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Mediastinal teratoma simulating adenomatous goiter
Mediastinal Teratoma Simulating Adenomatous Goiter TIMOTHY A. LAMPHIER,
M.D.,
Boston, Massachusetts the number of reported cases to 138. HedbIom [2] compiIed 185 and added six of his own in 1933. In 1936 Andrus and Heuer [3] reported 2 :r7 cases, and in 1944 Rusby [4], in an exceIIent review of the Iiterature, reported 252. Goorwitch and SokoI’s [I] report of 362 cases is the most recent.
ITH the possibIe exception of the Iipoma, tumors are probabIy the most common tumors found in the anterior mediastinum. ActualIy it is not to be inferred that this classification of tumors is frequentIy encountered, for extensive review of the Iiterature brings to Iight Iess than 400 recorded cases. According to Goorwitch and SokoI [I], 362 cases were reported by 1951 in a survey which incIuded European as we11 as American literature.
W teratoid
PATHOLOGY The statement that the origin of the tumor preceded the birth of the individual is probabIy true, for the present Iiterature indicates that the teratoid tumor is a resuIt of an embryoIogic defect. AIthough the tumor is present at birth, the majority are dormant unti1 adoIescence or earIy aduIt life. They are most commonIy seen cIinicaIIy in the third decade. They must not be discounted, however, from a differentia1 diagnosis simpIy on the basis of age, for WheatIey reports a mediastina1 teratoma removed from a seven month oId chiId. The tumor apparentIy has no prediIection for sex or for the side of the chest affected. HedbIom [2] divides teratoid tumors arbitrariIy into three types, depending upon their tissue of origin. They are: (I) epidermoid type, derived compIeteIy from the ectoderm; (2) dermoid type, derived from entoderm and mesoderm; and (3) teratoma, derived from a11 three germ layers. He emphasizes that in many cases the distinction between the types is diffIcuIt or impossibIe to make, and that in reality the majority of the tumors are teratomas incorrectIy IabeIed as dermoids. The teratoid tumors are divided further into two groups, soIid and cystic, with the Iatter the predominant form. The cysts are usuaIIy smooth, gIistening and encIosed in a fibrous capsuIe, but may occasionaIIy be ragged. The cyst may be attached to associated mediastinal
HISTORY The first teratoid tumor of the mediastinum (dermoid cyst) was reported by J. A. Gordon in an address to the Medico-ChirurgicaI Society of London in November, 1823. His address was later published in the 1827 edition of the Society’s Proceedings. He reported that the tumor, located in the anterior mediastinum, contained sebaceous materia1, hair, a bone which bore an extraordinary resembIance to the maxiIIa, and seven teeth. The next mention of such a tumor was by Spath in 1836. He noted that grossIy the growth contained cartiIage, fibrous and fatty tissue, and bone. There was no avaiIabIe microscopic study. Three years Iater Muny pubIished a concise cIinica1 record of such a cyst in the anterior mediastinum of a twentyeight year old woman. There have been occasional reports of this type of tumor from this point on, but it was not unti1 I 869 that NobiIing cIearIy estabIished teratoid tumor as a cIinica1 entity by correctIy diagnosing it in a case in which the chief feature of the patient’s history was expectoration of hair. In 1916 HertzIer coIIected seventy-two cases from the medica Iiterature and added one of his own. By 1928 Kerr and WarfieId increased 455
American
Journar
of Surgery,
Vohme
94, September,
19~7
Lamphier structures or to the sternum by a stalk of variabIe Iength and thickness that must be divided if surgica1 treatment is to be successfu1 and recurrence prevented. Because of the presence of this stalk, a tag of tumor tissue may have to be Ieft behind, but there is no mention of recurrence of the condition or of maIignant degeneration of tissue remaining in this fashion. AIso, the cyst itself may be adherent to surrounding mediastina1 structures. These adhesions may be so thin that they afford the surgeon no great inconvenience, or they may be so strong and heavy that compIete excision is rendered impossibIe because of the danger to surrounding vita1 structures. The inner Iining of the cyst may be of two forms: first, it may bear numerous papiIIomatous projections; or second, the waI1 may be smooth and contain onIy a single hard ridge running the Iength of the growth. From the projections or from the ridge spring the hairs, the presence of which afford an immediate diagnosis of the gross Iesion. The cysts are Iined with stratified squamous, cuboidal or coIumnar epithelium. Sebaceous materia1 and hairs are characteristicaIIy found in the cavity, but cIear yeIIowish or brown fluid may also be found. In the soIid parts of the tumor, a wide variation of tissue may be found. NyIander et a1. [6] Iist the tissues which they were abIe to differentiate microscopicaIIy in their thorough study of a Iarge series of cases. They found nerve tissue, skin, connective tissue, fat, bIood and Iymph vesseIs, Iymphatic tissue, smooth and striated muscle fibers, cartiIage, bone, intestine, respiratory structures, pancreas, Iiver and even placenta. In no case was a structure we11 enough differentiated to be recognizabIe grossIy, but there have been reports in the Iiterature of fuIIy formed bones and teeth. The compIeteIy soIid teratoma is rare, and shows a greater tendency toward maIignant change than does the dermoid. The tissues within them may appear to be we11 differentiated and aduIt, but the potentia1 maIignancy must not be ignored. When found in the younger age groups, the tumor is usuaIIy malignant from the start. MaIignancy in the aduIt is generaIIy accepted as the resuIt of the degeneration of a previousIy benign part, usuaIIy epitheIia1 in nature. About 12 per cent degenerate to maIignancy. The most common maIignant degenerations are either carcinomatous or sarcomatous, or
