio-
NEW SERIESVOL. LIV. No. I
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genie tumors is: (I) Squamous ceII carcinoma; (2) smaI1 ceI1 or undifferentiated The ceI1 carcinoma; (3) endocarcinoma. confusion regarding cIass&ation of bronchiogenic tumors by the microscopic appearance does not, however, affect certain important facts concerning their site of prediIection. For cIinica1 purposes, al1 bronchiogenic tumors can be divided into two groups: (I) Tumors of periphera1 origin, in which the Iesion originates in minor bronchi, or from the periphery of the Iung; (2) neopIasms originating in Iarge or major bronchi. TuttIe and Womack12 have pointed out the differences in symptomatoIogy and prognosis, depending upon whether the tumor originates in the periphery of the Iung or near the puImonary hiIum. They have shown that tumors originating in the major bronchi are more sIowIy growing, probabIy because of the tough cartiIage which must be penetrated if the neopIasm spreads by direct extension. Tumors of Iarge bronchi produce symptoms earIier because obstruction or partia1 obstruction of the bronchus wiI1 resuIt in ateIectasis or puImonary suppuration much earIier in the course of the disease than if the tumor is situated in a smaI1 periphera1 bronchus. In sIightIy more than haIf of the cases studied by TuttIe and Womack12 the tumor was situated in a major bronchus. Boyd13 and Edwards14 found a somewhat higher incidence of centraIIy Iocated tumors, the former finding onIy IO per cent of the tumors Iocated in the periphery, and the Iatter, 20 per cent. In 2 I 6 patients with bronchiogenic carcinoma reported by Gebauer,15 squamous ceI1 carcinomas were situated in the major bronchi in 70 per cent of the cases. Tumors cIassifIed as adenocarcinoma arose in secondary bronchi in go per cent of the cases, and in the main bronchi, in onIy IO per cent. Sixty-six per cent of tumors cIassified by Gebauer15 as smaI1 ceI1 carcinomas began in the main stem bronchi, Controversia1 opinions concerning histoIogic cIass&ations make evaIuation of these
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data diffIcuIt. It is apparent, however, that in genera1 the more sIowIy growing and better differentiated carcinomas have a tendency to arise in the major bronchi, whereas the rapidIy growing, undifferentiated varieties of cancer usuaIIy originate in the smaIIer bronchi. The reIative frequency of invoIvement of the right and left Iung does not vary enough to be of great practica1 importance. Fischerl” studied 3,735 cases and found the right Iung involved in 53 per cent of the cases, the Ieft in 45 per cent, and a biIatera1 Iesion overriding the carina in 2 per cent. Ochsner and DeBakey’ coIIected 4,732 cases from the Iiterature, and found rightsided invoIvement in 58.4 per cent, and Ieft sided tumors in 41.6 per cent. In 784 of Fischer’sI cases, 18 per cent of the tumors originated in the right main bronchus, and 14 per cent of the tumors were in the Ieft main bronchus. The most frequent site of origin was in the right upper Iobe bronchus, where approximateIy Ig per cent of the tumors were situated. The Ieft upper Iobe bronchus was the site of origin in 16.4 per cent of cases, the right Iower lobe in 16 per cent, the Ieft Iower Iobe in 13 per cent, and onIy in approximateIy 0.2 of the cases was the middIe Iobe bronchus invoIved. It is interesting to note that the superior divisions or dorsa1 divisions of the Iower Iobes are an infrequent site of tumor formation. In a series of I 17 cases at the Barnes HospitaI, we have seen onIy one instance of bronchiogenic carcinoma originating in this bronchia segment. This finding is particuIarIy interesting because the superior divisions of the Iower Iobe are probabIy more frequentIy invoIved in Iung abscess formation than any other portion of the Iung. Posterior Superior S&us Tumors. Tumors originating in the posterior superior suIcus of the thorax at the apex of the chest often produce a rather typica symptom compIex consisting of uniIatera1 pain in the shouIder girdIe, Horner’s syndrome, and paresis of the hand. Roentgenograms wiI1 usuaIIy_show a mass confined to the posterior superior sulcus of the chest when these
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of Surgery
symptoms are present. Pancoast17 first described the symptom compIex associated with the abnormaI roentgenographic shadow, and it became the custom to refer to the condition as a Pancoast tumor. Ray’* and others who have studied the so-caIIed Pancoast tumor and its associated symptom compIex are convinced that in the great majority of cases, the symptoms and roentgenographic findings are the resuIt of invasion of the pIeura by an ordinary bronchiogenic carcinoma and that neurofibromas, or any other tumor situated in the posteromesia1 portion of the chest, are capabIe of producing the same symptoms. It wouId seem, therefore, more proper to regard the term, Pancoast tumor, as a topica cIassification rather than a pathoIogic entity and to mention the condition in connection with bronchiogenic carcinomas. RARE
CONDITIONS PULMONARY
WHICH
SIMULATE
TUMORS
Tuberculomas or caIcified puImonary abscesses may be indistinguishabIe from true puImonary neopIasms unti1 they are removed. Graham and SingerIg reported three such cases in 1936, and Iater HaightzO reported a caIcified pseudotumor of the Iung which proved to be a tubercuIoma. Thoracic aneurysms may aIso be confused with intrathoracic neopIasms, aIthough usuaIIy the true nature of the Iesion can be determined by roentgenoIogica1 examination. Grahamzl has reported two instances of nonpuIsating aneurysms of the ductus arteriosus in which the positive diagnosis couId be estabIished onIy by expIoration of the thorax. In two other cases at the Barnes HospitaI, thoracic expIoration was necessary to estabIish a positive diagnosis of aortic aneurysm in which puIsation couId not be determined by ffuoroscopy or by roentgen kymograms. MEDIASTINAL
TUMORS
Tumors of the mediastinum may be benign or maIignant, but, except for rather rare cystic types of tumors, practicaIIy a11
OCTOAER. 1941
Tumors
mediastina1 tumors are potentiaIIy malignant. The site of origin of mediastina1 neopIasms is rather constant for each group, and a convenient method for describing them is to divide the various types into: (I) Tumors which usuaIIy originate in the anterior mediastinum; (2) posterior mediastina1 tumors; (3) a misceIIaneous group of comparatively rare neopIasms which may be Iocated in any part of the mediastinum. ANTERIOR
MEDIASTINAL
TUMORS
