Successful removal of a retroperitoneal teratoma from an infant is described. Various theories dealing with the origin of such tumors are discussed. Blood supply, hemopoiesis and circulation of the tumor have been investigated

Enzmann, E. V.

Magen, M. M.

Miroyiannis, S. D.

Name not available

Report on a Retroperitoneal Teratoma (Foetus in foetu)

University of Northern Iowa

Proceedings of the Iowa Academy of Science Volume 67 Annual Issue Article 68 1960 Report on a Retroperitoneal Teratoma (Foetus in foetu) E. V. Enzmann College of Osteopathic Medicine and Surgery S. D. Miroyiannis College of Osteopathic Medicine and Surgery M. M. Magen College of Osteopathic Medicine and Surgery Let us know how access to this document benefits you Copyright ©1960 Iowa Academy of Science, Inc. Follow this and additional works at: https://scholarworks.uni.edu/pias Recommended Citation Enzmann, E. V.; Miroyiannis, S. D.; and Magen, M. M. (1960) "Report on a Retroperitoneal Teratoma (Foetus in foetu)," Proceedings of the Iowa Academy of Science, 67(1), 544-552. Available at: https://scholarworks.uni.edu/pias/vol67/iss1/68 This Research is brought to you for free and open access by the Iowa Academy of Science at UNI ScholarWorks. It has been accepted for inclusion in Proceedings of the Iowa Academy of Science by an authorized editor of UNI ScholarWorks. For more information, please contact scholarworks@uni.edu. Report on a Retroperitoneal Teratoma (Foetus in f oetu) E. V. ENZMANN, S. D. MIROYIANNIS and M. M. MAGEN1 Abstract. Successful removal of a retroperitoneal teratoma from an infant is described. Various theories dealing with the origin of such tumors are discussed. Blood supply, hemopoiesis and circulation of the tumor have been investigated. Teratomata have been defined as tumors which are composed of several tissues not usually encountered at the location where such growths arise, and which are capable of a certain amount of differ-entiation. The degree of differentiation and the primary site of the tumors form the basis of the somewhat arbitrary classification as dermoid cysts, solid teratoma, foetus in foetu, as well as gonadal, sacrococcygeal, retroperitoneal and other categories. The most interesting of these tumors are the so-called retroperi-toneal teratomata, since in some of these development has proceeded far enough to produce a nearly complete human foetus; hence the terms "enclosed twin", or "foetus in foetu". These tumors occur chiefly in the upper abdominal region and are located dorsal to the peritoneum, usually on the left side of the body, near the spleen, pancreas, or kidney. Lord ( 1951) reported forty-two cases of retroperitoneal tera-toma; Michalany et al. ( 19 51) listed 5 8 cases and Charles ( 19 53) eighty-two such tumors. Only nine specimens were recognized by Lord as true "foetus in foetu" on the basis of the presence of a body axis within the tumor. Though these growths contain derivatives of all three germ lay-ers and almost any type of tissue or even organ, they never possess all of them. To the embryologist they are of special interest be-cause they represent a distorted picture of normal development; the student of pathology is interested in them because of their tendency to become malignant and because their further study may contrib-ute greatly to the solution of the origin of cancerous growths. The present paper contains a report of a retroperitoneal teratoma, which we have classified as foetus in foetu because it possesses a rudimentary body axis, a spinal cord with indistinct regional differ-entiation, and appendages. 1College of Osteopathic Medicine and Surgery, Des Moines, Iowa. 544 1Enzmann et al.: Report on a Retroperitoneal Teratoma (Foetus in foetu)Published by UNI ScholarWorks, 19601960] TERATOMA 545 PRESENTATION On April 28, 1959, a twenty-five day old girl was admitted to the Wilden Hospital of Des Moines. The child had been losing weight since birth and had refused to eat. Examination pointed to the presence of a tumor in the area of the left kidney. The tumor and the kidney were successfully removed. The child is alive and growing normally. The tumor weighed 363 grams and measured 9 by 9 by 6 centi-meters. It consisted of a mass of tissue enclosed in a fibrous sac which was double-walled in some areas. The fibrous envelope was rough on the outside but smooth and shiny on the inside, resembling an early chorion-amnion. There was no indication of a true umbili-cal cord though the specimen bore a small appendage on its anterior surface near the site where the umbilical cord normally takes origin. The blood supply came from the renal artery; the venous re-turn was not determined. The cranial part of the specimen con-sisted of an irregular mass of cysts of various sizes and colors. Some of the cysts were filled with a gelatinous mass, others with a clear yellow fluid. Many of the cysts contained fibrous trabeculae span-ning their lumina. The caudal part of the tumor consisted of two malformed legs with feet and digits, and buttocks, as well as a