chorioepitheIiomatous. Metastases occur in the Iungs and the mediastina1 Iymph nodes. No cures have resuIted in those tumors which have undergone maIignant changes. The fact that there may be a chorioepitheIiomatous eIement affords a diagnostic tooI, for the Aschheim-Zondek test is only positive in the male in cases of testicuIar teratoma, extragenita1 teratoma and chorioepitheIioma. The tumor may undergo secondary infection in two ways. First, a fistuIa may be formed between the cyst cavity and the bronchus. This fistuIous tract may Iead to direct infection of the cyst from the outside. Second, the cyst may be infected through the bIood and Iymph streams suppIying the tumor tissue. The tumor grows to an extremeIy Iarge size. Fox and Hospers [7] report a soIid teratoma which weighed g kg. Nandrot [8] aspirated 3 kg. of sebaceous materia1 and Iater excised a tumor, which then weighed 8 kg., making a tota of I I kg. This mediastina1 tumor is the Iargest on record. PATHOGENESIS
Although it is generaIIy agreed that the teratoid tumor is the resuIt of an embryologic defect, the precise etioIogy is stiI1 a matter of conjecture. There have been severa theories advanced, but it is not the purpose of this paper to review a11 of them and weigh their relative merits. One theory hoIds that these tumors arise from rests or dispIacements of bronchogenic ceIIs, probabIy arising from the third and fourth bronchia arches, which are brought into the thorax by the descent of the diaphragm and the heart. As a result, tumor tissue is found in the superior and anterior mediastinum anterior to the great vesseIs and often in contact with the pericardium. It may aIso occur in conjunction with the thymus. The fact that thyroid tissue is so frequentIy found Iends strong support to this theory. Other theories are the germ ceI1 hypothesis, the hypothesis of extragonada1 germ cells, the hypothesis of fertiIized poIar bodies, the hypothesis of dispIaced bIastomeres, the morphogenetic hypothesis, the hypothesis of tissue disIocation with Iiberation of organizers, and the hypothesis of “fetus in fetu.” The highIy differentiated, extremeIy compIex teratoma, which is occasionaIIy found, may we11 be the resuIt of a fertiIized poIar body 456
MediastinaI
Teratoma
Simulating
Goiter
and possibIy a pIeura1 effusion. Often a pulsating mass is seen in the chest. It must be remembered, however, that there may be no signs at a11 unti1 the thorax is x-rayed.
which grows and becomes more or Iess a parasitic fetus, the so-cahed fetus in fetu, or the incIuded twin. CLINICAL
Adenomatous
PICTURE
Many of the symptoms and signs produced by the tumor are directIy referabIe to its growth and pressure on the surrounding structures. According to Rusby [4], a cough which may be dry or productive is the most frequent and outstanding symptom. This cough may be constant, but it is more frequentIy paroxysma and is often brought on by a change in position of the patient. Often, the coughing is Ieast in the sitting position, and is aggravated when the patient Iies down. The sputum may be clear, bIood-tinged, or may contain cIay-Iike particIes and hairs. The Iatter is pathognomonic of mediastina1 teratoma, and is the resuit of the formation of a fistuIa between the tumor cavity and a bronchus. The hairs, if expectorated, are onIy produced after a great deal of coughing. It is thought that the sebaceous materia1 clogs the fistuIa and is only dislodged after an unusuaIIy strong cough. Cough is often associated with dyspnea. Dyspnea is commonIy regarded as being the result of pressure on surrounding Iung tissue or on the large puImonary vesseIs, or both. Associated chest pain, in a large majority of the cases, is pleuritic in nature and is aggravated by movements of the chest. Some patients, however, compIain of a pain which is of angina1 type, with radiation down the uInar aspect of the Ieft arm. Neck veins may become engorged and the face swoIIen if venous obstruction is sufficient. If venous obstruction is marked, ankle edema, ascites and even anasarca may resuIt. According to Rusby [4], paIpitation of the heart is frequent and due to pressure on the heart itseIf or dispIacement of it by the tumor mass. Often an arrhythmia is produced, which may take the form of a paroxysma tachycardia. PupiIIary changes, according to Rusby [4], are quite rare, aIthough Heuer recorded one instance of a fuI1 Horner’s syndrome being produced. There is often pressure on the Ieft recurrent IaryngeaI nerve which gives rise to a husky, hoarse voice. PhysicaIIy, the patient may present duIIness to percussion over the mass, marked fuIIness or protrusion of the affected side of the thorax, diminished to absent voice and breath sounds,
DIAGNOSIS
The diagnosis often is diffrcuIt or impossibIe to make before the tumor is operativeIy excised. There are three procedures, however, which may Iead one to a positive diagnosis before operation. The first of these, which has aIready been mentioned, is a positive AschheimZondek test in the presence of a known tumor mass in the mediastinum. Another is the presence of hairs in the sputum. A third is the aspiration of the contents of the cyst cavity and the examination of the aspirated material for hairs and sebaceous materia1. RoentgenoIogic examination may often give definite diagnostic cIues as to the nature of the pathoIogic process. According to Good [9], in aImost one-half of teratoid tumors there is roentgenoIogic evidence of a caIcific process. This caIcification may be seen in the cyst waI1, in bone formed within the tumor, or in teeth seen in the tumor substance. He cIaims that the presence of a we11 formed tooth is pathognornonic of a teratoid tumor if the Iocation of the tumor is the anterior mediastinum. On x-ray examination, the tumor may be ovoid or IobuIated, with either distinct or indistinct borders. In many cases, especiaIIy those with fistuIa formation, an air-fluid IeveI may be demonstrated in the c,yst cavity. COMPLICATIONS
