- Email: [email protected]
Oral Teratomas: A Report of 5 Cases
J Oral Maxillofac Surg 70:2809-2813, 2012
Oral Teratomas: A Report of 5 Cases Cristina Bonet,* David Peñarrocha-Oltra,† J.M. Minguez,‡ Beatriz Vera-Sirera,§ Miguel Peñarrocha-Diago,储 and María Peñarrocha-Diago¶ Purpose: To present a case series of 5 patients with oral teratomas, discussing the treatment and
follow-up. Report of Cases: Five patients (4 girls and 1 boy) with oral teratomas presented at the Maxillofacial Surgery Department of a University Hospital with a reference population of 1,000,000 between 1980 and 2002. The associated lesions, clinical examination, histopathology, treatment and follow-up were registered and described. The newborns presented associated congenital malformations such as cleft palate, bifid tongue, dorso-nasal fistula and nasal dermoid cyst. In all 5 cases the tumor mass was excised at its base with surrounding normal tissue under general anesthesia combining conventional and electric scalpels. Histological analysis resulted in different compositions of multiple tissues typical of teratomas. After a mean follow-up of 8 years no sign of tumor recurrence had been detected. Conclusion: Teratomas were a rare finding within a large population of newborn patients. Five tumors were excised and histologically diagnosed as teratomas. No recurrence occurred after 8 years of follow-up. © 2012 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 70:2809-2813, 2012
Teratomas are neoplasms composed of multiple tissues from all 3 germinal layers (ectoderm, mesoderm, and endoderm), with differentiation to identifiable tissues and organs with variable levels of maturity.1,2 They occur in about 1:4,000 births, Received from the Valencia University Medical and Dental School, Valencia, Spain. *Master of Oral Surgery and Implantology, Department of Stomatology, Valencia University Medical and Dental School, Valencia, Spain. †Master of Oral Surgery and Implantology, Department of Stomatology, Valencia University Medical and Dental School, Valencia, Spain. ‡Associate Professor of Oral Surgery, Faculty of Dentistry, Cardenal Herrera CEU University, Valencia, Spain. §Resident of the Master in Endodontology, Department of Cariology Endodontology Pedodontology, ACTA Graduate School of Dentistry, Amsterdam, Holland. 储Chairman of Oral Surgery, Department of Stomatology, Valencia University Medical and Dental School, Valencia, Spain. ¶Associate Professor of Oral Surgery, Valencia University Medical and Dental School, Valencia, Spain. Address correspondence and reprint requests to Dr Peñarrocha Diago: Unidad Médico Quirúrgica, Clínica Odontológica, Gascó, Oliag 1, 46010 Valencia, Spain; e-mail: [email protected] © 2012 American Association of Oral and Maxillofacial Surgeons
0278-2391/12/7012-0$36.00/0 doi:10.1016/j.joms.2012.01.028
with a female predominance,2,3 most commonly in the sacrococcygeal region. Teratomas are commonly benign; however, malignant teratomas have been described.4 Oral teratomas are extremely rare and seen almost exclusively in infants, usually in neonates; most cases include the tongue.4 These tumors occur in approximately 1:35,000 to 1:200,000 of live births, comprising 2% to 9% of all teratomas.2 Teratomas have been classified into 4 subtypes: 1) dermoid, which contains tissues of mesodermal and epidermal origin; 2) teratoid, which contains tissue from the 3 primary germ layers but is poorly differentiated; 3) true teratoma, which is similar to the teratoid but differentiates histologically into recognizable tissues; and 4) epignathus, which is tridermal in origin but differentiates into recognizable organs, sometimes with limbs or even a visible second fetus.5 Prenatal ultrasonography may detect teratomas.5 Epignathi usually are diagnosed at birth. These tumors frequently accompany other anomalies such as cleft palate, bifid tongue, and double tongue.2,6 Sometimes the protruding tumor mass dislocates the temporomandibular joint.7-9 A giant epignathus can led to death in the neonatal period because of respiratory obstruction1,2,7; thus, the newborn should undergo surgery within the first days of life. The definitive treatment of teratoma is
2809
2810
9
3
7 5
5
Follow-Up (yrs)
complete surgical excision, and the prognosis is excellent. Recurrence has not been reported.4,5 This report describes 5 clinical cases of oral teratoma, including treatment and follow-up.