lower anterior body wall. All of these structures were covered with normal appearing epidermis. Some harder substance could be felt within the legs and buttocks, giving the impression of cartilaginous rudiments of leg bones and pelvic girdle (Figures 1-4) .2 A large number of tissue samples were taken from areas which suggested by their general appearance and location that they might be specific organs, such as brain, spinal cord, tongue, liver, etc. A histological study of these samples revealed the presence of derivatives of all three germ layers. Some of the skin contained hair follicles and sebaceous glands; other skin samples were devoid of such structures. Most of the stratified squamous epithelium was heavily cornified and in many places horny epithelial pearl had formed. The cavities of the cysts as well as the lining of the fibrous envelope consisted of flat cells resembling mesothelium. The body axis was represented by a caudal stump which con-tained a caudal spinal cord with meninges and a large fibrous sac filled with brain tissue. The cross section of the spinal cord showed some regional differentiation; ill-defined fibre tracts, neu-roglia and a spinal canal with ependymal lining could be recognized. 2The drawings (Figures 1-4) were made by Mr. G. R. Weiner; the photo-graphs were taken by Mr. L. Baldwin. We express our gratitude for their help and cooperation. 2Proceedings of the Iowa Academy of Science, Vol. 67 [1960], No. 1, Art. 68https://scholarworks.uni.edu/pias/vol67/iss1/68546 3 Figure I. Figure 2. Figure 3. Figure 4. IOWA ACADEMY OF SCIENCE [Vol. 67 2 Undissected specimen of a "foetus in foetu" viewed in craniad direction, showing buttocks and feet. The same specimen in dorsal view The ragged edge on the right shows where some cysts have been dissected away. Left foot, showing imperfect development of digits. Right leg of specimen after partial dissection. Blood vessels were present iu great numbers, especially in the fibrous envelope of the tumor. There were veins, sinusoidal spaces, hemorrhagic areas (especially in the deeper areas of the tumor) and arteries with degenerate and hyalinized walls. The blood cells were of the same type as those of the host (Figs. 6, 7.) The connective tissue consisted in many places of a loose ar-rangement of collagenous fibres and fibrocytes. Some connective tissue was mesenchymal in character with stellate cells. Degen-erate striated muscle was present. The gut was represented by a 3Enzmann et al.: Report on a Retroperitoneal Teratoma (Foetus in foetu)Published by UNI ScholarWorks, 19601960] TERA TOMA 547 portion of large intestine with well developed smooth muscle, a myenteric plexus, normal appearing mucosa, and large and small submucosal lymph nodules. The epithelium contained large num-bers of goblet cells. The cross section of the umbilicus-like appendage (pedicel) was filled with loose connective tissue and had no traces of blood ves-sels. No bones or cartilages were present; the harder areas of the legs and pelvic part consisted of dense fibrous tissue. Figure 5. Sect ion of the large intestine showing a large submucosal lymph nodule. Figure 6. Three arterioles with degenerate walls from the interior area of the tumor. Figure 7. Sinusoid spaces, venules and extravasations of blood from the lower abdominal area. 4Proceedings of the Iowa Academy of Science, Vol. 67 [1960], No. 1, Art. 68https://scholarworks.uni.edu/pias/vol67/iss1/68548 IOWA ACADEMY OF SCIENCE [Vol. 67 DISCUSSION Numerous theories have been put forward to account for the origin of teratoid tumors. The most widely accepted are the fol-lowing: ( 1) Double fertilization, of either two ova or an ovum and one of its polar bodies, with subsequent migration to an un-usual site. ( 2) A normally fertilized egg falling apart in the blastomere stage, the fragments developing somewhere outside the uterus. ( 3) Ovarian or tubal pregnancy. ( 4) Development of a totipotent cell at the original site of the primary gonads or at the actively growing ends of the body axis. The fertilization of a polar body, while theoretically possible, is not a likely explanation of the origin of a teratoma. In vitro exper-iments by one of us never showed penetration of polar bodies by sperm, though ova were fertilized with ease. It is also hard to imagine how a fertilized egg or a fertilized polar body could ac-complish long distance migrations and form a teratoma in such unlikely places as the orbit of the eye, the nasal cavity, or the brain. (Vide Kimmel, 1950.) Recent development of the technique of determining sex by ex-amination of various types of body cells makes it possible to rec-ognize the sex of a tumor. This method of sex determination could be used to decide between some of the theories listed above. One would expct, for instance, that individuals produced from a divided blastula or those originating by parthenogenesis (or ephebo-genesis, the male analogue of parthonogenesis) would always provide offspring of the same sex (as in identical twins). Myers (1959) carried out