Shigeta Rie, Orgun Doruk, Mizuno Hiroshi, Hayashi Ayato
Department of Plastic and Reconstructive Surgery, Juntendo University School of Medicine, Tokyo, Japan.
Department of Plastic and Reconstructive Surgery, Juntendo University Urayasu Hospital, Chiba, Japan.
Plast Reconstr Surg Glob Open. 2018 Dec 13;6(12):e2059. doi: 10.1097/GOX.0000000000002059. eCollection 2018 Dec.
Mammary analogue secretory carcinoma (MASC) of salivary glands is a newly recognized tumor entity. We report a child who was initially diagnosed with lymphangioma and referred to our institute for sclerotherapy, only to find out that the tumor was in fact MASC after excision. This case of MASC is in a 7-year-old boy, the youngest case so far reported. He referred to his primary care physician with a infra-auricular swelling, and it was diagnosed as lymphatic malformation he was referred to our institution for sclerotherapy. For Doppler and ultrasound magnetic resonance imaging, there was a distinct cystic lesion with a heterogeneous solid lesion inside. Minimally invasive treatment such as sclerotherapy was thought to be more desirable due to a pediatric case, the risk of postoperative facial paralysis and scar. However, even the successful treatment of cystic lesion with sclerotherapy, solid lesion of the tumor could be remained without pathological findings. Otolaryngologist also thought the importance of pathological diagnosis, and we finally chose surgical excision. The tumor was ultimately diagnosed as MASC considering histological and genetic findings. For child case, we tend to treat patient less invasively, and it might bring a risk of MASC being incorrectly treated nonsurgically such as with sclerotherapy. This could lead to tumor progression and wider radical excision at last. We believe that histological diagnosis should become the priority in similar cases of mixed solid and cystic tumors to avoid incorrect treatment, and we need to choose surgical excision by understanding the character of salivary gland tumor occurring in childhood.
涎腺乳腺样分泌癌(MASC)是一种新认识的肿瘤实体。我们报告一名儿童,最初被诊断为淋巴管瘤并转诊至我院接受硬化治疗,切除后发现肿瘤实际上是MASC。该例MASC发生在一名7岁男孩,是迄今为止报道的最年轻病例。他因耳下肿胀就诊于初级保健医生,被诊断为淋巴管畸形,随后转诊至我院接受硬化治疗。经多普勒超声和磁共振成像检查,发现一个明显的囊性病变,内部有一个不均匀的实性病变。由于是儿科病例,存在术后面瘫和瘢痕的风险,因此认为硬化治疗等微创治疗更为可取。然而,即使硬化治疗成功消除了囊性病变,肿瘤的实性病变仍可能残留且无病理诊断结果。耳鼻喉科医生也认识到病理诊断的重要性,最终我们选择了手术切除。综合组织学和遗传学检查结果,肿瘤最终被诊断为MASC。对于儿童病例,我们往往倾向于采用侵入性较小的治疗方法,这可能会带来将MASC误作为非手术治疗(如硬化治疗)的风险,进而导致肿瘤进展,最终需要进行更广泛的根治性切除。我们认为,在类似的实性和囊性混合肿瘤病例中,组织学诊断应成为首要考虑因素,以避免错误治疗,并且我们需要通过了解儿童涎腺肿瘤的特点来选择手术切除。