Clinical characteristics of 91 patients of otorhinolaryngology head and neck malignant tumors in children
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摘要: 目的 分析儿童耳鼻咽喉头颈部恶性肿瘤的临床特点、治疗方式及预后情况，以期提高该类疾病的诊治水平。方法 回顾分析2014—2020年在昆明市儿童医院和昆明医科大学第一附属医院住院治疗、最终病理确诊为头颈部恶性实体肿瘤的患儿91例，按照性别、年龄、部位、病理类型、治疗方法等进行统计分析。结果 91例患儿的主要临床表现以面颈部肿块为主，其他有鼻塞、吞咽不适、持续间断发热等。CT和MRI检查示肿瘤直径大小1.2 cm×2.0 cm~5.0 cm×12.0 cm，平均2.8 cm×3.2 cm，远处转移19例。最主要的组织来源是软组织(56例)和上皮组织(35例)。共6种病理类型，最常见的是肉瘤(41例)，其次分别为神经母细胞瘤(15例)、乳头状癌(14例)，鳞状细胞癌(10例)、黏液表皮样癌(8例)和腺癌(3例)。根据组织来源分类，对性别、病理类型进行统计分析，结果显示性别和病理类型的差异均有统计学意义(P＜0.01)。结论 儿童耳鼻咽喉头颈恶性肿瘤发病年龄、原发部位、组织来源和病理类型有其自身特性，应对其综合评估，多学科联合治疗。Abstract: Objective To analyze the clinical characteristics, treatment and prognosis of the otolaryngology head and neck malignant tumors in children, in order to improve the diagnosis and treatment of the diseases.Methods The patients of otorhinolaryngology head and neck malignant solid tumors under 14 years old hospitalized in Kunming Children's Hospital and the First Affiliated Hospital of Kunming Medical University from 2014 to 2020 were retrospectively analyzed. All cases were statistically analyzed according to gender, age, location, pathological type and treatment method.Results The main clinical manifestations of 91 children were mainly facial and neck masses, including nasal congestion, swallowing discomfort, and continuous intermittent fever. CT and MRI examination showed that the diameter of the tumor was 1.2 cm ×2.0 cm to 5.0 cm×12.0 cm, with a mean of 2.8 cm×3.2 cm, and 19 cases had distant metastasis. The main tissue sources were soft tissue (56 cases) and epithelial tissue (35 cases). There were 6 pathological types, the most common was sarcoma (41 cases), followed by neuroblastoma (15 cases), papillary carcinoma (14 cases), squamous cell carcinoma (10 cases), mucoepidermoid carcinoma (8 cases), and adenocarcinoma (3 cases). According to the classification of tissue origin, the statistical analysis of gender and pathological type showed statistically significant differences in both gender and pathological types(P < 0.01).Conclusion The age of onset, primary site, tissue origin and pathological type of otolaryngology head and neck malignancy in children have their own characteristics, which should be comprehensively evaluated and treated with multidisciplinary treatment.
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Key words:
- head and neck neoplasms /
- child /
- clinical characteristics
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图 6 横纹肌肉瘤 6a：瘤细胞成片、成巢分布，箭头所示瘤细胞偏位红染胞质；6b：瘤细胞Myogenin(+)；6c：瘤细胞MyoD1(+)； 图 7 髓系肉瘤 7a：瘤细胞弥漫分布，箭头所示瘤细胞核为圆形、卵圆形，核深染，胞质少，核圆形、不规则形或分叶状，可见核仁；7b：瘤细胞MPO(+)；7c：瘤细胞LCA(+)； 图 8 PNET 8a：肿瘤细胞呈片巢状，箭头所示核为圆形、卵圆形，核深染，胞质少；8b：肿瘤细胞CD99弥漫膜阳性(+)；8c：肿瘤细胞Fli-1(+)； 图 9 节细胞神经母细胞瘤 9a：不同阶段神经节细胞成巢分布，箭头所示多个核不成熟神经节细胞；9b：箭头所示不成熟的神经节细胞CD56(+)；9c：箭头所示趋于成熟的神经节细胞Syn(+)； 图 10 黏液表皮样癌 10a：瘤细胞呈团片巢状分布，局部腺样分布，细胞多为透亮、圆形、卵圆形，箭头所示核分裂；10b：瘤细胞CK8/18(+)；10c：瘤细胞CK7(+)。
表 1 儿童头颈部恶性肿瘤组织来源、性别及年龄分布
组织来源 病理类型 性别 年龄/岁 合计 男 女 0~3 4~6 7~12 13~14 间叶组织肿瘤 横纹肌肉瘤 15 10 10 9 4 2 25 尤文肉瘤 6 3 0 1 3 5 9 骨恶性肉瘤 1 1 0 0 0 2 2 髓系肉瘤 2 1 2 1 0 0 3 滑膜肉瘤 1 0 0 0 0 1 1 面部皮肤恶性肉瘤 0 1 1 0 0 0 1 胚胎组织肿瘤 神经母细胞瘤 6 9 6 6 2 1 15 上皮组织肿瘤 乳头状癌 0 14 0 0 7 7 14 鳞状细胞癌 6 4 0 0 7 3 10 黏液表皮样癌 4 4 0 0 1 7 8 腺癌 2 1 0 0 1 2 3 合计 43 48 19 17 25 30 91 
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表 2 儿童头颈部恶性肿瘤原发部位
例(%) 病理类型 耳部 咽喉部 头面部 鼻腔鼻窦 颈部 合计 肉瘤 2(2.2) 3(3.3) 10(11.0) 16(17.6) 10(11.0) 41(45.1) 神经母细胞瘤 0(0) 0(0) 1(1.1) 3(3.3) 11(12.1) 15(16.5) 乳头状癌 0(0) 0(0) 0(0) 0(0) 14(15.4) 14(15.4) 鳞状细胞癌 2(2.2) 5(5.5) 1(1.1) 1(1.1) 1(1.1) 10(11.0) 黏液表皮样癌 0(0) 2(2.2) 5(5.5) 0(0) 1(1.1) 8(8.8) 腺癌 1(1.1) 0(0) 1(1.1) 0(0) 1(1.1) 3(3.3) 合计 5(5.5) 10(11.0) 18(19.8) 20(22.0) 38(41.8) 91(100.0)
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表 3 91例患儿的治疗及预后情况
病理类型 例数 病程 症状 治疗 随访时间/月 预后 肉瘤 41 7 d~3个月 面颈部肿物20例，鼻塞伴涕血16例，鼻腔6例，张口呼吸伴打鼾3例 手术+化疗 8~44 存活27例，死亡12例，失访2例 神经母细胞瘤 15 1 d~2个月 面颈部肿物12例，鼻塞3例，其中3例有触痛，2例发热(38.2~39.0℃)，1例消瘦 手术+化疗 9~58 存活12例，死亡2例，失访1例 乳头状癌 14 1~12个月 颈部肿物14例，伴有声嘶3例 手术 10~95 均存活 鳞状细胞癌 10 1~2个月 鼻塞、涕血6例，耳道流脓血2例，面部肿物1例，1例颈部肿物为鳞状细胞癌复发转移瘤，其中3例伴有触痛 手术+放化疗 8~93 存活7例，死亡2例，失访1例 黏液表皮样癌 8 1~6个月 面颈部无痛性肿物6例，呼吸不畅伴打鼾2例 手术 10~35 均存活 腺癌 3 2~12个月 均表现为面颈部无痛性肿物 手术 9~45 均存活
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