Lymphomas. A common variety of anterior mediastina1 tumor arises from Iymphatic tissue, and may be cIassified under the inclusive term of Iymphomas. This group incIudes Iymphosarcoma, Hodgkin’s disease, so-caIIed Ieukosarcoma, thymomas or any neopIasm which may originate in the Iymphatic tissue situated in the mediastinum. Differentiation of the tumors of the Iymphoma group from other types of anterior mediastina1 tumors before treatment is undertaken is of primary importance because, in generaI, tumors composed of Iymphatic tissue wiI1 react to roentgenotherapy and are not amenabIe to surgica1 extirpation. It is, therefore, the custom of many experienced thoracic surgeons to submit a11 patients with anterior mediastina tumors to a test of roentgenotherapy. If the neopIasm decreases in size, the futiIity of surgica1 intervention is estabIished. Confusion and controversy regarding the origin and relative frequency of tumors which arise from the Iymphoid tissue in the mediastinum make a reliabIe estimate of the reIative frequency of the various types of Iymphomas impossibIe. BaIdridge and Awe 22 have a workabIe CIassification for Iymphogenous tissue, recognizing as the Hodgkin granuIoma a scIerosing type of Iesion, the IymphobIastic type as Iymphosarcoma and the endothelia1 type as IymphoepitheIioma. The Iymphocytic type, they consider a manifestation of Ieukemia with or without bIood changes. Ewing’sz3 cIassification is based on three anatomic eIements, nameIy, Iymphocytes,
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retie :uIum ceIIs of the foIIicIes and puIp, and ceIIs. It is, however, often endc )theIiaI histoIogicaIJy the imps IssibIe to cIassify
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sarcoma, and twenty-nine tas Hodgl sin’s granuIoma. In a series of eighl ty-seven c:ases of mediastina1 tumors studied at the Ba rnes
FIG. I. LateraI roentgenogram showing anterior position of the mediastinum.
Iymphomas of the anterior mediastinum which do not have other genera1 manifestations of disease of the Iymphatic system, or in which tissue is not avaiIabIe for biopsy. The same diffrcuIties exist when one attempts to cIassify tumors of the thymus gIand. Heuer and Andrus24 have been abIe to cohect 230 cases of maIignant thymomas. In their series of 144 cases of mediastinaJ tumors, studied at the New York Hospital, 32 per cent were considered Iymphomas. Of these, eighteen were diagnosed Iympho-
American
of a teratoid turn01
HospitaI, in thirty-eight instances the tumor was cIassified as a Iymphoma. In this group there was no other evidence of disease of the Iymphatic system which could be demonstrated by invoIvement of other Iymph-nodes or by changes in hemograms. In a11 instances the Iesion diminished in size when roentgenotherapy was given. Dermoids and Teratomas. Harringtonz5 empIovs the term, teratoid tumors, for al1 dermoids and teratomas of the mediastinum. He feeIs that it is more accurate
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than the incIusive term, dermoid tumors, because the majority of these neopIasms are derived from al1 three germinal Iayers.
FIG. 2. Photograph of teratoma of the mediastinum after successful surgical removd.
The structures of the teratoid tumors vary from thin waIIed cysts fiIIed with cIoudy fluid, or which may contain ectoderma1 derivatives such as skin, hair, or teeth, to soIid tumors, depending upon the varieties of tissue which predominate. Heuer24 has stated that the teratoid mediastina1 tumors are the most common neopIasms of the mediastinum, but at the Barnes HospitaI Chest Service, we have found that tumors of the Iymphoma group occur more frequentIv than the teratoid variety. With the exception of the Iymphomas, however, the incidence of teratoid tumors was more than twice that of any other kind of mediastina1 neopIasm. Of 233 coIIected cases, incIuding twenty-five at the Barnes Hospital, in onIy three instances has the tumor been reported to be in a posterior position in the mediastinum. These were in the cases of Krause,26 Von Torok,27 and of Smith.28 In a series of I 16 cases studied by HedbIom,2g a11 of the tumors were in an anterior position. The mass was predominantIy rightsided in sixty-three instances, and more apparent on the Ieft in fifty-three cases. The teratoid tumors are thought to take their origin from ceI1 rests probabIy of
Tumors
Oc~osaR.,941
branchiogenic eIements which are drawn into the thorax by the descent of the heart and diaphragm. (Figs. I and 2.) Mediastinal Thyroids. Thyroid tissue situated in a substerna position may produce an anterior mediastina1 tumor which cannot be diagnosed definiteIy unti1 the mass has been removed. In the great majority of cases, however, invoIvement of the thyroid tissue in the neck is rather obvious and wiI1 indicate the true nature of the Iesion. TotaI or compIete thoracic goiter may occur without cervica1 goiter, but this condition is rare. Cysts of the Mediastinum. Thin waIIed cysts containing fluid have been given a variety of names incIuding cystic hygromas, cystic Iymphangiomas, and ciliated epitheIia1 cysts. Cysts of the respiratory epitheIium are often caIIed bronchiogenic cysts. LamberP has recentIy introduced chnvincing evidence that many cysts of the mediastinum arise from the pericardium through the defects in the process of its deveIopment. Forty-one instances of mediastina1 cysts cIassifIed as either cystic bronchiogenic cysts or cystic hygromas, Iymphangiomas are recorded in the Iiterature. Five cases seen at the Barnes HospitaI in which operation was performed successfuIIy have been recIassified and are considered to originate from the pericardium, since Lambert’s30 conception of their origin has been known. POSTERIOR
MEDIASTINAL
TUMORS
The most important group of neopIasms which arise from the posterior mediastinum are the primary nerve tumors. These neopIasms take origin from any of the eIements of nerve tissue and may, therefore, be more preciseIy cIassified as gangIioneuromas, neurogenic sarcomas, neurofibromas, sympathicobIastoma, etc., depending upon the ceIIuIar eIement which predominates. Harrington 25has empIoyed the term perineura1 fibroblastoma in describing fourteen primary nerve tumors which he has treated surgicaIIy. He justifies the term perineura1 fibrobIastoma by the fact that in each
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instance the tumor was thought to arise from the sheath of the nerves. Thirteen of the group were benign tumors and one was
FIG. 3. Lateral
roentgenogram
showing
Tumors
Journal
of Surgery
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exactIy the incidence of each tY pe of primary nerve tumor which is ret on ded in the Iiterature. Including thirtet :n cases
usuaI posterior tumor.
