- Email: [email protected]
Teratoma of the Tongue Causing Respiratory Distress in a Neonate
Teratoma of the Tongue Surg 2003;15:274-279. Asian J Oral Maxillofac 2003;15:1xx-1xx. CASE REPORTS
Teratoma of the Tongue Causing Respiratory Distress in a Neonate 1
Masahiro Umemura,1 Kazuo Shimozato,2 Ichiro Oh-Iwa,1 Shigeyoshi Fujiwara,1 Hideo Ohshige1 Oral and Maxillo-facial Surgery, Japanese Red Cross Nagoya First Hospital, and 2The 2nd Department of Oral and Maxillofacial Surgery, School of Dentistry, Aichi-Gakuin University, Nagoya, Japan
Abstract A rapidly enlarging tongue teratoma causing respiratory distress during the early neonatal period is reported. The baby required intubation on the seventh day and a tracheostomy on the twenty-first day after birth. The tumour was diagnosed as an immature teratoma (grade 3) after biopsy and removed with a 2 mm margin of normal tissue on the forty-ninth day after birth. The tumour measured 70 x 70 x 60 mm, and appeared to arise from the dorsum of the tongue on the right side. The baby recovered well, was extubated, and was discharged from hospital on the seventy-second day after birth. Key words: Neonate, Respiratory distress, Teratoma, Tongue
Introduction Teratomas in childhood, similar to neuroblastomas, are generally regarded as a single group, which includes both benign and malignant lesions.1 The variable and complicated presentation of teratomas has resulted in controversies in their classification and difficulties in predicting prognosis. Some lesions may recur and invade the peritoneum in spite of their clinically benign features.2-4 This tumour is known to occur in the reproductive organs, the sacrococcygeal area, mediastinum, and the retroperitoneal space, but rarely in the head and neck region.1,5,6 This report is of a neonate with a teratoma in the tongue. This tumour caused severe impairment of swallowing and airway obstruction due to its very rapid growth.
Case Report The baby girl was born on 18 June 1996 by normal delivery at 39 weeks of gestation, with a body weight of 3302 g. Following delivery, breathing difficulty on suckling was recognised. After discharge from the maternity clinic, the degree of respiratory compromise increased. The patient was then admitted to a regional paediatric centre and required tracheal Correspondence: Masahiro Umemura, 3-35, Michishita-cho, Nakamura-ku, Nagoya City, Aichi, 453-8511, Japan. Tel: (81 52) 481 5111 ext 1421 E-mail: [email protected]
274
intubation for airway control on day 7 after birth. At the initial presentation to the paediatric centre, an enlarged tongue was recognised. Following computed tomography (CT) examination, ‘intra-oral tumour’ was suggested. Rapid enlargement of the tongue and airway obstruction necessitated tracheostomy on the twenty-first day after birth. The patient was then transferred to the Japanese Red Cross Nagoya First Hospital via the paediatric medical centre, for surgical treatment on 16 July 1996. At presentation to the department, the patient appeared stable with satisfactory airway control. Blood chemistry revealed high α-fetoprotein (AFP) of 2290 ng/ml. Magnetic resonance imaging (MRI) confirmed a tumour in the posterior part of the tongue (Figure 1a). The oral cavity was filled by the tumour, with the anterior part of the tongue pushed out of the rima oris (Figure 1b). Inability of the patient to swallow necessitated nasogastric tube feeding. An incisional biopsy under general anaesthesia was performed on the day after admission. Operative findings confirmed the presence of a tumour in the posterior part of the tongue, infiltrating into the tongue muscles. However, the extent of the tumour could not be delineated (Figure 1b). Histopathological diagnosis was a grade 3 immature teratoma (Table 1). Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
Umemura, Shimozato, Oh-Iwa, et al
a
Grade
Tumour characteristics
0
All tissues mature: no mitotic activity
1
Minor foci abnormally cellular or embryonal tissue mixed with mature elements: rare mitoses
2
Moderate quantities of embryonal tissue mixed with mature elements: moderate mitotic activity
3
Large quantities of embryonal tissue present
Table 1. Classification of teratoma.
