Becht Rafał, Kiełbowski Kajetan, Żychowska Justyna, Poncyljusz Wojciech, Łanocha Aleksandra, Kozak Katarzyna, Gabrysz-Trybek Ewa, Domagała Paweł
Rafał Becht Department of Clinical Oncology, Chemotherapy and Cancer Immunotherapy, Pomeranian Medical University in Szczecin, Unii Lubelskiej 1, Szczecin 71-252, Poland.
Department of Clinical Oncology, Chemotherapy and Cancer Immunotherapy, Pomeranian Medical University in Szczecin, Szczecin, Poland.
Ther Adv Med Oncol. 2024 Nov 20;16:17588359241298489. doi: 10.1177/17588359241298489. eCollection 2024.
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare and aggressive subtype of inflammatory myofibroblastic tumor. The disease is associated with rearrangements of the anaplastic lymphoma kinase (ALK). In this paper, we present the clinicopathological features and treatment of a female patient diagnosed with EIMS. In 2019, an 18-year-old female patient was admitted to the hospital with abdominal pain. Radiological examinations confirmed a large pelvic mass which was subsequently resected. After re-evaluation of the initial histologic diagnosis, the final diagnosis of EIMS was established. Consequently, due to the lack of response to chemotherapy and deteriorating clinical condition, she began the therapy with ALK inhibitors. In total, the patient was treated with crizotinib, alectinib, and lorlatinib. As a result, after over 4 years since the initial diagnosis, she is still alive with significantly improved clinical condition and quality of life. This paper demonstrates the clinical benefits of sequential therapy of ALK inhibitors and an exceptionally long response to lorlatinib, a third-generation tyrosine kinase inhibitor.
上皮样炎性肌纤维母细胞肉瘤（EIMS）是炎性肌纤维母细胞瘤的一种罕见且侵袭性亚型。该疾病与间变性淋巴瘤激酶（ALK）重排有关。在本文中，我们介绍了一名被诊断为EIMS的女性患者的临床病理特征及治疗情况。2019年，一名18岁女性患者因腹痛入院。影像学检查证实盆腔有一个大肿块，随后进行了切除。在对最初的组织学诊断进行重新评估后，确立了EIMS的最终诊断。因此，由于对化疗无反应且临床状况恶化，她开始使用ALK抑制剂进行治疗。该患者总共接受了克唑替尼、阿来替尼和劳拉替尼治疗。结果，自最初诊断以来4年多过去了，她仍然存活，临床状况和生活质量有了显著改善。本文展示了ALK抑制剂序贯治疗的临床益处以及对第三代酪氨酸激酶抑制剂劳拉替尼的超长反应。
