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Fetus in fetu or giant epignathus protruding from the mouth
Fetus in Fetu or Giant Epignathus ByOsman
Protruding
From the Mouth
Faruk Senyiiz, Riza Rizalar, Sinan Celayir, and Feriha 6z Istanbul, Turkey
0 A huge and extremely organoid mass, protruding from the mouth of a newborn and causing respiratory embarrassment, was resected. It was difficult to make a clear distinction whether this mass was fetus in fetu or a highly organoid epignathus dealing with the findings obtained from its gross and histological examination of the mass. Cap yright o 1992 by W.B. Saunders Company INDEX WORDS:
Fetus in fetu; epignathus;
teratoma.
T
HE TERM FETUS IN FETU is reserved for the malformed masses in which the differentiation of the teratoma is carried to a high degree with the presence of axial differentiation of limbs and organs.’ Teratomas are composed of poorly organized tissues derived from each of three layers of the embryonic disc.“.” It is sometimes difficult to make a clear distinction between teratomas and structures that result from abortive attempts of twinning because of the existency of highly organoid appearance.2 CASE REPORT This 3.4-kg boy was born by normal vaginal delivery at 37 weeks’ gestation in another hospital. There was a giant, irregular mass covered partly by hair-bearing skin, protruding from the mouth attached by a broad pedicle to the right side of the mouth (Fig 1). During his transfer to our hospital he had had respiratory difficulty when he was lying in the supine position. Because of the respiratory embarrassment, an endotracheal tube was inserted which allowed him to breath spontaneously. After a short time for preparation, the baby was taken to the operating room. Following induction of general anesthesia the tumor was noted to be attached to the buccal surface of the right cheek and the right side of the mandibular bone. There was a narrow opening, 1.5 cm in diameter between oral cavity and oropharynx at the left side of this broad based attachment. The mass was excised using diatermy and suture ligation because of the existency of two large vessels. A small part of the mass was left in the right cheek. The defect so created was covered with a flap of mucosa elevated from the buccal sulcus. The specimen measured 17 x 8 x 5 cm and weighed 900 g. RESULTS
X-ray of the resected specimen demonstrated a smaller lobe having some cystic spaces and a flat bone, resembling the mandibular bone, and a larger lobe, showing huge and well formed brain mass with a solid tissue appearance, and a triangular bone, like a part of vertabral body connected with the other long bones (Fig 2). The mass measured 17 x 8 x 5 cm with two parts. The smaller part measured 8.5 x 6 x 5 cm and was covered by 2 cm long hair bearing skin. There was a skin extension measured 1.8 x 1 x 6 cm. Very near to .hmalofPediatr~c Surgery. Vol27, No 12 (December), 1992: pp 1493.1495
Fig 1. Huge mass protruding from the mouth
this skin extension, there were eyelid-like skin folds and very long sulcus covered by mucosa. There were many well formed teeth connected to a long bone located deepy in this sulcus. The larger lobe measured 11.5 x 8 x 5 cm, and was totally composed of the brain and trigonal and long spongious bones, and scapula-shaped bone (Fig 3). Cut sections of the brain showed the existence of a cavity 2 cm in diameter, full of clear fluid. Just under the brain, and centrally located, there was a triangularshaped bone, imitating a part of vertebra, connected
From the Departments of Pediatric Surgery and Pathology. Cerrahpaga Medical Faculty, University of Istanbul, Istanbul, Turkey. Address reprint requests to Dr Osman Faruk Senyiiz. Okumy Adam Sokak N.3 D.5, Fatih, Istanbul, Turkey. Copyright o I992 by W.B. Saunders Company 0022-346819212712-0002$03.00l0 1493
SENYijZETAL
Fig 3. Gross examination of the resected mass. From top to bottom: smeller lobe:heir beering skin (s), eyelid-like skin folds (e), finger-like skin extension (f), long sulcus covered by mucosa (SC), resection area (r) Larger lobe consisted of brain lobes (b). Inside, deeply and centrally located bones.