outside the oral cavity. The tumour was thus completely exposed (Figures 2a and b). The degree of mouth opening was increased by the tumour mass beyond a normal physiological range, with bilateral temporomandiblar joint anterior dislocation. The epiglottis was now easily visible just posterior to the tumour. The degree of ‘exposure’ of the tumour proper simplified planning for surgical excision. With the possibility of reduced surgical morbidity, the family accepted surgical treatment.
b
Figure 1. (a) Magnetic resonance image showing tumour arising from the posterior part of oral tongue. (b) Tumour occupying the oral cavity, and the mobile normal tongue is pushed out through the rima oris deviating to the left side, forcing the mouth to remain open.
The intraoperative findings were discussed with the baby’s parents. The risks associated with the rapid enlargement of the tumour and possible surgical morbidity were explained. Surgery was initially strongly refused by the patient’s mother. A test dose of radiotherapy of 9 Gy was prescribed. Shrinkage of the tumour was not observed following the radiotherapy. Subsequent growth of the tumour resulted in further rotation of the tongue proper Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
On 7 August 1996, the tumour was excised under general anaesthesia. A 2 mm surgical margin in all directions was included. The tumour was successfully resected en block without any cut-through into the tumour mass . Branches of the hypoglossal nerve and the lingual artery to the tongue muscles of the right side were included in the specimen (Figure 3). The intraoperative blood loss was 20 ml, and the operation time was 18 minutes. The tumour measured 70 x 60 x 50 mm and arose from the middle part of the right body of the tongue. The anterior half of the mobile tongue was grossly displaced by the tumour to the left side (Figure 2b). Histopathological Findings The cut surface showed mucous cells of various sizes (Figure 3b). The tumour contained ectodermal, endodermal, mesenchymal, and neuroectodermal components with their immature derivatives (Figures 4, 5, and 6). The neuroectodermal component showed immature neuroepithelium, suggested by the presence of neuroblasts, rosette formation, and neural tube structures, with abundant nuclear degeneration, atrophy, and fission (Figure 4 ). These findings were also expressed in the smooth muscle and cartilage tissues of the mesenchymal component and the epithelial tissue of the endodermal component (Figures 5 and 6). Numerous small vesicles lined by columnar and squamous epithelium containing mucus 275
Teratoma of the Tongue
a
a
b
c b
Figure 3. (a) Resection specimen. (b) The cut surface shows mucous cells suggesting very rapid growth. (c) Surgical wound closure. Figure 2. (a) Extensive tumour growth out of the oral cavity, rotating oral floor and tongue components over the mandible, the epiglottis is visible just posterior to the tumour. (b) The tumour arises from the middle part of the right oral tongue, growing mainly toward the right side.
were evident in the epithelial tissue of endodermal origin (Figure 6). Postoperative Course The temporomandibular joints repositioned spontaneously on the fourth postoperative day, enabling 276
normal jaw movement and mouth closure. In spite of partial motor palsy of the right tongue due to resection of the hypoglossal nerve branches to the proper tongue muscles, suckling movement was preserved. Rehabilitation of oral feeding using a nursing bottle supplemented with nasogastric feeding was started on day 12 postoperatively. On day 14 postoperatively, the trachea was extubated. On day 27, the nasogastric tube was Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
Umemura, Shimozato, Oh-Iwa, et al
a
a
b
b
c
Figure 5. Abundant nuclear degeneration, atrophy and fission also expressed in (a) smooth muscle (hematoxylin and eosin x 400); and (b) chondrium, of the mesenchymal component (hematoxylin and eosin x 400).
