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Huge teratoma of the nasopharynx
American Journal of Otolaryngology –Head and Neck Medicine and Surgery 28 (2007) 177 – 179 www.elsevier.com/locate/amjoto
Case reports
Huge teratoma of the nasopharynx Azarsina Hossein, MDa,T, Ajalloueyan Mohammad, MDb a
Department of Anesthesiology, Baghiyatollah Medical Science University, Tehran, Iran Department of Otolaryngology, Baghiyatollah Medical Science University, Tehran, Iran
b
Abstract
Congenital immature teratoma of the nasopharynx is a very rare form of extragonadal teratoma. In this article, management of a newborn with nasopharyngeal teratoma who underwent tumor resection is reported. The tumor, which originated from the nasopharynx, almost filled the oral cavity and protruded through the mouth, with its external part comparable to the size of the head. She could breath when the head and mass were turned to the left. For safe management of these cases, prenatal evaluation, careful preoperative assessment of the airway, sufficient preparation, and intubation by an expert are mandatory. Perioperative bleeding and obstruction of airway by the tumor or its remnant could render the case difficult. D 2007 Elsevier Inc. All rights reserved.
1. Introduction Teratomas are the most common extragonadal germ cell tumors of childhood, consisting of tissues from at least 2 of the 3 germ layers [1]. They can occur in almost any region of the body and in any organ, but they are most commonly observed in the paraxial and midline locations, and they involve head and neck once in every 40 000 birth [1,2]. Teratomas occurring in infancy and early childhood are usually extragonadal, whereas older children and adults usually present with gonadal teratomas. The most common site of occurrence in neonates is in the sacrococcygeal and presacral region [2- 6]. Teratoma of the nasopharynx is an extremely rare tumor of the oral cavity and is composed of ectoderm, mesoderm, and endoderm with differentiation to identifiable tissues and organs [3,7 -9]. In this article, management of a newborn with nasopharyngeal mass who underwent tumor resection is reported. 2. Case report A female newborn was admitted to otolaryngology clinic because of a huge mass protruding from her mouth. She was born via normal vaginal delivery to a 27-year-old woman,
T Corresponding author. 36 Attar St Sanjabi St, South Dibaji Ave, Sadr Freeway, Tehran, Iran. Tel.: +98 21 2587117; fax: +98 21 8790298. E-mail address: [email protected] (A. Hossein). 0196-0709/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2005.05.006
gravida 1, at 39 weeks of gestation, with Apgar scores of 9 at 1 and 5 minutes. Physical examination revealed a 1900-g healthy female newborn, except for a mass measuring approximately 10 15 15.5 cm that is attached to the nasopharynx with a stalk (Fig. 1). Any associated congenital malformation was not detected, except for a simple cleft palate. Family medical history was not contributory, except for a case of cleft palate in her father’s family; her parents are in good health condition. Maternal serum a-fetoprotein level was 1365 IU/mL. Excision of the tumor was scheduled on the second day of her life. Mask ventilation was impossible. Awake laryngoscopy was considered impossible. Intubation by direct laryngoscopy was not possible. Flexible neonatal bronchoscope was not available. A soft metallic rod was introduced into the trachea through a very narrow tunnel via pediatric suspension laryngoscope as a guide for tracheal tube. Then a 4F orotracheal tube was pushed over the rod and inhalation anesthesia started. A surgical table also was prepared for tracheotomy in case of probable intubation failure. Surgical intervention was limited to excision of tumor from stalk and homeostasis. Estimated blood loss was about 20 mL. The trachea was extubated on the second postoperative hour. The hospital course was prolonged because of aspiration pneumonia (Figs. 2 and 3). Histopathological examination revealed typical features of teratoma, with tridermal derivatives predominantly consisting of well-differentiated embryonic components, including organoid aggregates of bone, cartilage, embryonic
178
A. Hossein, A. Mohammad / American Journal of Otolaryngology –Head and Neck Medicine and Surgery 28 (2007) 177–179
Fig. 1. Huge head size mass protruding from the mouth.