As has aIready been mentioned, this usuaIIy benign condition may undergo many compIications. For one, the tumor may become infected. This inflammatory process may cause a great increase in size, Ieading to pressure symptoms within the thorax; or it may weaken the cyst waI1 in such a manner as to bring about rupture of the cyst into adjoining structures such as the pericardium, the superior vena cava and the pIeura1 cavity. Inflammatory change may bring about the deveIopment of adhesions to adjacent structures which may cause the surgeon a great dea1 of diffrcuIty. Parts of the tumor may undergo maIignant degeneration. When this happens, there is aImost invariabIy a rapidIy fata termination. 447
Lamphier
FIG. I. Gland-Iike space, lined by pseudostratified ciIiated coIumnar epithelia1 ceIIs, such as are found Iining the tracheobronchial tree (entodermal origin). PhIoxine methyIene blue stain, origina magnification X 160. DIFFERENTIAL
DIAGNOSIS
There are severa factors which must be considered in the diagnosis of this Iesion. Because the condition is usuaIIy manifested by a series of non-specific symptoms, the diagnosis is often impossibIe to make. Tumors of the Iymphoma series, notably Iymphosarcoma and Hodgkin’s disease, must be ruIed out. These respond to deep x-ray treatment and the drugs of the nitrogen mustard series, whereas teratoid growths do not. GangIioneuroma is infrequentIy seen, and is usuaIIy Iocated in the posterior mediastinum when present. Since the teratoid tumor often is seen as a puIsating mass, a syphiIitic . or arterioscIerotic aortic aneurysm must be considered. Wassermann and Hinton tests are an aid here. Often, the Iipoma cannot be excluded and attempts at aspiration yieId nothing. Thymoma is usuaIIy maIignant and is sometimes accompanied by myasthenia gravis. Biopsy is the onIy way to distinguish an occasional huge bronchogenic carcinoma from the teratoid tumor. In areas of the country where Echinococcus granuIosus is prevaIent, the hydatid cyst must be excIuded. Another possibiIity, especiaIIy in the Great Lakes region, is a retrosterna1 goiter. TubercuIosis of the mediastina gIands, xanthoma and neurofibroma are seen aImost excIusiveIy in the posterior medias-
tinum. The possibiIity of a soIitary cyst of the Iung or bronchus and a pericardia1 cyst must be considered. The teratoma shown microscopicaIIy in Figures I to 3 was not located in the mediastinum, but may be regarded as a prototypi: of mediastina teratomas. CASE REPORT
J. R., a twenty-six year oId white man, was originaIIy seen on October zz, 1955. His chief compIaint was a mass in his neck of one year’s duration. For the previous month he had begun to have difTicuIty swaIIowing. He aIso stated that he had been troubIed for the past few weeks with irritabiIity, “jumpiness,” nervousness, etc. Past history was irreIevant except that right inguina1 herniorrophy had been performed in 1952 at an Army HospitaI in Munich, Germany. PhysicaI examination reveaIed a we11 nourished and we11 deveIoped white maIe who appeared to be of the stated age. His weight was 145 pounds. His height was 5 feet, 7 inches and his bIood pressure was 160/1 IO. A grade III enIargement of what was thought to be both IateraI Iobes of the thyroid gIand with a Iarge substerna extension was noted. There were no associated noduIes and the mass was soft,
MediastinaI
Teratoma
SimuIating
Adenomatous
Goiter
FIG. 2. Area of bone formation, set in a matrix of fibrous connective tissue, with bone fragments bordered by a line of osteobIasts (mesodermal origin). PhIoxine methylene blue stain, original magnification X 160.
FIG. 3. Area containing gIial-type ceIIs, as found in the central nervous system. Abundant Iarger ce1I.s resemble astrocytes; smaller ones resemble oligodendrogX 450. lia. PhIoxine methylene bIue stain, original magnification
which was thought to be probably secondary to.cystic degeneration. A basa1 metabolic study was reported as pIus 35, but cIinicaIIy the patient did not appear to be toxic. Therefore, radioactive iodine stud-
ies were carried out and reported as ‘‘within norma limits.” Chest x-rays, especiaIIy of the I;uperior thoracic strait, were reported by Dr. W. Arthur Young as foIIows: “The outIine of the cardio459
Lamphier vascuIar system is not remarkabIe. The bronchia1 pattern is moderateIy prominent in each lung, being more so on the right. There is no x-ray evidence of consohdation, infiItration or pleura1 effusion. Both hilar shadows are somewhat prominent, especiaIIy on the Ieft superiorly, and for this yeason, additiona chest x-rays at some future date shouId be done if cIinicaIIy indicated.” The patient was admitted to the Booth MemoriaI HospitaI, BrookIine, Massachusetts, on October 27, 1936, and on the foIIowing day what was thought to be an adendmatous goiter with cystic degeneration and substerna extension was removed as foIIows: Under adequate inhaIation anesthesia, and with the patient intubated and in the usua1 position for thyroidectomy, the anterior aspect of the neck was prepared with ether and zephiran@ and properIy draped. A Iow coIIar skin incision was made and carried down through the subcutaneous fat and pIatysma muscIe to the anterior IeveI of the deep cervica1 fascia. The upper ffap was then dissected proximaIIy as far as the cricoid cartiIage. The media1 aspect of both sternocIeidomastoid muscIes was then freed up. The deep cervica1 fascia was divided verticaIIy in the midIine, after which the strap muscIes were divided high between Kocher cIamps. It was then reveaIed that the trachea was markedIy deviated to the Ieft because of the Iarge mass invoIving the right IateraI Iobe of the thyroid. The right IateraI Iobe was grasped with doubIe hooks and rotated mediaIIy, after which the superior thyroid poIe was doubly Iigated with heavy catgut. By sharp dissection, the right IateraI Iobe was freed up from the carotid sheath. Extensive adhesions were found between the carotid sheath and the right IateraI lobe. The inferior thyroid artery was Iigated in continuity; then by sIiarp and bIunt dissection the substerna extension was freed up down to the aortic arch. AI1 bIeeding vesseIs were cIamped and Iigated with fine and heavy pIain catgut sutures. Because of persistent oozing, a geIfoam@ pad was pIaced in the defect in the superior thyroid strait and the strap muscles were brought together with Iateral mattress, heavy, pIain catgut sutures. The deep cervica1 fascia was brought together in the midIine with interrupted heavy, plain catgut sutures, and the skin with MicheI cIips. A smaI1 Penrose drain was aIlowed to make its exit from the wound