considered maIignant. PracticaIIy a11 primary nerve tumors have the common factor of being Iocated in the posterior mediastinum and of being maIignant or potentiaIIy mahgnant. Not infrequentIy, tumors which arise from the spina or sympathetic nerves invoIve not onIy the posterior mediastinum but the vertebra1 canaI, and assume an hour-gIass or dumbbe11 shape. DifficuIties in exact histoIogica1 cIassification make it impossibIe to evaIuate
American
position
of a primary
nerve
treated surgicaIIy at the Barnes HospitaI, 135 instances of neurogenic tumors of the posterior mediastinum are recorded. Twenty-two of these are considered neurofibromas, seventy gangIioneuromas, nineteen are considered neurinomas, (recorded by Heuer and Andrus) 24 and the remaining twenty-four as perineura1 GbrobIastomas or simpIy as primary nerve tumors. Not infrequentIy, neurofibromas of the mediastinum may be associated with other stigmata of Von ReckIinghausen’s disease. This was the
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case in one of our thirteen cases, and the condition has been recorded frequentIy in the literature. (Figs. 3 and 4.)
Tumors
Carcinoma of the Mediastinum. Since it has been appreciated that puImonary carcinoma is common and often invades the
FIG. 4. Photograph of neurofibroma of posterior tinum after successful removal. RARE
MEDIASTINAL
TUMORS
Mediastinal Fibromas and Sarcomas. Thirty-two cases of fibroma of the mediastinum have been reported. In many instances, however, the diagnosis couId be questioned, and it seems IikeIy that confusion with fibrosarcoma or neurofibromas. had occurred in many instances. The same diffIcuIty arises when one attempts to evaIuate the misceIIaneous group of tumors incIuded under the diagnosis of sarcoma of the mediastinum. These may arise from the maIignant degeneration of chondromas, primary nerve tumors, or from any of the connective tissue eIements of the Iymphnodes or of the thymus. In our experience, it is often impossibIe to make an exact histoIogic cIass&ation of rapidIy growing mediastina1 sarcomas. In many instances, however, the positions and cIinica1 aspects of the neopIasms have suggested that their origins were either from nerve tissue or the periosteum of the ribs.
Ocroeen,,941
medias-
mediastinum, fewer cases of carcinoma of the mediastinum have been reported. There is evidence that the thymus gIand is the most common source of primary carcinomas of the mediastinum. In many it is impossibIe to instances, however, establish the exact origin. By far the most common cause of invasion of the mediastinum with carcinoma is extension or metastasis from a primary bronchiogenic tumor. Mediastinal Tumors Arising from Bone and Cartilage. Chondromas, osteochondromas, chondromyxomas, enchondromas or chondromyxosarcomas may take their origin from the Costa1 cartiIages, sternum or spine and present in the mediastinum. AI1 varieties are rare. Chondromas are more common in the anterior mediastinum arising from the Costa1 cartiIages, and a11 others of the group are more commonIy Iocated in a posterior position. Heuer2” was abIe to coIIect only fourteen cases of mediastina1 tumor which might be considered to originate from bone or cartiIage.
NEW Snnr~s Var. LIV, No. I
Intrathoracic
BIades-Intrathoracic
Cysts of Intestinal Structure.
NichoIls,31 of London, has reported the successfu1 remova of an intrathoracic stomach and was abIe to coIIect tweIve cases of intrathoracic cysts of intestina1 structure from the Iiterature. Graham32 at the Barnes HospitaI has had two cases of intrathoracic stomachs, one in an infant, and the other in a young man in whom the tumor of the mediastinum was discovered during an examination for admission to service in the army. In this case, the intrathoracic stomach was removed successfuIIy, and the patient has now been admitted to miIitary service. Two expIanations have been advanced for the deveIopment of intrathoracic tumors of intestina1 structure: (I) That they are ceIIs from the foregut which remain in the thorax during the course of deveIopment; (2) that the ceIIs are derived from the viteIIo-intestina1 duct. In a11 of the cases reported by NichoIIs”’ and in Graham’s32 two cases, the tumor was Iocated in the posterior mediastinum. TweIve of the fifteen known cases were right-sided. Lipomas of the Mediastinum. Intrathoracic Iipomas may occur in a variety of forms, but are comparativeIy rare. Their anatomic Iocation may be of three types: (I) Tumors in which an intrathoracic mass is continuous with an extrathoracic extension, causing the neopIasm to assume an hour-glass shape; (2) thoracic Iipomas which extend into the neck; (3) Iipomas which Iie entireIy within the thoracic cage. McCorkIe et aI. have been abIe to record onIy thirty-four instances of thoracic Iipoma from the worId Iiterature, incIuding a case of their own. At the Barnes HospitaI we have seen onIy two cases of Iipomas of the mediastinum. Mediastinal Xanthomas. Another unusua1 type of intrathoracic new growth has been caIIed xanthoma by some, and sarcoma by others. The characteristic histoIogic finding in xanthomatous neoplasms is the xanthoma or foam ceI1. PhiIIips34 recorded two cases which he had observed and coIIected 3 simiIar cases from the Iitera-
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American Journnl of Surgery
I47
ture. He considered this type of neopIasm to be nonmaIignant and advised discontinuance of the term sarcomas in describing them. TUMORS
ORIGINATING
IN
THE
CHEST
WALL
It is frequentIy dificuIt to determine from pubIished accounts of intrathoracic tumors whether their origin was actualIy in the mediastinum or from the chest waI1. It is understandabIe why this situation can exist because it is often diffIcuIt to determine this point at operation or autopsy when the tumor is of Iarge size. Tumors may arise from the ribs, the pIeura or the nerves in the chest waI1 and manifest themserves as intrathoracic masses. Benign intrathoracic tumors arising from the chest waI1 are rare. Harper3j was abIe to coIIect sixty chondrcmas, nine fibromas, seven osteotomas, and eIeven benign giant ceI1 tumors reported in the worId literature. These tumors usuaIIy do not invade the thorax but present as externa1 masses. The tendency for a11 chondromas or osteochondromas of the chest waI1 to undergo maIignant degeneration has been we11 estabIished, and many beIieve that a Iarge proportion of cases of intrathoracic sarcomas begin as benign chondromas or neurofibromas. A great variety of tumors which may arise from the pIeura have been described. These may be benign or malignant, primary or metastatic. Fibromas, Iipomas, myxomas, angiomas, various types of sarcomas, incIuding myxosarcoma, round ceI1, spindIe ceII and fibrosarcoma have been described. AI1 of these tumors are rare and require no particuIar comment. EpitheIioma of the pIeura is probabIy a misnomer. Robertson36 has shown that primary maIignant tumors of pleura1 tissue are probabIy aIways sarcomas and that the great majority of so-caIIed endotheiiomas of the pIeura represent extension or impIantations of carcinoma from another primary source, usuaIIy the Iung.
