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一种罕见的乳腺肿瘤:实性神经内分泌癌。

A rare breast tumor: solid neuroendocrine carcinoma.

作者信息

Sanguinetti Alessandro, Santoprete Stefano, Lucchini Roberta, Triola Roberta, Loreti Fabio, Avenia Nicola

出版信息

Ann Ital Chir. 2013 Jan-Feb;84(1):81-5.

Abstract

BACKGROUND

Solid neuroendocrine carcinoma of breast (NECB) is extremely rare. In this paper, we present a case of inflammatory primary solid neuroendocrine carcinoma of breast managed by surgery and chemotherapy and a brief review of the epidemiology, clinical features, diagnosis, pathologic features, treatment, and prognosis of solid NECB.

METHODS

A 63-year-old woman was admitted in our institution with inflammatory primary solid neuroendocrine carcinoma of breast. A bulky mass of 6,5 cm tumor was located in the upper-outer and intern quadrant of her right breast. The patient underwent neo-adjuvant chemotherapy, and subsequent radical mastectomy with axillary lymph node dissection. Microscopically, the tumor was classified as solid cohesive, the tumor cells were positive for neuroendocrine markers chromogranin A and synaptophysin. 19 lymph nodes of 27 were metastatic.

RESULTS

Local recurrence and metastatic progression was noted only one month after the surgery, the patient was managed by chemotherapy and hormone-therapy. She is still alive, 24 months after diagnosis.

CONCLUSIONS

Solid neuroendocrine carcinoma is a subtype of mammary carcinoma with several distinctive features. Because of the rarity of this disease, there is no standard treatment, they are characterized by a higher propensity for local and distant recurrence, This case reinforces the importance to explore the novels therapeutics regimen and one of ways to explore is the use of VP16-cisplatine as treatment as it was partially efficacy for this kind of tumor.

摘要

背景

乳腺实性神经内分泌癌(NECB)极为罕见。在本文中,我们报告一例经手术和化疗治疗的原发性炎性乳腺实性神经内分泌癌病例,并简要回顾实性NECB的流行病学、临床特征、诊断、病理特征、治疗及预后。

方法

一名63岁女性因原发性炎性乳腺实性神经内分泌癌入住我院。其右乳外上象限及内象限有一个6.5厘米的巨大肿块。患者接受了新辅助化疗,随后进行了根治性乳房切除术及腋窝淋巴结清扫术。显微镜下,肿瘤被分类为实性凝聚型,肿瘤细胞神经内分泌标志物嗜铬粒蛋白A和突触素呈阳性。27个淋巴结中有19个发生转移。

结果

术后仅1个月就出现局部复发和转移进展,患者接受了化疗和激素治疗。诊断后24个月,她仍然存活。

结论

实性神经内分泌癌是乳腺癌的一种亚型,具有一些独特特征。由于这种疾病罕见,尚无标准治疗方法,其特点是局部和远处复发倾向较高。该病例强化了探索新治疗方案的重要性,探索途径之一是使用VP16 - 顺铂进行治疗,因为它对这类肿瘤有部分疗效。

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