Opiła Radosław, Feszak Sylwia, Wawryków Paweł, Peregud-Pogorzelski Jarosław
Department of Pediatrics, Pediatric Oncology, and Immunology, Pomeranian Medical University, 71-252 Szczecin, Poland.
Children (Basel). 2023 Mar 28;10(4):628. doi: 10.3390/children10040628.
Sialoblastoma is an extremely rare embryonal tumor derived from salivary gland primordial cells. Treatment usually consists of surgery alone; however, in some cases, chemotherapy is required and is administered with good response. We present a case of a 5-week-old girl diagnosed with a parotid gland tumor and co-existing nevus sebaceous on the face. Initial tumorectomy was microscopically non-radical and histopathology revealed sialoblastoma. The patient received adjuvant chemotherapy consisting of vincristine, actinomycin, and cyclophosphamide. Due to imaging studies being inconclusive regarding response and possible residual disease, a second surgery (total parotidectomy) was performed. The histopathology results showed fields of necrosis in the parotid gland but no neoplastic cells in the material. The patient remains under watchful observation and there is no evidence of relapse 12 months after the second surgery. The adjuvant chemotherapy regimen with vincristine, actinomycin, and cyclophosphamide is a viable option of treatment in children with sialoblastoma.
涎腺胚细胞瘤是一种极其罕见的胚胎性肿瘤,起源于唾液腺原始细胞。治疗通常仅包括手术;然而,在某些情况下,需要进行化疗,且化疗反应良好。我们报告一例5周大的女孩,诊断为腮腺肿瘤并伴有面部皮脂腺痣。初次肿瘤切除术在显微镜下未达到根治,组织病理学显示为涎腺胚细胞瘤。患者接受了由长春新碱、放线菌素和环磷酰胺组成的辅助化疗。由于影像学研究对于反应和可能的残留疾病尚无定论,遂进行了第二次手术(腮腺全切除术)。组织病理学结果显示腮腺有坏死区域,但送检材料中未发现肿瘤细胞。患者仍在密切观察中,第二次手术后12个月无复发迹象。长春新碱、放线菌素和环磷酰胺组成的辅助化疗方案是涎腺胚细胞瘤患儿可行的治疗选择。