Irace Alexandria L, Adil Eelam A, Archer Natasha M, Silvera Victoria M, Perez-Atayde Antonio, Rahbar Reza
Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA.
Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA; Otolaryngology, Harvard School of Medicine, 25 Shattuck Street, Boston, MA 02115, USA.
Int J Pediatr Otorhinolaryngol. 2016 Aug;87:44-9. doi: 10.1016/j.ijporl.2016.04.037. Epub 2016 Apr 29.
Sialoblastoma is a rare congenital salivary gland tumor of epithelial origin. The objectives of this study are to review the literature regarding clinical presentation of sialoblastoma, evaluate the effectiveness of various treatment methods, and present guidelines for evaluation and management in the pediatric population.
Case presentation and literature review.
A comprehensive search was conducted to identify cases of pediatric sialoblastoma in the English-language literature. The presentation, evaluation, and management of reported cases were analyzed. We also report an invasive and recurrent case in a pediatric patient to highlight the aggressive nature of these lesions.
Sixty-two cases of pediatric sialoblastoma were reviewed. The age at initial presentation ranged from before birth to 15 years. The parotid gland was the most common location (n = 47). Surgical excision was the primary treatment in all patients. Nine patients developed metastatic disease of the lung, lymph nodes, or bone. Almost a third of patients had recurrence and over two thirds of patients were tumor-free for at least 1 year following their last treatment intervention.
Prompt and complete surgical excision should be recommended to prevent local and systemic recurrence of pediatric sialoblastoma. Chemotherapy has also shown promise in several cases, and clinical genomics may shed light on more therapy options. Patients should be closely followed for at least 12 months following diagnosis, or longer depending on the histopathological staging of the tumor.
涎母细胞瘤是一种罕见的先天性上皮源性涎腺肿瘤。本研究的目的是回顾关于涎母细胞瘤临床表现的文献，评估各种治疗方法的有效性，并提出针对儿童患者的评估和管理指南。
病例报告和文献综述。
进行全面检索以确定英文文献中儿童涎母细胞瘤的病例。分析所报告病例的表现、评估和管理。我们还报告了一名儿科患者的侵袭性复发病例，以突出这些病变的侵袭性。
回顾了62例儿童涎母细胞瘤病例。初次就诊年龄从出生前到15岁不等。腮腺是最常见的部位（n = 47）。所有患者的主要治疗方法是手术切除。9例患者出现肺、淋巴结或骨转移。近三分之一的患者复发，超过三分之二的患者在最后一次治疗干预后至少1年无肿瘤。
应建议及时、完整地手术切除，以预防儿童涎母细胞瘤的局部和全身复发。化疗在一些病例中也显示出前景，临床基因组学可能会揭示更多的治疗选择。诊断后应密切随访患者至少12个月，或根据肿瘤的组织病理学分期延长随访时间。
