Dermarderosian Aren, Boghossian Raffi, Tai Derek, Sadjadi Javid, Akhtari Mojtaba
University of California Los Angeles, USA.
Rose and Alex Pilibos Armenian School, Los Angeles, CA, USA.
J Investig Med High Impact Case Rep. 2025 Jan-Dec;13:23247096251350571. doi: 10.1177/23247096251350571. Epub 2025 Jun 25.
Littoral cell angioma (LCA) is a rare primary splenic vascular neoplasm originating from the littoral cells of the reticuloendothelial system. Splenectomy is the accepted mode of definitive diagnosis and treatment. With fewer than 200 reported cases, LCA remains poorly understood. Herein, we provide an enhanced insight into its histology and highlight the role of nuclear imaging in aiding LCA diagnosis. A 63-year-old female with a history of stage II multiple myeloma (MM) and rheumatoid arthritis was incidentally found to have a slowly enlarging splenic mass over a 6-year period. Given her candidacy for autologous hematopoietic stem cell transplantation for MM, further evaluation of the splenic lesion was pursued using nuclear medicine (NM) liver-spleen scan, which revealed a photopenic region consistent with a benign hemorrhagic mass. Subsequent splenectomy and histopathological analysis confirmed the diagnosis of LCA, with immunohistochemistry demonstrating CD68+ and CD31+ expression, highlighting LCA's unique dual histiocytic and endothelial character. This case highlights the diagnostic challenge posed by LCA due to its nonspecific clinical presentation and imaging findings. While splenectomy remains the gold standard for diagnosis, our findings suggest that NM liver-spleen scan imaging may aid in differentiating LCA from malignant splenic masses preoperatively. Furthermore, this case reinforces the association between LCA and hematologic malignancies, supporting the hypothesis that immune dysregulation may play a role in its pathogenesis. This underscores the importance of considering LCA in the differential diagnosis of splenic masses, particularly in cases involving a history of malignancy and/or immune system abnormalities.
边缘细胞血管瘤（LCA）是一种罕见的原发性脾脏血管肿瘤，起源于网状内皮系统的边缘细胞。脾切除术是公认的确诊和治疗方式。由于报道的病例少于200例，LCA仍未被充分了解。在此，我们对其组织学进行了深入研究，并强调了核成像在辅助LCA诊断中的作用。一名63岁女性，有II期多发性骨髓瘤（MM）和类风湿性关节炎病史，在6年期间偶然发现脾脏肿块逐渐增大。鉴于她因MM而有自体造血干细胞移植的适应证，遂使用核医学（NM）肝脾扫描对脾脏病变进行进一步评估，结果显示一个与良性出血性肿块相符的放射性缺损区。随后的脾切除术和组织病理学分析证实了LCA的诊断，免疫组织化学显示CD68+和CD31+表达，突出了LCA独特的双组织细胞和内皮特征。该病例突出了LCA因其非特异性临床表现和影像学表现所带来的诊断挑战。虽然脾切除术仍然是诊断的金标准，但我们的研究结果表明，NM肝脾扫描成像可能有助于术前将LCA与恶性脾脏肿块区分开来。此外，该病例强化了LCA与血液系统恶性肿瘤之间的关联，支持免疫失调可能在其发病机制中起作用的假说。这强调了在脾脏肿块的鉴别诊断中考虑LCA的重要性，特别是在有恶性肿瘤病史和/或免疫系统异常的病例中。