at the right IateraI poIe of the wound. A dry, steriIe pressure-type dressing was appIied. PostoperativeIy the patient did we11 and he was discharged from the hospita1 improved on his fifth postoperative day. The pathoIogic report was as foIIows: CIinicaI impression : Thyroid adenoma, right, with substerna extension and cystic degeneration. GrossIy, the specimen consisted of a portion of thyroid tissue weighing 26 gm. and incIuded the waI1 of an empty open cyst. RecognizabIe thyroid tissue measured 3.0 by 2.5 by 1.5 cm. The cystic portion measured 4.5 by 4.0 by 2.5 cm. The thyroid tissue was pinkish red and gIistening. The outer surface of the cystic structure was, for the most part, smooth and yeIIowish white. There appeared to be a second and coIIapsed cyst attached to the Iarge cyst noted in conjunction with the thyroid tissue. This cyst measured approximateIy 1.5 cm. in its greatest diameter. MuItipIe sections reveaIed gIistening yeIIowish white tissue between the cyst waI1 and the thyroid. The Iining surface of the Iarger cyst was hemorrhagic and rough. A third cyst was found in the substance of the waI1 of the mass. This was muItiIocuIar in appearance. The waIIs of the IocuIes were composed of a soft yeIIowish green, somewhat geIatinous materia1, and in the cystic area a dirty .grey-brown fluid was encountered. PreIiminary gross diagnosis: Adenoma, thyroid, showing cystic and hemorrhagic necrosis. FinaI diagnosis: Cystic teratoma attached to thyroid and thymic t:ssue compIicated by chronic inff ammation. The teratoma contained recognizabIe pancreatic tissue, smaI1 intestina1 mucosa1 gIands, smooth muscIe, skeIeta1 mu!cIe and fibrous tissue. The Iesion was benign. One year Iater, foIIow-up studies revealed a non-disfiguring Iow coIIar skin scar and no evidence of recurrence. MediastinaI x-rays at this time were reported as normaI. TREATMENT
AI1 authors seem to agree unanimously that surgica1 intervention is the onIy treatment of any use in this condition. Rusby [4] Iists three accepted Iines of surgica1 approach. The first, rib resection and drainage, has IittIe to offer if this is the onIy treatment rendered the patient. The resuIts are poor, but this method is acceptabIe if it is used as a preIiminary to more Second, if the tumor is extensive surgery. 460
MediastinaI
Teratoma
SimuIating
firmly- bound down to adjacent vital structures, the usual procedure is that of partia1 removal with marsupialization. Indeed, this may be the only possible course to take. This step does not preclude the possibility of a more radica1 intervention at a later date. Many surgeons employ this as the first step in a muItipIe stage, resuItremoval of the tumor. If this ing in complete is the onI?- treatment, the resuIts are onIy sIightIy better than those obtained with rib resection and drainage. The surgeon’s aim shouId aIways be compIete excision of the mass. Rusby thinks the transpIeura1 approach is best if concomitant infection is feared or the neopIasm is Iarge. For tumors Iocated in the upper retrosternum, the transverse cervical coIIar incision with spIitting of the sternum is the accepted method. The trap-door incision is effective for a growth Iocated in the thoracic substernal region, but not in the lateral thoracic region. Harrington [ro] is an exponent of the posterior approach, for he beIieves that there is a signihcantIy better exposure obtained in this manner. CONCLUSION
A mediastina1 teratoma is described which preoperativeIy was diagnosed as an adenomatous goiter. NeedIe biopsy of tumors in the neck is to be discouraged as probabIy in this instance an erroneous diagnosis of adenomatous goiter with cystic degeneration wouId have been made if onIy needIe biopsy had been performed. The history, pathoIogy, pathogenesis and cIinica1 picture of mediastina1 teratomas are reviewed.
Adenomatous
Goiter
REFERENCES I. GOORWITCH, J.,
and SOKOL, J. ExtragenitaI (mediastinal) teratocarcinoma. J. Internet. Coil. Surgeons, 17: 343-352, 1952. 2. HEDBLO~U,C. A. Intrathoracic dermoid cysts and teratomata. J. Tboracic Surg., 3: 22, 1933. 1:. ANDRUS. W. DEW. and HEUER. G. J. Sureical treatment of tumors of the mediastinum. Surg., Gynec. ~3 Obst., 63: 469-482, 1936. 4. RUSBY, N. L. Dermoid cysts and teratomata of the mediastinum. J. Tboracic Surg., 13: 169-222, d
1944. 5. WHEATLEY, G. M. MediastinaI teratoma in a chiId seven months oId. Am. J. LG. Child., 54: 10571065, 1937. 6. NYLANDER, P. E. A., TOEVONIN, S., TURUNEN, M. and HJELT, L. Teratoid tumors of the mediastinum. Ann. cbir. et gynaec. Fenniae, 42: 141-156, 1953. 7. Fox, J. P. and HOSPERS, C. A. SoIid teratoid tumors of the anterior mediastinum. Am. J. Cancer,
28: 273, 1936. 8. NANDROT, C. Contribution
a I’etude deskystes dermoides du mediastinum anterieur. Paris, 1937. Thesis. g. GOOD, C. A. The roentgenoIogic diagnosis of surgical Iesions of the mediastinum. Section on Roentgenology, Mayo Clinic, Rochester, Minnesota. Read March 2, 1950, before Annual Clinical Conference of Chicago MedicaI Society. IO. HARRINGTON, S. W. Surgical treatment of intrathoracic tumors and tumors of chest waI1. Arch. Surg., 14: 406-431, 1927. I I. BKINDLEY, G. V. and HENDERSON, J. A. Mediastina tumors. Am. Surgeon, 20: 260-269, 1954. 12. BURNETT, W. E., ROSEMOND, G. P. and BUCHER, R. M. The diagnosis of mediastinal tumors. S. Clin. North America, 32: 1673-1694, 1952. IX. DORAN. W. T. and LESTER. C. W. Mediastinal teratoma. J. Tboracic Surg., 8: 309-315, 1939. 1.4. BLADES, B. ReIative frequency and site of predilection of intrathoracic tumors. Am. J. Sure., 54: r39-148. 1941. 15. THOMPSON, J. V. MediastinaI tumors and cysts. Internat. Abstr. Surg., 84: Ig5-221, 1947. .a
,
M.D.,
Boston, Massachusetts the number of reported cases to 138. HedbIom [2] compiIed 185 and added six of his own in 1933. In 1936 Andrus and Heuer [3] reported 2 :r7 cases, and in 1944 Rusby [4], in an exceIIent review of the Iiterature, reported 252. Goorwitch and SokoI’s [I] report of 362 cases is the most recent.
