炎性乳腺癌诊疗现状与进展

Current status and advances in the diagnosis and treatment of inflammatory breast cancer

摘要:

炎性乳腺癌（IBC）是一种罕见但侵袭性极强的乳腺癌亚型，临床表现进展迅速，预后极差。尽管其发病率仅占全部乳腺癌的2%~4%，但其相关病死率却高达8%~10%。IBC的病因复杂，涉及遗传、激素、环境及社会经济等多重因素，其病理特征以真皮内癌栓为主，分子分型以HER2阳性和三阴性为主，提示较强的侵袭性。IBC的诊断主要依赖临床表现与组织病理学，影像学检查如MRI和PET/CT有助于评估病情范围和转移灶。由于IBC初诊多为局部晚期或转移期，目前尚无特异性治疗方案，临床上多参照非IBC的治疗策略，采取以系统治疗为核心的多学科综合治疗模式。HER2阳性IBC可采用化疗联合双靶向治疗，三阴性IBC患者可能从免疫治疗中获益，而激素受体阳性亚型中CDK4/6抑制剂显示出潜在疗效。IBC的预后仍不理想，早期复发及远处转移常见，主要影响因素包括淋巴结受累、分子分型及新辅助治疗反应。随着TME研究和分子机制的深入，靶向治疗及个体化策略有望改善IBC患者的预后。本文从流行病学、病理学、诊断策略、治疗进展和预后因素等方面对IBC进行综述，旨在为其规范化诊治及未来研究提供参考。

Abstract:

Inflammatory breast cancer (IBC) is a rare but highly aggressive subtype of breast cancer characterized by rapid clinical progression and poor prognosis. Although it accounts for only 2%-4% of all breast cancer cases, it is responsible for 8%-10% of breast cancer-related mortality. The etiology of IBC is multifactorial, involving genetic, hormonal, environmental, and socioeconomic factors. Pathologically, IBC is marked by the presence of dermal lymphatic tumor emboli, and molecular subtypes are predominantly HER2-positive and triple-negative, indicating high tumor invasiveness. Diagnosis relies on characteristic clinical manifestations and histopathological confirmation, while imaging techniques such as MRI and PET/CT play important roles in evaluating disease extent and metastasis. Given that IBC is often diagnosed at a locally advanced or metastatic stage, there is currently no specific treatment protocol. Instead, management generally follows the treatment paradigm of non-IBC, emphasizing systemic therapy within a multidisciplinary framework. HER2-positive IBC benefits from chemotherapy combined with dual-targeted anti-HER2 therapy; triple-negative IBC may respond to immune checkpoint inhibitors; and CDK4/6 inhibitors show potential efficacy in hormone receptor-positive subtypes. Despite advancements, the prognosis remains poor, with a high risk of early recurrence and distant metastasis. Prognostic factors include lymph node involvement, molecular subtype, and response to neoadjuvant therapy. As research into the tumor microenvironment and molecular mechanisms deepens, targeted and individualized therapies hold promise for improving outcomes. This review summarizes the epidemiology, pathology, diagnostic criteria, treatment strategies, and prognostic factors of IBC, aiming to inform clinical practice and future research.