Misiak Jakub, Sokołowski Bernard, Skrobisz Norbert, Matczak Mateusz, Braun Marcin
Department of Pathology, Chair of Oncology, Medical University of Lodz, 92-213 Lodz, Poland.
Hematol Rep. 2024 Oct 18;16(4):648-655. doi: 10.3390/hematolrep16040063.
Angiosarcomas are highly aggressive malignancies with endothelial differentiation, presenting considerable challenges in oncology, especially when arising in rare locations such as the spleen. These tumors predominantly affect adults and are commonly found in the skin, breast, liver, or soft tissues, with more unusual occurrences in other organs. Angiosarcomas have a high propensity for metastasis, typically spreading to the liver, lungs, lymph nodes, and gastrointestinal tract. Splenic angiosarcoma, with fewer than 300 documented cases, is an especially rare and complex form of this malignancy.
This report details a case of splenic angiosarcoma in a 45-year-old male, where bone marrow metastases were the first clinical presentation, initially mimicking myelodysplastic syndrome (MDS) due to persistent pancytopenia.
The eventual identification of the splenic origin underscores the diagnostic difficulties and clinical challenges inherent in managing such atypical and rare presentations.
血管肉瘤是具有内皮分化的高度侵袭性恶性肿瘤，在肿瘤学中带来了相当大的挑战，尤其是当它发生在脾脏等罕见部位时。这些肿瘤主要影响成年人，常见于皮肤、乳腺、肝脏或软组织，在其他器官中的发生则较为罕见。血管肉瘤具有很高的转移倾向，通常会扩散到肝脏、肺、淋巴结和胃肠道。脾脏血管肉瘤记录在案的病例不到300例，是这种恶性肿瘤中一种特别罕见且复杂的形式。
本报告详细介绍了一名45岁男性脾脏血管肉瘤的病例，其中骨髓转移是最初的临床表现，由于持续性全血细胞减少，最初类似骨髓增生异常综合征（MDS）。
脾脏起源的最终确定凸显了处理此类非典型和罕见表现时固有的诊断困难和临床挑战。
