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Epipharyngeal teratoma in infancy
International Journal of Pediatric Otorhinolaryngology, 24 (1992) 171- 175 0 1992 Elsevier Science Publishers B.V. All rights reserved 0165.5876/92/$05.00
PEDOT
171
00798
Epipharyngeal GBbor Katona
a, Jen6 Hirschberg
teratoma in infancy a, Zolt&
Hosszti
a and L&z16 KirAly ’
Depariments of a Otorhinolaryngology and ’ Pathology, Heim Pa’1Hospital for Sick Children, Budapest (Hungary) (Received (Accepted
Kry words: Teratoma;
Infancy:
Surgical
17 September 9 November
1991) 1991)
management
Abstract
Epipharyngeal teratomas are rare congenital lesions that often cause respiratory distress, stridor at birth. The management of such anomalies should include establishment of secure upper airways, radiographic exclusion of midline CNS malformations and early surgical excision to prevent malignant transformation, asphyxia or permanent facial distortion. The diagnosis of congenital stridor. dyspnea and rhinitis should be performed very carefully.
Introduction
Cough and rhinitis are very frequent illnesses in infancy. They are mostly caused by banal infections of upper airway. Congenital stridor is seen also very often; in most cases caused by cartilage-weekness, which recover spontaneously. However, a correct diagnosis is important, because rarely there are serious, life-threatening causes in the background of mild symptoms [4]. The presented case is an example for this.
Case report
T.G., a 2-month-old boy was admitted to the pediatric department of another hospital because of congenital stridor, rhinitis and mild dyspnea. The symptoms were explained by the hyperplasia of the thymic gland and by the rachitic Correspondence
IO: GQbor Katona,
Budapest,
6llCii u. 86. H-1089
Hungary
Fig. 1. Medio-sagittal MRI picture of the head. A tumour mass is seen in the epipharyngeal region in close connection with the soft palate.
constitution of the child. Six months later he was admitted again because of the increasing, permanent complaints. At that time the prominence of the velum was recognized and the patient was sent to our department with suspicion of an epipharyngeal tumour. Bilateral purulent rhinitis was seen in the mild dystrophical child with no ability of nasal breathing. Stridor, dyspnea were observed. In the laboratory parameters (blood picture, hepatic and renal function) there were no pathological changes. A marked swelling of the velum could be seen, especially on the left side. Ears, larynx, hypopharynx were normal, and there were no palpable lymph nodes on the neck. According to the neurological investigation there was no sign of intracranial propagation. The sagittal MRI showed a large tumour mass in the epipharyngeal region (Fig. 1). MRI showed no sign of cranial base dyscontinuity, so a probe excision was performed for histological examination to decide about the further therapy. Surprisingly enough the histology revealed pure brain tissue in the removed specimen. In addition, after the procedure uncertain breathing and a circulatory problem appeared, necessitating a short intensive therapy. Believing that the child had an encephalocele, we sent him to the National Institute for Neurosurgery for further therapy. After repeated, accurate serial CT scans, a cranial discontinuity and a neurological malformation were excluded. Consulting with the neurosurgeon we decided for the total exstirpation of the tumour.
173
Fig. 2. Removed teratoma
The operation was performed in the Rose position, in intratracheal narcosis, by transpalatinal approach. A 3 x 2 x 1 cm tumour was removed (Fig. 2). The soft palate was reconstructed. There were no complications in the postoperative period and after a week the child was emmitted with good nasal breathing and without complaints. The histological examination of the specimen revealed a teratoma containing brain, cartilage, glandular and connective tissues (Figs. 3 and 4). No signs of malignancy was detectable. 6 months after the operation the child is free of symptoms.
Discussion Teratomas of the head and neck are a particular type of developmental malformation or tumour that are composed of cells with a full-range histogenic potential. They occur almost exclusively in infancy [ll]. The oropharyngeai or epipharyngeal teratomas are known about as epignatus [7]. There are reports in the literature of teratomas of the tongue [lo], the orbit [12], the maxillary sinus [12], the mediastinum [l], the oropharynx 181, the intracranium [5], and mostly the sacrococcygeal area [3,6]. The routine use of ultrasonography during pregnancy allows an early diagnosis of most of these teratomas with high degree of certainity [61. Several authors described a small, but significant risk of malignant transformation of germ cell tumours [2,3,9]. Touron et al. [9] presented a case of cervical teratoma of a newborn with large congenital metastatic leasions in the liver and the lung. Hager and Menardi [2] reported malignant transformation 9 of 33 teratoma cases. They proposed early surgical intervention and discussed the other ways of therapy (chemotherapy, second-look operation) in these casts. In our
Fig. 3. Teratoid
tumour containing
rife nervous ganglion cells. Haematoxilin-eosin microscopy.
Fig. 4. Chondroid tissue enclosed by glandular tissue. Haematoxilin-eosin
staining, light
staining, light microscopy.
175
patient dyspnea and stridor were not very significant in the beginning and the first histological result also delayed correct diagnosis and adequat therapy. Nevertheless, the tumour did not grow significantly in the observation period and the second histology did not reveal any signs of malignancy. The case emphasizes the importance of careful diagnosis in congenital stridor, rhinitis and dyspnea and calls the attention to the possibility of an epipharyngeal tumour in infancy.
