Angiosarcoma of soft tissue - A study of 80 cases
Angiosarcoma of soft tissue - A study of 80 cases
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DOI：
10.1097/00000478-199806000-00005
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发表时间：
1998-06-01
影响因子：
5.6
通讯作者：
Kindblom, LG
中科院分区：
文献类型：
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作者：
Meis-Kindblom, JM;Kindblom, LG
软组织血管肉瘤的临床病理学、免疫组织化学和超微结构特征尚不明确。报道了 80 例累及深部皮下组织、骨骼肌、腹膜后、肠系膜和纵隔的血管肉瘤。病变发生在50例男性和30例女性患者中，年龄5-97岁；发病高峰期是七十岁。其中 20 例病例记录有多种相关病症，包括其他肿瘤病史（部分接受过放射治疗）、合成血管移植物、遗传性疾病以及既往外伤或手术史。血管肉瘤好发于四肢（n = 43例）、躯干（n = 28例）和头颈部（n = 9例）区域，其中大腿和腹膜后是最常见的部位。它们通常表现为持续数周的增大、疼痛性肿块，偶尔与急性出血、贫血或凝血病有关。肿瘤直径为 1-15 厘米（中位数 5 厘米），并且经常出血且呈多结节状。病例内部和病例之间存在广泛的形态学谱，包括类似于海绵状血管瘤和毛细血管瘤、Dabska肿瘤、梭形细胞和上皮样血管内皮瘤、各种梭形细胞肉瘤或癌的区域。从组织学角度来看，上皮样血管肉瘤是最常见的模式。 70%的病例有上皮样细胞，呈巢状、簇状、乳头状和张开的血管通道排列。在一些病例中，出血往往会掩盖诊断，并且常常与乳头状内皮增生样区域相关。所有 42 个病例都进行了免疫组织化学染色，至少对因子 VIII 相关抗原进行局部染色，几乎所有病例都对波形蛋白进行了强烈染色，从而增强了内皮细胞和血管腔的形成。 74% 的病例检测到 CD34 抗原，72% 检测到 BNH9，35% 检测到细胞角蛋白。未检测到上皮膜抗原、S-100蛋白和HMB45。百分之五十五的肿瘤具有层粘连蛋白的胞质内聚集物。 α-平滑肌肌动蛋白的免疫染色显示多种肿瘤中存在显着的粒细胞成分（24%）。带有 MIB1 的 Ki67 免疫染色显示 72% 的病例具有高增殖活性（大于或等于 10%）。 20%的病例观察到p53免疫反应性（>20%核染色）。对12例低分化区域进行的超微结构研究显示，细胞群通常为上皮样，被基底层包围，与周细胞密切相关，以及细胞间和细胞内腔，有或没有红细胞。还注意到丰富的中间丝轮、偶尔的张力丝状结构和胞饮囊泡。与其他人的发现相反，没有发现韦贝尔-帕拉德尸体。对 49 例 (61%) 病例的随访显示，53% 的患者在中位间隔 11 个月时死于疾病，而 31% 的患者在中位间隔 46 个月时没有疾病证据。其余患者要么患病存活（14%），要么存活但疾病状态未知（2%）。 20%的病例出现局部复发，49%的病例出现远处转移，最常见的是肺部转移，其次是淋巴结、软组织、骨、肝脏和其他部位。这些结果表明软组织血管肉瘤是一种高级肉瘤。患者年龄较大、肿瘤位于腹膜后、肿瘤尺寸较大以及在大于或等于 10% 的肿瘤细胞群中检测到 MIB1 均与较差的预后相关。
The clinicopathologic, immunohistochemical, and ultrastructural features of soft tissue angiosarcomas are not well defined. Eighty cases of angiosarcoma that involved the deep subcutis, skeletal muscle, retroperitoneum, mesentery, and mediastinum are reported. The lesions occurred in 50 male and 30 female patients who were 5-97 years of age; the peak incidence was in the seventh decade of life. A variety of associated conditions were documented in 20 of these cases, including a history of other neoplasms (some irradiated), synthetic vessel grafts, heritable conditions, and prior trauma or surgery. The angiosarcomas occurred in the extremities (n = 43 cases), trunk (n = 28), and the head and neck (n = 9) regions, with the thigh and the retroperitoneum being the most common sites. They often were characterized as enlarging, painful masses of several weeks' duration and were occasionally associated with acute hemorrhage, anemia, or a coagulopathy. The tumors measured 1-15 cm in diameter (median 5 cm) and frequently were hemorrhagic and multinodular. There was a wide morphologic spectrum within and between cases, including areas similar to cavernous and capillary hemangioma, Dabska tumor, spindle cell and epithelioid hemangioendathelioma, various spindle cell sarcomas, or carcinoma. Histologically, epithelioid angiosarcoma was the most frequently observed pattern; 70% of cases had epithelioid cells that were arranged in nests, clusters, papillae, and gaping vascular channels. Hemorrhage tended to obscure the diagnosis in several cases and often was associated with papillary endothelial hyperplasia-like areas. All 42 cases studied immunohistochemically stained at least focally for Factor VIII-related antigen, and nearly all stained strongly for vimentin, which accentuated the endothelial cells and vessel lumen formation. CD34 antigen was detected in 74% of cases, BNH9 in 72%, and cytokeratins in 35%. Epithelial membrane antigen, S-100 protein, and HMB45 were not detected. Fifty-five percent of the tumors had intracytoplasmic aggregates of laminin. Immunostains for alpha-smooth muscle actin demonstrated a prominent peticytic component in several tumors (24%). Ki67 immunostains with MIB1 indicated high proliferative activity (greater-than-or-equal-to 10%) in 72% of cases. p53 immunoreactivity (>20% nuclear staining) was observed in 20% of cases.Ultrastructural studies performed on poorly differentiated areas of 12 cases showed groups of cells, which were frequently epithelioid, surrounded by basal lamina, and closely associated with pericytes, along with intercellular and intracellular lumina with or without red blood cells. Whorls of abundant intermediate filaments, occasional tonofilamentlike structures, and pinocytotic vesicles also were noted. In contrast to the findings of others, Weibel-Palade bodies were not seen. Follow-up in 49 cases (61%) showed that 53% of patients were dead of disease at a median interval of 11 months, whereas 31% had no evidence of disease at a median interval of 46 months. The remaining patients were either alive with disease (14%) or alive but disease status was unknown (2%). There were local recurrences in 20% of cases and distant metastases in 49%, most frequently to the lungs, followed by the lymph nodes, soft tissues, bone, liver, and other sites. These results indicate that angiosarcoma of soft tissue is a high-grade sarcoma. Older patient age, tumor location in the retroperitoneum, and larger tumor size as well as detection of MIB1 in greater-than-or-equal-to 10% of the tumor cell population were all associated with a poorer prognosis.