8
ORAL TERATOMAS
Stratified squamous epithelium, salivary gland, calcification, smooth muscle fibers 1.5 ⫻ 2.0 2⫻2
Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
24 F 5
Abbreviations: AS, age at surgery in days; F, female; M, male.
Between hemilingual structures Vomer
Striated muscle tissue, adipose tissue, salivary gland, stratified epithelium 2⫻2 F 4
20
Double tongue, cleft palate, dorsal hemivertebra, vitreous hemorrhages Double tongue, cleft palate
Posterior pharyngeal wall Between hemilingual structures Between hemilingual structures M 3
3 F 2
2
Double tongue, cleft palate, nasal dermoid cyst
Posterior pharyngeal wall
1.5 ⫻ 1.5 2.5 ⫻ 2.0
Fibroconjunctive stroma, adipose tissue and bundles of muscular fibers Ciliate, cylindrical, pseudostratified epithelium; adipose tissue, smooth, and striated muscle; mature cartilage Polypoid mass, skin, hair 2⫻2 F 1
2
Cleft palate, dorsonasal fistula
Posterior pharyngeal wall
2⫻2
Histology Size (cm) Location (Pedicle) Associated Malformation AS
The etiology of epignathus is unknown; the most popular theory is that an epignathus may arise from a disorganized growth of pluripotential cells in the region of the Rathke pouch.6,7,9 Oral teratoma is a rare congenital tumor that is seen almost exclusively in infants, usually in neonates.10 The incidence ranges from 1:35,000 to 1:200,000 of live births.2,11,12 There is a female predominance of 6:1.3,10 The cases presented represent an incidence of 5 cases in a reference population of 1,000,000 inhabitants over a period of 22 years. There was female predominance of 4:1. Teratomas are rare in the head and neck region, representing less than 5% of all cases.4 The clinical diagnosis in all 5 cases was made at birth; the urgency for surgery was determined by the degree of respiratory and feeding difficulties. Three cases (Figs 1-3) underwent urgent surgery within the first days of life because of the critical
Gender
Discussion
Case
The authors describe 5 cases of oral teratoma that presented from 1980 through 2002 at the maxillofacial surgery department of a university hospital with a reference population of 1,000,000 inhabitants. The cases involve 4 girls and 1 boy, none of whom presented any family history of interest. The associated lesions, clinical examination, histopathology, treatment, and follow-up are described. The newborns presented associated congenital malformations such as cleft palate, bifid tongue, dorsonasal fistula, and nasal dermoid cyst (Table 1). In cases 3 and 5, the teratomas had a double pedicle, and the remaining cases presented a single pedicle. The tumor size ranged from 2.5 ⫻ 2.0 cm to 1.5 ⫻ 2.0 cm (Table 1). In cases 1, 2, and 3 (Figs 1-3), because of the large size of the mass protruding from the mouth and the episodes of respiratory difficulty experienced by the infants, tumor excision was scheduled during the first days of life. In all 5 cases, the tumor mass was excised at its base with the surrounding normal tissue under general anesthesia. A conventional scalpel was used in combination with an electronic scalpel (Valleylab Force 30, Covidien Surgical Solutions Group, Boulder, Colorado) to achieve coagulation and thus hemostatic control; complete resection was performed to avoid possible recurrences. The results of the histologic examination of the tumors are presented in Table 1. At 6 months of age, cases 3, 4, and 5 (Figs 3-5) underwent surgery to repair the double tongues. The next stage in the surgical management was the surgical closure of the cleft palate at approximately 18 months of age. No sign of tumor recurrence was detected during a mean follow-up of 8 years.
