Page 1 of 2 Case report Licensee OA Publishing London 2013. Creative Commons Attribution License (CC-BY) Competing interests: none declared. Conflict of interests: none declared. All authors contributed to the conception, design, and preparation of the manuscript, as well as read and approved the final manuscript. All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure. For citation purposes: Hu R, Jiang RS. The recurrence of a soft palate teratoma in a neonate: a case report. Head Neck Oncol. 2013 Feb 06;5(2):16. The recurrence of a soft palate teratoma in a neonate: a case report R Hu 1 , RS Jiang 1 * Abstract Teratomas are rare malformations containing cells from ectodermal, mes- odermal and endodermal layers, seldom seen in the soft palate. A 2-day-old girl presenting a teratoma arising from the soft palate was surgically treated. Unfortunately, recurrence was observed during follow-up. Introduction Teratomas are rarely observed in the soft palate. A teratoma, as defined by Weaver et al. 1 , is a tumour consisting of multiple tissues that are not indig- enous to their site of origin. The most important complication of oral tera- tomas is respiratory compromise, which is the main cause of death in neonates 2,3 . Surgical resection is the treatment of choice for oral terato- mas. Although the follow-up is short in most cases, there are no reports of recurrence of oral teratomas 4,5 . A case of a soft palate teratoma in a neonate is reported herein; recurrence was observed in 4 years follow-up. Case report A female infant was born to a 20-year- old mother by vaginal delivery after 37 +3 weeks gestation with birth weight of 3.05 kg. A congenital tumour was found in the mouth, which prevented oral feeding. Oral clinical examination revealed an asympto- matic mass measuring 3.0 × 4.0 × 2.5 cm, originating from the soft palate near the midline (Figure 1). The mother did not undergo a three-dimensional (3D)-ultrasonography examination during her pregnancy. The prenatal and perinatal courses were uncompli- cated, and the family history was neg- ative. The father was 27 years old and in good health. The infant was admit- ted to our department on the second day after birth because of irregular respiratory cycle. Shortness of breath and lip cyanosis was observed, espe- cially when the infant was being fed. Due to repeated episodes of apnea, tra- cheal intubation ventilator support was provided and the infant was transferred to a neonatal intensive care unit. Com- puted tomography showed an oral cav- ity mass without intracranial extension. On the seventh day after birth, under general anaesthesia with intubation, the mass was excised from the soft pal- ate finding a short peduncle 1 cm in diameter and the mucosal defect was repaired by suturing a transferred local palatal flap (Figure 2). No cerebrospi- nal fluid leakage occurred from the excision site. After surgery, the wound healed well and the baby tolerated oral feeds. No respiratory compromise occurred any more. Histopathological examination revealed a mature tera- toma composed of mature respiratory epithelium, glandular tissues, neuro- glial tissue, choroid plexus, muscle and blood vessels (Figure 3). Some margins showed nerve and muscle. Signs of recurrence were found in the second year of follow-up. Magnetic resonance imaging (MRI) indicated maxillary tumour with cystic and solid areas with fat density (Figure 4). The parents wanted to “wait and watch”, while hoping for a better prognosis, instead of opting for imme- diate reoperation. Discussion Teratomas are rare malformations containing cells from ectodermal, mesodermal and endodermal layers with a variable degree of differentia- tion 6 . They have an incidence of 1:4000 live births; less than 2% of these arise from the oropharyngeal cavity 1,7 . Nasopharynx is one of the most fre- quent sites for head and neck terato- mas and has a 6:1 female predominance. In contrast, oral teratomas do not present a clear gender predilection 8 . Oral teratomas arise anywhere in the oronasal cavity and are regarded as expanding, cavity-filling lesions, espe- cially in the neonatal period 9 . The main therapy of teratomas is complete surgical excision, which depends on the site of the tumour. Unless the teratoma is expanding massively into the cranial area, resection of tumour may be attempted. Initial treatment should be directed toward airway manage- ment and feeding problems 10 . When a neonate is experiencing respira- tory difficulty, the first priority should be stabilization of the airway 11 . Our case presented with an obstructive mass causing respira- tory embarrassment and immediate threat to life, which demanded establishment of an airway with tracheostomy. Histologically, teratomas may pre- sent different characteristics. In oral teratomas, the most common tissues observed are nerves and cartilages. Other tissues commonly seen are mus- cles, bones and respiratory epithelia 9 . Our case showed mature respiratory epithelia, glandular tissues, neuroglial tissues, choroid plexuses, muscles and blood vessels. Teratomas are associated with concomitant malformations in 6% of all cases, with cleft palate being the most commonly associated anomaly 5 . In the present case, congenital cardiac septal * Corresponding author Email: [email protected] 1 Department of Reconstructive Plastic Surgery, the Children’s Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang Province, China
Welcome message from author
This document is posted to help you gain knowledge. Please leave a comment to let me know what you think about it! Share it to your friends and learn new things together.
