|Year : 2015 | Volume
| Issue : 4 | Page : 1040
Congenital nasopharyngeal teratoma causing airway obstruction in the newborn
Mou Das1, Debashish Mukherjee1, Shatavisha Das Gupta1, Suchandra Mukherjee2, Uttara Chatterjee1, Chhanda Datta1
1 Department of Pathology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
2 Department of Pediatrics, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
|Date of Web Publication||15-Feb-2016|
Shatavisha Das Gupta
15A/7, East Road, Santoshpur, Kolkata - 700 075, West Bengal
Source of Support: None, Conflict of Interest: None
Teratomas are the most common congenital tumors, but teratomas of the nasopharynx are rare in neonates. The present report is about an 18-day-old girl child with a nasopharyngeal teratoma protruding from the oral cavity. The tumor almost completely obstructed the airways and necessitated immediate intervention. The tumor was successfully removed by the transpalatal route. Histological examination showed that it was a mature teratoma. The case with its related differential diagnosis is discussed here.
Keywords: Nasopharynx, neonates, teratoma
|How to cite this article:|
Das M, Mukherjee D, Gupta SD, Mukherjee S, Chatterjee U, Datta C. Congenital nasopharyngeal teratoma causing airway obstruction in the newborn. J Can Res Ther 2015;11:1040
|How to cite this URL:|
Das M, Mukherjee D, Gupta SD, Mukherjee S, Chatterjee U, Datta C. Congenital nasopharyngeal teratoma causing airway obstruction in the newborn. J Can Res Ther [serial online] 2015 [cited 2019 Sep 18];11:1040. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/1040/139269
| > Introduction|| |
Teratoma is the most common extragonadal germ cell tumor of childhood, consisting of tissues from at least two of the three germ layers. Teratomas are seen more often in the perinatal period than any other time in childhood. About half of the childhood teratomas are congenital and among these, majority are mature teratomas. Though mature teratomas are considered benign on the basis of histological findings, they may cause death if the vital structures are involved. Unique clinical presentations are associated with large, space occupying congenital teratomas. We describe a case of congenital nasopharyngeal teratoma, in an 18-day-old girl child. Nasopharyngeal teratomas arise from the skull base or the posterior pharyngeal wall and extend inferiorly to cause obstruction to the upper aerodigestive tract.
| > Case Report|| |
An 18-day-old girl child presented with respiratory distress, difficulty in swallowing, regurgitation of milk and failure to thrive. The mother's antenatal history was uneventful and the prenatal ultrasonography was unremarkable. She was born by normal vaginal delivery in a peripheral health center. The neonate was referred to our tertiary care center for failure to thrive and episodes of respiratory distress.
At admission, the baby was emaciated in appearance although, birth weight was normal and there was no obvious, visible congenital abnormality. Facial dysmorphism was not evident. Neurological examination was normal. On examination, a large lobulated mass was seen protruding into the oral cavity from the pharynx. The computed tomography (CT) scan, done on the 23rd day, revealed a large, oval soft tissue density mass lesion noted at left prestyloid parapharyngeal space extending into the naso and oropharyngeal cavity causing compromise of the airspace [Figure 1]. Portion of the lesion was seen to be hanging within the oropharyngeal cavity. The mass measured 3.8 × 2 cm approximately.
|Figure 1: CT scan showing large, oval, soft tissue density mass lesion at left prestyloid parapharyngeal space, extending into the naso and oropharyngeal cavity|
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The mass was removed by transpalatal route. We received about 20 ml of tissue fragments with variegated as well as glistening mucoid areas. Histopathology revealed that the tumor was composed of a heterogeneous mixture of different tissue components. These included brain tissue, choroid plexus, pigmented epithelium, cartilage, skin, respiratory and salivary glands and fibro-adipose tissue. Immature elements as well as primitive neuroectoderm were lacking even after extensive sampling. Based on these features a diagnosis of a mature teratoma was made [Figure 2]. The baby had an uneventful symptom-free postoperative period and is doing fine.