ITH the possibIe exception of the Iipoma, tumors are probabIy the most common tumors found in the anterior mediastinum. ActualIy it is not to be inferred that this classification of tumors is frequentIy encountered, for extensive review of the Iiterature brings to Iight Iess than 400 recorded cases. According to Goorwitch and SokoI [I], 362 cases were reported by 1951 in a survey which incIuded European as we11 as American literature.
W teratoid
PATHOLOGY The statement that the origin of the tumor preceded the birth of the individual is probabIy true, for the present Iiterature indicates that the teratoid tumor is a resuIt of an embryoIogic defect. AIthough the tumor is present at birth, the majority are dormant unti1 adoIescence or earIy aduIt life. They are most commonIy seen cIinicaIIy in the third decade. They must not be discounted, however, from a differentia1 diagnosis simpIy on the basis of age, for WheatIey reports a mediastina1 teratoma removed from a seven month oId chiId. The tumor apparentIy has no prediIection for sex or for the side of the chest affected. HedbIom [2] divides teratoid tumors arbitrariIy into three types, depending upon their tissue of origin. They are: (I) epidermoid type, derived compIeteIy from the ectoderm; (2) dermoid type, derived from entoderm and mesoderm; and (3) teratoma, derived from a11 three germ layers. He emphasizes that in many cases the distinction between the types is diffIcuIt or impossibIe to make, and that in reality the majority of the tumors are teratomas incorrectIy IabeIed as dermoids. The teratoid tumors are divided further into two groups, soIid and cystic, with the Iatter the predominant form. The cysts are usuaIIy smooth, gIistening and encIosed in a fibrous capsuIe, but may occasionaIIy be ragged. The cyst may be attached to associated mediastinal
HISTORY The first teratoid tumor of the mediastinum (dermoid cyst) was reported by J. A. Gordon in an address to the Medico-ChirurgicaI Society of London in November, 1823. His address was later published in the 1827 edition of the Society’s Proceedings. He reported that the tumor, located in the anterior mediastinum, contained sebaceous materia1, hair, a bone which bore an extraordinary resembIance to the maxiIIa, and seven teeth. The next mention of such a tumor was by Spath in 1836. He noted that grossIy the growth contained cartiIage, fibrous and fatty tissue, and bone. There was no avaiIabIe microscopic study. Three years Iater Muny pubIished a concise cIinica1 record of such a cyst in the anterior mediastinum of a twentyeight year old woman. There have been occasional reports of this type of tumor from this point on, but it was not unti1 I 869 that NobiIing cIearIy estabIished teratoid tumor as a cIinica1 entity by correctIy diagnosing it in a case in which the chief feature of the patient’s history was expectoration of hair. In 1916 HertzIer coIIected seventy-two cases from the medica Iiterature and added one of his own. By 1928 Kerr and WarfieId increased 455
American
Journar
of Surgery,
Vohme
94, September,
19~7
Lamphier structures or to the sternum by a stalk of variabIe Iength and thickness that must be divided if surgica1 treatment is to be successfu1 and recurrence prevented. Because of the presence of this stalk, a tag of tumor tissue may have to be Ieft behind, but there is no mention of recurrence of the condition or of maIignant degeneration of tissue remaining in this fashion. AIso, the cyst itself may be adherent to surrounding mediastina1 structures. These adhesions may be so thin that they afford the surgeon no great inconvenience, or they may be so strong and heavy that compIete excision is rendered impossibIe because of the danger to surrounding vita1 structures. The inner Iining of the cyst may be of two forms: first, it may bear numerous papiIIomatous projections; or second, the waI1 may be smooth and contain onIy a single hard ridge running the Iength of the growth. From the projections or from the ridge spring the hairs, the presence of which afford an immediate diagnosis of the gross Iesion. The cysts are Iined with stratified squamous, cuboidal or coIumnar epithelium. Sebaceous materia1 and hairs are characteristicaIIy found in the cavity, but cIear yeIIowish or brown fluid may also be found. In the soIid parts of the tumor, a wide variation of tissue may be found. NyIander et a1. [6] Iist the tissues which they were abIe to differentiate microscopicaIIy in their thorough study of a Iarge series of cases. They found nerve tissue, skin, connective tissue, fat, bIood and Iymph vesseIs, Iymphatic tissue, smooth and striated muscle fibers, cartiIage, bone, intestine, respiratory structures, pancreas, Iiver and even placenta. In no case was a structure we11 enough differentiated to be recognizabIe grossIy, but there have been reports in the Iiterature of fuIIy formed bones and teeth. The compIeteIy soIid teratoma is rare, and shows a greater tendency toward maIignant change than does the dermoid. The tissues within them may appear to be we11 differentiated and aduIt, but the potentia1 maIignancy must not be ignored. When found in the younger age groups, the tumor is usuaIIy malignant from the start. MaIignancy in the aduIt is generaIIy accepted as the resuIt of the degeneration of a previousIy benign part, usuaIIy epitheIia1 in nature. About 12 per cent degenerate to maIignancy. The most common maIignant degenerations are either carcinomatous or sarcomatous, or
