Hintze Justin M, Afshar Salim, Taghinia Amir, Labow Brian, Green Mark, Robson Caroline D, Marcus Karen, Mack Jennifer, Perez-Atayde Antonio, Rahbar Reza
Department of Otolaryngology and Communication Enhancement, USA; Harvard Medical School, Boston, MA, USA.
Department of Plastic and Oral Surgery, USA; Harvard Medical School, Boston, MA, USA.
Int J Pediatr Otorhinolaryngol. 2023 May;168:111547. doi: 10.1016/j.ijporl.2023.111547. Epub 2023 Apr 15.
Mandibular tumors in the pediatric population are rare. These malignancies are variable in their histology, and combined with their rarity, has made it difficult to describe their clinical course, and treatment guidelines. The aim of this paper is to describe the experience of Boston Children's Hospital, a pediatric tertiary referral center, with treating malignant mandibular malignancies, as well as provide multi-disciplinary team approach in managing this clinical entity.
A retrospective search was performed for mandibular malignancies in pediatric patients between 1995 and 2020 via the pathological database at Boston Children's Hospital. Only patients with malignant solid mandibular neoplasms were included, leaving 15 patients for final analysis.
The median age at presentation was 10.1 ± 10.3 years. Nine of 15 patients (60%) presented with jaw mass which was the most common clinical presentation. The most commonly identified histological diagnosis was rhabdomayosarcoma and osteosarcoma (n = 4, 26% each). A mandibulectomy was performed in 12 (80%) cases. Reconstruction of the mandible was performed using a fibular free flap in 6 (40%) cases, and a plate in 3 (20%) cases. Mean follow-up was 4.6 ± 4.9 years.
Malignant tumors most commonly present with a jaw mass, however asymptomatic and incidental presentations follow closely and pathologies can vary greatly. Surgical resection and reconstruction is often indicated, multidisciplinary tumor board review is required to determine when children are best treated with neo-/adjuvant treatment with chemo- and radiotherapy.
小儿下颌骨肿瘤较为罕见。这些恶性肿瘤的组织学表现多样,再加上其罕见性,使得描述其临床病程和治疗指南变得困难。本文旨在描述小儿三级转诊中心波士顿儿童医院治疗下颌骨恶性肿瘤的经验,并提供管理这一临床实体的多学科团队方法。
通过波士顿儿童医院的病理数据库,对1995年至2020年间儿科患者的下颌骨恶性肿瘤进行回顾性检索。仅纳入患有下颌骨实体恶性肿瘤的患者,最终分析共15例患者。
就诊时的中位年龄为10.1±10.3岁。15例患者中有9例(60%)以颌部肿块就诊,这是最常见的临床表现。最常确诊的组织学诊断是横纹肌肉瘤和骨肉瘤(各4例,占26%)。12例(80%)患者进行了下颌骨切除术。6例(40%)患者采用游离腓骨瓣进行下颌骨重建,3例(20%)患者采用钢板重建。平均随访时间为4.6±4.9年。
恶性肿瘤最常见的表现是颌部肿块,但无症状和偶然发现的情况也很常见,病理类型差异很大。通常需要进行手术切除和重建,需要多学科肿瘤委员会进行评估,以确定儿童何时接受新辅助/辅助化疗和放疗效果最佳。