Fig 2. X-ray examination of the resected specimen: smaller lobe having a flat bone, representing the mandibula (m), and some cystic spaces. Larger lobe, showing huge and well formed brain mass as solid tissue appearance (b), and a triangular bone (t) connected to the other bones.
with the various length of long bones (Fig 4). No vertebral column was discernible except the presence of the triangular-shaped osseous structure. Histological findings included eyelid-like skin folds with nonkeratinized stratified squamous epithelium, mesenchymal connective tissue and secretory glandular lobes. Between these two folds there was stratified squamous cornea1 epithelium. Sections obtained superficial to the brain showed brain tissue covered by leptomeninges and keratinizing stratified squamous epithelium with hair follicles and sweat glands. The bone marrow demonstrated active hematopoiesis. The sulcus that was apparent in gross examina-
tion was covered by pseudostratified ciliated, and partly by stratified epithelium. The brain was composed of white and gray substances, some sections with choriedeal plexus.
Fig 4. Bones from the larger lobe of the mass: triangular-shaped bone (t), scapula-shaped bone (5).
1495
FETUS IN FETU OR EPIGNATHUS
There were mature spongious bones with cartilage caps and bone marrow with active hematopoiesis. DISCUSSION
Fetus in fetu is distinguished from teratoma by the presence of part or the whole of a vertebral axial skeleton and appropriate arrangement of limbs and other organs with respect to the axis.4,5 Teratoma is defined as a true tumor composed of multiple tissues foreign to those characteristic of the part from which it is derived.2,3Jj Giant epignathi are very rare teratomas that they have been classified as epignathus in the presence of the parasite attached to the palate of the autosite, causing respiratory obstruction. It has also been pointed out that these masses rarely have recognizable fetal parts.2,3 In the present case, the resected huge mass was composed of very well-developed brain halves covered by leptomeninges and partly by hair bearing skin and a triangular bone. It might be a part of vertebra, centrally located just under the brain, well formed long and spongious bones connected to this triangular one, their cartilage caps and bone marrow with active hematopoiesis.
In addition to these, there were eyelid-like skin folds with lacrimal glands located very near to the long sulcus covered by oral and respiratory epithelium, many well-formed teeth and mandibula-like flat bone. These findings seemed us that this mass was imitating its counterpart, in other words, fetus in fetu as Ouimet and RUSSO,~Eng et al,’ and Simpson8 have described their cases instead of as teratoma because of the presence of organ-like structures even in the absence of vertebral axis. On the other hand, if the vertebral axis is considered as the sole criteria between these questionable cases, this mass is supposed to be diagnosed as giant and extremely mature epignathus with a part of vertebral body connected to the other bones without having organized axial skeleton. Another fact is the attachement of the parasite to the autosite. There are no significant connection to the autosite in cases of fetus in fetu.2,7,9But the broad attachment to the autosite was apparent in our case, as the other masses called teratoma. At the end of this report we still do not know for sure whether this case should be diagnosed as fetus in fetu or giant and extremely mature epignathus.
REFERENCES 1. Ouimet A, Russo P: Fetus in fetu or not? .I Pediatr Surg 91926-927, 1989 2. Potter EL, Craig JM: Pathology of the Fetus and the Infant (ed 3). Chicago, IL, Year Book, 1976 pp 188-236 3. Valente A. Grant Cl, Orr JD, et al: Neonatal tonsillar teratoma. J Pediatr Surg 4:364-366, 1988 4. Sutthiwan P, Sutthiwan I, Tree-trakan T: Fetus in fetu. J Pediatr Surg 3:290-292, 1983 5. George V, Khanna M, Dutta T: Fetus in fetu. J Pediatr Surg 3:288-289. 1983
6. Hirabayashi S, Ueda K: Nasopharyngeal teratoma to the lower jaw. Plast Reconstr Surg 6:939-941, 1985
attached
7. Eng HL, Chuang JH, Lee TY. et al: Fetus in fetu: A case report and review of the literature. J Pediatr Surg 3296-299. 1989 8. Simpson JS: Conjoined twins, in Holder TM, Ashcraft KW (eds): Pediatric Surgery. Philadelphia. PA. Saunders, 19X0. pp 1104-1113 9. Numanoglu I, Yavuz A. Gokdemir Surg 51472-473, 1970
A: Fetus in fetu. J Pediatr
Protruding
From the Mouth
Faruk Senyiiz, Riza Rizalar, Sinan Celayir, and Feriha 6z Istanbul, Turkey
0 A huge and extremely organoid mass, protruding from the mouth of a newborn and causing respiratory embarrassment, was resected. It was difficult to make a clear distinction whether this mass was fetus in fetu or a highly organoid epignathus dealing with the findings obtained from its gross and histological examination of the mass. Cap yright o 1992 by W.B. Saunders Company INDEX WORDS:
Fetus in fetu; epignathus;
teratoma.