|Figure 2: (a) Photomicrograph showing mature cartilage, glands and fibro collagenous tissue (H and E, ×100) (b) Photomicrograph showing choroid plexus and squamous epithelium (H and E, ×400)(c) Photomicrograph showing mature glial tissue, squamous epithelium and cartilage (H and E, ×100) (d) Photomicrograph' showing glandular epithelium and fibroconnective tissue (H and E, ×400)|
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| > Discussion|| |
Teratoma is the most common germ cell tumor of the fetus and newborn and usually presents as an obvious mass with signs and symptoms referable to the location of origin. The usual sites are the gonads, the retro peritoneum and the sacrococcygeal regions. The incidence of teratomas is 1:4000 births, with the sacrococcygeal area being the most common site. In the neonatal age group, the most common site is the sacrococcygeal region constituting about 79.7% of all cases. The other sites are the cervical region, followed by the retro peritoneum, mediastinum, head (face), orbit, abdominal wall and rare cases of nasopharyngeal teratomas. Teratomas of the head and neck account for less than 2% of reported cases of congenital teratomas, and are most commonly found in the cervical neck., Rare cases of multiple nasopharyngeal teratomas have also been reported. A female predominance of 6:1 has been widely reported in the literature.,,
Nasopharyngeal teratomas are true neoplasms, arising in the vicinity of Rathke's pouch and the anterior end of notochord., Occasionally, they have an intracranial portion connected by an isthmus traversing the sphenoid. They may resemble fetal parts and have been designated as epignathi. Large nasopharyngeal teratomas prevent fetal swallowing, which leads to polyhydramnios and this is a poor prognostic feature which, however, was not present in our case. These tumors can be easily diagnosed by prenatal sonography. A large, solid and cystic, sometimes calcified mass projecting out from the oral cavity suggests the prenatal diagnosis. Mortality rate can be high if these expanding cavity filling lesions cause airway obstruction. Less than 10% of newborns have other associated congenital malformations like cleft palate, hypertelorism, congenital heart abnormalities, umbilical hernia and facial hemangiomas, unlike the present case. These facial defects are attributed to the mechanical effect of the nasopharyngeal teratoma on the developing structures.
Early diagnosis and surgical excision are the mainstay of treatment. A thorough preoperative imaging study must be performed prior to any attempt at removal of the teratoma. Preoperative CT and magnetic resonance imaging (MRI) are imperative to rule out intracranial extension of the tumors.,,, The extent of the surgery is dictated by the exact size, and the location of the lesion. The surgical intervention should be very prompt, since a delay can lead to severe life-threatening complications. Majority of the teratomas in the neonatal period are histologically benign and are composed of mature tissues, without immature neural elements and therefore no postoperative chemotherapy is required. However, a few reports of immature teratomas and associated yolk sac tumor with mature teratoma exist. Hence a thorough sampling of the specimen should be done so as not to miss the immature elements. A useful clue, indicating the presence of the yolk sac tumor component is a raised serum alpha fetoprotein (AFP) in the newborn, which in our case was not elevated.
Our patient presented with a protruding mass in the oral cavity and on examination it was seen to involve the nasal region as well. Successful surgery was carried out through the transpalatal route and the entire mass was removed. Histological examination proved it to be a mature teratoma. A dermoid cyst is one of the main considerations in the differential diagnosis of nasopharyngeal masses in the newborn. The dermoid is related to teratoma, but it does not contain tissues representative of all the three germ cell layers. Histologically, it consists only of skin, with hair and other dermal appendages. The dermoid, depending on their size and location can produce intermittent respiratory obstruction and feeding problems.
The differential diagnoses for the nasopharyngeal teratomas include the lesions that can also present with an oropharyngeal mass like encephalocele and nasal gliomas, which are benign congenital malformations, are rare as well. Both of these lesions, predominantly appear in the pediatric population, causing nasal obstruction, difficulty in feeding and nasal cosmetic deformities. Most importantly, these lesions can be foci of infections. It is of paramount importance to rule out the possibility of such lesions, given the propensity of intracranial communication in these lesions, thus putting the patient at risk for intracranial infections and associated complications. In both these lesions, variable proportion of glial tissue is present characterized by neuroglial fibers and astrocytes. Although, glial tissue is present in this case, the presence of other multiple mature tissue elements proved it to be a teratoma and ruled out the possibility of heterotrophic glial tissue (nasal gliomas).
Other possible differential diagnoses of nasopharyngeal masses in the infant includes meningoencephalocele, congenital rhabdomyosarcoma, hemangiomas, neurofibromatosis, congenital epulis, congenital epignathus (which may be multiple) and lymphatic malformation.,,,
| > Conclusion|| |
Nasopharyngeal teratoma is a rare benign entity. Most of the published literature on this topic is represented by sporadic case reports. The recommended treatment for such lesions is early diagnosis and complete surgical removal of the tumor. The single most important factor affecting outcome, is whether the teratoma could be resected successfully during initial surgery.
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[Figure 1], [Figure 2]