Discussion
Figure 4. (a) Neuroectodermal component shows mostly immature nature of neuroepithelium, as suggested by presence of neuroblasts (hematoxylin and eosin x 200); (b) rosette formation (hematoxylin and eosin x 400); or (c) neural tube structure with abundant findings of nuclear degeneration, atrophy and fission (hematoxylin and eosin x 200).
removed as oral feeding was considered satisfactory (700 ml/7 times/day). The patient was discharged from the paediatric surgical centre on 10 September 1996, with slight motor palsy of the right tongue (Figure 7). There was no sign of recurrence after 5 years. Local developmental problems, deglutition, and articulation disorders were not observed. The partial right tongue motor disorder persisted. Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
The incidence of teratoma in neonates is 1 per 20,000 to 40,000 births. Those occurring in the head and neck region account for 3% to 6%.1,7-9 Among those in the head and neck region, 18 patients with teratomas involving the tongue have been reported in the past 80 years.6,10-17 Head and neck teratomas are complicated by obstruction of the upper aero-digestive tract and/or compression of vital organs. It therefore may not be straightforward to arrive at a definitive diagnosis.18,19 Impending airway obstruction often necessitates endotracheal intubations or emergency tracheotomy.18,19 Rapid tumour growth increases the surgical sequelae of injury to vital anatomic structures, severe blood loss and post-surgical deformities.10,20 Teratomas arise from multipotential stem cells. The tumour is composed of a variety of tissues, which develop independently from surrounding anatomical sites.5,6,14,18,20 The multipotential nature of the stem cell explains the presence of tissues of ecto-, meso-, and endodermal origin.14,18,20 Widely used 277
Teratoma of the Tongue
a
Figure 7. Residual motor palsy of the right tongue.
b
Figure 6. Large number of small vesicles lined by (a) columnar (hematoxylin and eosin x 200) and (b) squamous epithelium containing mucus is evident in the epithelial tissue of endodermal origin (hematoxylin and eosin x 200).
classifications of teratoid tumours (germ cell tumour) divide these tumours into benign, maturated teratoma, low malignant immature teratoma, and malignant tumour (germinoma, embryonal-carcinoma, yolk sac tumour, and choriocarcinoma).6,21-23 In the benign maturated teratoma, the component tissues are mixed in various proportions, and are sufficiently maturated to be identified.6,22,23 In immature teratoma, at least 1 of the 3 germ layer-derived components contains immature elements.6,22,23 The immature teratoma in childhood is generally regarded as a benign tumour in contrast to that occurring in an adult, which is usually regarded as a malignancy.6,23,24 The presence of immature components in teratoma strongly suggests its malignant nature. However, the behaviour of the tumour varies from relatively benign to aggressive. Aggressive tumours are associated with local recurrence with peritoneal dissemination.2-4,18,25 It has been suggested that the clinical behaviour of the 278
tumour correlates with the proportion of immature cells. Based on this, a classification into 4 grades was proposed (Table 1). Grade 0 to 1 tumours show minimal malignant potential, while grades 2 to 3 tumours show malignant courses. 25,26 This classification is thought to be useful for adult patients, but not for children.23,24 The immature structures most commonly seen in the immature teratoma are of neural origin. These include structures similar to neural tube cells, rosette-forming cells, and neuroepithelial cells.6,16 In addition, undifferentiated mesenchymal cells and immature myocytes could be present.6,11,12,13 The present tumour was classified as a grade 3 immature teratoma based on the above-mentioned criteria (Figures 4, 5, and 6). The treatment approach for immature teratoma of children remains controversial. Paediatric patients usually have a better prognosis than adults.23,24 The most important treatment may be surgical resection, especially for low-grade malignancies.3,6,10,22-24,26 Systemic chemotherapy may also be added for advanced or peritoneally disseminated cases.24,27,28 The role and efficacy of radiation therapy for paediatric patients is not yet established.4,22 In the present patient, potential malignancy was suggested by the clinical course of the tumour. Further follow up is needed to evaluate the outcome of the treatment.
