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乳腺原发性骨肉瘤:50例临床病理分析

Primary osteogenic sarcoma of the breast: a clinicopathologic analysis of 50 cases.

作者信息

Silver S A, Tavassoli F A

机构信息

Department of Gynecologic and Breast Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.

出版信息

Am J Surg Pathol. 1998 Aug;22(8):925-33. doi: 10.1097/00000478-199808000-00002.

DOI:10.1097/00000478-199808000-00002
PMID:9706972
Abstract

Extraskeletal osteosarcomas are rare. Few primary mammary osteosarcomas have been reported; many of these have been described in association with a biphasic tumor. Fifty pure osteosarcomas of the breast, diagnosed between 1957 and 1995, were reviewed after excluding those of biphasic origin. The absence of epithelial differentiation was confirmed using a panel of immunohistochemical markers in 32 cases and using ultrastructural evaluation in an additional four cases. Tumors occurred in 49 women and one man; age ranged from 27 to 89 years (median, 64.5 years). One patient received radiotherapy for ipsilateral breast carcinoma 9 years before presentation. Patients were treated by excisional biopsy (n = 13), tylectomy (n = 5) or mastectomy (n = 32). All axillary nodes, dissected in 20 patients, were free of tumor. One patient had extramammary spread at diagnosis. The neoplasms were 1.4 cm to 13.0 cm (mean, 4.6 cm), and 60% were grossly circumscribed. Tumors were classified as fibroblastic (n = 28), osteoclastic (n = 14), or osteoblastic (n = 8). Of 39 patients with available follow-up information, locally recurrent (n = 11) and metastatic (n = 15) disease developed in 23 (59%) at a mean of 10.5 and 14.5 months from diagnosis. Eight (73%) patients in whom local recurrence developed were treated with excisional biopsy or tylectomy; of these, seven had a positive margin. Sixteen (41%) patients died of disease at a mean of 17.1 months, within 20 months of onset of metastases, most commonly to the lung. Mammary osteosarcomas are aggressive tumors with a propensity for blood-borne rather than lymphatic spread. Total excision without axillary dissection is advised.

摘要

骨外骨肉瘤罕见。原发性乳腺骨肉瘤报道较少;其中许多与双相性肿瘤相关。对1957年至1995年间诊断的50例乳腺单纯骨肉瘤进行回顾,排除双相起源者。32例通过一组免疫组化标志物证实无上皮分化,另外4例通过超微结构评估证实。肿瘤发生于49名女性和1名男性;年龄范围为27至89岁(中位数为64.5岁)。1例患者在就诊前9年因同侧乳腺癌接受过放疗。患者接受了切除活检(n = 13)、乳房局部切除术(n = 5)或乳房切除术(n = 32)。20例患者接受了腋窝淋巴结清扫,所有腋窝淋巴结均无肿瘤。1例患者在诊断时已有乳腺外转移。肿瘤大小为1.4 cm至13.0 cm(平均4.6 cm),60%在大体上边界清晰。肿瘤分为纤维母细胞型(n = 28)、破骨细胞型(n = 14)或成骨细胞型(n = 8)。在有随访信息的39例患者中,23例(59%)出现局部复发(n = 11)和转移(n = 15),分别平均在诊断后10.5个月和14.5个月出现。8例(73%)出现局部复发的患者接受了切除活检或乳房局部切除术;其中7例切缘阳性。16例(41%)患者平均在17.1个月死于疾病,在转移发生后20个月内,最常见转移至肺。乳腺骨肉瘤是侵袭性肿瘤,倾向于血行转移而非淋巴转移。建议行完整切除且不进行腋窝清扫。

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