Cervical Giant Immature Teratoma in a Newborn: A Challenge for Survival.

Teratomas are a conglomerate of heterogenous cells arising from totipotent germ cells. Cervical teratomas occur 1 per 20,000 to 40,000 live births constituting 1.6-9.3% of all paediatric teratomas. They can present antenatally with cervical mass or polyhydramnios and postnatally with respiratory distress and facial disfigurement. Complete excision of the tumour prevents malignant transformation. Timing of the surgery is based on severity of airway compromise. Surgical outcome and survival depend on pre-existing pressure effects, operative injuries to the vital structures and also co-existing comorbidities. One such complicated case of giant cervical teratoma is described here. Copyright:

INTRODUCTION

Cervical teratomas constitute 1.6%–9.3% of all pediatric teratomas.[12] They can present antenatally with cervical mass or polyhydramnios and postnatally with respiratory distress and facial disfigurement. Surgical outcome and survival depend on preexisting pressure effects, operative injuries to the vital structures, and also coexisting comorbidities. One such complicated case of giant cervical teratoma is described here.

CASE REPORT

A full-term female neonate was born with a huge lobulated cervical mass [Figure 1]. At birth, the baby was desaturating and was immediately intubated. Alpha-fetoprotein (AFP) was 65,000 ng/ml and beta human chorionic gonadotropin was 35 mIU/ml. The child was operated within 16 h of life due to tracheal compression. Complete excision of the tumor measuring 15 cm × 12 cm × 8 cm, adherent to pretracheal fascia, left carotid sheath, [Figure 1] displacing the trachea and the carotid vessels, with preservation of neurovascular structures, was successfully done. Histopathology showed fibrocartilaginous tissue and few cystic spaces [Figure 2] lined by mucin-secreting columnar epithelium along with extensive areas of glial tissue admixed with cluster of small-to-round cells having hyperchromatic nuclei in rossettoid pattern consistent with immature teratoma WHO grade 3.[3]

Figure 1

Preoperative appearance and completely excised gross specimen of the tumor

Figure 2

Histopathology demonstrating the features of immature teratoma. (a) Glandular epithelium – endoderm (short arrow); (b) cartilage – mesoderm (thin long arrow); (c) immature neural elements – ectoderm (arrowhead)

Postoperatively, the child required prolonged ventilation for 14 days due to tracheomalacia. Two-dimensional echo revealed severe pulmonary arterial hypertension (PAH) which was managed with ventilation, sildenafil, and bosentan. PAH and tracheomalacia regressed completely over the next 4 weeks. Neuropraxia of marginal mandibular, glossopharyngeal, vagus, and hypoglossal nerves presenting as transient mouth and tongue deviation, respiratory distress, regurgitation, and swallowing difficulties resolved completely by the time of discharge. The child is currently 6 months postoperative and AFP now is <16 ng/ml and she has no airway or facial abnormality.

DISCUSSION

Literature search revealed only a few surviving cases of giant cervical immature teratoma.[2] Antenatal scans at times may not identify the mass as in the cases reported by Gezer et al.[4] The reasons for morbidity and mortality of giant cervical teratomas are airway obstruction, pressure on cervical neurovascular structures and comorbid factors

In antenatally diagnosed cases, airway is secured either by ex utero intrapartum treatment procedure or by extracorporeal membrane oxygenation.[5] If airway is not secured, an aggressive surgical exploration has to be done by a team with adequate expertise.[6]

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.

CONCLUSION

Giant cervical immature teratoma with PAH in a surviving neonate as reported here is of interest because of its rarity and survival is possible with aggressive multidisciplinary approach.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.