Transcript
HNO16_Feb13.inddLicensee OA Publishing London 2013. Creative
Commons Attribution License (CC-BY)
Co m
pe tin
g in
te re
st s:
n on
e de
cl ar
ed . C
on fli
ct o
ed ic
al E
th ic
s (A
M E)
e th
ic al
ru le
s of
d is
cl os
ur e.
For citation purposes: Hu R, Jiang RS. The recurrence of a soft palate teratoma in a neonate: a case report. Head Neck Oncol. 2013 Feb 06;5(2):16.
The recurrence of a soft palate teratoma in a neonate: a case report R Hu1, RS Jiang1*
Abstract Teratomas are rare malformations containing cells from ectodermal, mes- odermal and endodermal layers, seldom seen in the soft palate. A 2-day-old girl presenting a teratoma arising from the soft palate was surgically treated. Unfortunately, recurrence was observed during follow-up.
Introduction Teratomas are rarely observed in the soft palate. A teratoma, as defined by Weaver et al.1, is a tumour consisting of multiple tissues that are not indig- enous to their site of origin. The most important complication of oral tera- tomas is respiratory compromise, which is the main cause of death in neonates2,3. Surgical resection is the treatment of choice for oral terato- mas. Although the follow-up is short in most cases, there are no reports of recurrence of oral teratomas4,5. A case of a soft palate teratoma in a neonate is reported herein; recurrence was observed in 4 years follow-up.
Case report A female infant was born to a 20-year- old mother by vaginal delivery after 37+3 weeks gestation with birth weight of 3.05 kg. A congenital tumour was found in the mouth, which prevented oral feeding. Oral clinical examination revealed an asympto- matic mass measuring 3.0 × 4.0 × 2.5 cm, originating from the soft palate near the midline (Figure 1). The mother did not undergo a three-dimensional (3D)-ultrasonography examination
during her pregnancy. The prenatal and perinatal courses were uncompli- cated, and the family history was neg- ative. The father was 27 years old and in good health. The infant was admit- ted to our department on the second day after birth because of irregular respiratory cycle. Shortness of breath and lip cyanosis was observed, espe- cially when the infant was being fed. Due to repeated episodes of apnea, tra- cheal intubation ventilator support was provided and the infant was transferred to a neonatal intensive care unit. Com- puted tomography showed an oral cav- ity mass without intracranial extension. On the seventh day after birth, under general anaesthesia with intubation, the mass was excised from the soft pal- ate finding a short peduncle 1 cm in diameter and the mucosal defect was repaired by suturing a transferred local palatal flap (Figure 2). No cerebrospi- nal fluid leakage occurred from the excision site. After surgery, the wound healed well and the baby tolerated oral feeds. No respiratory compromise occurred any more. Histopathological examination revealed a mature tera- toma composed of mature respiratory epithelium, glandular tissues, neuro- glial tissue, choroid plexus, muscle and blood vessels (Figure 3). Some margins showed nerve and muscle. Signs of recurrence were found in the second year of follow-up. Magnetic resonance imaging (MRI) indicated maxillary tumour with cystic and solid areas with fat density (Figure 4). The parents wanted to “wait and watch”, while hoping for a better prognosis, instead of opting for imme- diate reoperation.