References 1. Hashizume K. Teratomas and germ cell tumors in children in Japan: an analysis of data gatherd by the Committee on Tumors Registration of the Japanese Society of Pediatric Surgeons [article in Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
Umemura, Shimozato, Oh-Iwa, et al
Japanese]. Jpn J Prediatr Surg 1995;27:861-865. 2. Nagashima K, Yamaguchi K, Hasui K, Oota K. Malignant glimatosisi peritonei originating from cystic ovarian teratoma [article in Japanese]. Acta Path Jpn 1974;24:529-539 . 3. Billmire DF, Grosfeld JL. Teratomas in childhood — analysis of 142 cases. J? Pediatr Surg 1986; 21:548-551. 4. Brodeur GM, Howarth CB, Pratt CB, Caces J, Hustu HO. Malignant germ cell tumors in 57 children and adolescents. Cancer 1981;48: 1890-1898 . 5. Tapper D, Lack EE. Teratomas in infancy and childhood — a 54-year experience at the Children’s Hospital Medical Center. Ann Surg 1983;98:398-410. 6. Lalwani AK, Engel TL. Teratoma of the tongue: a case report and review of the literature. Int J Pediatr Otorhinolaryngol 1992;24:261-268. 7. Teal LN, Angtuaco TL, Jimenez JF, Quirk Jr JG. Fetal teratomas. Antenatal diagnosis and clinical management. J Clin Ultrasound 1988;16:329-332. 8. Dunn CJ, Nguyen DL, Leonard JC. Ultrasound dignosis of immature cervical teratoma?A case report. Am J Perionatol 1992;9:445-447. 9. Grosfeld JL, Ballantine TVN, Lowe D, Baehner RL. Benign and malignant teratomas in children: Analysis of 85 patients. Surgery 1976;80:297-305. 10. McMahon MJ, Chescheir NC, Kuller JA, Wells SR, Wright LN, Nakayama DK. Perinatal management of a lingual teratoma. Obstet Gynecol 1996;87:848-851. 11. Towne BM, Hurst R. Firm mass on the Posterior Tongue. J Oral Maxillofac Surg 1997;55:987-991. 12. Valtonen H, Nuutinen J, Karja J, Collan Y. Congenital dermoid cyst of the tongue. J Laryngol Otol 1986;100:965-969. 13. Shaari CM, Ho BT, Shah K, Biller HF. Lingual dermoid cyst. Otolaryngol Head Neck Surg 1995; 112:476-478. 14. Kountakis SE, Minotti AM, Mailland A, Stiernberg CM. Teratomas of the head and neck. Am J Otolaryngol 1994;15:292-296. 15. Tumushime-Buturo CG, Nkanza NK. Glial teratoma thought to be a lingual thyroid. J Laryngol Otol 1989;103:620-621. 16. Rodin AE, Singla P. Teratomas of the tongue present at birth. Pediatr Pathol 1985;3:291-298.
Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
17. Dudgeon DL, Isaacs H Jr, Hays DM. Multiple teratomas of the head and neck. J Pediatr 1974;85: 139-140. 18. Jordan RB, Gauderer MWL. Cervical teratomas: an analysis. Literature review and proposed classification. J Pediatr Surg 1988;23:583-591. 19. Zerella JT, Finberg FJ. Obstruction of the neonatal airway from teratomas. Surg Gynecol Obstet 1990;170:126-131. 20. Nakada K, Fujioka T, Kashimura T, Ohkawa I, Sato Y, Yamate N. Teratoma of the head and neck [article in Japanese]. Jpn J Prediatr Surg 1995; 27:931-937. 21. Mahour GH, Wooley MM, Trivedi SN, Landing BH. Teratomas in infancy and childhood: experience with 81 cases. Surgery 1974;76:309-318. 