A Rare Nasopharyngeal Teratoma Arising From the Vomer.

Teratomas are rare germ cell neoplasms derived from the 3 germinal layers (ectoderm, mesoderm, and endoderm). Nasopharyngeal teratoma is a very rare teratoma arising anywhere from the oronasal cavity, regarded as an expanding, avity filling lesion, with a high mortality rate because of severe airway obstruction, especially in the neonatal period and make up only 2% of all teratomas. The authors present a case of an infant girl with a single, finger-like, hairy teratoma arising from the vomer and protruding from the mouth with bilateral complete cleft palate, cleft lip, and cleft alveolus. Complete intraoral resection of the teratoma and cleft lip repair was conducted simultaneously. Reconstruction of the cleft palate was performed at a later stage. Recurrence occurred 9 months after surgery and extended complete surgical excision was performed after recurrence, with no recurrence observed again to date. Histopathologic examination confirmed the diagnosis of congenital mature teratoma.

Congenital nasopharyngeal teratoma causing airway obstruction in the newborn.

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.

Huge nasopharyngeal teratoma with a cleft palate and bifid tongue in a patient with pierre robin syndrome.

PURPOSE: Nasopharyngeal teratoma is a very rare neoplasm that develops on the oronasal cavity as an expanding and cavity-filling lesion. We present a rare case of nasopharyngeal teratoma associated with a cleft palate and bifid tongue in a patient with Pierre Robin syndrome. METHODS: A preterm male neonate of 33 weeks and 6 days was referred to our department. A protruding solid mass with approximately 7.0 × 4.0-cm size arising from the vomer and combined cleft palate and tongue covered with pinkish skin with lanugo were observed. In addition, there was a bandlike tissue connecting vomer and sublingual area, which divided and restricted the anterior portion of the tongue. RESULTS: An early surgical intervention was decided because of problems with airways and nutrition caused by the mass. The mass was completely removed from the vomer. The histologic examination of the mass was consistent with mature teratoma. After his first operation, he was finally diagnosed with the Pierre Robin syndrome. After 4 months, a tongue reduction was conducted for macroglossia, and after 9 months, remnant teratoma excision and a palate repair were conducted using 2-flap palatoplasty. CONCLUSIONS: A nasopharyngeal teratoma is a rare case and induces a cleft palate and bifid tongue in a preterm infant, leading to severe airway and nutritional problems. A teratoma interferes with the fusion of embryonic tissues in the early developmental period, in particular, with the palatal fusion severely prevented if the teratoma is accompanied by a Pierre Robin syndrome such as in this case.

Congenital hairy polyp and autoamputation in an infant with acute otitis media.

Hairy polyp is a rare, benign tumor that frequently localizes in the nasopharynx and oropharynx. The embryogenesis of hairy polyp is not known precisely. The clinical profile can vary depending on the size and location of the tumor. In this report, we present a case with hairy polyp who was admitted with acute otitis media and completely recovered after spontaneous autoamputation.

Neonatal airway lesions: our experience and a review of the literature.

OBJECTIVES: This paper reports on two rare cases of neonatal airway lesions with differing aetiology that were successfully managed by surgery, and provides a review of the literature on neonatal stridor and airway lesions. CASE REPORTS: In the first case report, a newborn presented with a nasopharyngeal teratoma. In the second case report, a newborn presented with a congenital laryngeal saccular cyst. Difficulties in the diagnosis of these lesions, and surgical and anaesthetic challenges in their management are discussed. CONCLUSION: Every case of neonatal airway distress must be evaluated and the cause of stridor needs to be established. It is important that rare lesions such as teratomas and laryngeal cysts are not overlooked; a high index of suspicion for these congenital anomalies is necessary. These airway lesions should be managed in an institutional setting by a multidisciplinary team.

Congenital salivary gland anlage tumor of the nasopharynx.

Nasal and upper respiratory tract obstruction in the neonatal period can result from a variety of conditions, and may be present with variable symptoms. Salivary gland anlage tumor, also referred as congenital pleomorphic adenoma, is a very rare benign congenital tumor of the nasopharynx, which may produce nasal obstruction and other associated, nonspecific symptoms. We report a case of congenital salivary gland anlage tumor causing a severe neonatal respiratory distress with pulmonary hypertension. The tumor was removed and the outcome was favourable without recurrence at five years of the follow up.

Nasopharyngeal teratoma associated with cleft palate in newborn: report of 2 cases.

