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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 16  |  Issue : 1  |  Page : 91-94

A large obstructive hard palate Teratoma in a neonate


Department of Oral and Maxillofacial Surgery, INHS Asvini, Mumbai, Maharashtra, India

Date of Submission16-Aug-2021
Date of Acceptance21-Jan-2022
Date of Web Publication05-Apr-2022

Correspondence Address:
Pushpa Kumari
INDC Danteshwari, INHS Asvini, Near RC Church, Mumbai - 400 005, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jodd.jodd_37_21

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  Abstract 


A neonate female was born with congenital swelling of the hard palate. The baby did not cry immediately after birth, and she was in respiratory distress. The patient required positive pressure ventilation in bag and mask ventilation followed by endotracheal intubation. Noncontrast computed tomography of the head on the 7th day of life showed a soft tissue density fat-containing lesion over the roof of the oral cavity and bony defect in the hard palate. It was provisionally diagnosed as a case of soft tissue mass – hamartoma of the hard palate associated with complete cleft palate and acyanotic heart disease. The patient underwent excision of the mass on the 9th day of life. Histopathological report showed mature teratoma. Teratoma is a rare congenital malformation. At present, after 2 years of age, the patient shows no signs of recurrence.

Keywords: Congenital hard palate swelling, endotracheal intubation, respiratory distress, teratoma


How to cite this article:
Kumari P, Yadav AS, Randhawa JS, Saxena V, Mehta R, Mukund B. A large obstructive hard palate Teratoma in a neonate. J Dent Def Sect. 2022;16:91-4

How to cite this URL:
Kumari P, Yadav AS, Randhawa JS, Saxena V, Mehta R, Mukund B. A large obstructive hard palate Teratoma in a neonate. J Dent Def Sect. [serial online] 2022 [cited 2022 Jun 30];16:91-4. Available from: http://www.journaldds.org/text.asp?2022/16/1/91/342643




  Introduction Top


Congenital tumors are rare in the oral cavity and offer diagnostic surprise and therapeutic challenge to the clinician. “Epignathus” is the tumor arising in the oral cavity from palate, tongue, or floor of the mouth. It is a congenital malformation classified as a mature teratoma.[1]

Teratomas are typically benign neoplasms that contain elements derived from all three embryonic germ layers: endoderm, mesoderm, and ectoderm. They are composed of multiple tissues that are foreign to the anatomic site in which they are found.[2]

A teratoma may develop in any area of the body along the midline.[1] They have a reported incidence of 1:4000 live births, with at least 2% of these in the head and neck[1] region. The most common sites are the sacrococcyx, mediastinum, gonads, or retroperitoneum.[3]

We describe a case report of a rare nonsyndromic, centrally located, congenital hard palate lesion - teratoma in a neonate associated with congenital heart defects.


  Patient Information Top


A female child born at 40 weeks of gestation by spontaneous vaginal delivery did not cry immediately after birth. She had respiratory distress, was initially supported by bag and mask ventilation, and then was intubated. She had an obstructing intraoral lesion and was admitted to the neonatal intensive care unit.

Clinical findings

Examination showed a tongue-shaped obstructive, lobulated, soft tissue mass originating from the roof of the oral cavity, measuring about 20 mm × 10 mm [Figure 1]. It lacked a pedicle, i.e., it was sessile. Overlying mucosa was normal in appearance similar to the adjacent oral mucosa. However, the mass had areas of hair growth.
Figure 1: Preoperative picture of the patient at day 9

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A defect in the alveolus could be appreciated on the anterior aspect of the hard palate. Examination of the posterior hard palate was difficult due to obscuring soft tissue mass. Rest of the oral cavity and lower jaw was normal in appearance. The nasal cavity, lips, and rest of the face were normal in appearance.

The neonate developed pneumonia, for which intravenous antibiotics were started.

The mother was antenatally booked and immunized and was euthyroid and euglycemic. Antenatal ultrasonography (USG) at 12 weeks showed single live intrauterine fetus (SLIUF) with nuchal translucency - 0.7 mm and antenatal USG at 36 weeks showed SLIUF with cephalic presentation and S/D ratio - 2.4.

Diagnostic assessment

Clinical examination at birth on auscultation revealed ejection systolic murmur. Computed tomography of the head showed a soft tissue density protruding from the oral cavity. It was viewed three-dimensionally and seen to arise from the palate. It consisted of cystic lesion and areas of calcification. Noncontrast computed tomography of the head showed no intracranial extension [Figure 2]. On further comprehensive examination, 2D Echo showed ventricular septal defect (VSD).
Figure 2: NCCT Head, preoperative

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Therapeutic intervention

Maintaining saturation was difficult for the newborn, and also, breastfeeding was an issue as the patient was intubated. She was fed formula milk through Ryle's tube. With initial evaluation and opinion of pediatric surgeon and maxillofacial surgeon, a provisional diagnosis of vomerine hamartoma and a differential diagnosis of teratoma were made, and the infant was taken up for excisional biopsy under general anesthesia on the 9th day of life. The mass including the surrounding mucosa of the cleft was excised. In the deeper layers, the mass was poorly demarcated from the hard palate. The surgical soft tissue defect was left to heal by secondary intention.

The temporomandibular joints moved well. After the tumor had been removed, a complete unilateral cleft involving the hard palate and soft palate became apparent. Postoperatively, the palatal wound epithelialized well.