References 1 Dumbell, H.R., Coleman, A.C., Pudifin, J.M. and Winship. W.S. Prenatal ultrasonographic diagnosis and successful management of mediastinal teratoma: A case report. S. Afr. Med. J., 7X (1990) 481-483. 2 Hager, J. and Menardi, G. Keimzelltumoren im Kindesalter. 2. Kinderchir., 44 (1989) 25-29. 3 Heikinheimo, M., Rajantie, J., Kuusela, P. and Siimes. M.A. Early detection of malignant transformation after sacrococcygeal teratoma (letter) Pediatr. Haematol. Oncol., 4 (1987) 171-172. 4 Hirschberg, J. and Szende, T. Pathological Cry. Stridor and Cough in Infants. Akademiai Kiado, Budapest, 1982. 5 Hunt. S.J., Johnson, P.C., Coons, S.W. and Pittman, H.W. Neonatal intracranial teratomas. Surg. Neurol.. 34 (1990) 336-342. 6 Kainer, F., Winter, R., Hofman, H.M. and Karpf, E.F. Das Sacrococcygeale Teratom. Prenatale Diagnose und Prognose. Zentralbr. Gynaekol., 112 (1990) 609-616. 7 Levine. A.B.. Alvarez, M., Wedgwood, J., Berkowitz, R.L. and Holzman, 1. Contemporary management of a potentially lethal fetal anomaly: a successful perinatal approach to epignathus. Obstet. Gynecol. 76 (1990) 962-966. 8 Sauter, E.R., Diaz, J.H., Arensman, R.M., Butcher. R.B., Guarisco, J.L. and Hayes. D.H. The perioperative management of neonates with congenital oropharyngeal teratomas. J. Pediatr. Surg.. 25 (1990) 925-928. 9 Touran. T., Applebaum, H., Frost, D.B., Richardson, R.. Taber, P. and Rowland. J. Congenital metastatic cervical teratoma: diagnostic and management considerations. J. Pediatr. Surg. 24 (19x0) 21-23. 10 Tumushime-Buturo, C.G. and Nkanza, N.K. Glial teratoma thought to be a lingual thyroid. J. Laryngol. Otol., 103 (1989) 620-621. 11 Ward, R.F. and April, M. Teratomas of the head and neck. Otolaryngol. Clin. North Am.. 22 (1Y8Y) 621-629. 12 Weiss, A.H., Greenwald, M.J., Margo, C.E. and Myers. W. Primary and secondary orbital tcratomas. J. Pediatr. Ophthalmol. 26 (1989) 44-40.
PEDOT
171
00798
Epipharyngeal GBbor Katona
a, Jen6 Hirschberg
teratoma in infancy a, Zolt&
Hosszti
a and L&z16 KirAly ’
Depariments of a Otorhinolaryngology and ’ Pathology, Heim Pa’1Hospital for Sick Children, Budapest (Hungary) (Received (Accepted
Kry words: Teratoma;
Infancy:
Surgical
17 September 9 November
1991) 1991)
management
Abstract
Epipharyngeal teratomas are rare congenital lesions that often cause respiratory distress, stridor at birth. The management of such anomalies should include establishment of secure upper airways, radiographic exclusion of midline CNS malformations and early surgical excision to prevent malignant transformation, asphyxia or permanent facial distortion. The diagnosis of congenital stridor. dyspnea and rhinitis should be performed very carefully.
Introduction
Cough and rhinitis are very frequent illnesses in infancy. They are mostly caused by banal infections of upper airway. Congenital stridor is seen also very often; in most cases caused by cartilage-weekness, which recover spontaneously. However, a correct diagnosis is important, because rarely there are serious, life-threatening causes in the background of mild symptoms [4]. The presented case is an example for this.
Case report
T.G., a 2-month-old boy was admitted to the pediatric department of another hospital because of congenital stridor, rhinitis and mild dyspnea. The symptoms were explained by the hyperplasia of the thymic gland and by the rachitic Correspondence
IO: GQbor Katona,
Budapest,
6llCii u. 86. H-1089
Hungary
Fig. 1. Medio-sagittal MRI picture of the head. A tumour mass is seen in the epipharyngeal region in close connection with the soft palate.
constitution of the child. Six months later he was admitted again because of the increasing, permanent complaints. At that time the prominence of the velum was recognized and the patient was sent to our department with suspicion of an epipharyngeal tumour. Bilateral purulent rhinitis was seen in the mild dystrophical child with no ability of nasal breathing. Stridor, dyspnea were observed. In the laboratory parameters (blood picture, hepatic and renal function) there were no pathological changes. A marked swelling of the velum could be seen, especially on the left side. Ears, larynx, hypopharynx were normal, and there were no palpable lymph nodes on the neck. According to the neurological investigation there was no sign of intracranial propagation. The sagittal MRI showed a large tumour mass in the epipharyngeal region (Fig. 1). MRI showed no sign of cranial base dyscontinuity, so a probe excision was performed for histological examination to decide about the further therapy. Surprisingly enough the histology revealed pure brain tissue in the removed specimen. In addition, after the procedure uncertain breathing and a circulatory problem appeared, necessitating a short intensive therapy. Believing that the child had an encephalocele, we sent him to the National Institute for Neurosurgery for further therapy. After repeated, accurate serial CT scans, a cranial discontinuity and a neurological malformation were excluded. Consulting with the neurosurgeon we decided for the total exstirpation of the tumour.
