Case Reports Journal Article Epublish 1 citation
PubMed: 39574496 DOI: 10.1177/17588359241298489
Epithelioid inflammatory myofibroblastic sarcoma with exceptionally long response to lorlatinib-a case report.
Ther Adv Med Oncol 16:17588359241298489
Authors: Rafał Becht, Kajetan Kiełbowski, Justyna Żychowska, Wojciech Poncyljusz, Aleksandra Łanocha, Katarzyna Kozak, Ewa Gabrysz-Trybek, Paweł Domagała
Affiliations
- 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.
- Department of Diagnostic Imaging and Interventional Radiology, Pomeranian Medical University in Szczecin, Szczecin, Poland.
- Department of Hematology and Transplantology, Pomeranian Medical University in Szczecin, Szczecin, Poland.
- Department of Soft Tissue/Bone Sarcoma and Melanoma, The Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland.
- Department of Pathology, Pomeranian Medical University in Szczecin, Szczecin, Poland.
Abstract
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.
MeSH
Sarcoma, Diagnosis, Therapeutics