A 26-day-old male neonate presented with intermittent vomiting of approximately two weeks duration, and malena for one day. The general physical and systemic examinations were unremarkable. An initial contrast-enhanced computed tomography (CECT) scan showed upper esophageal dilation, unclear lower esophagus, and a large tumor mass between the esophagus and gastric fundus. The upper gastrointestinal barium study (Fig. 1a) revealed a large tumor mass in the lower esophagus involving the cardia and gastric fundus. The laboratory tests were within normal limits, including liver function tests, lactate dehydrogenase, ferritin, carcino-embryonic antigen, and neuron-specific enolase. Alpha fetoprotein was elevated with 5284 ng/mL. A biopsy was taken from the margins of the tumor mass, and immuno-histochemistry revealed an immature teratoma (Fig. 2).The tumor mass was surgically excised. The post-operative period was uneventful. The infant was discharged home after 2 weeks. He was breastfeeding well and there was no vomiting.

Fig. 1 Upper gastrointestinal barium study showing (a) tumor mass in the lower esophagus involving the cardia and gastric fundus before surgery (arrows ); (b) a fistula (arrows) located at the lower end of the esophagus, fundus being pulled up to the diaphragm, and an esophageal hiatal hernia at 1-month follow up.

Fig. 2 Immunohistochemistry reveals an immature teratoma (arrow shows the bone content).  (See website for color image).

At the three-month follow-up, alpha fetoprotein levels had reduced to normal, 2.83 ng/mL. At follow-ups at 6 months and 1 year, the boy was in a better condition, accepting normal food and his growth and development were normal. An upper gastrointestinal barium study at 1 month after surgery revealed a fistula located at the lower end of the esophagus, fundus pulled up to the diaphragm, and an esophageal hiatal hernia (Fig. 1b). The infant is being followed closely and the decision to repair the fistula is pending.

Teratomas most often occur in a para-axial location, in the midline from the brain to the sacral area, or in the gonads. Teratoma at the esophagus and stomach is extremely rare in children, accounting for less than 1% of all teratomas. To date, only one such case has been reported in the English literature [2]. Bernat, et al. [3] reported seven cases of benign esophageal tumors treated in a hospital from 1972 to 1990, only one of them was finally diagnosed as mature teratoma. He reported complete cure in all patients after surgery [3].

Gastric teratoma is believed to arise from the pluripotent cells of the gastric visceral wall [4]. The majority is benign and immature. The site of gastric teratoma is variable, most commonly arising from the greater curvature and posterior wall. The clinical features of esophagogastric junction teratoma in the neonate of the present case appeared at three weeks of life, with vomiting and melena, and without abdominal distension, constipation, or respiratory distress.

The melena could be due to mucosal bleeding at the tumor site. The CECT scan suggested the possibility of a leiomyosarcoma or neuroblastoma. The final diagnosis of teratoma can only be confirmed by histopathological examination of tissue. The alpha fetoprotein levels reflect the treatment response after excision, and may be of significance when chemotherapy is recommended in immature teratomas. Complete excision of the teratoma carries a good prognosis.

Few post-operative complications were noted in the present case. An inadequately dissected fundus may pull it into the chest, which could increase the anastomotic tension between the esophagus and stomach, and lead to the postoperative fistula. On the other hand, the hiatal hernia could have been induced by an inadequately repaired esophageal hiatus. In addition, difficulties during surgery, especially exposing the mass, looking for a surgical entry point, and complete removal of the tumor may have added to the risk of postoperative complications.

While resecting such masses, full dissociation of the fundus and pulling it into the chest may reduce esophageal and gastric anastomotic tension and thereby avoid the occurrence of postoperative fistula.

1. Peterson CM1, Buckley C, Holley S, Menias CO. Teratomas: A multimodality review. Curr Probl Diagn Radiol. 2012;41:210-9.

2. Giacomoni MA, Zaffaroni G. Teratoma of the gastro-esophageal junction in an infant. Arch Ital Chir. 1968;94:781-7.

3. Bernat M1, Strutyñska-Karpiñska M, Lewandowski A, Blaszczuk J, Grabowski K, Czapla L. Benign esophageal tumors. Wiad Lek. 1993;46:24-7.