Dental Service, Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York.
Department of Pediatrics, Memorial Sloan Kettering Cancer Center, New York, New York.
Pediatr Blood Cancer. 2021 Apr;68(4):e28918. doi: 10.1002/pbc.28918. Epub 2021 Jan 28.
Although metastatic involvement of bony sites including cranial bones is common in neuroblastoma (NB), mandibular metastases (MM) are uncommon, and specific outcomes have not been reported upon in the modern therapeutic era.
In this retrospective study, medical records on patients with MM from NB were reviewed. Statistical analysis was performed using the Kaplan-Meier method.
Of 29 patients, nine (31%) had MM at diagnosis, whereas in 20 (69%) MM were first detected at NB relapse at a median time of 26 (6-89) months from diagnosis. Median maximal diameter of lesions was 3 (range 0.8-4.9) cm. MM were unilateral in 83% of patients, with ascending ramus (55%) and mandibular body (38%) being the two most common sites. All patients received systemic chemotherapy, and 26 (93%) patients received radiotherapy to MM. At a median follow-up of 37.3 (24.2-219.5) months, eight of nine patients with MM at diagnosis did not experience mandibular progressive disease. Eighteen of 20 patients with MM at relapse received therapeutic radiotherapy; objective responses were noted in 78%. Seventy-two percent (5/18) had not experienced relapse within the radiation field at a median of 12 (2-276) months postradiotherapy. Dental findings at follow-up after completion of NB therapy included hypodontia, hypocalcification of enamel, and trismus. Median 3-year overall survival in patients with relapsed MM was 51 ± 12% months from relapse.
MM when detected at diagnosis is associated with a prognosis similar to that for other skeletal metastases of NB. Radiotherapy is effective for control of MM detected both at diagnosis and relapse. Significant dental abnormalities posttherapy warrant regular dental evaluations and appropriate intervention.
神经母细胞瘤（NB）常发生颅骨等骨部位的转移，而下颌骨转移（MM）较为罕见，在现代治疗时代尚无相关具体预后报道。
本回顾性研究分析了 NB 伴 MM 患者的病历资料。采用 Kaplan-Meier 法进行统计学分析。
29 例患者中，9 例（31%）在诊断时即存在 MM，20 例（69%）则在 NB 复发时首次发现 MM，诊断后至复发的中位时间为 26（6-89）个月。病变最大直径的中位数为 3（范围 0.8-4.9）cm。83%的患者 MM 为单侧，其中升支（55%）和下颌体（38%）是最常见的两个部位。所有患者均接受全身化疗，26（93%）例患者接受 MM 放疗。中位随访时间为 37.3（24.2-219.5）个月时，9 例诊断时存在 MM 的患者中，无 8 例出现下颌进行性疾病。20 例复发时存在 MM 的患者中，18 例接受了治疗性放疗；客观缓解率为 78%。放疗后 12（2-276）个月的中位时间内，18 例中有 78%（15/18）未在放疗野内复发。NB 治疗完成后的随访中，口腔检查发现缺牙、釉质钙化不全和牙关紧闭。复发 MM 患者的中位 3 年总生存率为复发后 51±12%个月。
诊断时发现 MM 与 NB 其他骨骼转移的预后相似。放疗对诊断时和复发时发现的 MM 均有效。治疗后出现明显的牙齿异常，需要定期进行口腔评估和适当干预。