Discussion Teratomas are rare malformations containing cells from ectodermal,
mesodermal and endodermal layers with a variable degree of differentia- tion6. They have an incidence of 1:4000 live births; less than 2% of these arise from the oropharyngeal cavity1,7. Nasopharynx is one of the most fre- quent sites for head and neck terato- mas and has a 6:1 female predominance. In contrast, oral teratomas do not present a clear gender predilection8. Oral teratomas arise anywhere in the oronasal cavity and are regarded as expanding, cavity-filling lesions, espe- cially in the neonatal period9.
The main therapy of teratomas is complete surgical excision, which depends on the site of the tumour. Unless the teratoma is expanding massively into the cranial area, resection of tumour may be attempted. Initial treatment should be directed toward airway manage- ment and feeding problems10. When a neonate is experiencing respira- tory difficulty, the first priority should be stabilization of the airway11. Our case presented with an obstructive mass causing respira- tory embarrassment and immediate threat to life, which demanded establishment of an airway with tracheostomy.
Histologically, teratomas may pre- sent different characteristics. In oral teratomas, the most common tissues observed are nerves and cartilages. Other tissues commonly seen are mus- cles, bones and respiratory epithelia9. Our case showed mature respiratory epithelia, glandular tissues, neuroglial tissues, choroid plexuses, muscles and blood vessels.
Teratomas are associated with concomitant malformations in 6% of all cases, with cleft palate being the most commonly associated anomaly5. In the present case, congenital cardiac septal
* Corresponding author Email: [email protected] 1 Department of Reconstructive Plastic Surgery,
the Children’s Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang Province, China
Page 2 of 2
Licensee OA Publishing London 2013. Creative Commons Attribution License (CC-BY)
Co m
pe tin
g in
te re
st s:
n on
e de
cl ar
ed . C
on fli
ct o
ed ic
al E
th ic
s (A
M E)
e th
ic al
ru le
s of
d is
cl os
ur e.
For citation purposes: Hu R, Jiang RS. The recurrence of a soft palate teratoma in a neonate: a case report. Head Neck Oncol. 2013 Feb 06;5(2):16.
defects and patency of ductusarteriosus were detected. Richieri-Costa et al.12 have also reported the observation of cardiac abnormalities.
Teratomas are mostly benign in the neonatal period 5%; of the cases present malignity criteria on histopa- thology13. Usually, benign teratomas consist of mature tissue components, while those with malignant potential contain immature tissues; there is a higher incidence of malignancy in adults. Incomplete resection and presence of primitive neural tissue entail the risk of a malignant relapse14,15. Becker et al.5 reported the recurrence of a congenital epignathus post-operation; this did not necessar- ily imply malignancy, although the clinician should continue follow-up screening.
It has been 3 years since the recur- rence of oral teratoma; the girl is still in good condition without any drugs or surgery. Because the risk of
malignant change is evident, long- term follow-up is mandatory.
Acknowledgements We thank the patient and her parents for participation. We also thank Mr Grahay Rester for critical reading of the manuscript.
