Boshier Piers R, Sayers Rosie, Hadjiminas Dimitri J, Mackworth-Young Charles, Cleator Susan, Leff Daniel R
Department of Surgery and Cancer, Imperial College London, London, UK.
Department of Breast Surgery, Imperial College NHS Healthcare Trust, Charing Cross Hospital, London, UK.
Exp Hematol Oncol. 2016 Jun 22;5:16. doi: 10.1186/s40164-016-0045-2. eCollection 2015.
Inflammatory breast cancer is a complex pathological entity associated with poor outcomes. This loco-regional disease is characterised by a rapid clinical course in the presence breast erythema and infiltration of dermal lymphatics by tumours cells. Herein we describe a case of inflammatory breast cancer with a rare presentation and disease course defined by a profound systemic inflammatory response in the absence of an infective cause.
The patient presented with pyrexia and malaise following a recent tissue diagnosis of inflammatory breast cancer. At the time of admission the patient demonstrated clinical features of the systemic inflammatory response syndrome (SIRS) in the presence of a negative septic screen. Her condition deteriorated despite systemic broad spectrum intravenous antibiotics and she underwent surgical debulking of a 180 × 135 × 100 mm (821 g) primary tumour composed of oedematous, friable and haemorrhagic tissue (pT4,N1a,M0; oestrogen/progesterone/HER-2 receptor negative). Following surgery, the clinical picture dramatically improved with cessation of SIRS and normalisation of inflammatory markers. After 4 weeks the patient required readmission to hospital due to recurrent SIRS and negative septic screen. The patient received treatment with systemic chemotherapy showing transient clinical improvement and suppression of SIRS. Despite on going chemotherapy, systemic antibiotics and a trial of steroid therapy the patient died 5 months after her initial presentation to hospital. At the time of death she demonstrated persistent SIRS with elevated inflammatory markers.
This is the first case report of inflammatory breath cancer associated with SIRS in the absence of clinically confirmed infection. Important learning points highlighted by this case are: (a) recognition of the diagnostic and therapeutic uncertainties that still exist in the context of inflammatory breast cancer; (b) appreciation of the potential paraneoplastic systemic inflammatory manifestations of this disease, and finally; (c) the importance a multidisciplinary and multimodal approach to treatment.
炎性乳腺癌是一种预后较差的复杂病理实体。这种局部区域性疾病的特点是临床病程迅速，伴有乳腺红斑以及肿瘤细胞浸润真皮淋巴管。在此，我们描述一例炎性乳腺癌病例，其表现罕见，疾病进程由无感染病因情况下的严重全身炎症反应所界定。
该患者在近期组织诊断为炎性乳腺癌后出现发热和不适。入院时，患者在脓毒症筛查阴性的情况下表现出全身炎症反应综合征（SIRS）的临床特征。尽管给予了全身性广谱静脉抗生素治疗，其病情仍恶化，随后对一个大小为180×135×100毫米（821克）的原发性肿瘤进行了减瘤手术，该肿瘤由水肿、易碎和出血组织构成（pT4，N1a，M0；雌激素/孕激素/HER-2受体阴性）。手术后，随着SIRS的停止和炎症标志物的正常化，临床情况显著改善。4周后，患者因复发性SIRS和脓毒症筛查阴性再次入院。患者接受了全身化疗，临床有短暂改善且SIRS得到抑制。尽管持续进行化疗、全身使用抗生素并尝试了类固醇治疗，但患者在首次入院5个月后死亡。死亡时，她表现出持续的SIRS，炎症标志物升高。
这是首例在无临床确诊感染情况下与SIRS相关的炎性乳腺癌病例报告。该病例突出的重要经验教训有：（a）认识到炎性乳腺癌背景下仍然存在的诊断和治疗不确定性；（b）认识到该疾病潜在的副肿瘤性全身炎症表现，最后；（c）多学科和多模式治疗方法的重要性。