22. Cohen MD (editor). Imaging of children with cancer. St Louis: Mosby-Year Book; 1992. 23. Sasaki Y, Tanaka Y, Kashimura T, Misugi K. Histological classification of teratoma group tumors [article in Japanese]. Jpn J Prediatr Surg 1995;27:866-872. 24. Ablin AR, Krailo MD, Ramsay NK, Malogolowkin MH, Isaacs H, Raney RB, Adkins J, Hays DM, Benjamin DR, Grosfeld JL. Results of treatment of malignant germ cell tumors in 93 children: a report from the Childrens Cancer Study Group. J Clin Oncol 1991;9:1782-1792. 25. Norris HJ, Zirkin HJ, Benson WL. Immature (malignant) teratoma of the ovary: a clinical and pathologic study of 58 cases. Cancer 1976;37: 2359-2372. 26. Robby SJ, Scully RE. Ovarian teratoma with glial implants on the peritoneum. Analysis of 12 cases. Human Pathology 1970;1:643-653 27. Bosl GJ, Gluckman R, Geller NL, Golbey RB, Whitmore WF Jr, Herr H, Sogani P, Morse M, Martini N, Bains M. VAB-6: an effective chemotherapy regimen for patients with germcell tumors. J Clin Oncol 1986;4:1493-1499. 28. Stoter G, Koopman A, Vendrik CPJ, Struyvenberg A, Sleyfer DT, Willemse PHB, Koops HS, van Oosterom AT, ten Bokkel Huinink WW, Pinedo HM. Ten-year survival and late sequelae in testicular cancer patients treated with cisplatin, vinblastine, and bleomycin. J Clin Oncol 1989;7:1099-1104.
279
Teratoma of the Tongue Causing Respiratory Distress in a Neonate 1
Masahiro Umemura,1 Kazuo Shimozato,2 Ichiro Oh-Iwa,1 Shigeyoshi Fujiwara,1 Hideo Ohshige1 Oral and Maxillo-facial Surgery, Japanese Red Cross Nagoya First Hospital, and 2The 2nd Department of Oral and Maxillofacial Surgery, School of Dentistry, Aichi-Gakuin University, Nagoya, Japan
Abstract A rapidly enlarging tongue teratoma causing respiratory distress during the early neonatal period is reported. The baby required intubation on the seventh day and a tracheostomy on the twenty-first day after birth. The tumour was diagnosed as an immature teratoma (grade 3) after biopsy and removed with a 2 mm margin of normal tissue on the forty-ninth day after birth. The tumour measured 70 x 70 x 60 mm, and appeared to arise from the dorsum of the tongue on the right side. The baby recovered well, was extubated, and was discharged from hospital on the seventy-second day after birth. Key words: Neonate, Respiratory distress, Teratoma, Tongue
Introduction Teratomas in childhood, similar to neuroblastomas, are generally regarded as a single group, which includes both benign and malignant lesions.1 The variable and complicated presentation of teratomas has resulted in controversies in their classification and difficulties in predicting prognosis. Some lesions may recur and invade the peritoneum in spite of their clinically benign features.2-4 This tumour is known to occur in the reproductive organs, the sacrococcygeal area, mediastinum, and the retroperitoneal space, but rarely in the head and neck region.1,5,6 This report is of a neonate with a teratoma in the tongue. This tumour caused severe impairment of swallowing and airway obstruction due to its very rapid growth.