References 1. Weaver RG, Meyerhoff WL, Gates GA. Teratomas of head and neck. Surg Forum. 1976;27(62):539–42. 2. Wakhlu A, Wakhlu AK. Head and neck terotmas in children. Pediatr Surg Int. 2000;16(5-6):333–7. 3. Yoon JK, Kim J, Park C. Congenital immature teratoma of the tongue: an autopsy case. Oral Surg Oral Med Oral
Pathol Oral Radiol Endod. 2002 Dec;94(6): 741–5. 4. Cay A, Bektas D, Imamoglu M, Bahadir O, Cobanoglu U, Sarihan H. Oral terotoma: a case report and literature review. Pediatr Surg Int. 2004 Apr;20(4):304–8. 5. Becker S, Schön R, Gutwald R, Otten JE, Maier W, Hentschel R, et al. A congenital teratoma with a cleft palate: report of a case. Br J Oral Maxillofac Surg. 2007 Jun; 45(4):326–7. 6. Freitas Rda S, Alonso N, Azzolini Tde F, Gianini-Romano G, Tolazzi AR, Busato L, et al. Epignathus: two cases. Br J Oral Maxillofac Surg. 2008 Jun;46(4):317–9. 7. Sauter ER, Diaz JH, Arensman RM, Butcher RB 3rd, Guarisco JL, Hayes DH. The perioperative management of neonates with congenital oropharyngealteratomas. J Pediatr Surg. 1990 Sep;25(9):925–8. 8. Lopes MA, Pereira CM, da Cruz Perez DE, Vargas PA, de Almeida OP. Benign teratoma of the buccal mucosa in a 9-year- old girl: report of case and review of the lit- erature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005 Nov;100(5):598–602. 9. Kothari PR, Jiwane A, Kulkarni B. Congenital naso-pharyngeal teratoma with cleft palate. J Indian Assoc Pediatr Surg. 2004;9(1):42–4. 10. Iken T, Alagöz MS, Günlemez A, Unal C, Sen C, Onyedi M, et al. A congenital true teratoma with cleft lip, palate, and colu- mellar sinus. J Craniofac Surg. 2007 Sep; 18(5):1083–5. 11. Makki FM, Al-Mazrou KA. Nasopharyn- geal teratoma associated with cleft palate in a newborn. Eur Arch Otorhinolaryngol. 2008 Nov;265(11):1413–5. 12. Richieri-Costa A, Zechi-Ceide RM, Guion-Almeida ML. Oral teratoma, dextro- cardia, and congenital heart defect: a non- random association or serendipity? Clin Dysmorphol. 2008 Apr;17(2):149–50. 13. Clement K, Chamberlain P, Boyd P, Molyneux A. Prenatal diagnosis of an epignathus: a case report and review of the literature. Ultrasound Obstet Gynecol. 2001 Aug;18(2):178–81. 14. Benson RE, Fabbroni G, Russell JL. A large teratoma of the hard palate: a case report. Br J Oral Maxillofac Surg. 2009 Jan;47(1):46–9. 15. Lo Curto M, D’Angelo P, Cecchetto G, Klersy C, Dall’lgna P, Federico A, et al. Mature and immature teratomas: results of the first paediatric Italian study. Pediatr Surg Int. 2007 Apr;23(4):315–22.
Figure 1: Presence of a congenital tumour in the mouth preoperatively.
Figure 4: MRI indicated maxillary tumour (recurrence).
Figure 2: Mucosal defect in the soft palate after the teratoma was removed.
Co m
pe tin
g in
te re
st s:
n on
e de
cl ar
ed . C
on fli
ct o
ed ic
al E
th ic
s (A
M E)
e th
ic al
ru le
s of
d is
cl os
ur e.
For citation purposes: Hu R, Jiang RS. The recurrence of a soft palate teratoma in a neonate: a case report. Head Neck Oncol. 2013 Feb 06;5(2):16.
The recurrence of a soft palate teratoma in a neonate: a case report R Hu1, RS Jiang1*
Abstract Teratomas are rare malformations containing cells from ectodermal, mes- odermal and endodermal layers, seldom seen in the soft palate. A 2-day-old girl presenting a teratoma arising from the soft palate was surgically treated. Unfortunately, recurrence was observed during follow-up.
Introduction Teratomas are rarely observed in the soft palate. A teratoma, as defined by Weaver et al.1, is a tumour consisting of multiple tissues that are not indig- enous to their site of origin. The most important complication of oral tera- tomas is respiratory compromise, which is the main cause of death in neonates2,3. Surgical resection is the treatment of choice for oral terato- mas. Although the follow-up is short in most cases, there are no reports of recurrence of oral teratomas4,5. A case of a soft palate teratoma in a neonate is reported herein; recurrence was observed in 4 years follow-up.
