Ex Utero Intrapartum Treatment (EXIT) in a rare infantile tongue fibrosarcoma and it’s management dilemma

Abstract

Ex utero intrapartum treatment (EXIT) has been described as a safe procedure to secure challenging fetal airways while on placental support. Here we present an extremely rare case of huge infantile tongue fibrosarcoma diagnosed prenatally and successfully delivered via EXIT. Initial diagnosis of infantile tongue haemangioma was made based on physical examination, radiological findings and high incidence of infantile haemangioma in the first year of life hence trial of oral propranolol was given. Tracheostomy was performed at 1 week of life in anticipation of airway compromise and failed extubation. A biopsy of the tongue tumour was performed to rule out soft tissue malignancy. Histopathological examination (HPE) revealed a malignant tumour suggestive of infantile fibrosarcoma. The management of infantile fibrosarcoma is a multidisciplinary team’s involvement. However, non-mutilating surgery should be the primary treatment for infantile fibrosarcoma aiming for complete excision. Neoadjuvant chemotherapy is indicated when upfront resection is unfeasible as in the present case.

Keywords:

• EXIT procedure
• infantile fibrosarcoma
• tongue
• soft tissue tumour

Acknowledgement

The authors would like to acknowledge Dato Dr Bavanandam Naidu (Fetomaternal specialist) and his team for their expertise in managing the mother, Dr Nazuha and the rest of anesthesiologist team for allowing surgery to proceed smoothly. The authors would also like to thank the paediatric intensivist Dr Eric Ang Boon Kuang together with his team who have been tirelessly involved in the care of the patient. We also would like to thank Director- General of Health Malaysia for his permission to publish this article.

Consent for publication

Written informed consent was obtained from the patient’s legal guardian for the publication of this case report and any accompanying images.

Author contributions

NR was involved in the collection of the data, literature search, writing of the original draft, surgical assistance, patient follow-up and the corresponding author. FAR, NG and SSMH were involved in the patient management, operating surgeon, follow-up and review of the draft. GBS was involved in reviewing the final draft. All authors read and approved the final manuscript.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

This manuscript has not received any external or industrial support or funding.