T
HE TERM FETUS IN FETU is reserved for the malformed masses in which the differentiation of the teratoma is carried to a high degree with the presence of axial differentiation of limbs and organs.’ Teratomas are composed of poorly organized tissues derived from each of three layers of the embryonic disc.“.” It is sometimes difficult to make a clear distinction between teratomas and structures that result from abortive attempts of twinning because of the existency of highly organoid appearance.2 CASE REPORT This 3.4-kg boy was born by normal vaginal delivery at 37 weeks’ gestation in another hospital. There was a giant, irregular mass covered partly by hair-bearing skin, protruding from the mouth attached by a broad pedicle to the right side of the mouth (Fig 1). During his transfer to our hospital he had had respiratory difficulty when he was lying in the supine position. Because of the respiratory embarrassment, an endotracheal tube was inserted which allowed him to breath spontaneously. After a short time for preparation, the baby was taken to the operating room. Following induction of general anesthesia the tumor was noted to be attached to the buccal surface of the right cheek and the right side of the mandibular bone. There was a narrow opening, 1.5 cm in diameter between oral cavity and oropharynx at the left side of this broad based attachment. The mass was excised using diatermy and suture ligation because of the existency of two large vessels. A small part of the mass was left in the right cheek. The defect so created was covered with a flap of mucosa elevated from the buccal sulcus. The specimen measured 17 x 8 x 5 cm and weighed 900 g. RESULTS
X-ray of the resected specimen demonstrated a smaller lobe having some cystic spaces and a flat bone, resembling the mandibular bone, and a larger lobe, showing huge and well formed brain mass with a solid tissue appearance, and a triangular bone, like a part of vertabral body connected with the other long bones (Fig 2). The mass measured 17 x 8 x 5 cm with two parts. The smaller part measured 8.5 x 6 x 5 cm and was covered by 2 cm long hair bearing skin. There was a skin extension measured 1.8 x 1 x 6 cm. Very near to .hmalofPediatr~c Surgery. Vol27, No 12 (December), 1992: pp 1493.1495
Fig 1. Huge mass protruding from the mouth
this skin extension, there were eyelid-like skin folds and very long sulcus covered by mucosa. There were many well formed teeth connected to a long bone located deepy in this sulcus. The larger lobe measured 11.5 x 8 x 5 cm, and was totally composed of the brain and trigonal and long spongious bones, and scapula-shaped bone (Fig 3). Cut sections of the brain showed the existence of a cavity 2 cm in diameter, full of clear fluid. Just under the brain, and centrally located, there was a triangularshaped bone, imitating a part of vertebra, connected
From the Departments of Pediatric Surgery and Pathology. Cerrahpaga Medical Faculty, University of Istanbul, Istanbul, Turkey. Address reprint requests to Dr Osman Faruk Senyiiz. Okumy Adam Sokak N.3 D.5, Fatih, Istanbul, Turkey. Copyright o I992 by W.B. Saunders Company 0022-346819212712-0002$03.00l0 1493
SENYijZETAL
Fig 3. Gross examination of the resected mass. From top to bottom: smeller lobe:heir beering skin (s), eyelid-like skin folds (e), finger-like skin extension (f), long sulcus covered by mucosa (SC), resection area (r) Larger lobe consisted of brain lobes (b). Inside, deeply and centrally located bones.