Case Report The baby girl was born on 18 June 1996 by normal delivery at 39 weeks of gestation, with a body weight of 3302 g. Following delivery, breathing difficulty on suckling was recognised. After discharge from the maternity clinic, the degree of respiratory compromise increased. The patient was then admitted to a regional paediatric centre and required tracheal Correspondence: Masahiro Umemura, 3-35, Michishita-cho, Nakamura-ku, Nagoya City, Aichi, 453-8511, Japan. Tel: (81 52) 481 5111 ext 1421 E-mail: [email protected]
274
intubation for airway control on day 7 after birth. At the initial presentation to the paediatric centre, an enlarged tongue was recognised. Following computed tomography (CT) examination, ‘intra-oral tumour’ was suggested. Rapid enlargement of the tongue and airway obstruction necessitated tracheostomy on the twenty-first day after birth. The patient was then transferred to the Japanese Red Cross Nagoya First Hospital via the paediatric medical centre, for surgical treatment on 16 July 1996. At presentation to the department, the patient appeared stable with satisfactory airway control. Blood chemistry revealed high α-fetoprotein (AFP) of 2290 ng/ml. Magnetic resonance imaging (MRI) confirmed a tumour in the posterior part of the tongue (Figure 1a). The oral cavity was filled by the tumour, with the anterior part of the tongue pushed out of the rima oris (Figure 1b). Inability of the patient to swallow necessitated nasogastric tube feeding. An incisional biopsy under general anaesthesia was performed on the day after admission. Operative findings confirmed the presence of a tumour in the posterior part of the tongue, infiltrating into the tongue muscles. However, the extent of the tumour could not be delineated (Figure 1b). Histopathological diagnosis was a grade 3 immature teratoma (Table 1). Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
Umemura, Shimozato, Oh-Iwa, et al
a
Grade
Tumour characteristics
0
All tissues mature: no mitotic activity
1
Minor foci abnormally cellular or embryonal tissue mixed with mature elements: rare mitoses
2
Moderate quantities of embryonal tissue mixed with mature elements: moderate mitotic activity
3
Large quantities of embryonal tissue present
Table 1. Classification of teratoma.
outside the oral cavity. The tumour was thus completely exposed (Figures 2a and b). The degree of mouth opening was increased by the tumour mass beyond a normal physiological range, with bilateral temporomandiblar joint anterior dislocation. The epiglottis was now easily visible just posterior to the tumour. The degree of ‘exposure’ of the tumour proper simplified planning for surgical excision. With the possibility of reduced surgical morbidity, the family accepted surgical treatment.
b
Figure 1. (a) Magnetic resonance image showing tumour arising from the posterior part of oral tongue. (b) Tumour occupying the oral cavity, and the mobile normal tongue is pushed out through the rima oris deviating to the left side, forcing the mouth to remain open.
The intraoperative findings were discussed with the baby’s parents. The risks associated with the rapid enlargement of the tumour and possible surgical morbidity were explained. Surgery was initially strongly refused by the patient’s mother. A test dose of radiotherapy of 9 Gy was prescribed. Shrinkage of the tumour was not observed following the radiotherapy. Subsequent growth of the tumour resulted in further rotation of the tongue proper Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
On 7 August 1996, the tumour was excised under general anaesthesia. A 2 mm surgical margin in all directions was included. The tumour was successfully resected en block without any cut-through into the tumour mass . Branches of the hypoglossal nerve and the lingual artery to the tongue muscles of the right side were included in the specimen (Figure 3). The intraoperative blood loss was 20 ml, and the operation time was 18 minutes. The tumour measured 70 x 60 x 50 mm and arose from the middle part of the right body of the tongue. The anterior half of the mobile tongue was grossly displaced by the tumour to the left side (Figure 2b). Histopathological Findings The cut surface showed mucous cells of various sizes (Figure 3b). The tumour contained ectodermal, endodermal, mesenchymal, and neuroectodermal components with their immature derivatives (Figures 4, 5, and 6). The neuroectodermal component showed immature neuroepithelium, suggested by the presence of neuroblasts, rosette formation, and neural tube structures, with abundant nuclear degeneration, atrophy, and fission (Figure 4 ). These findings were also expressed in the smooth muscle and cartilage tissues of the mesenchymal component and the epithelial tissue of the endodermal component (Figures 5 and 6). Numerous small vesicles lined by columnar and squamous epithelium containing mucus 275
Teratoma of the Tongue
a
a
b
c b
Figure 3. (a) Resection specimen. (b) The cut surface shows mucous cells suggesting very rapid growth. (c) Surgical wound closure. Figure 2. (a) Extensive tumour growth out of the oral cavity, rotating oral floor and tongue components over the mandible, the epiglottis is visible just posterior to the tumour. (b) The tumour arises from the middle part of the right oral tongue, growing mainly toward the right side.
