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[乳腺血管肉瘤。附8例报告及文献复习]

[Angiosarcoma of the breast. Apropos of 8 cases and review of the literature].

作者信息

Molitor J L, Llombart A, Guinebretière J M, Zemoura L, Spielmann M, Contesso G, de Vathaire F, Zelek L, Kaylitalire L, Le Chevalier T, Genin J

机构信息

Département de médecine, Institut Gustave-Roussy, rue Camille-Desmoulins, Villejuif, France.

出版信息

Bull Cancer. 1997 Feb;84(2):206-11.

PMID:9180846
Abstract

The 8 cases of primary breast angiosarcoma which were diagnosed, treated and had their follow-up at the Gustave-Roussy Institute between 1954 and 1995 are reported. The age at presentation ranged from 32 to 68 years. In 4 patients the vascular nature of their mammary lesion was conspicuous by a violaceous discolorations of the overlying skin. No patient had enlarged ipsilateral axillary lymph nodes. One patient had metastases. In 2 out of 5 patients who had a partial surgical or fine-needle biopsy before treatment, the diagnosis was missed, and adequate treatment was unduly delayed. The tumor most often presents as an ill defined area made of dense endothelium-lined papillae. A remarkable picture of "soap bubbles" has been identified in 4 cases. The "meshes" of fatty areas appear to be reinforced as they are infiltrated by tumor cells. This appearance may be specific of mammary angiosarcoma. By the French Cancer Centers' grading system for soft tissue sarcomas in general, grade is III in 3 tumors, II in 2 tumors, I in 3 tumors. Five tumors were treated by total mastectomy only. In 3 cases a total mastectomy was followed by radiation therapy to the chest wall. At diagnosis a chemotherapy was administered only to the patient who had metastases. Median disease-free survival was 9 months. Median overall survival was 13 months. From a review of the literature a simple mastectomy appears to be necessary as well as enough for local treatment. Patients with a grade III angiosarcoma of the breast should be included into a therapeutical trial of adjuvant chemotherapy for soft tissue sarcomas in general.

摘要

本文报告了1954年至1995年间在古斯塔夫 - 鲁西研究所诊断、治疗并随访的8例原发性乳腺血管肉瘤。患者就诊时年龄在32岁至68岁之间。4例患者乳腺病变的血管性质因皮肤出现紫蓝色变色而明显。所有患者同侧腋窝淋巴结均未肿大。1例患者出现转移。5例患者中有2例在治疗前接受了部分手术或细针穿刺活检,但诊断失误,导致适当治疗被过度延迟。肿瘤通常表现为界限不清的区域,由密集的内皮细胞衬里乳头组成。4例患者出现了典型的“肥皂泡”样表现。脂肪区域的“网眼”在被肿瘤细胞浸润时似乎得到强化。这种表现可能是乳腺血管肉瘤所特有的。按照法国癌症中心对一般软组织肉瘤的分级系统,3例肿瘤为Ⅲ级,2例为Ⅱ级,3例为Ⅰ级。5例肿瘤仅接受了全乳切除术。3例患者在全乳切除术后接受了胸壁放疗。诊断时仅对出现转移的患者进行了化疗。无病生存期的中位数为9个月。总生存期的中位数为13个月。通过文献回顾发现,单纯乳房切除术似乎对于局部治疗既必要又足够。乳腺Ⅲ级血管肉瘤患者应纳入一般软组织肉瘤辅助化疗的治疗试验。

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