The patient was unable to take feeds orally because of complete cleft palate, and hence, initial postoperative feeds were through nasogastric tube [Figure 3]. The patient was on special need feeder bottle designed for cleft cases.
Figure 3: Post operative picture of the patient

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Follow-up and outcomes

Histopathological examination

Sections from submitted tissues showed keratinized stratified squamous epithelium and subepithelium revealed numerous pilosebaceous units. Underlying Fibroadipose tissue showed multiple cystic spaces lined by pseudostratified squamous epithelium [Figure 4] and [Figure 5]. Cartilage and bone were also identified. No foci of immature elements were seen. The tissue was diagnosed as mature teratoma.
Figure 4: Histopathological slide of specimen

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Figure 5: High power magnification of the cystic wall

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After 2 years of birth, the patient does not show any sign of recurrence.

Cleft palate was repaired at 8 months of age. However, the patient shows difficulty in swallowing and is presently on peg feed.


  Discussion Top


Teratomas may be solid, cystic, or mixed. Teratomas are classified as mature or immature. The incidence of malignancy in neonatal teratomas is very low, they are usually benign, and the incidence of malignancy in teratomas in adults is high.[4]

Calcification is more common in benign than malignant teratomas. The presence of primitive neural tissue also suggests malignancy. The presence of a teratoma should raise the suspicion of other germ cell tumors such as yolk sac tumor in neonates.[5] The lesion in the present case had areas of calcifications, and no primitive neural tissue was seen.

Embryologically, the palate is believed to develop between 6th and 8th week; consequently, this causes a distinction between the nasal and oral cavities, and the development is completed by the 12th week. It has been suggested that the tissues of a teratoma are derived from totipotent cells sequestered during embryogenesis.[6],[7]

Teratoma in the oral cavity is a very rare disease. An incidence of between 1:35,000 and 1:200,000 live births has been found. Most reports described a slight female predominance.[3],[8]

The teratoma may be diagnosed antenatally on ultrasound or magnetic resonance imaging which permits early multidisciplinary management. In the present case, the tumor presents as a cystic lesion. This diagnosis is confirmed by polyhydramnios,[9] which is thought to be a consequence of impaired fetal swallowing, and it has been proposed that cardiac decompensation caused by circulation within a large vascular teratoma may also contribute.

The differential diagnosis is limited but includes hamartoma, dermoid cyst, and heterotopic gastrointestinal cyst.[9]

Teeth within the mass was confirmed as teratoma.[10]

Antenatal diagnosis allows a controlled environment to be planned for the birth with multidisciplinary care.[6] In our case, it was diagnosed postnatally. On further evaluation, the baby had VSD.

This teratoma may present as an obstructive mass causing respiratory embarrassment and an immediate threat to life, which demands immediate establishment of an airway, often with tracheostomy. It may also present as a small pedunculated tumor with no functional obstruction. Excision is the treatment of choice, as there may be a small chance of malignant transformation the longer they are left.[11] Prognosis worsens as the size of the tumor increases. The ultimate prognosis of lesions with intracranial involvement is poor.[12],[13]

Benign teratomas may recur after excision.[12] Carney et al. discussed three cases of mature sacrococcygeal teratomas that recurred in children. This does not necessarily imply malignancy, although the clinician should maintain follow-up.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hoeffel CC, Nguyen KQ, Phan HT, Truong NH, Nguyen TS, Tran TT, et al. Fetusinfetu: A case report and literature review. Pediatrics 2000;105:1335-44.  Back to cited text no. 1
    
2.
Takeuchi K, Masuda Y, Narita F, Kiyoshi K, Mizutori M, Maruo T. Prenatal evaluation of bidirectional epignathus: Comparison of ultrasonography and magnetic resonance imaging. Fetal Diagn Ther 2003;18:26-8.  Back to cited text no. 2
    
3.
Weaver RG, Meyerhoff WL, Gates GA. Teratomas of the head and neck. Surg Forum 1976;27:539-42.  Back to cited text no. 3
    
4.
Shafer WG, Hine MK, Levy BM, Tomich CE, editors. Textbook of Oral Pathology. 78-79, 9th ed. London: WB Saunders; 1983. p. 312-3.  Back to cited text no. 4
    
5.
Vandenhaute B, Leteurtre E, Lecomte-Houcke M, Pellerin P, Nuyts JP, Cuisset JM, et al. Epignathus teratoma: Report of three cases with a review of the literature. Cleft Palate Craniofac J 2000;37:83-91.  Back to cited text no. 5
    
6.
Zhang GZ, Din GC, Zhao YF. Giant epignathus teratoma: Report of a case. J Oral Maxillofac Surg 2007;65:337-40.  Back to cited text no. 6
    
7.
Sumiyoshi S, Machida J, Yamamoto T, Fukano H, Shimozato K, Fujimoto Y, et al. Massive immature teratoma in a neonate. Int J Oral Maxillofac Surg 2010;39:1020-3.  Back to cited text no. 7
    
8.
Williams LJ, Yankowitz J, Robinson RA, Benda JA. Nasopharyngeal teratoma. Report of a case with prenatal diagnosis and postnatal management. J Reprod Med 1997;42:587-92.  Back to cited text no. 8
    
9.
Beutel K, Partsch CJ, Jänig U, Nikischin W, Suttorp M. Oral mature teratoma containing epididymal tissue in a female neonate. Lancet 2001;357:283-4.  Back to cited text no. 9
    
10.
Zerella JT, Finberg FJ. Obstruction of the neonatal airway from teratomas. Surg Gynecol Obstet 1990;170:126-31.  Back to cited text no. 10
    
11.
Tamura T, Yamataka A, Okazaki T, Hosoda Y, Lane GJ, Miyano T. Management of a prenatally diagnosed huge teratoma arising from the soft palate. Asian J Surg 2006;29:212-5.  Back to cited text no. 11
    
12.
Carney JA, Thompson DP, Johnson CL, Lynn HB. Teratomas in children: Clinical and pathologic aspects. J Pediatr Surg 1972;7:271-82.  Back to cited text no. 12
    
13.
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;45:326-7.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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