Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
Division of Hematology, Cancer and Blood Disease Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
Pediatr Blood Cancer. 2018 Jul;65(7):e27051. doi: 10.1002/pbc.27051. Epub 2018 Apr 6.
Malignant soft tissue tumors are rare and difficult to diagnose in children. These can initially be misdiagnosed as benign vascular anomalies. Management of these two conditions differs drastically and delay in diagnosis may impact overall survival.
To predict qualities that may increase the index of suspicion for malignancy in patients presenting with lesions initially considered vascular anomalies.
A retrospective review at a quaternary hemangioma and vascular malformation center of all patients who presented between 2008 and 2016 with an initial diagnosis of a benign vascular malformation, which on further work-up was noted to be a malignancy. Demographics, clinical presentation, and laboratory and radiologic studies were analyzed.
Eleven patients were identified; the median age at presentation was 2 months (0-24years). Ten out of 11 lesions had rapid growth, which prompted biopsy. Pain was an inconsistent finding (36%). Tumor markers were positive in only one case. Median follow-up was 3 years (range 6 months to 8 years); seven patients have no evidence of disease, two patients are under treatment for progression or relapse of disease, and two patients have died.
Although malignant vascular tumors are rare, a clear index of suspicion needs to be maintained particularly with rapid growth or increasing symptoms. Differentiation of malignant tumor from benign lesions relies on the comprehensive evaluation of clinical manifestations, evolution of the lesion, and abnormal clinical behavior, by an experienced multidisciplinary vascular malformation team. There should be a low threshold for biopsy of unclear vascular lesions.
儿童期的软组织恶性肿瘤罕见且难以诊断。这些肿瘤最初可能被误诊为良性血管异常。这两种疾病的治疗方法截然不同，如果诊断延迟可能会影响整体生存率。
预测在最初被认为是血管异常的病变患者中，可能增加恶性肿瘤怀疑指数的特征。
在一家四级血管性肿瘤和血管畸形中心，对 2008 年至 2016 年间所有以良性血管畸形初步诊断、进一步检查后发现为恶性肿瘤的患者进行回顾性分析。分析患者的人口统计学、临床表现、实验室和影像学研究。
共确定了 11 名患者；发病时的中位年龄为 2 个月（0-24 岁）。11 个病灶中有 10 个迅速生长，促使进行了活检。疼痛是一种不一致的发现（36%）。仅 1 例肿瘤标志物阳性。中位随访时间为 3 年（6 个月至 8 年）；7 例患者无疾病证据，2 例患者因疾病进展或复发而正在接受治疗，2 例患者死亡。
尽管恶性血管肿瘤罕见，但特别是对于快速生长或症状加重的患者，需要保持明确的怀疑指数。通过有经验的多学科血管畸形团队，对临床表现、病变演变和异常临床行为进行全面评估，有助于将恶性肿瘤与良性病变区分开来。对于不明原因的血管性病变，活检的门槛应该较低。