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REFERENCES I. KOLETSKY, S. Primary carcinoma of the lung: a cIinica1 and pathoIogic study of one hundred cases. Arch. Inc. Med., 62: 636, 1938. Vircbows 2. KIKUTH, W. Ueber Lungencarcinom. Arch. j. patb. Anat., 225: 107, 1925. 3. HIRSCHE, H. F. and HALPERT, B. Regional Distribution of Malignant, Neoplasms: The Cancer ProbIem in New Haven. Orange, Conn., 1935. Wilson H. Lee Company. 4. MATZ, P. B. Incidence of primary bronchogenic carcinoma. J. A. M. A., I I I : 2086, 1938. 5. WELLER, C. V. Pathology of primary carcinoma of the Iung, Arch. Patb., 7: 478, 1929. 6. HOLZER, H. Zur Frage der Haufigkeit des Bronchialkrebses. Med. Klin., 21: 1235, 1925. 7. OCHSNER, A. and DEBAKEY, M. Carcinoma of Iung. Arch. Surg., 42: 209, 1941. 8. BRINES, 0. A. and KENNING, J. C.:Bronchogenic carcinoma. Am. J. Ch. Path., 7: 120, 1937. 9. BRUNN, H. and GOLDMAN, A. The differentiation of benign from malignant polypoid bronchial tumors. SUrg., GyneC. &’ Obst., 71: 703, 1940. IO. WOMACK, N. A. and GRAHAM, E. A. Mixed tumors of the lung. Artb. Patb., 26: 165, 1938. II. HALPERT, B. PathoIogic aspects of bronchiogenic carcinoma. New Orleans M. c~ S. J., 91: 439, 1939. IZ. TUTTLE, W. McC. and WOMACK, N. A. Bronchiogenie carcinoma: a classification in reIation to treatment and prognosis. J. Tboracic Surg., 4: 125, 1934. 13. BOYD, W. Notes on pathoIogy of primary carcinoma of Iung. Canad. M. A. J., 23: 210, 1930. 14. EDWARDS, A. T. Cited by Crafoord. MaIignant disease of the Iung. J. Tboracic Surg., 4: 107, 1934; Tumors of the lung. Brit. J. Surg., 26: 166, 1938. Cancer of the Iungs and pIeurae. Practitioner, 143: 29, 1939. 15. GEBAUER, P. W. A bronchoscopic mirror for upper Iobe VisuaIization. J. Tboracic Surg., 9: 89, 1939.
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16. FISCHER, W., HARKE, F. and LUBARSCH, 0. Handbuch der Speziellen Pathologischen Anatomie and HistoIogie. BerIin, 193 I. Julius Springer. 17. PANCOAST, H. K. and PENDERGRASS,E. P. Am. J. Roentgenol., 27: 357, 1932. 18. RAY, B. Surg., Gynec.
FREQUENCY
AND SITE OF PREDILECTION
OF INTRATHORACIC
TUMORS*
BRIAN BLADES, M.D. ST. LOUIS, MISSOURI RIMARY intrathoracic tumors are common. They may originate in the Iung, in the mediastinum or from the various eIements of the chest waII. By far the most important and frequent intrathoracic neopIasms are tumors of the Iung, which are more properIy termed bronchiogenie tumors because it has been estabIished that practicaIIy a11 Iung tumors are of bronchia origin and rareIy arise from the Iung parenchyma. Second in frequency are the various tumors of the mediastinum, and, third, the reIativeIy rare neopIasms which begin in the chest waI1 and invade the thoracic cavity.
P
BRONCHIOGENIC TUMORS Bronchiogenic tumors have unti1 recentIy been considered a rare form of disease. During the past ten years, however, it has become more and more apparent that the incidence of endobronchia1 neopIasms is increasing and that bronchiogenic carcinoma is one of the most common forms of cancer. At the present time, carcinoma of the Iung is of about the same frequency as carcinoma of the coIon, and constitutes between 5 and IO per cent of a11 cancers. KoIetsky’ in a study of 7,685 consecutive cases studied at autopsy found bronchiogenie carcinoma second onIy to carcinoma of the stomach. Kikuth,2 Hirsche and HaIpert,3 Matz,4 and many others, at postmortem observations, have found carcinoma of the Iung either second or third in frequency, being preceded onIy by carcinoma of the stomach and carcinoma of the large bowe1. Another important observation has been made in various series of autopsy studies, * From the Chest Service of Barnes
nameIy, that more recent autopsy reports demonstrate a progressive increase in the frequency of puImonary tumors. For exampIe, WeIIer5 at the University of Michigan found carcinoma of the lung occurred in o. I per cent of the first 1,000 autopsies performed at that institution. In the second 1,000 autopsies, the incidence was 0.5 per cent, and in 450 cases of the third 1,000 cases studied, the incidence was 0.8 per cent. HoIzeP reports that in autopsy studies conducted between 1895 and 1904, onIy 1.04 per cent of a11 carcinomas discovered at postmortem were bronchiogenic in origin. Corresponding data from 1915 to 1924 showed an increase to 6.9 per cent. Ochsner and DeBakey’ have further emphasized the steady increase of bronchiogenie carcinoma by a review of the death rates per IOO,OOOpopuIation in the United States who have died of maIignant tumors of various kinds and are recorded by the mortaIity statistics of the Bureau of Census. These data show that from rgzo to 1936 there was no appreciabIe increase in the incidence of carcinoma of the stomach, uterus or gaI1 bIadder, whereas there was a marked increase in the incidence of carcinoma of the Iung. The same authors have recentIy reported that in their experience carcinoma of the Iung has increased approximateIy five times in the past seven years. This statement was based on a study of necropsy materia1 at the Charity Hospita1 in New OrIeans. Brines and Kenning8 in a study of 936 patients with various types of tumors found carcinoma of the Iung fourth in frequency. At the Barnes HospitaI Chest Service, sixty-two proven cases of bronchiogenic carcinoma were seen in a
Hospital and the Department of Surgery, of Medicine, St. Louis, Missouri.