Teratoma is a true neoplasm that consists of tissues from all 3 embryonic germ layers: ectoderm, mesoderm, and endoderm. Nasopharyngeal teratoma is very rare and accounts for only 2% of all teratomas. We present 2 cases of nasopharyngeal teratomas associated with cleft palate in newborns. The first case was a mushroom-like teratoma arising from the base of the sphenoid bone with cleft soft palate. This case was unique and interesting because no earlier paper has reported a teratoma with such a clinical feature. The second case was a teratoma from the nasal septum of an incomplete cleft palate. The combination of the mass and cleft palate made oral feeding difficult. Complete intraoral resection was performed in both cases. Typical components of true teratoma were identified by microscopic examination after operation. Cleft palate reconstruction was performed before 1 year. Clinical follow-up continues, and no recurrence has been observed to date.

Neonatal airway obstruction caused by rapidly growing nasopharyngeal teratoma.

UNLABELLED: A case report is presented of a rapidly growing congenital nasopharyngeal teratoma (epignathus) in a preterm infant, leading to severe upper airway obstruction. Prenatal diagnosis by ultrasonography did not reveal the condition because the tumour masses were initially small and there was no polyhydramnios. Epignathus is a rare cause of upper airway obstruction of the newborn that can grow rapidly in the neonatal period and should be treated surgically. CONCLUSION: Epignathus is a rare cause of upper airway obstruction of the newborn that can grow rapidly in the neonatal period.

The EXIT (ex utero intrapartum treatment) procedure.

Fetuses with upper airway obstruction have a high mortality rate if proper airway management is not immediately administered after delivery. The EXIT (ex utero intrapartum treatment) procedure is a new technique that establishes the fetal airway while uteroplacental circulation is still maintained. The prognosis of such neonates has much improved after the introduction of this procedure. We report two neonates with prenatal diagnosis of upper airway obstruction; they were born smoothly via the EXIT procedure. The first was a case of epignathus, a rare type of nasopharyngeal or oropharyngeal teratoma derived from all three germ cell layers with variable maturity. The second was a case of giant lymphangioma that resulted from localized malformations in the development of the lymphatic system. Furthermore, compared with routine cesarean section, the short-term maternal outcomes were not different with regard to infection rate, estimated blood loss, wound complication, and postpartum hospital stay.

Respiratory distress secondary to nasopharyngeal glial heterotopia.

Nasal glial heterotopia (nasal glioma) is a rare congenital malformation of neural origin. We present a newborn baby with life-threatening respiratory distress secondary to nasopharyngeal glial heterotopia that obstructed the nasopharyngeal or nasal airway. A high degree of suspicion, early diagnosis and surgical management are essential to cure this rare and potentially life-threatening disorder.

A congenital true teratoma with cleft lip, palate, and columellar sinus.

Teratomas, the most common extragonadal germ cell tumor of childhood, involve at least two of the ectodermal, mesodermal, and endodermal layers. Of the teratomas seen in the first 2 months of life, 82% are sacrococcygeal. The head and neck region is the second most common location for teratomas in early infancy, accounting for five (14%) of those cases. We describe a female neonate with a teratoma of the nasopharyngeal area, bilateral cleft palate/lip, and columellar sinus pathologies. The mass, which was 8 x 5 x 7 cm and soft in consistency, blocked the airway and prevented oral feeding. On macroscopic examination of the excised mass, there was a notable typical cilia arrangement and lower eyelid appearance. The patient, who was diagnosed with a well-differentiated teratoma after the pathologic examination, did not have any complications in the postoperative period.

[Congenital hairy polyp of the nasopharynx--a case report].

INTRODUCTION: Hairy polyps are very rare benign tumors that can occur anywhere in the body, and they are the most common congenital nasopharyngeal masses. CASE REPORT: A two-day-old full-term female was admitted to the newborn Special Care Unit with noisy breathing, intermittent upper airway obstruction and feeding difficulty. A 3.4 kg infant was born by spontaneous vaginal delivery to a healthy mother, following a normal pregnancy. On admission, she presented with noisy inspiratory breathing which was louder and more labored in prone position. Flexible endoscopy showed a pale sausage-like mass protruding from nasopharynx just behind the soft palate, to the oropharynx and back. A computed tomography scan showed a 1.8 cm round mass in the right nasopharynx, with central fat density and no intracranial communication. The mass was excised transorally with no evidence of a residual tumor. The infant made a full and uneventful postoperative recovery. DISCUSSION: Hairy polyps usually occur as isolated defects and they are not associated with other congenital anomalies. Genetic predisposition has not been established. It does not have malignant alteration. However, there is some confusion about the histological classification of these lesions. One theory supports the idea that hairy polyps originate from disturbed development of stomodeum in the 28th week of gestation and regression of the nasopharyngeal membrane. The second theory supports the idea of origin from the first or second bronchial arch. Probably the best assumption would be that it develops as a special and unique entity. CONCLUSION: Hairy polyp is an unusual malformation that most frequently appears during the first days after birth. Complete resection provides permanent cure.

