Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2011  |  Volume : 16  |  Issue : 3  |  Page : 104--105

Malignant epignathus


Harijan Hanumantha Rayudu1, Kilashnath Reddy2, Kasa Lakshmi3, Santhosh Varma4,  
1 Department of Pediatric Surgery, Vishwa Bharathi Hospital, Kurnool, Andhra Pradesh, India
2 Department of Anesthesiology, Vishwa Bharathi Hospital, Kurnool, Andhra Pradesh, India
3 Department of Pathology, Vishwa Bharathi Hospital, Kurnool, Andhra Pradesh, India
4 Department of Radiology, Vishwa Bharathi Hospital, Kurnool, Andhra Pradesh, India

Correspondence Address:
Harijan Hanumantha Rayudu
50/760A-113, Gayathri Estate, Kurnool - 518 002, Andhra Pradesh
India

Abstract

Report of a neonate with a huge mass protruding from the oral cavity. The mass has originated from the base of the tongue. Successful excision and histopathological examination revealed it to be a malignant epignathus.



How to cite this article:
Rayudu HH, Reddy K, Lakshmi K, Varma S. Malignant epignathus.J Indian Assoc Pediatr Surg 2011;16:104-105


How to cite this URL:
Rayudu HH, Reddy K, Lakshmi K, Varma S. Malignant epignathus. J Indian Assoc Pediatr Surg [serial online] 2011 [cited 2021 Aug 3 ];16:104-105
Available from: https://www.jiaps.com/text.asp?2011/16/3/104/83490


Full Text

 Introduction



Epignathus tumors are extremely rare head and neck congenital teratomas, but are usually benign. [1] They may be large in size causing respiratory distress or difficulty in swallowing. Mostly they arise from the tongue. They are not known to be malignant. We report herein a case of malignant epignathus.

 Case Report



A 2-day-old male neonate weighing 3.2 kg was presented with fever and a huge mass protruding from the oral cavity. The patient was not in respiratory distress but was not able to swallow. The mass has originated from the base of the tongue, measuring 9 × 10 cm [Figure 1]. It was dark red and the surface was uneven and multilobulated. The mass had a mixed cystic and firm consistency. It was not pulsatile. No other congenital malformations were noted. Computed tomogram study demonstrated a large heterogenous mass arising from the base of the tongue. The mass was showing hyperdense calcific areas and fluid dense cystic areas. The serum alpha-fetoprotein was 1600 IU.{Figure 1}

The tumor was successfully excised totally from the base of the tongue under transnasal, endotracheal anesthesia. The cut section of the tumor showed multiloculated cysts containing serous fluid and solid areas. The postoperative recovery was uneventful [Figure 2]. The patient was discharged on the fifth postoperative day. The histopathological examination revealed it was a malignant epignathus [Figure 3] and [Figure 4].{Figure 2}{Figure 3}{Figure 4}

 Discussion



The commonest site of teratoma in the new born is sacrococcygeal teratoma. Teratomas of the tongue are extremely rare. [1] Malignant teratomas of the tongue are extremely rare and only two cases have been reported in the literature. [2] Epignathus is a terminology used for the tumors arising from soft and hard palate in the region of Rathke's pouch or in the nasopharynx in the region of basi-sphenoid, tongue, mandible, or tonsil. As the tumor is large in size they obstruct the air and food passages resulting in respiratory distress and impede swallowing. [3]

The specimen shows mostly solid areas, necrotic areas, and tiny cystic spaces. Histologically, the tumor cells are composed of mixture of embryonal and adult tissues derived from all the three germinal layers [Figure 3] and [Figure 4]. The main component in our child is primitive neuroepithelial elements. Surgery is the treatment of choice for epignathus. After complete excision, the tumors do not recur. In our case, the tumor was easily removed despite its large size.

References

1Kountakis SE, Minotti AM, Maillard A, Stiernberg CM. Teratomas of the head and neck. Am J Otolaryngol 1994;15:292-6.
2Millar AP, Ownes JB. Teratoma tongue. Cancer 1966;19:1583-6.
3Gupta M, Chaudhary N, Rai AK. Epignathus. Indian J Otolaryngol 2007;59:160-2.