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three-year period from 1933 to 1936. During the foIIowing three years, I I 7 bronchiogenie carcinoma cases were admitted for treatment. It becomes apparent, therefore, that the amazing increase in the incidence of bronchiogenic carcinoma cannot be attributed to improvements in diagnosis or increased awareness of the condition, but must be considered actua1 as demonstrated both by autopsy materia1 and cIinica1 observations. It is now generaIIy agreed that practicaIIy a11 puImonary tumors arise from the bronchi. There has, however, been much confusion in cIassification of Iung tumors because of the uncertainty of exact ceIIuIar origin and muItipIicity of names which have been appIied to the various Iesions based on their microscopic appearances. This controversy does not, however, incIude true squamous ceI1 cancer of the bronchus. Like squamous carcinoma in other Iocations, this variety of cancer occurs more frequentIy between the ages of forty and sixty, and is predominantIy a disease of maIes. Other bronchiogenic carcinomas have been designated as round ceI1 carcinomas, oat ceI1 carcinomas, adenocarcinomas, depending upon the various arrangements of the ceIIs. It is beyond the province of this paper to discuss in detai1 various controversia1 conceptions regarding cIass&ation of puImonary tumors. However, it is necessary to mention briefly a few of the recent conceptions of puImonary tumor formation to avoid confusion in the subsequent discussion of the IocaIization of these tumors. EndobronchiaI tumors which are sometimes cIassified as benign are rare as compared to true bronchiogenic carcinomas. The most common of this group is the so-caIIed bronchia adenoma. These tumors may arise in any portion of the bronchia tree, but are more commonIy found in the The anatomic origin of Iarger bronchi. bronchia adenomas may, therefore, be considered identica1 to that of bronchiogenie carcinomas. Unlike squamous ceI1 carcinoma of the bronchus, these tumors
Tumors
OCTOBER,194.1
occur frequentIy in femaIes and may appear at any age. Brunn and GoIdmang report an incidence of 64 per cent in femaIes in fortytwo coIIected cases. The tumors may be in the shape of a poIyp attached to the bronchia mucosa by a smaI1 pedicIe, but more often the intramura1 type is present where onIy a part of the tumor presents endobronchiaIIy. Not uncommonIy, these tumors assume a dumbbe11 pattern, with the greatest portion of the neopIasm extending outside of the bronchus into the parenchyma of the Iung. Brunn and GoIdmang and others beIieve that the bronchia adenoma has such a distinct difference in appearance and growth potentia1 that it deserves to be cIassified in an entireIy different category from true bronchiogenic carcinoma. There is, however, a sharp difference of opinion on this point, and others, notabIy Womack and Graham,‘O beIieve that many endobronchia1 tumors, incIuding bronchia adenomas, have a common origin, and are the resuIt of the faiIure of embryonic bronchia buds to deveIop in a norma way. When the entodermic eIements in the embryonic bud predominate, the tumor may be considered a bronchia adenoma, aIveoIar carcinoma, adenocarcinoma, etc. Mesodermic domination wiI1 resuIt in what has been caIIed by others chondroma, sarcoma, etc. Their conception is that these tumors shouId be considered potentiaIIy maIignant, aIthough they may be benign when first recognized. FrequentIy, these tumors resembIe the mixed tumors of the salivary gIands and it is not unusua1 to find in them cartiIage and even bone. When maIignant they manifest a markedIy invasive quaIity but often without microscopic evidence of great activity of growth. HaIpert’l expIains the formation of endobronchia1 tumors on the basis of what he terms reserve ceIIs.These are undifferentiated entoderma1 ceIIs capabIe of deveIoping into ciIiated epitheIium, cyIindrica1 ceIIs, gobIet ceIIs, or may differentiate into any kind of epitheIium that an entoderma1 ceI1 is capabIe of producing. Another common classification of bronchio-
NEW SERIESVOL. LIV. No. I
Blades-Intrathoracic
genie tumors is: (I) Squamous ceII carcinoma; (2) smaI1 ceI1 or undifferentiated The ceI1 carcinoma; (3) endocarcinoma. confusion regarding cIass&ation of bronchiogenic tumors by the microscopic appearance does not, however, affect certain important facts concerning their site of prediIection. For cIinica1 purposes, al1 bronchiogenic tumors can be divided into two groups: (I) Tumors of periphera1 origin, in which the Iesion originates in minor bronchi, or from the periphery of the Iung; (2) neopIasms originating in Iarge or major bronchi. TuttIe and Womack12 have pointed out the differences in symptomatoIogy and prognosis, depending upon whether the tumor originates in the periphery of the Iung or near the puImonary hiIum. They have shown that tumors originating in the major bronchi are more sIowIy growing, probabIy because of the tough cartiIage which must be penetrated if the neopIasm spreads by direct extension. Tumors of Iarge bronchi produce symptoms earIier because obstruction or partia1 obstruction of the bronchus wiI1 resuIt in ateIectasis or puImonary suppuration much earIier in the course of the disease than if the tumor is situated in a smaI1 periphera1 bronchus. In sIightIy more than haIf of the cases studied by TuttIe and Womack12 the tumor was situated in a major bronchus. Boyd13 and Edwards14 found a somewhat higher incidence of centraIIy Iocated tumors, the former finding onIy IO per cent of the tumors Iocated in the periphery, and the Iatter, 20 per cent. In 2 I 6 patients with bronchiogenic carcinoma reported by Gebauer,15 squamous ceI1 carcinomas were situated in the major bronchi in 70 per cent of the cases. Tumors cIassifIed as adenocarcinoma arose in secondary bronchi in go per cent of the cases, and in the main bronchi, in onIy IO per cent. Sixty-six per cent of tumors cIassified by Gebauer15 as smaI1 ceI1 carcinomas began in the main stem bronchi, Controversia1 opinions concerning histoIogic cIass&ations make evaIuation of these
