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The Desmoid Tumor Research Foundation

May 2021

Massive congenital cervicofacial desmoid-type fibromatosis in a 5-month-old infant

Author Information

Albokashy MS (1), Mohammed S Halawani MS(1), Eshky AT (1), Alsaad K (2), Khoja HA (3), Bawazir SM(1).

(1) Pediatric Div, Dept of Otolaryngology/Head and Neck Surgery, Prince Sultan Military Medical City, Riyadh, Saudi Arabia. (2) Dept of Oral and Maxillofacial Surgery, Prince Sultan Military Medical (3) Dept of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.City, Riyadh, Saudi Arabia.

Publication

J Surg Case Rep

Summary

Desmoid-type fibromatosis (DF) is a borderline tumor of soft tissues that has low malignant potential but described as infiltrative, locally aggressive and rapidly growing. In the pediatric population, it occurs in the head and neck. Presentation varies based on tumor size and location. Despite the high recurrence rate, surgical excision remains the modality of choice with. Here, we report a case of a 5-month-old boy, with extensive head and neck DF that was managed twice with conservative debulking surgery through a combined transoral-transcervical approach. On 2-year follow-up, he was gaining weight with no developmental delay and had no clinical evidence tumor regrowth.

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