Pediatric Head and Neck Fibrosarcomas: A Demographical, Treatment, and Survival Review and Review of a Rare Case

Publication date: Available online 2 October 2018

Source: International Journal of Pediatric Otorhinolaryngology

Author(s): Tyler A. Janz, Ramamoorthy Nagasubramanian, Julie L. Wei

Abstract

Objective

To examine pediatric head and neck fibrosarcoma cases and review the demographics, management, and survival for these patients.

Methods

Pediatric patients in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1973-2014 based on a diagnosis of a head and neck fibrosarcoma using ICD-O-3 head and neck primary sites and histology codes. Patients were included from ages 0-18 years. Additionally, a pediatric case of a head and neck infantile fibrosarcoma treated at the Nemours Children's hospital in Orlando, Florida is presented.

Results

One hundred-thirteen pediatric head and neck fibrosarcomas were identified within the SEER database over the study period. The mean age at diagnosis was 9.8 years (SD: 6.2, range: 0.0-18.0). The mean age at diagnosis for infantile fibrosarcomas was 1.7 years (SD: 3.2, range: 0.0-12.0). Fifty-one (45.1%) patients were female. A majority (N=67, 59.3%) of patients had dermatofibrosarcoma followed by 18 (15.9%) who had infantile fibrosarcomas. Nearly all patients (N=107, 94.7%) received surgical intervention. 27.5% of patients with an infantile fibrosarcoma received chemotherapy as a part of their care compared to 1.5% of patients with a dermatofibrosaroma (p=.004). The 5-year disease-specific survival was 97%.

Conclusions

and Relevance: Pediatric patients with head and neck fibrosarcomas are most likely to present in Caucasian males or female during late childhood or early adolescence. Infantile fibrosarcomas present in pediatric patients at a much earlier age. Surgical management is common for pediatric head and neck fibrosarcomas. Additionally, chemotherapy may be used for infantile fibrosarcomas of the head and neck. Survival rates for pediatric patients with a head and neck fibrosarcoma are excellent.

https://ift.tt/2IwpMrF