Tumors
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141
data diffIcuIt. It is apparent, however, that in genera1 the more sIowIy growing and better differentiated carcinomas have a tendency to arise in the major bronchi, whereas the rapidIy growing, undifferentiated varieties of cancer usuaIIy originate in the smaIIer bronchi. The reIative frequency of invoIvement of the right and left Iung does not vary enough to be of great practica1 importance. Fischerl” studied 3,735 cases and found the right Iung involved in 53 per cent of the cases, the Ieft in 45 per cent, and a biIatera1 Iesion overriding the carina in 2 per cent. Ochsner and DeBakey’ coIIected 4,732 cases from the Iiterature, and found rightsided invoIvement in 58.4 per cent, and Ieft sided tumors in 41.6 per cent. In 784 of Fischer’sI cases, 18 per cent of the tumors originated in the right main bronchus, and 14 per cent of the tumors were in the Ieft main bronchus. The most frequent site of origin was in the right upper Iobe bronchus, where approximateIy Ig per cent of the tumors were situated. The Ieft upper Iobe bronchus was the site of origin in 16.4 per cent of cases, the right Iower lobe in 16 per cent, the Ieft Iower Iobe in 13 per cent, and onIy in approximateIy 0.2 of the cases was the middIe Iobe bronchus invoIved. It is interesting to note that the superior divisions or dorsa1 divisions of the Iower Iobes are an infrequent site of tumor formation. In a series of I 17 cases at the Barnes HospitaI, we have seen onIy one instance of bronchiogenic carcinoma originating in this bronchia segment. This finding is particuIarIy interesting because the superior divisions of the Iower Iobe are probabIy more frequentIy invoIved in Iung abscess formation than any other portion of the Iung. Posterior Superior S&us Tumors. Tumors originating in the posterior superior suIcus of the thorax at the apex of the chest often produce a rather typica symptom compIex consisting of uniIatera1 pain in the shouIder girdIe, Horner’s syndrome, and paresis of the hand. Roentgenograms wiI1 usuaIIy_show a mass confined to the posterior superior sulcus of the chest when these
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of Surgery
symptoms are present. Pancoast17 first described the symptom compIex associated with the abnormaI roentgenographic shadow, and it became the custom to refer to the condition as a Pancoast tumor. Ray’* and others who have studied the so-caIIed Pancoast tumor and its associated symptom compIex are convinced that in the great majority of cases, the symptoms and roentgenographic findings are the resuIt of invasion of the pIeura by an ordinary bronchiogenic carcinoma and that neurofibromas, or any other tumor situated in the posteromesia1 portion of the chest, are capabIe of producing the same symptoms. It wouId seem, therefore, more proper to regard the term, Pancoast tumor, as a topica cIassification rather than a pathoIogic entity and to mention the condition in connection with bronchiogenic carcinomas. RARE
CONDITIONS PULMONARY
WHICH
SIMULATE
TUMORS
Tuberculomas or caIcified puImonary abscesses may be indistinguishabIe from true puImonary neopIasms unti1 they are removed. Graham and SingerIg reported three such cases in 1936, and Iater HaightzO reported a caIcified pseudotumor of the Iung which proved to be a tubercuIoma. Thoracic aneurysms may aIso be confused with intrathoracic neopIasms, aIthough usuaIIy the true nature of the Iesion can be determined by roentgenoIogica1 examination. Grahamzl has reported two instances of nonpuIsating aneurysms of the ductus arteriosus in which the positive diagnosis couId be estabIished onIy by expIoration of the thorax. In two other cases at the Barnes HospitaI, thoracic expIoration was necessary to estabIish a positive diagnosis of aortic aneurysm in which puIsation couId not be determined by ffuoroscopy or by roentgen kymograms. MEDIASTINAL
TUMORS
Tumors of the mediastinum may be benign or maIignant, but, except for rather rare cystic types of tumors, practicaIIy a11
OCTOAER. 1941
Tumors
mediastina1 tumors are potentiaIIy malignant. The site of origin of mediastina1 neopIasms is rather constant for each group, and a convenient method for describing them is to divide the various types into: (I) Tumors which usuaIIy originate in the anterior mediastinum; (2) posterior mediastina1 tumors; (3) a misceIIaneous group of comparatively rare neopIasms which may be Iocated in any part of the mediastinum. ANTERIOR
MEDIASTINAL
TUMORS
Lymphomas. A common variety of anterior mediastina1 tumor arises from Iymphatic tissue, and may be cIassified under the inclusive term of Iymphomas. This group incIudes Iymphosarcoma, Hodgkin’s disease, so-caIIed Ieukosarcoma, thymomas or any neopIasm which may originate in the Iymphatic tissue situated in the mediastinum. Differentiation of the tumors of the Iymphoma group from other types of anterior mediastina1 tumors before treatment is undertaken is of primary importance because, in generaI, tumors composed of Iymphatic tissue wiI1 react to roentgenotherapy and are not amenabIe to surgica1 extirpation. It is, therefore, the custom of many experienced thoracic surgeons to submit a11 patients with anterior mediastina tumors to a test of roentgenotherapy. If the neopIasm decreases in size, the futiIity of surgica1 intervention is estabIished. Confusion and controversy regarding the origin and relative frequency of tumors which arise from the Iymphoid tissue in the mediastinum make a reliabIe estimate of the reIative frequency of the various types of Iymphomas impossibIe. BaIdridge and Awe 22 have a workabIe CIassification for Iymphogenous tissue, recognizing as the Hodgkin granuIoma a scIerosing type of Iesion, the IymphobIastic type as Iymphosarcoma and the endothelia1 type as IymphoepitheIioma. The Iymphocytic type, they consider a manifestation of Ieukemia with or without bIood changes. Ewing’sz3 cIassification is based on three anatomic eIements, nameIy, Iymphocytes,
NEW 5;ERIBS VOL.. LIV.