were evident in the epithelial tissue of endodermal origin (Figure 6). Postoperative Course The temporomandibular joints repositioned spontaneously on the fourth postoperative day, enabling 276
normal jaw movement and mouth closure. In spite of partial motor palsy of the right tongue due to resection of the hypoglossal nerve branches to the proper tongue muscles, suckling movement was preserved. Rehabilitation of oral feeding using a nursing bottle supplemented with nasogastric feeding was started on day 12 postoperatively. On day 14 postoperatively, the trachea was extubated. On day 27, the nasogastric tube was Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
Umemura, Shimozato, Oh-Iwa, et al
a
a
b
b
c
Figure 5. Abundant nuclear degeneration, atrophy and fission also expressed in (a) smooth muscle (hematoxylin and eosin x 400); and (b) chondrium, of the mesenchymal component (hematoxylin and eosin x 400).
Discussion
Figure 4. (a) Neuroectodermal component shows mostly immature nature of neuroepithelium, as suggested by presence of neuroblasts (hematoxylin and eosin x 200); (b) rosette formation (hematoxylin and eosin x 400); or (c) neural tube structure with abundant findings of nuclear degeneration, atrophy and fission (hematoxylin and eosin x 200).
removed as oral feeding was considered satisfactory (700 ml/7 times/day). The patient was discharged from the paediatric surgical centre on 10 September 1996, with slight motor palsy of the right tongue (Figure 7). There was no sign of recurrence after 5 years. Local developmental problems, deglutition, and articulation disorders were not observed. The partial right tongue motor disorder persisted. Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
The incidence of teratoma in neonates is 1 per 20,000 to 40,000 births. Those occurring in the head and neck region account for 3% to 6%.1,7-9 Among those in the head and neck region, 18 patients with teratomas involving the tongue have been reported in the past 80 years.6,10-17 Head and neck teratomas are complicated by obstruction of the upper aero-digestive tract and/or compression of vital organs. It therefore may not be straightforward to arrive at a definitive diagnosis.18,19 Impending airway obstruction often necessitates endotracheal intubations or emergency tracheotomy.18,19 Rapid tumour growth increases the surgical sequelae of injury to vital anatomic structures, severe blood loss and post-surgical deformities.10,20 Teratomas arise from multipotential stem cells. The tumour is composed of a variety of tissues, which develop independently from surrounding anatomical sites.5,6,14,18,20 The multipotential nature of the stem cell explains the presence of tissues of ecto-, meso-, and endodermal origin.14,18,20 Widely used 277
Teratoma of the Tongue
a
Figure 7. Residual motor palsy of the right tongue.
b
Figure 6. Large number of small vesicles lined by (a) columnar (hematoxylin and eosin x 200) and (b) squamous epithelium containing mucus is evident in the epithelial tissue of endodermal origin (hematoxylin and eosin x 200).
classifications of teratoid tumours (germ cell tumour) divide these tumours into benign, maturated teratoma, low malignant immature teratoma, and malignant tumour (germinoma, embryonal-carcinoma, yolk sac tumour, and choriocarcinoma).6,21-23 In the benign maturated teratoma, the component tissues are mixed in various proportions, and are sufficiently maturated to be identified.6,22,23 In immature teratoma, at least 1 of the 3 germ layer-derived components contains immature elements.6,22,23 The immature teratoma in childhood is generally regarded as a benign tumour in contrast to that occurring in an adult, which is usually regarded as a malignancy.6,23,24 The presence of immature components in teratoma strongly suggests its malignant nature. However, the behaviour of the tumour varies from relatively benign to aggressive. Aggressive tumours are associated with local recurrence with peritoneal dissemination.2-4,18,25 It has been suggested that the clinical behaviour of the 278
tumour correlates with the proportion of immature cells. Based on this, a classification into 4 grades was proposed (Table 1). Grade 0 to 1 tumours show minimal malignant potential, while grades 2 to 3 tumours show malignant courses. 25,26 This classification is thought to be useful for adult patients, but not for children.23,24 The immature structures most commonly seen in the immature teratoma are of neural origin. These include structures similar to neural tube cells, rosette-forming cells, and neuroepithelial cells.6,16 In addition, undifferentiated mesenchymal cells and immature myocytes could be present.6,11,12,13 The present tumour was classified as a grade 3 immature teratoma based on the above-mentioned criteria (Figures 4, 5, and 6). The treatment approach for immature teratoma of children remains controversial. Paediatric patients usually have a better prognosis than adults.23,24 The most important treatment may be surgical resection, especially for low-grade malignancies.3,6,10,22-24,26 Systemic chemotherapy may also be added for advanced or peritoneally disseminated cases.24,27,28 The role and efficacy of radiation therapy for paediatric patients is not yet established.4,22 In the present patient, potential malignancy was suggested by the clinical course of the tumour. Further follow up is needed to evaluate the outcome of the treatment.