Table 1. CLINICAL AND HISTOLOGICAL DATA OF THE PATIENTS WITH CONGENITAL TERATOMAS
Report of Cases
BONET ET AL
2811
FIGURE 1. Case 1. A, Newborn with teratoma protruding from the oral cavity. B, Panoramic histologic view of tumoral lesion showing mature cartilage islands, nests of epithelial differentiation, and a stromal component with smooth muscle areas (hematoxylin and eosin stain; magnification, 25⫻). Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
situation of the neonate. These lesions must be diagnosed and excised early, because they have an excellent prognosis if treated properly.1,13 Giant epignathi have been reported to occupy the entire
oropharynx, resulting in a high mortality rate in the neonatal period.7,8 In the present cases and those described by other investigators, the most frequent congenital malformations were cleft palate, a double tongue, and supernumerary teeth in the upper and lower incisor regions.2,3,6,11,14 Cases have been described in which the location and volume of the tumor dislocated the temporomandibular joint.7-9 Different locations of the pedicle were noted in the cases presented. In 3 cases the pedicle was attached to the posterior pharyngeal wall (cases 1, 2, and 3), and in 2 cases between the 2 hemilingual structures (cases 3 and 5). In case 5, which had a double pedicle, the second pedicle was located on the vomer (Table 1). Vandenhaute et al,3 in 2000, observed that most teratomas have a pedicle at the base of the cranium, in
FIGURE 2. Case 2. A, A large teratoma located on the posterior pharyngeal wall caused difficulties with breathing and swallowing in the newborn. B, Image of the oral teratoma extirpated at the base of the pedicle.
FIGURE 3. Case 3. Newborn with 2 oral teratomas, 1 pediculated at the posterior pharyngeal wall and the other between the 2 hemilingual structures.
Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
2812
FIGURE 4. Case 4. Teratoma located on the tongue. Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
the nasopharyngeal posterior region, close to the Rathke pouch, whereas most published cases of oral teratomas have described an attachment to the base of the tongue.2,5,6 Lopes et al,4 in 2005, reported the
ORAL TERATOMAS
first case of a benign teratoma in the oral mucosa of a 9-year-old girl. Cases of giant epignathi have been described in the literature (14 ⫻ 8 ⫻ 6 cm and weighing 1 kg).7 In the present cases, the size ranged from 1.5 to 2.5 cm. It is important to make an accurate differential diagnosis that includes dermoid cyst, cystic hygromalymphangioma, hemangioma, giant epulis, ranula, salivary gland tumors, and rhabdomyosarcoma.2,6,15,16 The neonatal mortality associated with epignathus is extremely high because of airway obstruction. At present, ex utero intrapartum treatment is considered the best procedure for the safe management of airway obstruction at birth.7-9,17 The definitive treatment is complete surgical excision because the cellular elements that form these cysts are active cells and incomplete removal will result in recurrence.10,18 In the 5 cases presented, complete extirpation of the tumors was performed at the base of the pedicle to avoid recurrence. A review of the literature disclosed no published cases of recurrence or familial inheritance. The malignant transformation of teratomas originating in the oropharyngeal area also has not been described.11 During the follow-up of the present patients, no recurrences were detected. The aim of presenting these rare tumors is to emphasize the importance of recognizing the suggestive prenatal data and preparing for perinatal management, including the maintenance of fetal oxygenation and the aggressive early provision of an adequate airway.1,6,7,10
References
FIGURE 5. Case 5. A, Newborn with lingual teratoma, producing a double tongue. B, Histologic view of the tumor showing predominant epithelial differentiation with nests of keratinized squamous epithelium and areas of hair follicles (hematoxylin and eosin stain; magnification, 200⫻). Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