Fig 2. X-ray examination of the resected specimen: smaller lobe having a flat bone, representing the mandibula (m), and some cystic spaces. Larger lobe, showing huge and well formed brain mass as solid tissue appearance (b), and a triangular bone (t) connected to the other bones.
with the various length of long bones (Fig 4). No vertebral column was discernible except the presence of the triangular-shaped osseous structure. Histological findings included eyelid-like skin folds with nonkeratinized stratified squamous epithelium, mesenchymal connective tissue and secretory glandular lobes. Between these two folds there was stratified squamous cornea1 epithelium. Sections obtained superficial to the brain showed brain tissue covered by leptomeninges and keratinizing stratified squamous epithelium with hair follicles and sweat glands. The bone marrow demonstrated active hematopoiesis. The sulcus that was apparent in gross examina-
tion was covered by pseudostratified ciliated, and partly by stratified epithelium. The brain was composed of white and gray substances, some sections with choriedeal plexus.
Fig 4. Bones from the larger lobe of the mass: triangular-shaped bone (t), scapula-shaped bone (5).
1495
FETUS IN FETU OR EPIGNATHUS
There were mature spongious bones with cartilage caps and bone marrow with active hematopoiesis. DISCUSSION
Fetus in fetu is distinguished from teratoma by the presence of part or the whole of a vertebral axial skeleton and appropriate arrangement of limbs and other organs with respect to the axis.4,5 Teratoma is defined as a true tumor composed of multiple tissues foreign to those characteristic of the part from which it is derived.2,3Jj Giant epignathi are very rare teratomas that they have been classified as epignathus in the presence of the parasite attached to the palate of the autosite, causing respiratory obstruction. It has also been pointed out that these masses rarely have recognizable fetal parts.2,3 In the present case, the resected huge mass was composed of very well-developed brain halves covered by leptomeninges and partly by hair bearing skin and a triangular bone. It might be a part of vertebra, centrally located just under the brain, well formed long and spongious bones connected to this triangular one, their cartilage caps and bone marrow with active hematopoiesis.
In addition to these, there were eyelid-like skin folds with lacrimal glands located very near to the long sulcus covered by oral and respiratory epithelium, many well-formed teeth and mandibula-like flat bone. These findings seemed us that this mass was imitating its counterpart, in other words, fetus in fetu as Ouimet and RUSSO,~Eng et al,’ and Simpson8 have described their cases instead of as teratoma because of the presence of organ-like structures even in the absence of vertebral axis. On the other hand, if the vertebral axis is considered as the sole criteria between these questionable cases, this mass is supposed to be diagnosed as giant and extremely mature epignathus with a part of vertebral body connected to the other bones without having organized axial skeleton. Another fact is the attachement of the parasite to the autosite. There are no significant connection to the autosite in cases of fetus in fetu.2,7,9But the broad attachment to the autosite was apparent in our case, as the other masses called teratoma. At the end of this report we still do not know for sure whether this case should be diagnosed as fetus in fetu or giant and extremely mature epignathus.
REFERENCES 1. Ouimet A, Russo P: Fetus in fetu or not? .I Pediatr Surg 91926-927, 1989 2. Potter EL, Craig JM: Pathology of the Fetus and the Infant (ed 3). Chicago, IL, Year Book, 1976 pp 188-236 3. Valente A. Grant Cl, Orr JD, et al: Neonatal tonsillar teratoma. J Pediatr Surg 4:364-366, 1988 4. Sutthiwan P, Sutthiwan I, Tree-trakan T: Fetus in fetu. J Pediatr Surg 3:290-292, 1983 5. George V, Khanna M, Dutta T: Fetus in fetu. J Pediatr Surg 3:288-289. 1983
6. Hirabayashi S, Ueda K: Nasopharyngeal teratoma to the lower jaw. Plast Reconstr Surg 6:939-941, 1985
attached
7. Eng HL, Chuang JH, Lee TY. et al: Fetus in fetu: A case report and review of the literature. J Pediatr Surg 3296-299. 1989 8. Simpson JS: Conjoined twins, in Holder TM, Ashcraft KW (eds): Pediatric Surgery. Philadelphia. PA. Saunders, 19X0. pp 1104-1113 9. Numanoglu I, Yavuz A. Gokdemir Surg 51472-473, 1970
A: Fetus in fetu. J Pediatr