chorioepitheIiomatous. Metastases occur in the Iungs and the mediastina1 Iymph nodes. No cures have resuIted in those tumors which have undergone maIignant changes. The fact that there may be a chorioepitheIiomatous eIement affords a diagnostic tooI, for the Aschheim-Zondek test is only positive in the male in cases of testicuIar teratoma, extragenita1 teratoma and chorioepitheIioma. The tumor may undergo secondary infection in two ways. First, a fistuIa may be formed between the cyst cavity and the bronchus. This fistuIous tract may Iead to direct infection of the cyst from the outside. Second, the cyst may be infected through the bIood and Iymph streams suppIying the tumor tissue. The tumor grows to an extremeIy Iarge size. Fox and Hospers [7] report a soIid teratoma which weighed g kg. Nandrot [8] aspirated 3 kg. of sebaceous materia1 and Iater excised a tumor, which then weighed 8 kg., making a tota of I I kg. This mediastina1 tumor is the Iargest on record. PATHOGENESIS
Although it is generaIIy agreed that the teratoid tumor is the resuIt of an embryologic defect, the precise etioIogy is stiI1 a matter of conjecture. There have been severa theories advanced, but it is not the purpose of this paper to review a11 of them and weigh their relative merits. One theory hoIds that these tumors arise from rests or dispIacements of bronchogenic ceIIs, probabIy arising from the third and fourth bronchia arches, which are brought into the thorax by the descent of the diaphragm and the heart. As a result, tumor tissue is found in the superior and anterior mediastinum anterior to the great vesseIs and often in contact with the pericardium. It may aIso occur in conjunction with the thymus. The fact that thyroid tissue is so frequentIy found Iends strong support to this theory. Other theories are the germ ceI1 hypothesis, the hypothesis of extragonada1 germ cells, the hypothesis of fertiIized poIar bodies, the hypothesis of dispIaced bIastomeres, the morphogenetic hypothesis, the hypothesis of tissue disIocation with Iiberation of organizers, and the hypothesis of “fetus in fetu.” The highIy differentiated, extremeIy compIex teratoma, which is occasionaIIy found, may we11 be the resuIt of a fertiIized poIar body 456
MediastinaI
Teratoma
Simulating
Goiter
and possibIy a pIeura1 effusion. Often a pulsating mass is seen in the chest. It must be remembered, however, that there may be no signs at a11 unti1 the thorax is x-rayed.
which grows and becomes more or Iess a parasitic fetus, the so-cahed fetus in fetu, or the incIuded twin. CLINICAL
Adenomatous
PICTURE
Many of the symptoms and signs produced by the tumor are directIy referabIe to its growth and pressure on the surrounding structures. According to Rusby [4], a cough which may be dry or productive is the most frequent and outstanding symptom. This cough may be constant, but it is more frequentIy paroxysma and is often brought on by a change in position of the patient. Often, the coughing is Ieast in the sitting position, and is aggravated when the patient Iies down. The sputum may be clear, bIood-tinged, or may contain cIay-Iike particIes and hairs. The Iatter is pathognomonic of mediastina1 teratoma, and is the resuit of the formation of a fistuIa between the tumor cavity and a bronchus. The hairs, if expectorated, are onIy produced after a great deal of coughing. It is thought that the sebaceous materia1 clogs the fistuIa and is only dislodged after an unusuaIIy strong cough. Cough is often associated with dyspnea. Dyspnea is commonIy regarded as being the result of pressure on surrounding Iung tissue or on the large puImonary vesseIs, or both. Associated chest pain, in a large majority of the cases, is pleuritic in nature and is aggravated by movements of the chest. Some patients, however, compIain of a pain which is of angina1 type, with radiation down the uInar aspect of the Ieft arm. Neck veins may become engorged and the face swoIIen if venous obstruction is sufficient. If venous obstruction is marked, ankle edema, ascites and even anasarca may resuIt. According to Rusby [4], paIpitation of the heart is frequent and due to pressure on the heart itseIf or dispIacement of it by the tumor mass. Often an arrhythmia is produced, which may take the form of a paroxysma tachycardia. PupiIIary changes, according to Rusby [4], are quite rare, aIthough Heuer recorded one instance of a fuI1 Horner’s syndrome being produced. There is often pressure on the Ieft recurrent IaryngeaI nerve which gives rise to a husky, hoarse voice. PhysicaIIy, the patient may present duIIness to percussion over the mass, marked fuIIness or protrusion of the affected side of the thorax, diminished to absent voice and breath sounds,
DIAGNOSIS
The diagnosis often is diffrcuIt or impossibIe to make before the tumor is operativeIy excised. There are three procedures, however, which may Iead one to a positive diagnosis before operation. The first of these, which has aIready been mentioned, is a positive AschheimZondek test in the presence of a known tumor mass in the mediastinum. Another is the presence of hairs in the sputum. A third is the aspiration of the contents of the cyst cavity and the examination of the aspirated material for hairs and sebaceous materia1. RoentgenoIogic examination may often give definite diagnostic cIues as to the nature of the pathoIogic process. According to Good [9], in aImost one-half of teratoid tumors there is roentgenoIogic evidence of a caIcific process. This caIcification may be seen in the cyst waI1, in bone formed within the tumor, or in teeth seen in the tumor substance. He cIaims that the presence of a we11 formed tooth is pathognornonic of a teratoid tumor if the Iocation of the tumor is the anterior mediastinum. On x-ray examination, the tumor may be ovoid or IobuIated, with either distinct or indistinct borders. In many cases, especiaIIy those with fistuIa formation, an air-fluid IeveI may be demonstrated in the c,yst cavity. COMPLICATIONS
As has aIready been mentioned, this usuaIIy benign condition may undergo many compIications. For one, the tumor may become infected. This inflammatory process may cause a great increase in size, Ieading to pressure symptoms within the thorax; or it may weaken the cyst waI1 in such a manner as to bring about rupture of the cyst into adjoining structures such as the pericardium, the superior vena cava and the pIeura1 cavity. Inflammatory change may bring about the deveIopment of adhesions to adjacent structures which may cause the surgeon a great dea1 of diffrcuIty. Parts of the tumor may undergo maIignant degeneration. When this happens, there is aImost invariabIy a rapidIy fata termination. 447