extensive studies on sex chromatin in teratomas and reported the following findings: In sixty-four teratomas from fe-males, typical female sex chromatin was present; in forty-one tero-tomas of males the nuclear sex chromatin was male in thirteen cases, uniformly female in ten cases and mixed in others. Myers assumed that the occurrence of mosaic tumors rules out parthenogenesis in tumors of male hosts. Duerken ( 1932) explained the development of chorion epithelioma or of dermoid cysts in testes and epididymis by parthenogenetic development of spermatozoa. Cowen et al. ( 1958) indicated that teratoid tumors of the epididymis may arise from the Muellerian ducts, remnants of the appendix, testis, or paradidymis. Intraovarian development without fertilization may furnish a plausible explanation for some ovarian teratomas. Loeb (1932) 5Enzmann et al.: Report on a Retroperitoneal Teratoma (Foetus in foetu)Published by UNI ScholarWorks, 19601960] TERATOMA 549 demonstrated that in the guinea pig about ten per cent of all cases show such development. The senior author has observed repeat-edly spurious development of Graafian follicles within the ovaries of rats and mice. Aften perfect spindles and equatorial plates could be seen. The stimulus to this development seemed to be the onset of atresia. Since the method of sex recognition by means of sex chromatin still leaves a large area of doubt ( vide discussion in Myers' paper, p. 5 2), the present authors have compiled from the literature those cases of retroperitoneal teratoma in which the sexes of both the host and the tumor were stated. In the case of the foetus in f oetu cited by Lord ( 1956), the tumor found in a seven-week old boy contained testes. In the Young teratoma (1806-1809) the tumor found in a nine-month-old boy had male genitalia. The Brunkow teratoma ( 1942) of a fourteen-year-old girl contained a rudimentary ovary. In these few instances the sex of the tumor determined by gross or by microscopic study was established with sex of the host. Several of the theories given above assume extensive migration. It is well known that teratomas occur in preferred sites such as the gonads, the growing ends of the body axis ( Colcock et al, 19 5 5). Since the region just below the diaphragm is also the seat of the primitive gonads in the embryo, it has been assumed by some writ-ers that not all of the primitive sex cells have been included in the descending gonads and that those left behind could well give rise to teratomas under certain conditions, including genetic tendencies of the bearer of the tumor. The preferred sites of various localities differ in their growth capacity and metabolic activity; hence, we may expect that the frequency distribution of tumors at various sites differs. This is indeed the case as was shown by Colcock et al. (1955), Hoge and Kay (1958), Peterson (1956), and others. The average sizes of the tumors also differ in various locations as shown in the following table based on measurements by various authors. Table 1 Average Size of Tumors From Various Sites Location of tumor scalp nose submental neck head mediastinum presacral epididymis retroperitoneal Diameter in cm. 2.5 2.3 3.0 10.0 1.5 9.0 17.0 2.0 19.l 6Proceedings of the Iowa Academy of Science, Vol. 67 [1960], No. 1, Art. 68https://scholarworks.uni.edu/pias/vol67/iss1/68550 IOWA ACADEMY OF SCIENCE [Vol. 67 Most of the theories which have been put forward to explain the lack of proper order in the arrangement of organs of teratomas (or absence of organs) emphasize the role of organizers. Little weight has been assigned to the nutritional supply, the vascular system of the tumors. An adequate blood supply is essential in fast growing embryos; the development of the circulatory system is given priority during the early stages of embryonic development. All teratomas described so far lack a placenta and placental circulation. Some of the specimens studied by others were sur-rounded by a fibrous capsule in which (or just outside of which) a network of blood vessels was found. Such a vascular plexus could hardly be called a vitelline circulation, as the presence of such a circulation would presume the presence of a yolk sac. On the other hand nearly all described specimens of retroperi-toneal teratomas possessed an appendage, usually named "pedicel", which has features reminiscent of a body stalk. The specimen de-scribed by us had a stalk originating from the lower anterior body wall, at a site where the primitive body stalk usually arises and where the umbilical cord is inserted before the lower body wall grows, with a resultant cephalad shift of the insertion. The histo-logical examination of this pedicel of our specimen did not show any blood vessels. On the other hand, the teratoma described by Young ( 1809) contained two blood vessels; the Schoenfeld specimen (1841) (vide Lord, 1951) had a stalk which extended to the host's liver and was connected to the umbilical vein. The Davis ( 1939) teratoma resembled that described by the preceding author. Most of