Myoepithelial tumors of soft tissue - A clinicopathologic and immunohistochemical study of 101 cases with evaluation of prognostic parameters
Myoepithelial tumors of soft tissue - A clinicopathologic and immunohistochemical study of 101 cases with evaluation of prognostic parameters
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DOI：
10.1097/00000478-200309000-00001
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发表时间：
2003-09-01
影响因子：
5.6
通讯作者：
Fletcher, CDM
中科院分区：
文献类型：
--
作者：
Hornick, JL;Fletcher, CDM
肌上皮瘤和混合瘤只是最近才认识到主要发生在软组织，只有少数病例已被描述。为了进一步描述这些肿瘤并评估预后参数，从作者的咨询文件中检索了101例软组织肌上皮肿瘤。重新检查苏木精和伊红切片，进行免疫组织化学，并从转诊医生处获得临床详细信息。53例患者为男性，48例为女性（平均年龄38岁;范围3-83岁）。肿瘤大小范围为0.7 - 20 cm（平均4.7 cm）。大多数肿瘤发生在四肢和肢带：下肢41例，上肢35例，头颈部15例，躯干10例。54个肿瘤位于皮下组织，37个位于深部软组织（10个深度不详）。大多数病例大体上界限清楚; 43例在显微镜下显示浸润边缘。组织学上，大多数肿瘤呈分叶状，由上皮样、卵圆形或梭形细胞的索或巢组成，具有网状结构和软骨粘液样或胶原/透明化基质。8例主要表现为梭形或浆细胞样细胞的实体增殖; 17例表现为导管分化（混合瘤）。其中6例有软骨，6例有骨，4例两者都有。每10个高倍视野中有丝分裂数为0 - 68个（平均4.7个）。具有良性细胞形态学或轻度细胞非典型性（低度）的肿瘤被归类为肌上皮瘤或混合瘤，而具有中度至重度非典型性（高度）的肿瘤被归类为肌上皮癌（具有囊泡或粗染色质的上皮样或梭形细胞，突出，通常是大核仁，或核多形性）或恶性混合瘤（细胞学上恶性的软骨或骨）。肌上皮瘤或混合瘤61例，肌上皮癌或恶性混合瘤40例。通过免疫组化，所有可用材料的病例均对上皮标记物呈反应性（角蛋白和/或上皮膜抗原）：90/97（93%）表达角蛋白（最常见的是AE 1/AE 3或PAN-K），97种中的84种（87%）S-100蛋白，51种中的44种（86%）钙调蛋白，83种中的52种（63%）上皮膜抗原，胶质细胞酸性蛋白40/87（46%），平滑肌肌动蛋白27/75（36%），p63 15/66（23%），结蛋白7/51（14%）。对64例患者进行了随访。在33例良性或低级别细胞学检查的病例中（平均随访36个月;范围4-168个月），6例局部复发（18%），无转移。没有与复发相关的临床或组织学特征。在31例细胞学恶性肿瘤患者中（平均随访50个月，范围4-252个月），13例局部复发（42%），10例转移（32%），迄今为止，4例患者死于转移瘤。这项研究扩大了软组织肌上皮肿瘤的范围，包括肌上皮癌和恶性混合瘤，这些肿瘤追求积极的临床过程。虽然大多数形态学上良性或低度恶性的软组织肌上皮肿瘤表现为良性，但局部复发的风险约为20%。
Myoepitheliomas and mixed tumors were only recently recognized to occur primarily in soft tissue, and only small case numbers have been described. To characterize these tumors further and to evaluate prognostic parameters, 101 myoepithelial tumors of soft tissue were retrieved from the authors' consult files. Hematoxylin and eosin sections were reexamined, immunohistochemistry was performed, and clinical details were obtained from referring physicians. Fifty-three patients were male and 48 female (mean age 38 years; range 3-83 years). Tumor size ranged from 0.7 to 20 cm (mean 4.7 cm). Most tumors arose in the extremities and limb girdles: 41 in the lower limbs, 35 in the upper limbs, 15 in the head and neck, and 10 in the trunk. Fifty-four tumors were situated in subcutis and 37 in deep soft tissue (depth unstated in 10). Most cases were grossly well circumscribed; 43 showed microscopically infiltrative margins. Histologically, most tumors were lobulated, composed of cords or nests of epithelioid, ovoid, or spindled cells with a variably reticular architecture and a chondromyxoid or collagenous/hyalinized stroma. Eight cases showed a predominantly solid proliferation of spindled or plasmacytoid cells; 17 demonstrated ductular differentiation (mixed tumors). Cartilage was present in 6 cases, 6 contained bone, and 4 others contained both. Mitoses ranged from 0 to 68 per 10 high power fields (mean 4.7 per 10 high power fields). Tumors with benign cytomorphology or mild cytologic atypia (low-grade) were classified as myoepithelioma or mixed tumor, whereas tumors with moderate to severe atypia (high-grade) were classified as myoepithelial carcinoma (epithelioid or spindled cells with vesicular or coarse chromatin, prominent, often large nucleoli, or nuclear pleomorphism) or malignant mixed tumor (cytologically malignant cartilage or bone). Sixty-one cases were myoepitheliomas or mixed tumors, and 40 were myoepithelial carcinomas or malignant mixed tumors. By immunohistochemistry, all cases with available material were reactive for epithelial markers (keratins and/or epithelial membrane antigen): 90 of 97 (93%) expressed keratins (most often AE1/AE3 or PAN-K), 84 of 97 (87%) S-100 protein, 44 of 51 (86%) calponin, 52 of 83 (63%) epithelial membrane antigen, 40 of 87 (46%) glial fibrillary acidic protein, 27 of 75 (36%) smooth muscle actin, 15 of 66 (23%) p63, and 7 of 51 (14%) desmin. Follow-up was available for 64 patients. Among 33 cases with benign or low-grade cytology (mean follow-up 36 months; range 4-168 months), 6 recurred locally (18%) and none metastasized. No clinical or histologic features correlated with recurrence. Among 31 cytologically malignant cases (mean follow-up 50 months; range 4-252 months), 13 recurred locally (42%) and 10 metastasized (32%); so far, 4 patients have died of metastatic tumor. This study expands the spectrum of myoepithelial tumors of soft tissue to include myoepithelial carcinomas and malignant mixed tumors, which pursue an aggressive clinical course. Although the majority of morphologically benign or low-grade myoepithelial neoplasms of soft tissue behave in a benign fashion, there is an approximate 20% risk for local recurrence.