The pediatric population, specifically under 15 years of age, accounts for only about 4% of all patients with salivary gland tumors.¹ In a review of the Salivary Gland Register of the University of Hamburg, only 2.5% of all epithelial neoplasia occurred in patients younger than 20 years.² Sialoblastoma is a very rare salivary tumor initially reported in 1966 by Vawter and Tefft under the nosology of embryoma. This tumor has also been referred to as congenital basal cell adenoma, basal cell adenoma, and basaloid adenocarcinoma. It may present congenitally or perinatally.³ Most commonly, it originates in the parotid but may also arise in the submandibular gland.^4,5

We originally reported a patient with a sialoblastoma of the left parotid diagnosed at 3 months of age.⁹ The tumor was locally invasive and recurred multiple times. Increasing tumor mitotic index and cytological pleomorphism was noted with recurrences (Figure 1). She was treated with incomplete surgical resection, chemotherapy (vincristine, adriamycin, cytoxan, etoposide and cisplatinum) and highdose radiation therapy at 3.5 years of age. Orbital recurrence developed 1.5 years later, which was completely resected with negative margins; adjuvant radiation therapy was administered. At 6.5 years of age, she developed metastasis to the right lung. There was complete radiologic response after six cycles of Ifosfamide, Carboplatinum and Etoposide; however the patient developed metastasis to the left lung two years later, which responded to two cycles of Cytoxan and Topotecan (Figure 2). The left lung metastases were resected with negative margins, and she received six more courses of adjuvant chemotherapy with the same agents. Unfortunately 6 months later, she developed a third pulmonary recurrence in the right upper lobe, this metastasis was treated by a wedge resection.

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