Fig. 3. Large mouth after enblock resection of the mass from the stalk.
teeth, liver, pancreatic, skin, and brain tissues. There are also numerous less-organoid but well-differentiated epithelial structures noted, including respiratory and intestinal epithelia, as well as mesenchymal components, including welldifferentiated smooth and cardiac muscles and vascular plexus. Immature neuroectodermal and mesenchymal components formed a small percentage of the tumor. There is no evidence of coexisting embryonic carcinoma. The patient was followed up every 2 weeks for 6 months and was reoperated to resect the remnant of tumor pedicle when 4.5 months old. When 18 months old, she was again brought to the theater for surgical look and cleft palate repair. No sign of tumor recurrence was detected.
lium, which can be cured by surgery alone. In the currently described case, all 3 germ cell layers were represented. The infant’s mother was a 27-year-old healthy homemaker with normal menstruation and pregnancy. She had no familial relation with her husband. There was no history of toxemia or diabetes mellitus during pregnancy. The mother did not take corticosteroids, sedatives, or oxytocin during pregnancy, but she took co-amoxiclav and cefixime for urinary tract infection during the third semester. Most extragonadal teratomas, including those of the head and neck, manifest during childhood, although they represent fewer than 5% of all pediatric neoplasms [5]. Most of them are related to the spine, head, and neck, unlike adult teratomas, which are mostly gonadal [3 -6]. Of teratomas seen in the first 2 months of life, 82% are sacrococcygeal [4 -6]. The head and neck region is the second most common location for teratomas in early infancy, accounting for 5% to 14% of those cases [3,4]. Sacrococcygeal teratomas have a female predominance of up to 87%, whereas craniofacial teratomas have an overall male-to-female ratio that varies from 1:1 to 2:1 [3,4]. Teratomas of the neck, which are usually large bulky masses are present at birth and are rare in patients older than 1 year [10]. This patient’s mass was huge and protruding from the mouth along with cleft palate, as seen in the case documented by Uchida et al [11]. Ultrasonography allows some of these tumors to be diagnosed prenatally [10]. Prenatal diagnosis allows for a carefully planned delivery to maintain an open airway and potentially improves perinatal outcome. McMahon et al [12] reported the perinatal management of a huge intrapharyngeal and intraoral teratoma that had a broad connection to the base of the tongue. Polyhydramnios (by interfering with fetal swallowing), stillbirth, and premature labor are frequent concomitant conditions and some fetuses may be too large for due dates [10]. Unfortunately, routine prenatal ultrasonography at 35 weeks failed to show this mass, and the history was negative for polyhydramnios.
3. Discussion Immature teratomas with malignant elements, which warrant aggressive adjuvant therapy, should be differentiated from immature teratomas with primitive neuroepithe-
Fig. 2. Oral intubation with the mass attached to nasopharynx.
A. Hossein, A. Mohammad / American Journal of Otolaryngology –Head and Neck Medicine and Surgery 28 (2007) 177–179
Teratomas have heterogeneous histological characteristics. Mature teratomas may contain all manners of adult tissues with varying degrees of organoid development, whereas immature lesions may produce embryonic or extraembryonic fetal tissue. Up to 90% of childhood teratomas contain derivatives from all 3 embryonic germ layers [3]. Approximately 20% to 40% of childhood teratomas contain some immature tissues [4,13]. In childhood, 75% to 85% of teratomas of the head and neck region usually contain neuroectodermal elements (both mature and immature) such as this case and others [4,13-15]. Approximately 80% of teratomas are benign [2]. This case had no histological evidence of malignancy. Benign teratomas may contain either mature tissues or immature elements (up to 50%). Malignant extragonadal teratomas occur in adults and older children but there are also isolated reports of neonatal malignant teratomas [4,10]. The recommended management for head and neck teratomas is surgical excision. This is curative and recurrence is rare [16]. When a neonate is experiencing respiratory difficulty, the first priority should be stabilization of the airway [1]. Prognosis depends on patient’s age, resectability of the tumor, and presence of metastases or metastatic potential [2]. High mortality rate in the adult group is due to high rate of malignant transformation. In contrast, death in neonates usually results from respiratory obstruction due to lesion bulk and location [1]. The aim of presenting this extremely rare tumor is to emphasize the role of exact prenatal evaluation to select the best delivery route, urgent intervention for maintaining airway in these cases of absolutely difficult intubation, and reduction of complications.