1. Marras T, Poenaru D, Kamal I: Perinatal management of nasopharyngeal teratoma. J Otolaryngol 24:310, 1995 2. Haghighi K, Milles M, Cleveland D, et al: Epignathus teratoma with bifid tongue and median glossal salivary mass: Report of a case. J Oral Maxillofac Surg 62:379, 2004 3. Vandenhaute B, Leteurtre E, Lecomte-Houcke M, et al: Epignathus teratoma: Report of three cases with a review of the literature. Cleft Palate Craniofac J 37:83, 2000 4. Lopes MA, Pereira CM, Da Cruz Perez DE, et al: Benign teratoma of the buccal mucosa in a 9-year-old girl: Report of case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 100:598, 2005 5. Hassan S, Sidek DS, Shah Jihan WD, et al: Massive lingual teratoma in a neonate. Singapore Med J 48:e212, 2007 6. Noguchi T, Jinbu Y, Itoh H, et al: Epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma: Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 101:481, 2006 7. Zhang GZ, Din GC, Zhao YF: Giant epignathus teratoma: Report of a case. J Oral Maxillofac Surg 65:337, 2007 8. Tamura T, Yamataka A, Okazaki T, et al: Management of a prenatally diagnosed huge teratoma arising from the soft palate. Asian J Surg 29:212, 2006 9. Sumiyoshi S, Yamamoto T, Shimozato K, et al: Massive immature teratoma in a neonate. Int J Oral Maxillofac Surg, 39: 1020-3, 2010 10. Zerella JT, Finberg FJ: Obstruction of the neonatal airway from teratomas. Surg Gynecol Obstet 170:126, 1990
BONET ET AL 11. Williams LJ, Yankowitz J, Robinson RA, et al: Nasopharyngeal teratoma. Report of a case with prenatal diagnosis and postnatal management. J Reprod Med 42:587, 1997 12. Levine AB, Alvarez M, Wedgwood J, et al: Contemporary management of a potentially lethal fetal anomaly: A successful perinatal approach to epignathus. Obstet Gynecol 76:962, 1990 13. Gunkel AR, Sturm C, Simma B, et al: [Transoral CO2 laser resection of extensive nasal and oropharyngeal teratoma]. Laryngorhinootologie 75:239, 1996 14. Chervenak FA, Isaacson G, Touloukian R, et al: Diagnosis and management of fetal teratomas. Obstet Gynecol 66:666, 1985
2813 15. Agris J, Renfro B, Grabb WC: Teratomatous choristoma and its associated syndromes. Case report. Plast Reconstr Surg 58:232, 1976 16. Celik M, Akkaya H, Arda I, et al: Congenital teratoma of the tongue: A case report and review of the literature. J Pediatr Surg 41:25, 2006 17. Benson RE, Fabbroni G, Russell JL: A large teratoma of the hard palate: A case report. Br J Oral Maxillofac Surg 47:46, 2009 18. Becker S, Schön R, Gutwald R, et al: A congenital teratoma with a cleft palate: Report of a case. Br J Oral Maxillofac Surg 45:326, 2007
Oral Teratomas: A Report of 5 Cases Cristina Bonet,* David Peñarrocha-Oltra,† J.M. Minguez,‡ Beatriz Vera-Sirera,§ Miguel Peñarrocha-Diago,储 and María Peñarrocha-Diago¶ Purpose: To present a case series of 5 patients with oral teratomas, discussing the treatment and
follow-up. Report of Cases: Five patients (4 girls and 1 boy) with oral teratomas presented at the Maxillofacial Surgery Department of a University Hospital with a reference population of 1,000,000 between 1980 and 2002. The associated lesions, clinical examination, histopathology, treatment and follow-up were registered and described. The newborns presented associated congenital malformations such as cleft palate, bifid tongue, dorso-nasal fistula and nasal dermoid cyst. In all 5 cases the tumor mass was excised at its base with surrounding normal tissue under general anesthesia combining conventional and electric scalpels. Histological analysis resulted in different compositions of multiple tissues typical of teratomas. After a mean follow-up of 8 years no sign of tumor recurrence had been detected. Conclusion: Teratomas were a rare finding within a large population of newborn patients. Five tumors were excised and histologically diagnosed as teratomas. No recurrence occurred after 8 years of follow-up. © 2012 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 70:2809-2813, 2012