[Foetal rhinopharyngeal teratoma: antenatal diagnosis and prognosis. Case report]. / Tératome foetal du rhinopharynx : diagnostic anténatal et pronostic. A propos d'un cas.

Fetal teratomas are commonly found in the sacrococcygeal area. More rarely, these tumors may be found in cervicofacial forms. Rhinopharyngeal teratoma represent an extremely rare congenital tumor, often resulting in intracranial extension, obstruction of the respiratory or the digestive pathways and rapid asphyxia following birth. We report a case of rhinopharyngeal teratoma identified in a foetus at 18 weeks of gestation. The diagnosis was made by antenatal ultrasound and IRM. Tumor invaded the cavum and the right infratemporal region with no intracranial extension. The pregnancy was interrupted at 27 weeks of gestation. Authors discuss the difficulties to establish antenatal diagnosis by ultrasound, and those to decide what treatment to carry out.

A very rare cause of recurrent apnea: congenital nasopharyngeal teratoma.

A three-month-old girl patient born at the 37th week of gestation as one of twins was admitted to our hospital with cardiac arrest. There was past medical history of multiple hospitalizations with symptoms of cyanosis, respiratory distress, and frequent and severe attacks of apnea since her birth. On nasopharyngeal computerized tomography a mass (25x24 mm) occupying the right side of the nasopharynx was detected. The pathological evaluation of the excised mass revealed benign teratoma. After the removal of the nasopharyngeal mass under general anesthesia, respiratory distress and attacks of apnea disappeared completely. In every neonate with unexplained stridor and recurrent apnea, beside the common causes like respiratory distress syndrome, sepsis, and asphyxia, the possibility of oropharyngeal and nasopharyngeal mass should be kept in mind.

Congenital nasopharyngeal immature teratoma: a first case report in Thailand.

The authors report the very rare case of a congenital immature teratoma arising from the nasopharyx in a full term female neonate. The tumor also extended to the oral cavity, particularly the right tonsillar fossa without intracranial involvement leading to upper airway obstruction and secondary Escherichia coli pneumonia. The immature part of the tumor in the head and neck region is not a poor prognostic indicator and chemotherapy is useless. In the presented case, the mass was widely excised without postoperative complications. To the best of our knowledge, this is the first reported case in Thailand.

Congenital salivary gland anlage tumor: a case series and review of the literature.

OBJECTIVE: To understand the clinical presentation and management of salivary gland anlage tumor (SGAT). DESIGN AND METHODS: This case series includes a report of a newborn male who presented with acute airway obstruction secondary to a nasopharyngeal mass which was discovered in the course of the clinical evaluation. Six additional cases of SGAT from the pathology consultation files of one of the authors (LPD) presenting in similar fashion are also included. The relevant literature from 1966 to the present has been reviewed through a Medline keyword search utilizing terms "salivary gland anlage tumor", "neonatal", and "nasopharynx." RESULTS: Endoscopic evaluation identified a nasopharyngeal mass tethered to the posterior septum. Although CT and MRI were helpful in identifying the mass and excluding involvement of the surrounding structures, the imaging characteristics of the mass itself were nonspecific. The patient was taken to the operating room and the polypoid mass was removed transorally after lysis of its septal attachment. Pathologic examination revealed a SGAT, a recently described entity in neonates and young infants, who present with early onset respiratory distress. Since the initial report of nine cases by one of the co-authors (LPD), seven additional cases including the present one have been seen in consultation. CONCLUSIONS: Salivary gland anlage tumor of the nasopharynx is a rare cause of neonatal airway obstruction. Endoscopic evaluation and imaging studies are helpful in the exclusion of other etiologies, some of which may have intracranial extension. Simple excision has been curative to date. There have been no reported recurrences in any of the previously studied cases with clinical follow-up dating more than 5 years.