179
References [1] Smith RJH, Robinson RA. Head and neck malignancies. In: Cummings CW, Fredrickson JM, Harker LA, et al, editors. Otolaryngology head and neck surgery. 3rd ed. St Louis (Mo)7 Mosby; 1998. p. 229 - 47. [2] Azizkhan RG, Caty MG. Teratomas in childhood. Curr Opin Pediatr 1996;8:287 - 92. [3] Tapper D, Lack EE. Teratomas in infancy and childhood. Ann Surg 1984;198:398 - 409. [4] Bale PM, Painter DM, Cohen D. Teratomas in childhood. Pathology 1975;7:209 - 18. [5] Billmire DF, Grosfeld JL. Teratomas in childhood: analysis of 142 cases. J Pediatr Surg 1986;21:548 - 51. [6] Grosfeld JL, Ballantine TVN, Lowe D, et al. Benign and malignant teratomas in children: analysis of 85 patients. Surgery 1976;80: 297 - 305. [7] Shaari CM, Ho BT, Shah K, et al. Lingual dermoid cyst. Otolaryngol Head Neck Surg 1995;112:476 - 8. [8] Batsakis JG, El Naggar AK, Hicks MJ. Epithelial choristomas and teratomas of the tongue. Ann Otol Rhinol Laryngol 1993;102:567 - 9. [9] Antoine GA, White JD, Heffner DK, et al. Teratoma of the tongue: a case report and literature review. Laryngoscope 1985;95:1262 - 3. [10] Donegan OJ. Congenital neck masses. In: Cummings CW, Fredrickson JM, Harker LA, et al, editors. Otolaryngology head and neck surgery. 2nd ed. St Louis (Mo)7 Mosby; 1993. p. 1554 - 65. [11] Uchida K, Urata H, Suzuki H. Teratoma of the tongue in neonates: report of a case and review of the literature. Pediatr Surg Int 1998;14: 79 - 81. [12] McMahon MJ, Chescheir NC, Kuller JA, et al. Perinatal management of a lingual teratoma. Obstet Gynecol 1996;87:848 - 51. [13] Isaacs H. Perinatal (congenital and neonatal) neoplasms: a report of 110 cases. Pediatr Pathol 1985;3:165 - 216. [14] Shaari CM, Ho BT, Shah K, et al. Lingual dermoid cyst. Otolaryngol Head Neck Surg 1995;112:476 - 8. [15] Rodin AE, Singla P. Teratomas of the tongue present at birth. Pediatr Pathol 1985;3:291 - 8. [16] Lalwani AK, Engel TL. Teratoma of the tongue: a case report and literature review. Int J Pediatr Otorhinolaryngol 1992;24:261 - 8.
Case reports
Huge teratoma of the nasopharynx Azarsina Hossein, MDa,T, Ajalloueyan Mohammad, MDb a
Department of Anesthesiology, Baghiyatollah Medical Science University, Tehran, Iran Department of Otolaryngology, Baghiyatollah Medical Science University, Tehran, Iran
b
Abstract
Congenital immature teratoma of the nasopharynx is a very rare form of extragonadal teratoma. In this article, management of a newborn with nasopharyngeal teratoma who underwent tumor resection is reported. The tumor, which originated from the nasopharynx, almost filled the oral cavity and protruded through the mouth, with its external part comparable to the size of the head. She could breath when the head and mass were turned to the left. For safe management of these cases, prenatal evaluation, careful preoperative assessment of the airway, sufficient preparation, and intubation by an expert are mandatory. Perioperative bleeding and obstruction of airway by the tumor or its remnant could render the case difficult. D 2007 Elsevier Inc. All rights reserved.