No.
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BIades-Intrathoracic
retie :uIum ceIIs of the foIIicIes and puIp, and ceIIs. It is, however, often endc )theIiaI histoIogicaIJy the imps IssibIe to cIassify
Tumors
Journd
of
Surgery
I43
sarcoma, and twenty-nine tas Hodgl sin’s granuIoma. In a series of eighl ty-seven c:ases of mediastina1 tumors studied at the Ba rnes
FIG. I. LateraI roentgenogram showing anterior position of the mediastinum.
Iymphomas of the anterior mediastinum which do not have other genera1 manifestations of disease of the Iymphatic system, or in which tissue is not avaiIabIe for biopsy. The same diffrcuIties exist when one attempts to cIassify tumors of the thymus gIand. Heuer and Andrus24 have been abIe to cohect 230 cases of maIignant thymomas. In their series of 144 cases of mediastinaJ tumors, studied at the New York Hospital, 32 per cent were considered Iymphomas. Of these, eighteen were diagnosed Iympho-
American
of a teratoid turn01
HospitaI, in thirty-eight instances the tumor was cIassified as a Iymphoma. In this group there was no other evidence of disease of the Iymphatic system which could be demonstrated by invoIvement of other Iymph-nodes or by changes in hemograms. In a11 instances the Iesion diminished in size when roentgenotherapy was given. Dermoids and Teratomas. Harringtonz5 empIovs the term, teratoid tumors, for al1 dermoids and teratomas of the mediastinum. He feeIs that it is more accurate
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than the incIusive term, dermoid tumors, because the majority of these neopIasms are derived from al1 three germinal Iayers.
FIG. 2. Photograph of teratoma of the mediastinum after successful surgical removd.
The structures of the teratoid tumors vary from thin waIIed cysts fiIIed with cIoudy fluid, or which may contain ectoderma1 derivatives such as skin, hair, or teeth, to soIid tumors, depending upon the varieties of tissue which predominate. Heuer24 has stated that the teratoid mediastina1 tumors are the most common neopIasms of the mediastinum, but at the Barnes HospitaI Chest Service, we have found that tumors of the Iymphoma group occur more frequentIv than the teratoid variety. With the exception of the Iymphomas, however, the incidence of teratoid tumors was more than twice that of any other kind of mediastina1 neopIasm. Of 233 coIIected cases, incIuding twenty-five at the Barnes Hospital, in onIy three instances has the tumor been reported to be in a posterior position in the mediastinum. These were in the cases of Krause,26 Von Torok,27 and of Smith.28 In a series of I 16 cases studied by HedbIom,2g a11 of the tumors were in an anterior position. The mass was predominantIy rightsided in sixty-three instances, and more apparent on the Ieft in fifty-three cases. The teratoid tumors are thought to take their origin from ceI1 rests probabIy of
Tumors
Oc~osaR.,941
branchiogenic eIements which are drawn into the thorax by the descent of the heart and diaphragm. (Figs. I and 2.) Mediastinal Thyroids. Thyroid tissue situated in a substerna position may produce an anterior mediastina1 tumor which cannot be diagnosed definiteIy unti1 the mass has been removed. In the great majority of cases, however, invoIvement of the thyroid tissue in the neck is rather obvious and wiI1 indicate the true nature of the Iesion. TotaI or compIete thoracic goiter may occur without cervica1 goiter, but this condition is rare. Cysts of the Mediastinum. Thin waIIed cysts containing fluid have been given a variety of names incIuding cystic hygromas, cystic Iymphangiomas, and ciliated epitheIia1 cysts. Cysts of the respiratory epitheIium are often caIIed bronchiogenic cysts. LamberP has recentIy introduced chnvincing evidence that many cysts of the mediastinum arise from the pericardium through the defects in the process of its deveIopment. Forty-one instances of mediastina1 cysts cIassifIed as either cystic bronchiogenic cysts or cystic hygromas, Iymphangiomas are recorded in the Iiterature. Five cases seen at the Barnes HospitaI in which operation was performed successfuIIy have been recIassified and are considered to originate from the pericardium, since Lambert’s30 conception of their origin has been known. POSTERIOR
MEDIASTINAL
TUMORS
The most important group of neopIasms which arise from the posterior mediastinum are the primary nerve tumors. These neopIasms take origin from any of the eIements of nerve tissue and may, therefore, be more preciseIy cIassified as gangIioneuromas, neurogenic sarcomas, neurofibromas, sympathicobIastoma, etc., depending upon the ceIIuIar eIement which predominates. Harrington 25has empIoyed the term perineura1 fibroblastoma in describing fourteen primary nerve tumors which he has treated surgicaIIy. He justifies the term perineura1 fibrobIastoma by the fact that in each
NEW
SERIES VOL. LIV
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BIades-Intrathoracic
instance the tumor was thought to arise from the sheath of the nerves. Thirteen of the group were benign tumors and one was
FIG. 3. Lateral
roentgenogram
showing
Tumors
Journal
of Surgery
‘45
exactIy the incidence of each tY pe of primary nerve tumor which is ret on ded in the Iiterature. Including thirtet :n cases
usuaI posterior tumor.
considered maIignant. PracticaIIy a11 primary nerve tumors have the common factor of being Iocated in the posterior mediastinum and of being maIignant or potentiaIIy mahgnant. Not infrequentIy, tumors which arise from the spina or sympathetic nerves invoIve not onIy the posterior mediastinum but the vertebra1 canaI, and assume an hour-gIass or dumbbe11 shape. DifficuIties in exact histoIogica1 cIassification make it impossibIe to evaIuate
American
position
of a primary
nerve
treated surgicaIIy at the Barnes HospitaI, 135 instances of neurogenic tumors of the posterior mediastinum are recorded. Twenty-two of these are considered neurofibromas, seventy gangIioneuromas, nineteen are considered neurinomas, (recorded by Heuer and Andrus) 24 and the remaining twenty-four as perineura1 GbrobIastomas or simpIy as primary nerve tumors. Not infrequentIy, neurofibromas of the mediastinum may be associated with other stigmata of Von ReckIinghausen’s disease. This was the
146
American
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BIades--1ntrathoracic
case in one of our thirteen cases, and the condition has been recorded frequentIy in the literature. (Figs. 3 and 4.)