Lamphier
FIG. I. Gland-Iike space, lined by pseudostratified ciIiated coIumnar epithelia1 ceIIs, such as are found Iining the tracheobronchial tree (entodermal origin). PhIoxine methyIene blue stain, origina magnification X 160. DIFFERENTIAL
DIAGNOSIS
There are severa factors which must be considered in the diagnosis of this Iesion. Because the condition is usuaIIy manifested by a series of non-specific symptoms, the diagnosis is often impossibIe to make. Tumors of the Iymphoma series, notably Iymphosarcoma and Hodgkin’s disease, must be ruIed out. These respond to deep x-ray treatment and the drugs of the nitrogen mustard series, whereas teratoid growths do not. GangIioneuroma is infrequentIy seen, and is usuaIIy Iocated in the posterior mediastinum when present. Since the teratoid tumor often is seen as a puIsating mass, a syphiIitic . or arterioscIerotic aortic aneurysm must be considered. Wassermann and Hinton tests are an aid here. Often, the Iipoma cannot be excluded and attempts at aspiration yieId nothing. Thymoma is usuaIIy maIignant and is sometimes accompanied by myasthenia gravis. Biopsy is the onIy way to distinguish an occasional huge bronchogenic carcinoma from the teratoid tumor. In areas of the country where Echinococcus granuIosus is prevaIent, the hydatid cyst must be excIuded. Another possibiIity, especiaIIy in the Great Lakes region, is a retrosterna1 goiter. TubercuIosis of the mediastina gIands, xanthoma and neurofibroma are seen aImost excIusiveIy in the posterior medias-
tinum. The possibiIity of a soIitary cyst of the Iung or bronchus and a pericardia1 cyst must be considered. The teratoma shown microscopicaIIy in Figures I to 3 was not located in the mediastinum, but may be regarded as a prototypi: of mediastina teratomas. CASE REPORT
J. R., a twenty-six year oId white man, was originaIIy seen on October zz, 1955. His chief compIaint was a mass in his neck of one year’s duration. For the previous month he had begun to have difTicuIty swaIIowing. He aIso stated that he had been troubIed for the past few weeks with irritabiIity, “jumpiness,” nervousness, etc. Past history was irreIevant except that right inguina1 herniorrophy had been performed in 1952 at an Army HospitaI in Munich, Germany. PhysicaI examination reveaIed a we11 nourished and we11 deveIoped white maIe who appeared to be of the stated age. His weight was 145 pounds. His height was 5 feet, 7 inches and his bIood pressure was 160/1 IO. A grade III enIargement of what was thought to be both IateraI Iobes of the thyroid gIand with a Iarge substerna extension was noted. There were no associated noduIes and the mass was soft,
MediastinaI
Teratoma
SimuIating
Adenomatous
Goiter
FIG. 2. Area of bone formation, set in a matrix of fibrous connective tissue, with bone fragments bordered by a line of osteobIasts (mesodermal origin). PhIoxine methylene blue stain, original magnification X 160.
FIG. 3. Area containing gIial-type ceIIs, as found in the central nervous system. Abundant Iarger ce1I.s resemble astrocytes; smaller ones resemble oligodendrogX 450. lia. PhIoxine methylene bIue stain, original magnification
which was thought to be probably secondary to.cystic degeneration. A basa1 metabolic study was reported as pIus 35, but cIinicaIIy the patient did not appear to be toxic. Therefore, radioactive iodine stud-
ies were carried out and reported as ‘‘within norma limits.” Chest x-rays, especiaIIy of the I;uperior thoracic strait, were reported by Dr. W. Arthur Young as foIIows: “The outIine of the cardio459
Lamphier vascuIar system is not remarkabIe. The bronchia1 pattern is moderateIy prominent in each lung, being more so on the right. There is no x-ray evidence of consohdation, infiItration or pleura1 effusion. Both hilar shadows are somewhat prominent, especiaIIy on the Ieft superiorly, and for this yeason, additiona chest x-rays at some future date shouId be done if cIinicaIIy indicated.” The patient was admitted to the Booth MemoriaI HospitaI, BrookIine, Massachusetts, on October 27, 1936, and on the foIIowing day what was thought to be an adendmatous goiter with cystic degeneration and substerna extension was removed as foIIows: Under adequate inhaIation anesthesia, and with the patient intubated and in the usua1 position for thyroidectomy, the anterior aspect of the neck was prepared with ether and zephiran@ and properIy draped. A Iow coIIar skin incision was made and carried down through the subcutaneous fat and pIatysma muscIe to the anterior IeveI of the deep cervica1 fascia. The upper ffap was then dissected proximaIIy as far as the cricoid cartiIage. The media1 aspect of both sternocIeidomastoid muscIes was then freed up. The deep cervica1 fascia was divided verticaIIy in the midIine, after which the strap muscIes were divided high between Kocher cIamps. It was then reveaIed that the trachea was markedIy deviated to the Ieft because of the Iarge mass invoIving the right IateraI Iobe of the thyroid. The right IateraI Iobe was grasped with doubIe hooks and rotated mediaIIy, after which the superior thyroid poIe was doubly Iigated with heavy catgut. By sharp dissection, the right IateraI Iobe was freed up from the carotid sheath. Extensive adhesions were found between the carotid sheath and the right IateraI lobe. The inferior thyroid artery was Iigated in continuity; then by sIiarp and bIunt dissection the substerna extension was freed up down to the aortic arch. AI1 bIeeding vesseIs were cIamped and Iigated with fine and heavy pIain catgut sutures. Because of persistent oozing, a geIfoam@ pad was pIaced in the defect in the superior thyroid strait and the strap muscles were brought together with Iateral mattress, heavy, pIain catgut sutures. The deep cervica1 fascia was brought together in the midIine with interrupted heavy, plain catgut sutures, and the skin with MicheI cIips. A smaI1 Penrose drain was aIlowed to make its exit from the wound
