Sporadic cutaneous angiosarcomas: A proposal for risk stratification based on 69 cases
Sporadic cutaneous angiosarcomas: A proposal for risk stratification based on 69 cases
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DOI：
10.1097/pas.0b013e3180f633a3
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发表时间：
2008-01-01
影响因子：
5.6
通讯作者：
Weiss, Sharon W.
中科院分区：
文献类型：
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作者：
Deyrup, Andrea T.;McKenney, Jesse K.;Weiss, Sharon W.
血管肉瘤传统上被认为是高度级别的病变，其组织学特征和分级对预后没有影响，因此，它们被排除在美国癌症联合委员会分期系统之外。因此，我们分析了69个在会诊中看到的皮肤血管肉瘤，这些肿瘤与淋巴水肿或既往放射治疗无关，以确定是否可以结合组织学和临床参数来区分惰性和侵袭性转化。分析的临床特征包括患者的年龄、病变的位置、大小、深度和灶性；研究的组织学特征包括生长模式(血管形成与实质)、核级别(高与低)、坏死(有/无)、细胞类型(上皮样或梭形)、炎性浸润性程度(最小与明显)和有丝分裂率。病变发生在成人(21-94岁)的头/颈(49例)、四肢(15例)和躯干(5例)，男性(41例男性；女性28例)居多。肿瘤大小0.3~15 cm(平均3.1 cm)，累及乳头状(n=9)、网状(n=16)或真皮/皮下深层(n=30)。它们可以是主要的血管形成(n=28)到固体(大于50%固体，n=41)。大多数病灶为高(n=65)而不是低(n=4)核级别，核分裂活跃(0~99/10高倍视野)，偶见坏死(n=14)和上皮样改变(n=21)。在大多数病例中，炎性浸润物很少。所有患者都获得了随访信息。18例(26%)复发，15例(22%)转移到以下部位：肺(6例)、淋巴结(7例)、肝(2例)、骨(2例)和腮腺(1例)。47例患者死亡(30例死于疾病)，22例存活(范围16~158个月；平均65个月；中位数36个月)。疾病相关的5年存活率为48%。通过单变量分析，只有年龄较大、解剖部位、坏死和上皮样特征与死亡率增加相关。根据肿瘤的坏死和/或上皮样特征，将肿瘤分为低风险组(n=41)和高风险组(n=28)。多变量分析显示，高危人群(风险比4.07，P=0.0004)和年龄>70岁(风险比2.79，P=0.012)与死亡率增加相关，肿瘤深度(P=0.048)与局部复发风险相关。高危组的预后明显差于低危组，3年生存率分别为24%和77%。具有高危特征的患者无一存活5年。总之，我们报告了结合临床和组织学特征可以将血管肉瘤患者分为两个危险组，这两个危险组与临床病程的显著差异密切相关。这些特征的重要性似乎随着肿瘤大小的增加而减弱，可能在最大尺寸小于5厘米的肿瘤中最有用。
Angiosarcomas have traditionally been considered high-grade lesions for which histologic features and grading have played no role in prognostication and, consequently, they have been excluded from the American Joint Committee on Cancer staging system. We have, therefore, analyzed 69 cutaneous angiosarcomas seen in consultation and not associated with lymphedema or prior radiation therapy to determine if a combination of histologic and clinical parameters could be used to differentiate indolent from aggressive turnors. The clinical features analyzed included patient age, location, size, depth, and focality of the lesion; the histologic features studied included pattern of growth (vasoformative vs. solid), nuclear grade (high vs. low), necrosis (present/absent), cell type (epithelioid or spindled), extent of inflammatory infiltrate (minimal vs. marked), and mitotic rate. Lesions occurred on the head/neck (49), extremities (15), and trunk (5) of adults (21 to 94y) and predominated in males (41 males; 28 females). Tumors ranged in size from 0.3 to 15-cm (average 3.1 cm) and involved the papillary (n = 9), reticular (n = 16), or deep dermis/ subcutis (n = 30). They could be predominantly vasoformative (n = 28) to solid (greater than 50% solid, n = 41). Most lesions were of high (n 65) as opposed to low (n = 4) nuclear grade, were mitotically active (0 to 99/10 high power fields), and occasionally displayed necrosis (n = 14) and epithelioid features (n = 21). Inflammatory infiltrates were minimal in most cases. Follow-up information was obtained for all patients. Recurrences developed in 18 patients (26%) and metastasis in 15 (22%) to the following sites: lung (6), lymph node (7), liver (2), bone (2), and parotid gland (1). Forty-seven patients died (30 of disease) and 22 were alive at last known follow-up (range, 16 to 158 mo; mean, 65; median, 36). Five-year disease-specific survival was 48%. By univariate analysis only older age, anatomic site, necrosis, and epithelioid features correlated with increased mortality. Tumors were stratified into low (n = 41) or high (n = 28) risk groups based on necrosis and/or epithelioid features. By multivariable analysis, high-risk group (hazard ratio 4.07, P = 0.0004) and age > 70 (hazard ratio 2.79, P = 0.012) were associated with increased mortality, and tumor depth (P = 0.048) correlated with the risk of local recurrence. The high-risk group had a significantly worse prognosis than the low-risk group with 3-year survival of 24% and 77%, respectively. No patients with high-risk features survived 5 years. In conclusion, we report that a combination of clinical and histologic features allows stratification of angiosarcoma patients into 2 risk groups that are strongly associated with marked differences in clinical course. These features seem to diminish in importance with increased tumor size and are probably most useful in tumors less than 5cm in maximum dimension.