Tumors
Carcinoma of the Mediastinum. Since it has been appreciated that puImonary carcinoma is common and often invades the
FIG. 4. Photograph of neurofibroma of posterior tinum after successful removal. RARE
MEDIASTINAL
TUMORS
Mediastinal Fibromas and Sarcomas. Thirty-two cases of fibroma of the mediastinum have been reported. In many instances, however, the diagnosis couId be questioned, and it seems IikeIy that confusion with fibrosarcoma or neurofibromas. had occurred in many instances. The same diffIcuIty arises when one attempts to evaIuate the misceIIaneous group of tumors incIuded under the diagnosis of sarcoma of the mediastinum. These may arise from the maIignant degeneration of chondromas, primary nerve tumors, or from any of the connective tissue eIements of the Iymphnodes or of the thymus. In our experience, it is often impossibIe to make an exact histoIogic cIass&ation of rapidIy growing mediastina1 sarcomas. In many instances, however, the positions and cIinica1 aspects of the neopIasms have suggested that their origins were either from nerve tissue or the periosteum of the ribs.
Ocroeen,,941
medias-
mediastinum, fewer cases of carcinoma of the mediastinum have been reported. There is evidence that the thymus gIand is the most common source of primary carcinomas of the mediastinum. In many it is impossibIe to instances, however, establish the exact origin. By far the most common cause of invasion of the mediastinum with carcinoma is extension or metastasis from a primary bronchiogenic tumor. Mediastinal Tumors Arising from Bone and Cartilage. Chondromas, osteochondromas, chondromyxomas, enchondromas or chondromyxosarcomas may take their origin from the Costa1 cartiIages, sternum or spine and present in the mediastinum. AI1 varieties are rare. Chondromas are more common in the anterior mediastinum arising from the Costa1 cartiIages, and a11 others of the group are more commonIy Iocated in a posterior position. Heuer2” was abIe to coIIect only fourteen cases of mediastina1 tumor which might be considered to originate from bone or cartiIage.
NEW Snnr~s Var. LIV, No. I
Intrathoracic
BIades-Intrathoracic
Cysts of Intestinal Structure.
NichoIls,31 of London, has reported the successfu1 remova of an intrathoracic stomach and was abIe to coIIect tweIve cases of intrathoracic cysts of intestina1 structure from the Iiterature. Graham32 at the Barnes HospitaI has had two cases of intrathoracic stomachs, one in an infant, and the other in a young man in whom the tumor of the mediastinum was discovered during an examination for admission to service in the army. In this case, the intrathoracic stomach was removed successfuIIy, and the patient has now been admitted to miIitary service. Two expIanations have been advanced for the deveIopment of intrathoracic tumors of intestina1 structure: (I) That they are ceIIs from the foregut which remain in the thorax during the course of deveIopment; (2) that the ceIIs are derived from the viteIIo-intestina1 duct. In a11 of the cases reported by NichoIIs”’ and in Graham’s32 two cases, the tumor was Iocated in the posterior mediastinum. TweIve of the fifteen known cases were right-sided. Lipomas of the Mediastinum. Intrathoracic Iipomas may occur in a variety of forms, but are comparativeIy rare. Their anatomic Iocation may be of three types: (I) Tumors in which an intrathoracic mass is continuous with an extrathoracic extension, causing the neopIasm to assume an hour-glass shape; (2) thoracic Iipomas which extend into the neck; (3) Iipomas which Iie entireIy within the thoracic cage. McCorkIe et aI. have been abIe to record onIy thirty-four instances of thoracic Iipoma from the worId Iiterature, incIuding a case of their own. At the Barnes HospitaI we have seen onIy two cases of Iipomas of the mediastinum. Mediastinal Xanthomas. Another unusua1 type of intrathoracic new growth has been caIIed xanthoma by some, and sarcoma by others. The characteristic histoIogic finding in xanthomatous neoplasms is the xanthoma or foam ceI1. PhiIIips34 recorded two cases which he had observed and coIIected 3 simiIar cases from the Iitera-
Tumors
American Journnl of Surgery
I47
ture. He considered this type of neopIasm to be nonmaIignant and advised discontinuance of the term sarcomas in describing them. TUMORS
ORIGINATING
IN
THE
CHEST
WALL
It is frequentIy dificuIt to determine from pubIished accounts of intrathoracic tumors whether their origin was actualIy in the mediastinum or from the chest waI1. It is understandabIe why this situation can exist because it is often diffIcuIt to determine this point at operation or autopsy when the tumor is of Iarge size. Tumors may arise from the ribs, the pIeura or the nerves in the chest waI1 and manifest themserves as intrathoracic masses. Benign intrathoracic tumors arising from the chest waI1 are rare. Harper3j was abIe to coIIect sixty chondrcmas, nine fibromas, seven osteotomas, and eIeven benign giant ceI1 tumors reported in the worId literature. These tumors usuaIIy do not invade the thorax but present as externa1 masses. The tendency for a11 chondromas or osteochondromas of the chest waI1 to undergo maIignant degeneration has been we11 estabIished, and many beIieve that a Iarge proportion of cases of intrathoracic sarcomas begin as benign chondromas or neurofibromas. A great variety of tumors which may arise from the pIeura have been described. These may be benign or malignant, primary or metastatic. Fibromas, Iipomas, myxomas, angiomas, various types of sarcomas, incIuding myxosarcoma, round ceI1, spindIe ceII and fibrosarcoma have been described. AI1 of these tumors are rare and require no particuIar comment. EpitheIioma of the pIeura is probabIy a misnomer. Robertson36 has shown that primary maIignant tumors of pleura1 tissue are probabIy aIways sarcomas and that the great majority of so-caIIed endotheiiomas of the pIeura represent extension or impIantations of carcinoma from another primary source, usuaIIy the Iung.
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oTSorgcry
BIades-Intrathoracic
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Tumors
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