1. Introduction Teratomas are the most common extragonadal germ cell tumors of childhood, consisting of tissues from at least 2 of the 3 germ layers [1]. They can occur in almost any region of the body and in any organ, but they are most commonly observed in the paraxial and midline locations, and they involve head and neck once in every 40 000 birth [1,2]. Teratomas occurring in infancy and early childhood are usually extragonadal, whereas older children and adults usually present with gonadal teratomas. The most common site of occurrence in neonates is in the sacrococcygeal and presacral region [2- 6]. Teratoma of the nasopharynx is an extremely rare tumor of the oral cavity and is composed of ectoderm, mesoderm, and endoderm with differentiation to identifiable tissues and organs [3,7 -9]. In this article, management of a newborn with nasopharyngeal mass who underwent tumor resection is reported. 2. Case report A female newborn was admitted to otolaryngology clinic because of a huge mass protruding from her mouth. She was born via normal vaginal delivery to a 27-year-old woman,
T Corresponding author. 36 Attar St Sanjabi St, South Dibaji Ave, Sadr Freeway, Tehran, Iran. Tel.: +98 21 2587117; fax: +98 21 8790298. E-mail address: [email protected] (A. Hossein). 0196-0709/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.amjoto.2005.05.006
gravida 1, at 39 weeks of gestation, with Apgar scores of 9 at 1 and 5 minutes. Physical examination revealed a 1900-g healthy female newborn, except for a mass measuring approximately 10 15 15.5 cm that is attached to the nasopharynx with a stalk (Fig. 1). Any associated congenital malformation was not detected, except for a simple cleft palate. Family medical history was not contributory, except for a case of cleft palate in her father’s family; her parents are in good health condition. Maternal serum a-fetoprotein level was 1365 IU/mL. Excision of the tumor was scheduled on the second day of her life. Mask ventilation was impossible. Awake laryngoscopy was considered impossible. Intubation by direct laryngoscopy was not possible. Flexible neonatal bronchoscope was not available. A soft metallic rod was introduced into the trachea through a very narrow tunnel via pediatric suspension laryngoscope as a guide for tracheal tube. Then a 4F orotracheal tube was pushed over the rod and inhalation anesthesia started. A surgical table also was prepared for tracheotomy in case of probable intubation failure. Surgical intervention was limited to excision of tumor from stalk and homeostasis. Estimated blood loss was about 20 mL. The trachea was extubated on the second postoperative hour. The hospital course was prolonged because of aspiration pneumonia (Figs. 2 and 3). Histopathological examination revealed typical features of teratoma, with tridermal derivatives predominantly consisting of well-differentiated embryonic components, including organoid aggregates of bone, cartilage, embryonic
178
A. Hossein, A. Mohammad / American Journal of Otolaryngology –Head and Neck Medicine and Surgery 28 (2007) 177–179
Fig. 1. Huge head size mass protruding from the mouth.
Fig. 3. Large mouth after enblock resection of the mass from the stalk.
teeth, liver, pancreatic, skin, and brain tissues. There are also numerous less-organoid but well-differentiated epithelial structures noted, including respiratory and intestinal epithelia, as well as mesenchymal components, including welldifferentiated smooth and cardiac muscles and vascular plexus. Immature neuroectodermal and mesenchymal components formed a small percentage of the tumor. There is no evidence of coexisting embryonic carcinoma. The patient was followed up every 2 weeks for 6 months and was reoperated to resect the remnant of tumor pedicle when 4.5 months old. When 18 months old, she was again brought to the theater for surgical look and cleft palate repair. No sign of tumor recurrence was detected.
lium, which can be cured by surgery alone. In the currently described case, all 3 germ cell layers were represented. The infant’s mother was a 27-year-old healthy homemaker with normal menstruation and pregnancy. She had no familial relation with her husband. There was no history of toxemia or diabetes mellitus during pregnancy. The mother did not take corticosteroids, sedatives, or oxytocin during pregnancy, but she took co-amoxiclav and cefixime for urinary tract infection during the third semester. Most extragonadal teratomas, including those of the head and neck, manifest during childhood, although they represent fewer than 5% of all pediatric neoplasms [5]. Most of them are related to the spine, head, and neck, unlike adult teratomas, which are mostly gonadal [3 -6]. Of teratomas seen in the first 2 months of life, 82% are sacrococcygeal [4 -6]. The head and neck region is the second most common location for teratomas in early infancy, accounting for 5% to 14% of those cases [3,4]. Sacrococcygeal teratomas have a female predominance of up to 87%, whereas craniofacial teratomas have an overall male-to-female ratio that varies from 1:1 to 2:1 [3,4]. Teratomas of the neck, which are usually large bulky masses are present at birth and are rare in patients older than 1 year [10]. This patient’s mass was huge and protruding from the mouth along with cleft palate, as seen in the case documented by Uchida et al [11]. Ultrasonography allows some of these tumors to be diagnosed prenatally [10]. Prenatal diagnosis allows for a carefully planned delivery to maintain an open airway and potentially improves perinatal outcome. McMahon et al [12] reported the perinatal management of a huge intrapharyngeal and intraoral teratoma that had a broad connection to the base of the tongue. Polyhydramnios (by interfering with fetal swallowing), stillbirth, and premature labor are frequent concomitant conditions and some fetuses may be too large for due dates [10]. Unfortunately, routine prenatal ultrasonography at 35 weeks failed to show this mass, and the history was negative for polyhydramnios.
