Congenital teratoma of the thyroid

CONGENITAL

TERATOMA

OF THE THYROID

E. H. MUNRO, M.D. AND RICHARD WALDAPFEL, Surgeon,

M.D.

St. Mary’s Hospital

GRAND JUNCTION, COLORADO

T

UMORS of the newborn have aroused the interest of the practitioner, oband pathologist since the stetrician, earIy days of medicine. The uncommonness of their occurrence and deveIopment expIains this interest suffrcientIy. The type of tumor reported in this paper-teratoma-occurs most frequentIy in the ovary and in the testicIe and next most frequently at the two ends of the original infoIding of epithehum in the primitive embryo, nameIy, in the sacrococcygea1 region and in the bronchogenic region. Teratomas of the thyroid gIand, however, are very rare. RusseII and Kennedy, l in a report of a case pubIished in 1913, state that in twenty-two annua1 voIumes of this journa1 onIy one reference has been made to it (Hewetson2 1903). Pusch and NeIson3 in a clinica revision up to 1933 could colIect onIy twenty-eight authentic cases from the entire worId’s Iiterature, the first case dating back as far as 1691. Another case was pubhshed since this comprehensive articIe by Potter4 in 1938. Most of the cases reported have been stiIIborn or in a prematureIy deIivered fetus. Almost without exception those born alive have not survived more than a few of the CIinic of Goetdays. Tammann, tingen, in 1925 reported a case with a tumor 4 cm. in diameter, successfulIy operated upon. There has never been a satisfactory expIanation as to the origin of these tumors. The most understandabIe theories are perhaps : (I) Separation of a totipotent bIastomere in the earIy stage of cel1 cIeavage and its migration to a point where it Iater develops, and (2) some kind of twinning with overgrowth of one twin. As to the occurrence in the thyroid, it wouId seem possibIe that a fertilized poIar

body could have been incIuded in the primitive infoIding of the epithelium in the branchogenic region which Iater forms the thyroid primordium and migrates down from the second branchia1 arch through the hyoid, past the Iarynx to the upper portion of the trachea. In the many compIex changes in this region, the thyroid anIage meets with many bizarre accidents and aberrant thyroid glands resuIt in the nasopharynx in, on or beneath the tongue, in the waII of a thyrogIossa1 cyst, preIaryngeaI, intratrachea1, intra-esophagea1, supra- or subcIavicuIar,-or in the posterior triangIe of the neck. The occurrence of such a variety of accidents to the thyroid in the embryo Iends argument to the theory of incIusion of a poIar body or of some other muhipotent ceIIs from the primitive germ Iayers within tissues undergoing invagination or fusion. Because of the rarity of these cases and our Iimited experience with them, it is desirabIe that new cases be reported Every new report is whenever found. apt to furnish more detaiIs heretofore unknown, and wiII contribute to our knowledge of the pathogenesis of these formations. CASE KEPOKT K. L. was four weeks of age when his mother brought him for the first examination. She reported that it was a normaI birth and K. was the third child. The others are heaIthy and normal. Neither parent was goitrous. The father’s sister and mother’s mother had goiters. When K. was born, the parents noticed a slight swelling of the left side of his neck and some breathing difficulties in certain positions. They thought it wouId disappear, but it did not. On the contrary, it seemed to increase in these four weeks. The breathing was worse in feeding and sIeeping on the right side. Sometimes the

271

baby really turned blue and became limp and unconscious. The first examination showed a sIightIy

FIG. I. Baby at first examination. Ieft thyroid Iobe.

Tumor

of

cyanotic, somewhat undernourished child with a Ieft sided tumor of the neck. (Fig. I.) It was the size of a fist, was covered by norma skin, and beIonged apparently to the Ieft lobe of the thyroid gland with which it moved upward in

FIG. 3. Tumor removed at operation; Iar surface.

nodu-

swaIIowing movements. The tumor itself was not movable nor separabIe from the thyrsid gland. No tenderness or inflammation was present. Nose and pharynx were normal. The Iaryngoscopy showed a normaI Iarynx which, perhaps

as a whole, was slightly dislocated to the r,ight side. There was a definite inspiratory str idor noticeahIe mainly when the baby’s head was

FIG. 2. Baby four months after operation. Tumor compIeteIy gone, chiId exceIIentIy developed and free of any tumor symptoms.

turned to the right side; in this position the baby was sIightIy cyanotic. Otherwise, the b aby appeared normaIIy deveIoped. The x-ray ex-

FIG. 4. Cross section of tumor. Left side hard, containing bony parts; right side spongy; numerous cavities; fibrous capsuIe surrounding entire tumor.

amination showed a sIight displacement of the trachea to the right side with Iittle narrow ;mg of its Iumen in the uppermost portion. ’The tumor was non-opaque, but within its Iir nits severa smaI1 densities were discernibIe.

NEH ~SEHIES VOL. LX IV, No. z

Munro,

Waldapfel-Teratoma

led to -I‘he cIinica1 and x-ray examination the diagnosis: IJnusual nodular goiter, perhaps feu tl adenoma.

American

Journal

of Surg SW

273

the trachea. Keeping up this pull, the operation couId be finished uneventfuIIy witho but resorting to tracheoscopy or tracheoto my. The

a

FIG. 5. Microscopica sections through tumor; complete absence of thyroid tissue except in B. A, embryonic nerve tissue (n) covered by squamous epithelium (e); gland tubuIe (gl) lined with squamous epitheIium. B, part near cap&e; c, capsular vein. There are severa rows of atrophic thyroid acini, the onIy pIace in the tumor where thyroid tissue couId be found. c, striated muscle (m) around a cavity. D, Iumina (I) Iined with columnar epithelium (ep) and containing mucus (mc); hyaline cartilage near by (c). The whole formation is suggestive of an abortive attempt at bronchus and Iung formation.

