Case Report: Hybrid low-grade fibromyxoid sarcoma and sclerosing epithelioid fibrosarcoma of the retroperitoneum
Burkitbayev Z. Zhaksylyk A. Ussipbekov B. Uskenbayev T. Kerimkulov A. Sarina Т. Gogolev A. Bolsinbekova S. Sipenova A.
2026Frontiers Media SA
Frontiers in Oncology
2026#16
Retroperitoneal Sarcomas are rare malignancies that comprise 1%–2% of all malignant tumors. Among them, Low-Grade Fibromyxoid Sarcoma (LGFMS) and Sclerosing Epithelioid Fibrosarcoma (SEF) are ultra-rare sarcomas, especially in retroperitoneum. Hybrid LGFMS/SEF is even rarer, with limited cases reported in literature. We present the case of a 63-years old male with complaints on left hypochondrial discomfort, weight loss, and generalized weakness. Imaging revealed a large retroperitoneal mass with suspicion of local invasion. The patient underwent en-bloc surgical resection, including distal pancreatectomy, splenectomy, and left adrenalectomy. Histopathological examination confirmed a hybrid LGFMS/SEF with characteristic biphasic morphology. Hybrid LGFMS/SEF tumors exhibit features of both LGFMS and SEF sarcoma subtypes. Complete surgical resection remains the primary treatment strategy for localized disease. Because of the rarity of the disease, long-term surveillance is recommended. For advanced diseases, there are limited efficient available treatments necessitating the research of targeted therapies. Copyright
en-bloc surgical resection , hybrid soft tissue tumor , low-grade fibromyxoid sarcoma , retroperitoneal sarcoma , sclerosing epithelioid fibrosarcoma
Text of the article Перейти на текст статьи
Department of Surgery, National Research Oncology Center, Astana, Kazakhstan
Department of Surgery, School of Medicine, Nazarbayev University, Astana, Kazakhstan
Department of Surgery
Department of Surgery
10 лет помогаем публиковать статьи Международный издатель
Книга Публикация научной статьи Волощук 2026 Book Publication of a scientific article 2026