173
Fig. 2. Removed teratoma
The operation was performed in the Rose position, in intratracheal narcosis, by transpalatinal approach. A 3 x 2 x 1 cm tumour was removed (Fig. 2). The soft palate was reconstructed. There were no complications in the postoperative period and after a week the child was emmitted with good nasal breathing and without complaints. The histological examination of the specimen revealed a teratoma containing brain, cartilage, glandular and connective tissues (Figs. 3 and 4). No signs of malignancy was detectable. 6 months after the operation the child is free of symptoms.
Discussion Teratomas of the head and neck are a particular type of developmental malformation or tumour that are composed of cells with a full-range histogenic potential. They occur almost exclusively in infancy [ll]. The oropharyngeai or epipharyngeal teratomas are known about as epignatus [7]. There are reports in the literature of teratomas of the tongue [lo], the orbit [12], the maxillary sinus [12], the mediastinum [l], the oropharynx 181, the intracranium [5], and mostly the sacrococcygeal area [3,6]. The routine use of ultrasonography during pregnancy allows an early diagnosis of most of these teratomas with high degree of certainity [61. Several authors described a small, but significant risk of malignant transformation of germ cell tumours [2,3,9]. Touron et al. [9] presented a case of cervical teratoma of a newborn with large congenital metastatic leasions in the liver and the lung. Hager and Menardi [2] reported malignant transformation 9 of 33 teratoma cases. They proposed early surgical intervention and discussed the other ways of therapy (chemotherapy, second-look operation) in these casts. In our
Fig. 3. Teratoid
tumour containing
rife nervous ganglion cells. Haematoxilin-eosin microscopy.
Fig. 4. Chondroid tissue enclosed by glandular tissue. Haematoxilin-eosin
staining, light
staining, light microscopy.
175
patient dyspnea and stridor were not very significant in the beginning and the first histological result also delayed correct diagnosis and adequat therapy. Nevertheless, the tumour did not grow significantly in the observation period and the second histology did not reveal any signs of malignancy. The case emphasizes the importance of careful diagnosis in congenital stridor, rhinitis and dyspnea and calls the attention to the possibility of an epipharyngeal tumour in infancy.
References 1 Dumbell, H.R., Coleman, A.C., Pudifin, J.M. and Winship. W.S. Prenatal ultrasonographic diagnosis and successful management of mediastinal teratoma: A case report. S. Afr. Med. J., 7X (1990) 481-483. 2 Hager, J. and Menardi, G. Keimzelltumoren im Kindesalter. 2. Kinderchir., 44 (1989) 25-29. 3 Heikinheimo, M., Rajantie, J., Kuusela, P. and Siimes. M.A. Early detection of malignant transformation after sacrococcygeal teratoma (letter) Pediatr. Haematol. Oncol., 4 (1987) 171-172. 4 Hirschberg, J. and Szende, T. Pathological Cry. Stridor and Cough in Infants. Akademiai Kiado, Budapest, 1982. 5 Hunt. S.J., Johnson, P.C., Coons, S.W. and Pittman, H.W. Neonatal intracranial teratomas. Surg. Neurol.. 34 (1990) 336-342. 6 Kainer, F., Winter, R., Hofman, H.M. and Karpf, E.F. Das Sacrococcygeale Teratom. Prenatale Diagnose und Prognose. Zentralbr. Gynaekol., 112 (1990) 609-616. 7 Levine. A.B.. Alvarez, M., Wedgwood, J., Berkowitz, R.L. and Holzman, 1. Contemporary management of a potentially lethal fetal anomaly: a successful perinatal approach to epignathus. Obstet. Gynecol. 76 (1990) 962-966. 8 Sauter, E.R., Diaz, J.H., Arensman, R.M., Butcher. R.B., Guarisco, J.L. and Hayes. D.H. The perioperative management of neonates with congenital oropharyngeal teratomas. J. Pediatr. Surg.. 25 (1990) 925-928. 9 Touran. T., Applebaum, H., Frost, D.B., Richardson, R.. Taber, P. and Rowland. J. Congenital metastatic cervical teratoma: diagnostic and management considerations. J. Pediatr. Surg. 24 (19x0) 21-23. 10 Tumushime-Buturo, C.G. and Nkanza, N.K. Glial teratoma thought to be a lingual thyroid. J. Laryngol. Otol., 103 (1989) 620-621. 11 Ward, R.F. and April, M. Teratomas of the head and neck. Otolaryngol. Clin. North Am.. 22 (1Y8Y) 621-629. 12 Weiss, A.H., Greenwald, M.J., Margo, C.E. and Myers. W. Primary and secondary orbital tcratomas. J. Pediatr. Ophthalmol. 26 (1989) 44-40.