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[乳腺恶性转移性叶状囊肉瘤。乌尔姆罕见肿瘤收藏:VI]

[Malignant metastasizing cystosarcoma phylloides of the breast. Ulm collection of rare tumors: VI].

作者信息

Baczako K, Fischer H

出版信息

Onkologie. 1984 Dec;7(6):382-4. doi: 10.1159/000215487.

Abstract

Malignant cystosarcoma phylloides represents the malignant form of mammary fibroadenoma. In spite of atypical epithelial components, the biological behaviour of the tumour depends on malignant transformation of the dominant mesenchymal stromal tissue in the tumour. The histological appearance, often extremely variable, usually corresponds to a fibrosarcoma. Lipoblastic, chondroid and myxoid structures or areas of ossification may be present, although a completely dedifferentiated cystosarcoma is rare. The epithelial structures which are typically canalicular may reveal hyperplastic and dysplastic changes as well as an apocrine change and occasionally squamous metaplasia. Besides aggressive local infiltration, hematogenous metastasis of the skeletal system and lungs is common. Therapy is dependent on the extent of local tumour growth as well as on the degree of metastasis and usually takes the form of radical mastectomy with lymphadenectomy and postoperative radiotherapy. Local recurrence is found in over 50% of the cases and the 5-year survival rate is in the order of 70%.

摘要

恶性叶状囊肉瘤是乳腺纤维腺瘤的恶性形式。尽管存在非典型上皮成分,但肿瘤的生物学行为取决于肿瘤中占主导地位的间充质基质组织的恶性转化。其组织学表现通常变化极大,通常与纤维肉瘤相符。可能存在脂肪母细胞、软骨样和黏液样结构或骨化区域,不过完全去分化的叶状囊肉瘤很少见。典型的小管状上皮结构可能会出现增生、发育异常改变以及大汗腺化生,偶尔还会有鳞状化生。除了局部侵袭性生长外,骨骼系统和肺部的血行转移也很常见。治疗取决于局部肿瘤生长的程度以及转移程度,通常采取根治性乳房切除术加淋巴结清扫术以及术后放疗的形式。超过50%的病例会出现局部复发,5年生存率约为70%。

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