Gatt Raphael, Casingena Luca, Pisani David, Agius Rachel, Cassar Noel
Mater Dei Hospital, Msida, Malta.
Surg Case Rep. 2023 Sep 20;9(1):166. doi: 10.1186/s40792-023-01734-4.
Undifferentiated pleomorphic sarcoma is an uncommon sarcoma and its presence in the spleen is even rarer, with only a handful of cases reported in English literature. It is typically only diagnosed following histological analysis. Its rarity also means that there is little consensus over ideal management.
This report presents a case of a 40-year-old Caucasian male who was found to have a splenic mass after presenting with non-specific abdominal pain and generalized malaise. Numerous imaging modalities were used which demonstrated a large partially solid and partially cystic lesion in spleen with no evidence of metastasis. As core biopsies were undiagnostic, he was planned for a diagnostic and therapeutic splenectomy. However, despite magnetic resonance imaging 11 days prior to his operation showed no evidence of liver metastasis, a massive splenic tumour with hepatic metastases was identified intraoperatively. An open splenectomy, distal pancreatectomy and liver metastasectomy was hence carried out. Histological analysis confirmed liver metastasis secondary to a splenic undifferentiated pleomorphic sarcoma. The patient recovered well and was discharged home. He presented again three weeks after his operation with lower back pain, abdominal pain and fever. Computed tomography demonstrated extensive recurrent disease burden in the peritoneum and liver. The patient passed away a month after surgery.
Splenic undifferentiated pleomorphic sarcoma is a rare tumour which may pose a significant diagnostic challenge on both clinical and histopathological grounds. Following diagnosis and treatment, its aggressive nature often results in a poor prognosis. Current literature fails to delineate any superior management strategy to increase survival.
未分化多形性肉瘤是一种罕见的肉瘤，其发生于脾脏的情况更为罕见，英文文献中仅报道过少数病例。通常只有在进行组织学分析后才能确诊。其罕见性也意味着对于理想的治疗方案几乎没有共识。
本报告介绍了一名40岁的白种男性病例，该患者在出现非特异性腹痛和全身不适后被发现脾脏有肿块。使用了多种成像方式，显示脾脏有一个大的部分实性和部分囊性病变，无转移迹象。由于核心活检未明确诊断，计划对其进行诊断性和治疗性脾切除术。然而，尽管术前11天的磁共振成像显示无肝转移证据，但术中发现了一个巨大的脾脏肿瘤伴肝转移。因此进行了开放性脾切除术、远端胰腺切除术和肝转移瘤切除术。组织学分析证实肝转移继发于脾脏未分化多形性肉瘤。患者恢复良好并出院回家。术后三周，他再次出现下背痛、腹痛和发热。计算机断层扫描显示腹膜和肝脏有广泛的复发疾病负担。患者在手术后一个月去世。
脾脏未分化多形性肉瘤是一种罕见肿瘤，在临床和组织病理学方面都可能带来重大诊断挑战。诊断和治疗后，其侵袭性往往导致预后不良。目前的文献未能明确任何提高生存率的优越管理策略。