Case report A female infant was born to a 20-year- old mother by vaginal delivery after 37+3 weeks gestation with birth weight of 3.05 kg. A congenital tumour was found in the mouth, which prevented oral feeding. Oral clinical examination revealed an asympto- matic mass measuring 3.0 × 4.0 × 2.5 cm, originating from the soft palate near the midline (Figure 1). The mother did not undergo a three-dimensional (3D)-ultrasonography examination
during her pregnancy. The prenatal and perinatal courses were uncompli- cated, and the family history was neg- ative. The father was 27 years old and in good health. The infant was admit- ted to our department on the second day after birth because of irregular respiratory cycle. Shortness of breath and lip cyanosis was observed, espe- cially when the infant was being fed. Due to repeated episodes of apnea, tra- cheal intubation ventilator support was provided and the infant was transferred to a neonatal intensive care unit. Com- puted tomography showed an oral cav- ity mass without intracranial extension. On the seventh day after birth, under general anaesthesia with intubation, the mass was excised from the soft pal- ate finding a short peduncle 1 cm in diameter and the mucosal defect was repaired by suturing a transferred local palatal flap (Figure 2). No cerebrospi- nal fluid leakage occurred from the excision site. After surgery, the wound healed well and the baby tolerated oral feeds. No respiratory compromise occurred any more. Histopathological examination revealed a mature tera- toma composed of mature respiratory epithelium, glandular tissues, neuro- glial tissue, choroid plexus, muscle and blood vessels (Figure 3). Some margins showed nerve and muscle. Signs of recurrence were found in the second year of follow-up. Magnetic resonance imaging (MRI) indicated maxillary tumour with cystic and solid areas with fat density (Figure 4). The parents wanted to “wait and watch”, while hoping for a better prognosis, instead of opting for imme- diate reoperation.
Discussion Teratomas are rare malformations containing cells from ectodermal,
mesodermal and endodermal layers with a variable degree of differentia- tion6. They have an incidence of 1:4000 live births; less than 2% of these arise from the oropharyngeal cavity1,7. Nasopharynx is one of the most fre- quent sites for head and neck terato- mas and has a 6:1 female predominance. In contrast, oral teratomas do not present a clear gender predilection8. Oral teratomas arise anywhere in the oronasal cavity and are regarded as expanding, cavity-filling lesions, espe- cially in the neonatal period9.
The main therapy of teratomas is complete surgical excision, which depends on the site of the tumour. Unless the teratoma is expanding massively into the cranial area, resection of tumour may be attempted. Initial treatment should be directed toward airway manage- ment and feeding problems10. When a neonate is experiencing respira- tory difficulty, the first priority should be stabilization of the airway11. Our case presented with an obstructive mass causing respira- tory embarrassment and immediate threat to life, which demanded establishment of an airway with tracheostomy.
Histologically, teratomas may pre- sent different characteristics. In oral teratomas, the most common tissues observed are nerves and cartilages. Other tissues commonly seen are mus- cles, bones and respiratory epithelia9. Our case showed mature respiratory epithelia, glandular tissues, neuroglial tissues, choroid plexuses, muscles and blood vessels.
Teratomas are associated with concomitant malformations in 6% of all cases, with cleft palate being the most commonly associated anomaly5. In the present case, congenital cardiac septal
* Corresponding author Email: [email protected] 1 Department of Reconstructive Plastic Surgery,
the Children’s Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang Province, China
Page 2 of 2
Licensee OA Publishing London 2013. Creative Commons Attribution License (CC-BY)
Co m
pe tin
g in
te re
st s:
n on
e de
cl ar
ed . C
on fli
ct o
ed ic
al E
th ic
s (A
M E)
e th
ic al
ru le
s of
d is
cl os
ur e.
For citation purposes: Hu R, Jiang RS. The recurrence of a soft palate teratoma in a neonate: a case report. Head Neck Oncol. 2013 Feb 06;5(2):16.
defects and patency of ductusarteriosus were detected. Richieri-Costa et al.12 have also reported the observation of cardiac abnormalities.
Teratomas are mostly benign in the neonatal period 5%; of the cases present malignity criteria on histopa- thology13. Usually, benign teratomas consist of mature tissue components, while those with malignant potential contain immature tissues; there is a higher incidence of malignancy in adults. Incomplete resection and presence of primitive neural tissue entail the risk of a malignant relapse14,15. Becker et al.5 reported the recurrence of a congenital epignathus post-operation; this did not necessar- ily imply malignancy, although the clinician should continue follow-up screening.