at the right IateraI poIe of the wound. A dry, steriIe pressure-type dressing was appIied. PostoperativeIy the patient did we11 and he was discharged from the hospita1 improved on his fifth postoperative day. The pathoIogic report was as foIIows: CIinicaI impression : Thyroid adenoma, right, with substerna extension and cystic degeneration. GrossIy, the specimen consisted of a portion of thyroid tissue weighing 26 gm. and incIuded the waI1 of an empty open cyst. RecognizabIe thyroid tissue measured 3.0 by 2.5 by 1.5 cm. The cystic portion measured 4.5 by 4.0 by 2.5 cm. The thyroid tissue was pinkish red and gIistening. The outer surface of the cystic structure was, for the most part, smooth and yeIIowish white. There appeared to be a second and coIIapsed cyst attached to the Iarge cyst noted in conjunction with the thyroid tissue. This cyst measured approximateIy 1.5 cm. in its greatest diameter. MuItipIe sections reveaIed gIistening yeIIowish white tissue between the cyst waI1 and the thyroid. The Iining surface of the Iarger cyst was hemorrhagic and rough. A third cyst was found in the substance of the waI1 of the mass. This was muItiIocuIar in appearance. The waIIs of the IocuIes were composed of a soft yeIIowish green, somewhat geIatinous materia1, and in the cystic area a dirty .grey-brown fluid was encountered. PreIiminary gross diagnosis: Adenoma, thyroid, showing cystic and hemorrhagic necrosis. FinaI diagnosis: Cystic teratoma attached to thyroid and thymic t:ssue compIicated by chronic inff ammation. The teratoma contained recognizabIe pancreatic tissue, smaI1 intestina1 mucosa1 gIands, smooth muscIe, skeIeta1 mu!cIe and fibrous tissue. The Iesion was benign. One year Iater, foIIow-up studies revealed a non-disfiguring Iow coIIar skin scar and no evidence of recurrence. MediastinaI x-rays at this time were reported as normaI. TREATMENT
AI1 authors seem to agree unanimously that surgica1 intervention is the onIy treatment of any use in this condition. Rusby [4] Iists three accepted Iines of surgica1 approach. The first, rib resection and drainage, has IittIe to offer if this is the onIy treatment rendered the patient. The resuIts are poor, but this method is acceptabIe if it is used as a preIiminary to more Second, if the tumor is extensive surgery. 460
MediastinaI
Teratoma
SimuIating
firmly- bound down to adjacent vital structures, the usual procedure is that of partia1 removal with marsupialization. Indeed, this may be the only possible course to take. This step does not preclude the possibility of a more radica1 intervention at a later date. Many surgeons employ this as the first step in a muItipIe stage, resuItremoval of the tumor. If this ing in complete is the onI?- treatment, the resuIts are onIy sIightIy better than those obtained with rib resection and drainage. The surgeon’s aim shouId aIways be compIete excision of the mass. Rusby thinks the transpIeura1 approach is best if concomitant infection is feared or the neopIasm is Iarge. For tumors Iocated in the upper retrosternum, the transverse cervical coIIar incision with spIitting of the sternum is the accepted method. The trap-door incision is effective for a growth Iocated in the thoracic substernal region, but not in the lateral thoracic region. Harrington [ro] is an exponent of the posterior approach, for he beIieves that there is a signihcantIy better exposure obtained in this manner. CONCLUSION
A mediastina1 teratoma is described which preoperativeIy was diagnosed as an adenomatous goiter. NeedIe biopsy of tumors in the neck is to be discouraged as probabIy in this instance an erroneous diagnosis of adenomatous goiter with cystic degeneration wouId have been made if onIy needIe biopsy had been performed. The history, pathoIogy, pathogenesis and cIinica1 picture of mediastina1 teratomas are reviewed.
Adenomatous
Goiter
REFERENCES I. GOORWITCH, J.,
and SOKOL, J. ExtragenitaI (mediastinal) teratocarcinoma. J. Internet. Coil. Surgeons, 17: 343-352, 1952. 2. HEDBLO~U,C. A. Intrathoracic dermoid cysts and teratomata. J. Tboracic Surg., 3: 22, 1933. 1:. ANDRUS. W. DEW. and HEUER. G. J. Sureical treatment of tumors of the mediastinum. Surg., Gynec. ~3 Obst., 63: 469-482, 1936. 4. RUSBY, N. L. Dermoid cysts and teratomata of the mediastinum. J. Tboracic Surg., 13: 169-222, d
1944. 5. WHEATLEY, G. M. MediastinaI teratoma in a chiId seven months oId. Am. J. LG. Child., 54: 10571065, 1937. 6. NYLANDER, P. E. A., TOEVONIN, S., TURUNEN, M. and HJELT, L. Teratoid tumors of the mediastinum. Ann. cbir. et gynaec. Fenniae, 42: 141-156, 1953. 7. Fox, J. P. and HOSPERS, C. A. SoIid teratoid tumors of the anterior mediastinum. Am. J. Cancer,
28: 273, 1936. 8. NANDROT, C. Contribution
a I’etude deskystes dermoides du mediastinum anterieur. Paris, 1937. Thesis. g. GOOD, C. A. The roentgenoIogic diagnosis of surgical Iesions of the mediastinum. Section on Roentgenology, Mayo Clinic, Rochester, Minnesota. Read March 2, 1950, before Annual Clinical Conference of Chicago MedicaI Society. IO. HARRINGTON, S. W. Surgical treatment of intrathoracic tumors and tumors of chest waI1. Arch. Surg., 14: 406-431, 1927. I I. BKINDLEY, G. V. and HENDERSON, J. A. Mediastina tumors. Am. Surgeon, 20: 260-269, 1954. 12. BURNETT, W. E., ROSEMOND, G. P. and BUCHER, R. M. The diagnosis of mediastinal tumors. S. Clin. North America, 32: 1673-1694, 1952. IX. DORAN. W. T. and LESTER. C. W. Mediastinal teratoma. J. Tboracic Surg., 8: 309-315, 1939. 1.4. BLADES, B. ReIative frequency and site of predilection of intrathoracic tumors. Am. J. Sure., 54: r39-148. 1941. 15. THOMPSON, J. V. MediastinaI tumors and cysts. Internat. Abstr. Surg., 84: Ig5-221, 1947. .a
,