Malignant epignathus.

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.

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

Big mass arising from oral cavity

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 [Figures 3 and 4].

Figure 2

After excision of mass

Figure 3

Mixture of embroyonal and adult tissue

Figure 4

Premitive neuroepithelial elements arrange in Rosettes—malignant

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 [Figures 3 and 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.