the other described specimens resembled ours and re-ceived their blood supply from one of the systemic vessels of the host. Apparently the nearest artery of the bearer supplies the tumor. Hoeven (1952) found that the tumor studied by him was nourished through the renal artery of the host, whereas Gross and Clathworthy ( 19 51 ) reported the blood supply as coming from the superior mesenteric. Michalany and others ( 1951) gave spinal arteries as the source. Our specimen was supplied from the renal artery. Lord held ( 19 51, p. 63 7) that there was no direct connection between the vascular system of the host and that of the tumor. If this were true it would raise the question of the origin of the blood cells of the tumor. Since many of the tumors reported in the literature contained well-developed bones, erythropoiesis might conceivably take place in them. Hemopoiesis occurs in foetal bones as early as the second month of life. The literature on teratomata generally fails to state 7Enzmann et al.: Report on a Retroperitoneal Teratoma (Foetus in foetu)Published by UNI ScholarWorks, 19601960] TERATOMA 551 whether bone marrow was present in the bones of most tumors. The only paper dealing with bone marrow was that by Fujikura et al. (1959), who found red marrow within a femur containing eosinophil granulocytes, myelocytes, and rare normoblasts. No megakaryocytes were found. -Our specimen did not contain bones and there was no evidence of erythropoiesis. We assume that the blood supply came directly from the host, as it most likely did in most of the described speci-mens. (It is of interest to note that the description of the Tokyo teratoma in the 1960 March issue of Newsweek mentioned the pres-ence of whiskers on the face of the enclosed twin. It is possible that such hair growth was induced by blood borne hormones of its host.) Some hemopoiesis does take place within the tumor. Our speci-men contained lymph nodules within the mucosa of its intestine (Fig. 7). Numerous mitoses could be seen within these struc-tures. Fujikura (cited above) also mentioned the presence of lymphoid tissue and lymph nodes. Several other writers have noted the presence within the tumor of thymus and of spleen tissue. It is questionable whether the blood of the foetus in f oetu is set in motion by a propelling system within the tumor. Several writers note the presence of a heart (Lord, 1950; Klebs, 1876) but the hearts were described as rudimentary. The Klebs specimen even contained an aorta. Our specimen contained a considerable number of blood vessels of various sizes, including arteries, sinus-like spaces, capillaries and v~ins (Fig. 8). It is of interest to note that all arterial walls showed extreme degeneration. In most cases all components ex-cept the endothelium were hyalinized. We have observed similar vessel degeneration in an embryo with an ectopic heart combined with incomplete aorta. It can be inferred that the blood flow in such vessels is very slow, if not stagnant. Literature Cited Brunkow, C. W. 1940. Conservative surgical therapy of tumors in infants and children. J. Surg. 48, 480. Charles, D. 1953. Retroperitoneal teratoma in infancy. Arch. of Dis. in Childhood 28, 1947. Colcock, P. B., Sass, R. E. and Staudinger, L. 1955. Dermoid cysts. New Engl. Jour. of Med. 252, 373. Cowen, R. 1958. Teratoma of the epididymis: a mature adult type inclusion tumor of the epididymis in an 18-month-old child. J. Urol. 79, 1001. Duer ken, 193 2. From Jordan. and Kindred, "Textbook of Embryology." Apple-ton, 1948. Fujikura, T., and Hunter, W. C. 1959. Retroperitoneal "fetus in fetu" Obst. and Gyn. 13, 547. 8Proceedings of the Iowa Academy of Science, Vol. 67 [1960], No. 1, Art. 68https://scholarworks.uni.edu/pias/vol67/iss1/68552 IOWA ACADEMY OF SCIENCE [Vol. 67 Gross, R. E., and Clathworthy, H. W. 1951. J. Pediat. 38, 502. Hoge, R. H., and Kay, S. 1958 Squamous cell carcinoma arising in benign cystic teratoma (dermoid) of the ovary Ann. Surg. 147, 697. Klebs, E. 1876. Handbuch der pathologischen Anatomy. Berlin, vol. 1, p. 1011 ( vide Lord). Kimmel, A. 1950. Cerebral tumor containing five human fetuses. Anat. Rec. 109, 141. Loeb, L. 1923. Anat. Rec. 25, 141. Lord, M. J. 1951. Intra abdominal foetus in foetu. J. Path. and Bact. 72, 350. Michalany, J., Navarre, E. F., and Filho, V. A. 1951. Primary retroperitoneal teratoma, successful removal from a boy three weeks old. Coll. of Surg. 16, 350. Myers, L. M. 1959. Sex chromatin in teratomas. J. Path. and Bact. 78, 43. Petersen, W. F. 1956. Solid, histological benign teratoma of the ovary. Re-port on four cases and review of the literature. Am. J. Obst. and Gyn. 72, 1094. "Tokyo Tumor." 1960. Report in Newsweek, March 14. Vol. 55, p. 88. Young, G. W. 1806-1809. Med. Chir. Trans. 1, 234, (Vide Lord). 9Enzmann et al.: Report on a Retroperitoneal Teratoma (Foetus in foetu)Published by UNI ScholarWorks, 1960

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Report on a Retroperitoneal Teratoma (Foetus in foetu)

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