Teratomas are neoplasms composed of multiple tissues from all 3 germinal layers (ectoderm, mesoderm, and endoderm), with differentiation to identifiable tissues and organs with variable levels of maturity.1,2 They occur in about 1:4,000 births, Received from the Valencia University Medical and Dental School, Valencia, Spain. *Master of Oral Surgery and Implantology, Department of Stomatology, Valencia University Medical and Dental School, Valencia, Spain. †Master of Oral Surgery and Implantology, Department of Stomatology, Valencia University Medical and Dental School, Valencia, Spain. ‡Associate Professor of Oral Surgery, Faculty of Dentistry, Cardenal Herrera CEU University, Valencia, Spain. §Resident of the Master in Endodontology, Department of Cariology Endodontology Pedodontology, ACTA Graduate School of Dentistry, Amsterdam, Holland. 储Chairman of Oral Surgery, Department of Stomatology, Valencia University Medical and Dental School, Valencia, Spain. ¶Associate Professor of Oral Surgery, Valencia University Medical and Dental School, Valencia, Spain. Address correspondence and reprint requests to Dr Peñarrocha Diago: Unidad Médico Quirúrgica, Clínica Odontológica, Gascó, Oliag 1, 46010 Valencia, Spain; e-mail: [email protected] © 2012 American Association of Oral and Maxillofacial Surgeons
0278-2391/12/7012-0$36.00/0 doi:10.1016/j.joms.2012.01.028
with a female predominance,2,3 most commonly in the sacrococcygeal region. Teratomas are commonly benign; however, malignant teratomas have been described.4 Oral teratomas are extremely rare and seen almost exclusively in infants, usually in neonates; most cases include the tongue.4 These tumors occur in approximately 1:35,000 to 1:200,000 of live births, comprising 2% to 9% of all teratomas.2 Teratomas have been classified into 4 subtypes: 1) dermoid, which contains tissues of mesodermal and epidermal origin; 2) teratoid, which contains tissue from the 3 primary germ layers but is poorly differentiated; 3) true teratoma, which is similar to the teratoid but differentiates histologically into recognizable tissues; and 4) epignathus, which is tridermal in origin but differentiates into recognizable organs, sometimes with limbs or even a visible second fetus.5 Prenatal ultrasonography may detect teratomas.5 Epignathi usually are diagnosed at birth. These tumors frequently accompany other anomalies such as cleft palate, bifid tongue, and double tongue.2,6 Sometimes the protruding tumor mass dislocates the temporomandibular joint.7-9 A giant epignathus can led to death in the neonatal period because of respiratory obstruction1,2,7; thus, the newborn should undergo surgery within the first days of life. The definitive treatment of teratoma is
2809
2810
9
3
7 5
5
Follow-Up (yrs)
complete surgical excision, and the prognosis is excellent. Recurrence has not been reported.4,5 This report describes 5 clinical cases of oral teratoma, including treatment and follow-up.
8
ORAL TERATOMAS
Stratified squamous epithelium, salivary gland, calcification, smooth muscle fibers 1.5 ⫻ 2.0 2⫻2
Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
24 F 5
Abbreviations: AS, age at surgery in days; F, female; M, male.
Between hemilingual structures Vomer
Striated muscle tissue, adipose tissue, salivary gland, stratified epithelium 2⫻2 F 4
20
Double tongue, cleft palate, dorsal hemivertebra, vitreous hemorrhages Double tongue, cleft palate
Posterior pharyngeal wall Between hemilingual structures Between hemilingual structures M 3
3 F 2
2
Double tongue, cleft palate, nasal dermoid cyst
Posterior pharyngeal wall
1.5 ⫻ 1.5 2.5 ⫻ 2.0
Fibroconjunctive stroma, adipose tissue and bundles of muscular fibers Ciliate, cylindrical, pseudostratified epithelium; adipose tissue, smooth, and striated muscle; mature cartilage Polypoid mass, skin, hair 2⫻2 F 1
2
Cleft palate, dorsonasal fistula
Posterior pharyngeal wall
2⫻2
Histology Size (cm) Location (Pedicle) Associated Malformation AS