It has been 3 years since the recur- rence of oral teratoma; the girl is still in good condition without any drugs or surgery. Because the risk of
malignant change is evident, long- term follow-up is mandatory.
Acknowledgements We thank the patient and her parents for participation. We also thank Mr Grahay Rester for critical reading of the manuscript.
References 1. Weaver RG, Meyerhoff WL, Gates GA. Teratomas of head and neck. Surg Forum. 1976;27(62):539–42. 2. Wakhlu A, Wakhlu AK. Head and neck terotmas in children. Pediatr Surg Int. 2000;16(5-6):333–7. 3. Yoon JK, Kim J, Park C. Congenital immature teratoma of the tongue: an autopsy case. Oral Surg Oral Med Oral
Pathol Oral Radiol Endod. 2002 Dec;94(6): 741–5. 4. Cay A, Bektas D, Imamoglu M, Bahadir O, Cobanoglu U, Sarihan H. Oral terotoma: a case report and literature review. Pediatr Surg Int. 2004 Apr;20(4):304–8. 5. Becker S, Schön R, Gutwald R, Otten JE, Maier W, Hentschel R, et al. A congenital teratoma with a cleft palate: report of a case. Br J Oral Maxillofac Surg. 2007 Jun; 45(4):326–7. 6. Freitas Rda S, Alonso N, Azzolini Tde F, Gianini-Romano G, Tolazzi AR, Busato L, et al. Epignathus: two cases. Br J Oral Maxillofac Surg. 2008 Jun;46(4):317–9. 7. Sauter ER, Diaz JH, Arensman RM, Butcher RB 3rd, Guarisco JL, Hayes DH. The perioperative management of neonates with congenital oropharyngealteratomas. J Pediatr Surg. 1990 Sep;25(9):925–8. 8. Lopes MA, Pereira CM, da Cruz Perez DE, Vargas PA, de Almeida OP. Benign teratoma of the buccal mucosa in a 9-year- old girl: report of case and review of the lit- erature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005 Nov;100(5):598–602. 9. Kothari PR, Jiwane A, Kulkarni B. Congenital naso-pharyngeal teratoma with cleft palate. J Indian Assoc Pediatr Surg. 2004;9(1):42–4. 10. Iken T, Alagöz MS, Günlemez A, Unal C, Sen C, Onyedi M, et al. A congenital true teratoma with cleft lip, palate, and colu- mellar sinus. J Craniofac Surg. 2007 Sep; 18(5):1083–5. 11. Makki FM, Al-Mazrou KA. Nasopharyn- geal teratoma associated with cleft palate in a newborn. Eur Arch Otorhinolaryngol. 2008 Nov;265(11):1413–5. 12. Richieri-Costa A, Zechi-Ceide RM, Guion-Almeida ML. Oral teratoma, dextro- cardia, and congenital heart defect: a non- random association or serendipity? Clin Dysmorphol. 2008 Apr;17(2):149–50. 13. Clement K, Chamberlain P, Boyd P, Molyneux A. Prenatal diagnosis of an epignathus: a case report and review of the literature. Ultrasound Obstet Gynecol. 2001 Aug;18(2):178–81. 14. Benson RE, Fabbroni G, Russell JL. A large teratoma of the hard palate: a case report. Br J Oral Maxillofac Surg. 2009 Jan;47(1):46–9. 15. Lo Curto M, D’Angelo P, Cecchetto G, Klersy C, Dall’lgna P, Federico A, et al. Mature and immature teratomas: results of the first paediatric Italian study. Pediatr Surg Int. 2007 Apr;23(4):315–22.
Figure 1: Presence of a congenital tumour in the mouth preoperatively.
Figure 4: MRI indicated maxillary tumour (recurrence).
Figure 2: Mucosal defect in the soft palate after the teratoma was removed.
Related Documents