3. Discussion Immature teratomas with malignant elements, which warrant aggressive adjuvant therapy, should be differentiated from immature teratomas with primitive neuroepithe-
Fig. 2. Oral intubation with the mass attached to nasopharynx.
A. Hossein, A. Mohammad / American Journal of Otolaryngology –Head and Neck Medicine and Surgery 28 (2007) 177–179
Teratomas have heterogeneous histological characteristics. Mature teratomas may contain all manners of adult tissues with varying degrees of organoid development, whereas immature lesions may produce embryonic or extraembryonic fetal tissue. Up to 90% of childhood teratomas contain derivatives from all 3 embryonic germ layers [3]. Approximately 20% to 40% of childhood teratomas contain some immature tissues [4,13]. In childhood, 75% to 85% of teratomas of the head and neck region usually contain neuroectodermal elements (both mature and immature) such as this case and others [4,13-15]. Approximately 80% of teratomas are benign [2]. This case had no histological evidence of malignancy. Benign teratomas may contain either mature tissues or immature elements (up to 50%). Malignant extragonadal teratomas occur in adults and older children but there are also isolated reports of neonatal malignant teratomas [4,10]. The recommended management for head and neck teratomas is surgical excision. This is curative and recurrence is rare [16]. When a neonate is experiencing respiratory difficulty, the first priority should be stabilization of the airway [1]. Prognosis depends on patient’s age, resectability of the tumor, and presence of metastases or metastatic potential [2]. High mortality rate in the adult group is due to high rate of malignant transformation. In contrast, death in neonates usually results from respiratory obstruction due to lesion bulk and location [1]. The aim of presenting this extremely rare tumor is to emphasize the role of exact prenatal evaluation to select the best delivery route, urgent intervention for maintaining airway in these cases of absolutely difficult intubation, and reduction of complications.
179
References [1] Smith RJH, Robinson RA. Head and neck malignancies. In: Cummings CW, Fredrickson JM, Harker LA, et al, editors. Otolaryngology head and neck surgery. 3rd ed. St Louis (Mo)7 Mosby; 1998. p. 229 - 47. [2] Azizkhan RG, Caty MG. Teratomas in childhood. Curr Opin Pediatr 1996;8:287 - 92. [3] Tapper D, Lack EE. Teratomas in infancy and childhood. Ann Surg 1984;198:398 - 409. [4] Bale PM, Painter DM, Cohen D. Teratomas in childhood. Pathology 1975;7:209 - 18. [5] Billmire DF, Grosfeld JL. Teratomas in childhood: analysis of 142 cases. J Pediatr Surg 1986;21:548 - 51. [6] Grosfeld JL, Ballantine TVN, Lowe D, et al. Benign and malignant teratomas in children: analysis of 85 patients. Surgery 1976;80: 297 - 305. [7] Shaari CM, Ho BT, Shah K, et al. Lingual dermoid cyst. Otolaryngol Head Neck Surg 1995;112:476 - 8. [8] Batsakis JG, El Naggar AK, Hicks MJ. Epithelial choristomas and teratomas of the tongue. Ann Otol Rhinol Laryngol 1993;102:567 - 9. [9] Antoine GA, White JD, Heffner DK, et al. Teratoma of the tongue: a case report and literature review. Laryngoscope 1985;95:1262 - 3. [10] Donegan OJ. Congenital neck masses. In: Cummings CW, Fredrickson JM, Harker LA, et al, editors. Otolaryngology head and neck surgery. 2nd ed. St Louis (Mo)7 Mosby; 1993. p. 1554 - 65. [11] Uchida K, Urata H, Suzuki H. Teratoma of the tongue in neonates: report of a case and review of the literature. Pediatr Surg Int 1998;14: 79 - 81. [12] McMahon MJ, Chescheir NC, Kuller JA, et al. Perinatal management of a lingual teratoma. Obstet Gynecol 1996;87:848 - 51. [13] Isaacs H. Perinatal (congenital and neonatal) neoplasms: a report of 110 cases. Pediatr Pathol 1985;3:165 - 216. [14] Shaari CM, Ho BT, Shah K, et al. Lingual dermoid cyst. Otolaryngol Head Neck Surg 1995;112:476 - 8. [15] Rodin AE, Singla P. Teratomas of the tongue present at birth. Pediatr Pathol 1985;3:291 - 8. [16] Lalwani AK, Engel TL. Teratoma of the tongue: a case report and literature review. Int J Pediatr Otorhinolaryngol 1992;24:261 - 8.