Journal
ACTA OTO-LARYNGOLOGICA CASE REPORTS
Volume 8, Issue 1, Pages 72-76Publisher
TAYLOR & FRANCIS LTD
DOI: 10.1080/23772484.2023.2217341
Keywords
EXIT procedure; infantile fibrosarcoma; tongue; soft tissue tumour
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This article presents a rare case of huge infantile tongue fibrosarcoma that was diagnosed prenatally and successfully delivered via ex utero intrapartum treatment (EXIT). The initial diagnosis was infantile tongue hemangioma based on physical examination, radiological findings, and high incidence of hemangioma in infants. A tracheostomy was performed due to anticipated airway compromise and failed extubation. Histopathological examination revealed a malignant tumor suggestive of infantile fibrosarcoma. The management of infantile fibrosarcoma requires a multidisciplinary team approach, with non-mutilating surgery as the primary treatment and neoadjuvant chemotherapy as an option when upfront resection is unfeasible.
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.
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