| 摘要: |
| 目的 根据国际血管瘤和脉管畸形研究学会（ISSVA）标准将肌内血管瘤患者分为血管瘤和血管畸形，比较不同类型间的临床及病理特征差异。方法 收集新疆医科大学第一附属医院2013年10月至2021年5月间依据WHO骨与软组织肿瘤标准诊断为肌内血管瘤的病例资料，根据ISSVA标准重新分类为血管瘤及血管畸形，对比分析两者在临床及病理特征方面的差异。结果 共纳入依据WHO骨与软组织肿瘤标准诊断为肌内血管瘤的患者40例，根据ISSVA标准可进一步分类为血管畸形36例和血管瘤4例。血管瘤患者以女性为主（男1例、女3例），好发于头颈部（4例均发生于面部）。血管畸形患者以静脉畸形为主（22例静脉畸形、1例动静脉畸形、13例混合型血管畸形），好发于四肢，尤以下肢为多见（19例发生于下肢，10例发生于上肢，4例发生于躯干，3例发生于面部）。从镜下特点来看，血管瘤镜下以血管增殖为特点，血管内皮细胞肿胀，可见核分裂象，血管增生呈分叶状分布。血管畸形镜下特点根据类型不同而存在差异，其中静脉畸形以薄壁、扩张静脉为特征，多伴血栓、静脉石；动静脉畸形血管壁厚薄不一，间质纤维、平滑肌增生；淋巴管畸形内淋巴管管腔大小不一，间质见淋巴细胞聚集灶。随访9个月至8年，血管瘤患者均无复发，血管畸形患者中有8例复发（复发率为22.2%）。结论 建议结合ISSVA标准对肌内血管瘤重新分类，新分类的血管瘤和血管畸形在发病部位、预后及病理形态方面均存在明显差异。 |
| 关键词: 疾病分类 肌内血管病变 肌内血管瘤 血管畸形 |
| DOI：10.16781/j.CN31-2187/R.20220024 |
| 投稿时间：2022-01-09修订日期：2022-07-01 |
| 基金项目: |
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| Clinicopathological analysis of intramuscular hemangioma reclassified by ISSVA criteria |
| MA Qian,WU Ya-shan,LIU Ting,HUANG Jing,LI Qiao-xin* |
(Department of Pathology, The First Affiliated Hospital of Xinjiang Medical University, Urumqi 830054, Xinjiang Uygur Autonomous Region, China
*Corresponding author) |
| Abstract: |
| Objective To reclassify intramuscular hemangioma patients with the International Society for the Study of Vascular Anomalies (ISSVA) criteria, and compare the clinicopathological differences between different types. Methods Intramuscular hemangioma cases diagnosed using the World Health Organization (WHO) classification of soft tissue and bone tumors were collected from Oct. 2013 to May 2021 in The First Affiliated Hospital of Xinjiang Medical University and re-classified as hemangioma or vascular malformations with the ISSVA criteria. The clinicopathological characteristics were compared between the 2 groups. Results A total of 40 intramuscular vascular cases diagnosed using the WHO criteria were enrolled, including 4 hemangioma cases and 36 vascular malformation cases reclassified by the ISSVA criteria. The hemangioma cases were mainly female (1 male and 3 females) and were mainly in the head and neck region (4 cases all in the face). The patients with vascular malformations were mainly venous malformations (22 venous malformations, 1 arteriovenous malformation, and 13 mixed vascular malformations). The lesions were more commonly seen in the extremities, especially in the lower limbs (19 in the lower limbs, 10 in the upper limbs, 4 in the trunk, and 3 in the face). The pathological morphology of hemangioma was characterized by vascular proliferation with lobed distribution under the microscope, in which vascular endothelial cells were plump and mitosis could be seen. While the microscopic morphological features were correlated with the specific subtype of vascular malformations, venous malformations were characterized by thin-walled and dilated veins, occasionally accompanied by thrombosis and phleboliths. The thickness of blood vessels varied for arteriovenous malformations, with fibers and smooth muscles proliferating into surrounding stroma. For lymphatic malformations, the diameters of lymphatics differed and foci of lymphocytic cells could be seen in the stroma. There was no recurrence in hemangioma patients, and the recurrence rate was 22.2% (8/36) for vascular malformation cases during the follow-up from 9 months to 8 years. Conclusion It is suggested to reclassify intramuscular hemangiomas with the ISSVA criteria. The new classification (hemangioma and vascular malformation) has significant differences in lesion location, prognosis and pathological morphology. |
| Key words: classification of disease intramuscular vascular lesion intramuscular hemangioma vascular malformation |
