Primary surgical resection with negative margins is considered the mainstay therapy for sialoblastomas.5,8,10,11
In cases complicated by metastases, recurrences, or difficulties in surgical access or resectability, additional chemotherapy and/or radiation may be necessary.12,13
Neoadjuvant chemotherapy and/or radiation may result in good response. Saribeyoglu et al.
reported on a patient with locally recurrent sialoblastoma who was treated with chemotherapy only, obviating the need for surgical intervention.13
Optimistically, that patient was in remission for 4 years at the time of publication. However, this is an isolated report of a durable response; most of the published reports lack long term follow-up. Scott et al.
reported a young girl with a sialoblastoma which had also metastasized to the lungs. The tumor was resected after a good response to neo-adjuvant chemotherapy; pulmonary metastases were treated by partial lobectomy.14
Only six months of follow up was reported for this patient. Ersoz et al.
describe a patient still receiving chemotherapy for pulmonary relapse at the time of the report.6
Likewise, Prigent et al.
described a patient with sialoblastoma and pulmonary metastases who was in remission one year following neoadjuvant chemotherapy and resection.12
A novel approach to tumor recurrence was recently reported by Shan et al. In an effort to preserve facial nerve function and avoid a second extensive resection, brachytherapy with a 125I seed implant was utilized, which lead to a complete clinical response after two months. The patient was disease-free 21 months after this intervention; however the durability of this response cannot is unknown.15
In our case, the increased tumor mitotic index and cytological pleomorphism preceded development of distant metastases. Despite the initial response to Ifosfamide, Carboplati num, and Etoposide, and Cytoxan and Topo tecan, chemotherapy and radiation, these modalities did not prevent ultimate disease-progression. Importantly, surgical resection of pulmonary metastasis has lead to a durable remission.
While treatment planning for children with sialoblastomas should be individualized for each patient, surgical resection of recurrent and metastatic disease provided our patient with a long-term remission.