Operation was recommended on account of the breathing difhcuIties. The parents wanted to take the baby home first, but were advised to watch the baby’s breathing and to report instantIy in case it became worse. They came back two weeks Iater when they discovered the baby’s condition sIightIy worse. The stridor was continually noticeabIe and the sweIIing had somewhat increased in size. Operation was performed on August 4, 1942. Everything was ready for an instant tracheoscopy and introduction of a narrow tube through the larynx into the trachea at a moment’s notice and for a tracheotomy as we11 shouId signs of suffocation appear during the operation. After administering the first few drops of ether, the breathing seemed to become worse and stiI1 looked critical during the incision of the skin, but it improved considerabIy, against expectation, when the tumor was grasped with the forceps and puIIed away from

operation consisted in removing the entire tumor from the Ieft lobe of the thyroid gland, to which it definiteIy belonged and by which it was surrounded in its whole circumference. After spIitting a thin layer of thyroid tissue, what appeared the capsuIe of the tumor was struck and the tumor couId be removed in its entirety with its capsuIe in blunt dissection. It seemed attached to the surrounding tissue onIy near the trachea. BIeeding was inconsiderable. The breathing became instantIy normal after separating the tumor from the trachea and remained so through the postoperative course of heaIing, which occurred uneventfully and per primam. (Fig. 2.) The removed tumor (Fig. 3) measured about 236 by I >d inches and showed a smooth but nodular surface in some of its portions. The specimen was fixed in formalin for a few days and then cut in two. It showed a very unusua1 picture on its cross section. (Fig. 4.)

274

AmrrirnnJwrd

01 Surgery

Munro,

Waldapfel-Teratoma

Within a thin fibrous capsule numerous cavities couId be seen and between them tissue of different consistency and structure. The knife

IllAY,I<,.$.$

mucus, suggested embryonic bronchial formation (Fig. SD); occurrence of foci of cartilage confirmed this suggestion and the whole section

FIG. 6. E, bone formation (b) of intercartilaginous (c) type. F, bone formation (b) of intramembranous type. c, budIike projection (n) of nerve tissue into a cavity. The bud is covered bv squamous eDithelium and contains bone in its root and’near by (b). .

felt some hard resistance like ossifications which beIonged to the Ieft part of the tumor in Figure 4; this part was made up of a tough fibrous tissue basis and could be differentiated microscopicaIIy from a right Iobular part which showed more spongy appearance and seemed gelatinous in its consistency. The microscopic examination was made from several portions of the tumor and showed compIete absence of thyroid tissue except in a smaII area of the capsuIe where several rows of atrophic thyroid acini were present. No colloid material was seen in (Fig. 5B.) any part of the various sections. The greater buIk of the tumor was made up of mostIy heterogenous tissue eIements with embryonic nerve tissue in the foreground (Figs. 5~ and 6~) which seemed aImost the matrix of the whole tumor. A net of Iumina lined with columnar epithelium and containing

may we11 represent an abortive attempt at formation of a lung. GIand tubules lined with squamous epithelium showed the character of the epidermis of the embryo. (Fig. SA.) An isIand of hyaline cartiIage could be seen, bone formation of intercartilaginous type (Fig. 6~) and of intermembranous type (Fig. 6~ and G), striated muscIe. (Fig. SC.) Pigmented columnar epitheIium and isIes of dark staining melanin represented very IikeIy embryonic retina. There were many bud or poIyp-like projections of connective and nerve tissue into the cavity spaces. (Fig. 6~.) Nowhere did the various eIements form a definite structure nor arrange themseIves to form an organ. COMMENT We

of the

are concerned thyroid gland

AND

here made

SUMMARY

with a growth up of eIements

N~wS~HI~~VOL.LXIV.

No. 2

Munro,

WaldapfelLTeratoma

entireIy stituents The showed dermal: pigmented gIands; muscle,

different from the norma conof thyroid tissue. various eIements of the tumor the following origin: (I) Ectonerve tissue, squamous epithelium, epithelium, hair foIIicIes, sweat (2) mesoderma1: cartiIage, bone, connective tissue, and (3) entodermal : columnar epitheIium of tracheobronchial

Amrrk~n Journ;~l ofSr,rg~'.,y 273

tion soon after birth which could be eliminated by surgica1 remova of the tumor. The rarity of the case justifies this report. The fact that this observed case is one of the very few which has been found and diagnosed in the living, and has been operated upon and cured, makes it still more worth whiIe recording.

character.

This survey shows that a11 three germinal Iayers were represented in the tumor and the tumor, therefore, must be regarded as a rea1 congenita1 teratoma or embryoma. CIinicaIIy it caused symptoms of suffoca-

REFERENCES I. RUSSELL

and

KENNEDY.

.I. Ohsf. C+ Gyne~., Brit.

Empire, 23: log, 1913. z. fjEWETSON.

J. Obsl. & Gpec.,

&it. Empire, 4: 355,

'903.

3. PUSCH and NELSON. Am.J. Cancer,23: 4. POTTER. Arch. Path/., 65: 689, 1938.

INTRAMEDULLARY tumours are usuaIly malignant, and are commonly gliomatous. Other varieties incIude tubercuIoma, cyst, and endotheIioma, which have probabIy originated from invagination of the pia mater which lies in the posterior spina sulcus,or else have become embedded in the cord.

791,1935