Pediatric head and neck sarcomas: a retrospective study from a national tertiary referral center.

Abstract

This study aims to analyze the demographics, therapeutic approaches, and outcomes of pediatric sarcomas of the head and neck treated at a single tertiary referral center. We retrospectively reviewed the medical charts of all pediatric patients diagnosed with head and neck sarcomas treated at the Tel Aviv Sourasky Medical Center during 2002-2021. Clinical data, oncologic and surgical treatments, and outcome measures were retrieved from electronic medical files. A total of 52 patients met the inclusion criteria. The mean age at diagnosis was 7.25 ± 6.04 years (range 2 months to 20 years), and the male-to-female ratio was 1.4: 1. The leading histological subtypes were rhabdomyosarcoma (RMS) (50%) followed by Ewing sarcoma (23%). The vast majority (96%) of patients were treated with multimodal therapy that included chemotherapy, surgical resection, and/or radiation therapy. Twenty-nine (55.8%) patients underwent surgical resection. The average follow-up was 4.75 years (range 5 months to 13.5 years). The 1-, 5-, and 10-year overall survival rates were 89.5%, 81.3%, and 62.5%, respectively. Male sex, Ewing sarcoma, and non-metastatic disease at diagnosis were associated with better disease-free survival (DFS) (p = 0.008, p = 0.048, and p = 0.038, respectively). Patients with sarcomas suitable for surgical resection and those who received adjuvant therapy had significantly better DFS (p = 0.003 and p = 0.025, respectively).

Conclusions: Head and neck sarcomas in the pediatric population are best managed by a multidisciplinary team. Surgical resection and the integration of adjuvant therapy in selected patients confer survival benefit.

What is known: • Head and neck sarcomas pose a considerable challenge to physicians due to their rarity, their heterogenic presentation, and their proximity to vital structures.

What is new: • A multimodal therapeutic approach that includes chemotherapy, radiotherapy, and surgical resection when feasible should be provided to these patients in order to optimize survival outcomes. Surgical resection and the integration of adjuvant therapy in selected patients confer survival benefit.