Kalakonda Anita J M, Mahajan Abhishek, Chow Shien, Olsson-Brown Anna C, Sacco Joseph J
Clatterbridge Cancer Centre NHS Foundation Trust, Liverpool, UK.
CRN North West Coast, NIHR, Liverpool, UK.
Case Rep Oncol. 2024 Mar 27;17(1):497-503. doi: 10.1159/000535328. eCollection 2024 Jan-Dec.
Rare cancers, in aggregate, represent a significant burden of disease in oncology and remain therapeutically challenging to manage due to a lack of clinical trials. Eccrine porocarcinoma is a rare cutaneous sweat-gland malignancy for which there remains no standard approach to metastatic disease.
We describe a patient diagnosed with metastatic disease, confirmed on bone biopsy; pathological analysis further revealed this was oestrogen receptor positive. She was commenced on the aromatase inhibitor letrozole, and denosumab, and showed a significant clinical and radiological response on bone scan within 7 months. At the time of report, over 2 years since commencing letrozole, she remains well with no evidence of progression.
Our experience adds to the literature suggesting anti-oestrogen therapy can have significant benefit in patients with ER-positive non-breast cancer and is in keeping with increasing interest in therapies agnostic to site of origin but guided by expression/mutation of oncogenic drivers.
总体而言,罕见癌症在肿瘤学中构成了重大疾病负担,由于缺乏临床试验,其治疗管理仍然具有挑战性。小汗腺癌是一种罕见的皮肤汗腺恶性肿瘤,对于转移性疾病尚无标准治疗方法。
我们描述了一名经骨活检确诊为转移性疾病的患者;病理分析进一步显示其为雌激素受体阳性。她开始使用芳香化酶抑制剂来曲唑和地诺单抗治疗,7个月内骨扫描显示出显著的临床和影像学反应。在报告时,自开始使用来曲唑已过去2年多,她情况良好,无疾病进展迹象。
我们的经验丰富了文献,表明抗雌激素疗法对雌激素受体阳性的非乳腺癌患者可能有显著益处,这与对不考虑肿瘤起源部位但由致癌驱动因子的表达/突变所指导的治疗方法的兴趣日益增加相一致。