The etiology of epignathus is unknown; the most popular theory is that an epignathus may arise from a disorganized growth of pluripotential cells in the region of the Rathke pouch.6,7,9 Oral teratoma is a rare congenital tumor that is seen almost exclusively in infants, usually in neonates.10 The incidence ranges from 1:35,000 to 1:200,000 of live births.2,11,12 There is a female predominance of 6:1.3,10 The cases presented represent an incidence of 5 cases in a reference population of 1,000,000 inhabitants over a period of 22 years. There was female predominance of 4:1. Teratomas are rare in the head and neck region, representing less than 5% of all cases.4 The clinical diagnosis in all 5 cases was made at birth; the urgency for surgery was determined by the degree of respiratory and feeding difficulties. Three cases (Figs 1-3) underwent urgent surgery within the first days of life because of the critical
Gender
Discussion
Case
The authors describe 5 cases of oral teratoma that presented from 1980 through 2002 at the maxillofacial surgery department of a university hospital with a reference population of 1,000,000 inhabitants. The cases involve 4 girls and 1 boy, none of whom presented any family history of interest. The associated lesions, clinical examination, histopathology, treatment, and follow-up are described. The newborns presented associated congenital malformations such as cleft palate, bifid tongue, dorsonasal fistula, and nasal dermoid cyst (Table 1). In cases 3 and 5, the teratomas had a double pedicle, and the remaining cases presented a single pedicle. The tumor size ranged from 2.5 ⫻ 2.0 cm to 1.5 ⫻ 2.0 cm (Table 1). In cases 1, 2, and 3 (Figs 1-3), because of the large size of the mass protruding from the mouth and the episodes of respiratory difficulty experienced by the infants, tumor excision was scheduled during the first days of life. In all 5 cases, the tumor mass was excised at its base with the surrounding normal tissue under general anesthesia. A conventional scalpel was used in combination with an electronic scalpel (Valleylab Force 30, Covidien Surgical Solutions Group, Boulder, Colorado) to achieve coagulation and thus hemostatic control; complete resection was performed to avoid possible recurrences. The results of the histologic examination of the tumors are presented in Table 1. At 6 months of age, cases 3, 4, and 5 (Figs 3-5) underwent surgery to repair the double tongues. The next stage in the surgical management was the surgical closure of the cleft palate at approximately 18 months of age. No sign of tumor recurrence was detected during a mean follow-up of 8 years.
Table 1. CLINICAL AND HISTOLOGICAL DATA OF THE PATIENTS WITH CONGENITAL TERATOMAS
Report of Cases
BONET ET AL
2811
FIGURE 1. Case 1. A, Newborn with teratoma protruding from the oral cavity. B, Panoramic histologic view of tumoral lesion showing mature cartilage islands, nests of epithelial differentiation, and a stromal component with smooth muscle areas (hematoxylin and eosin stain; magnification, 25⫻). Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
situation of the neonate. These lesions must be diagnosed and excised early, because they have an excellent prognosis if treated properly.1,13 Giant epignathi have been reported to occupy the entire
oropharynx, resulting in a high mortality rate in the neonatal period.7,8 In the present cases and those described by other investigators, the most frequent congenital malformations were cleft palate, a double tongue, and supernumerary teeth in the upper and lower incisor regions.2,3,6,11,14 Cases have been described in which the location and volume of the tumor dislocated the temporomandibular joint.7-9 Different locations of the pedicle were noted in the cases presented. In 3 cases the pedicle was attached to the posterior pharyngeal wall (cases 1, 2, and 3), and in 2 cases between the 2 hemilingual structures (cases 3 and 5). In case 5, which had a double pedicle, the second pedicle was located on the vomer (Table 1). Vandenhaute et al,3 in 2000, observed that most teratomas have a pedicle at the base of the cranium, in
FIGURE 2. Case 2. A, A large teratoma located on the posterior pharyngeal wall caused difficulties with breathing and swallowing in the newborn. B, Image of the oral teratoma extirpated at the base of the pedicle.
FIGURE 3. Case 3. Newborn with 2 oral teratomas, 1 pediculated at the posterior pharyngeal wall and the other between the 2 hemilingual structures.
Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
2812
FIGURE 4. Case 4. Teratoma located on the tongue. Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
the nasopharyngeal posterior region, close to the Rathke pouch, whereas most published cases of oral teratomas have described an attachment to the base of the tongue.2,5,6 Lopes et al,4 in 2005, reported the
ORAL TERATOMAS
first case of a benign teratoma in the oral mucosa of a 9-year-old girl. Cases of giant epignathi have been described in the literature (14 ⫻ 8 ⫻ 6 cm and weighing 1 kg).7 In the present cases, the size ranged from 1.5 to 2.5 cm. It is important to make an accurate differential diagnosis that includes dermoid cyst, cystic hygromalymphangioma, hemangioma, giant epulis, ranula, salivary gland tumors, and rhabdomyosarcoma.2,6,15,16 The neonatal mortality associated with epignathus is extremely high because of airway obstruction. At present, ex utero intrapartum treatment is considered the best procedure for the safe management of airway obstruction at birth.7-9,17 The definitive treatment is complete surgical excision because the cellular elements that form these cysts are active cells and incomplete removal will result in recurrence.10,18 In the 5 cases presented, complete extirpation of the tumors was performed at the base of the pedicle to avoid recurrence. A review of the literature disclosed no published cases of recurrence or familial inheritance. The malignant transformation of teratomas originating in the oropharyngeal area also has not been described.11 During the follow-up of the present patients, no recurrences were detected. The aim of presenting these rare tumors is to emphasize the importance of recognizing the suggestive prenatal data and preparing for perinatal management, including the maintenance of fetal oxygenation and the aggressive early provision of an adequate airway.1,6,7,10
References
FIGURE 5. Case 5. A, Newborn with lingual teratoma, producing a double tongue. B, Histologic view of the tumor showing predominant epithelial differentiation with nests of keratinized squamous epithelium and areas of hair follicles (hematoxylin and eosin stain; magnification, 200⫻). Bonet et al. Oral Teratomas. J Oral Maxillofac Surg 2012.
1. Marras T, Poenaru D, Kamal I: Perinatal management of nasopharyngeal teratoma. J Otolaryngol 24:310, 1995 2. Haghighi K, Milles M, Cleveland D, et al: Epignathus teratoma with bifid tongue and median glossal salivary mass: Report of a case. J Oral Maxillofac Surg 62:379, 2004 3. Vandenhaute B, Leteurtre E, Lecomte-Houcke M, et al: Epignathus teratoma: Report of three cases with a review of the literature. Cleft Palate Craniofac J 37:83, 2000 4. Lopes MA, Pereira CM, Da Cruz Perez DE, et al: Benign teratoma of the buccal mucosa in a 9-year-old girl: Report of case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 100:598, 2005 5. Hassan S, Sidek DS, Shah Jihan WD, et al: Massive lingual teratoma in a neonate. Singapore Med J 48:e212, 2007 6. Noguchi T, Jinbu Y, Itoh H, et al: Epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma: Report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 101:481, 2006 7. Zhang GZ, Din GC, Zhao YF: Giant epignathus teratoma: Report of a case. J Oral Maxillofac Surg 65:337, 2007 8. Tamura T, Yamataka A, Okazaki T, et al: Management of a prenatally diagnosed huge teratoma arising from the soft palate. Asian J Surg 29:212, 2006 9. Sumiyoshi S, Yamamoto T, Shimozato K, et al: Massive immature teratoma in a neonate. Int J Oral Maxillofac Surg, 39: 1020-3, 2010 10. Zerella JT, Finberg FJ: Obstruction of the neonatal airway from teratomas. Surg Gynecol Obstet 170:126, 1990
BONET ET AL 11. Williams LJ, Yankowitz J, Robinson RA, et al: Nasopharyngeal teratoma. Report of a case with prenatal diagnosis and postnatal management. J Reprod Med 42:587, 1997 12. Levine AB, Alvarez M, Wedgwood J, et al: Contemporary management of a potentially lethal fetal anomaly: A successful perinatal approach to epignathus. Obstet Gynecol 76:962, 1990 13. Gunkel AR, Sturm C, Simma B, et al: [Transoral CO2 laser resection of extensive nasal and oropharyngeal teratoma]. Laryngorhinootologie 75:239, 1996 14. Chervenak FA, Isaacson G, Touloukian R, et al: Diagnosis and management of fetal teratomas. Obstet Gynecol 66:666, 1985
2813 15. Agris J, Renfro B, Grabb WC: Teratomatous choristoma and its associated syndromes. Case report. Plast Reconstr Surg 58:232, 1976 16. Celik M, Akkaya H, Arda I, et al: Congenital teratoma of the tongue: A case report and review of the literature. J Pediatr Surg 41:25, 2006 17. Benson RE, Fabbroni G, Russell JL: A large teratoma of the hard palate: A case report. Br J Oral Maxillofac Surg 47:46, 2009 18. Becker S, Schön R, Gutwald R, et al: A congenital teratoma with a cleft palate: Report of a case. Br J Oral Maxillofac Surg 45:326, 2007