Atypical and malignant glomus tumors - Analysis of 52 cases, with a proposal for the reclassification of glomus tumors
Atypical and malignant glomus tumors - Analysis of 52 cases, with a proposal for the reclassification of glomus tumors
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DOI：
10.1097/00000478-200101000-00001
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发表时间：
2001-01-01
影响因子：
5.6
通讯作者：
Weiss, SW
中科院分区：
文献类型：
--
作者：
Folpe, AL;Fanburg-Smith, JC;Weiss, SW
偶尔的球囊瘤表现出不寻常的特征，如体积大、位置深、浸润性生长、有丝分裂活性、核多形性和坏死。虽然有少数疑似恶性的血管球瘤被描述过，但血管球瘤恶性的组织学标准从未被详细阐述过。作者研究了52例不寻常的血管球瘤（从他们的咨询文件中检索），这些肿瘤先前因核异型性、浸润性生长或有丝分裂活性而被诊断为“非典型”或“恶性”。他们评估了小球瘤的大小、深度、生长模式、细胞度、核分级、每50倍视场（HPF）有丝分裂图像的数量、非典型有丝分裂图像、血管间隙受损伤和坏死来确定恶性肿瘤的标准。计算估计相对危险度，采用Fisher精确检验进行统计分析。女性27例，男性25例，年龄8 ~ 83岁，中位年龄43岁。谣言测量为0.2至12厘米（中位尺寸为2厘米），主要发生在四肢，在浅表（n = 35）和深层（n = 17）软组织。不典型的特征通常在中心可见，边缘呈良性的血管球瘤。随访资料（n = 35例，5个月-23年，平均5.5年）显示7例复发，8例转移，7例疾病死亡。深部肿瘤（p = 0.005）、大于2 cm的肿瘤（p = 0.004）和非典型有丝分裂的肿瘤（p = 0.004）的5年累积转移风险显著增加。有丝分裂活性大于5个/50 HPF、高细胞性、存在坏死、中高核分级接近但未达到显著性。单纯高核分级、浸润性生长和血管间隙累及与转移无关。作者提出了以下分类方案和标准。恶性血管球瘤：肿瘤位于深部，大小大于2厘米，或非典型有丝分裂象，或中至高核分级，大于或等于5个有丝分裂象/50 HPF。交变球囊瘤：无其他恶性特征的高核级肿瘤。恶性潜能不确定的血管球瘤：缺乏恶性血管球瘤或共生性血管球瘤的标准，但有丝分裂活性高且仅位于浅表，或仅大尺寸，或仅位于深部的肿瘤。血管瘤病：以弥漫性血管瘤病和血管瘤细胞过多为组织学特征的肿瘤。根据这种分类方案，38%的肿瘤符合恶性肿瘤的诊断标准。相反，转移性疾病未见于任何被归类为共生性血管球瘤、恶性潜能不确定的血管球瘤或血管瘤病的标本。
Occasional glomus tumors display unusual features, such as large size, deep location, infiltrative growth, mitotic activity, nuclear pleomorphism, and necrosis. Although a small number of purportedly malignant glomus tumors have been described, histologic criteria for malignancy in glomus tumors have never been elaborated. The authors studied 52 unusual glomus tumors (retrieved from their consultation files) previously diagnosed as "atypical" or "malignant" by virtue of nuclear atypia, infiltrative growth, or mitotic activity. They evaluated size, depth, growth pattern, cellularity, nuclear grade, number of mitotic figures per 50 high-power fields (HPF), atypical mitotic figures, vascular space involvement, and necrosis to define criteria for malignancy in glomus tumors. Estimated relative risk was calculated and the Fisher exact test was used for statistical analysis. The 27 female patients and the 25 male patients ranged in age from 8 to 83 years (median age, 43 years). The rumors measured from 0.2 to 12 cm (median size, 2 cm) and occurred predominantly in the extremities, in both the superficial (n = 35) and deep (n = 17) soft tissues. Atypical features were usually observed centrally with a rim of benign-appearing glomus tumor. Follow-up information (n = 35; range, 5 months-23 years; mean 5.5 years) showed seven recurrences, eight metastases, and seven deaths from disease. Five-year cumulative metastatic risk increased significantly for tumors with a deep location (p = 0.005), with a size of more than 2 cm (p = 0.004), and with atypical mitotic figures (p = 0.004). Mitotic activity of more than 5 mitoses/50 HPF, high cellularity, the presence of necrosis, and moderate to high nuclear grade approached but did not reach significance. High nuclear grade alone, infiltrative growth, and vascular space involvement were not associated with metastasis. The authors propose the following classification scheme and criteria. Malignant glomus tumor: Tumors with a deep location and a size of more than 2 cm. or atypical mitotic figures, or moderate to high nuclear grade and greater than or equal to5 mitotic figures/50 HPF. Symplastic glomus tumor: Tumors with high nuclear grade in the absence of any other malignant feature. Glomus tumor of uncertain malignant potential: Tumors that lack criteria for malignant glomus tumor or symplastic glomus tumor but have high mitotic activity and superficial location only, or large size only, or deep location only. Glomangiomatosis: Tumors with histologic features of diffuse angiomatosis and excess glomus cells. Using this classification scheme, metastasis was observed in 38% of tumors fulfilling the criteria for malignancy. In contrast, metastatic disease was not seen in any specimen classified as symplastic glomus tumor, glomus tumor of uncertain malignant potential, or glomangiomatosis.