References 1. Hashizume K. Teratomas and germ cell tumors in children in Japan: an analysis of data gatherd by the Committee on Tumors Registration of the Japanese Society of Pediatric Surgeons [article in Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
Umemura, Shimozato, Oh-Iwa, et al
Japanese]. Jpn J Prediatr Surg 1995;27:861-865. 2. Nagashima K, Yamaguchi K, Hasui K, Oota K. Malignant glimatosisi peritonei originating from cystic ovarian teratoma [article in Japanese]. Acta Path Jpn 1974;24:529-539 . 3. Billmire DF, Grosfeld JL. Teratomas in childhood — analysis of 142 cases. J? Pediatr Surg 1986; 21:548-551. 4. Brodeur GM, Howarth CB, Pratt CB, Caces J, Hustu HO. Malignant germ cell tumors in 57 children and adolescents. Cancer 1981;48: 1890-1898 . 5. Tapper D, Lack EE. Teratomas in infancy and childhood — a 54-year experience at the Children’s Hospital Medical Center. Ann Surg 1983;98:398-410. 6. Lalwani AK, Engel TL. Teratoma of the tongue: a case report and review of the literature. Int J Pediatr Otorhinolaryngol 1992;24:261-268. 7. Teal LN, Angtuaco TL, Jimenez JF, Quirk Jr JG. Fetal teratomas. Antenatal diagnosis and clinical management. J Clin Ultrasound 1988;16:329-332. 8. Dunn CJ, Nguyen DL, Leonard JC. Ultrasound dignosis of immature cervical teratoma?A case report. Am J Perionatol 1992;9:445-447. 9. Grosfeld JL, Ballantine TVN, Lowe D, Baehner RL. Benign and malignant teratomas in children: Analysis of 85 patients. Surgery 1976;80:297-305. 10. McMahon MJ, Chescheir NC, Kuller JA, Wells SR, Wright LN, Nakayama DK. Perinatal management of a lingual teratoma. Obstet Gynecol 1996;87:848-851. 11. Towne BM, Hurst R. Firm mass on the Posterior Tongue. J Oral Maxillofac Surg 1997;55:987-991. 12. Valtonen H, Nuutinen J, Karja J, Collan Y. Congenital dermoid cyst of the tongue. J Laryngol Otol 1986;100:965-969. 13. Shaari CM, Ho BT, Shah K, Biller HF. Lingual dermoid cyst. Otolaryngol Head Neck Surg 1995; 112:476-478. 14. Kountakis SE, Minotti AM, Mailland A, Stiernberg CM. Teratomas of the head and neck. Am J Otolaryngol 1994;15:292-296. 15. Tumushime-Buturo CG, Nkanza NK. Glial teratoma thought to be a lingual thyroid. J Laryngol Otol 1989;103:620-621. 16. Rodin AE, Singla P. Teratomas of the tongue present at birth. Pediatr Pathol 1985;3:291-298.
Asian J Oral Maxillofac Surg Vol 15, No 4, 2003
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