■原发性脾血管肉瘤(PSA)是一种罕见的肿瘤。它是来源于脾窦内皮细胞的恶性肿瘤。PSA的病因不明,高度恶性,容易早期转移,不典型的临床症状和影像学表现,和困难的早期诊断。本文报告1例PSA伴肝内转移的18F-FDGPET/CT表现,成像,和病理数据;并回顾文献。
■一名64岁男性患者于2022年3月13日出现左下腹胀痛,无明显原因。疼痛持续、迟钝,坐着吃饭后加重。血常规检查结果为RBC→3.33×1012/L,WBC^12.32×109/L,和PLT△40×109/L肿瘤标志物显示CA125^47.0U/ml,AFP(-),CEA(-),CA199(-),和CA724(-)。腹部CT非对比增强扫描显示脾脏体积明显增大,形态不规则,有多发结节和块状低密度阴影,界限不清,密度不均匀,和多个坏死区域。增强CT显示脾脏弥漫性不均匀轻度增强,增强程度随时间增加。肝脏可见多个结节状低密度阴影,18F-FDGPET/CT显示脾脏有多个结节和大量病变,并有多个坏死区。肝脏有多个结节性病变,FDG代谢水平升高,脾脏病变的SUVmax为9.0,肝脏病变的SUVmax为5.6.18F-FDGPET/CT诊断为脾恶性肿瘤伴肝转移。最后,经过多学科的讨论,决定进行腹腔镜全脾切除和门静脉灌注化疗。病理检查显示肿瘤细胞呈圆形,椭圆形,或者梭形,有明显的非典型性,排入电缆或吻合的血管腔。最终诊断为原发性脾血管肉瘤伴大量坏死。手术后,患者接受抗肿瘤联合治疗,5个月后死亡。
■PSA的发生率很低,其临床和放射学表现缺乏特异性。18F-FDGPET/CT显像对PSA有一定的诊断价值,对术前分期有显著的实用性,指导活检程序,评估术后治疗反应,监测疾病复发。在脾脏内存在显示坏死区域的占位性病变时,应考虑PSA。在对比增强扫描中显示渐进增强,并证明FDG摄取的异质性增加。
UNASSIGNED: Primary splenic angiosarcoma (PSA) is a rare neoplasm. It is a malignant tumor derived from endothelial cells of the splenic sinuses. PSA has an unknown etiology, a high degree of malignancy, easy early metastasis, atypical clinical symptoms and imaging findings, and difficult early diagnosis. This paper reports the 18F-FDG PET/CT findings of a case of PSA with intrahepatic metastasis; summarizes its clinical, imaging, and pathological data; and reviews the literature.
UNASSIGNED: A 64-year-old male patient presented with left lower abdominal distending pain without obvious causes on 13 March 2022. The pain was persistent and dull and worsened after sitting and eating. Blood routine examination results were RBC ↓ 3.33 × 1012/L, WBC ↑ 12.32 × 109/L, and PLT ↓ 40 × 109/L. The tumor markers indicated CA125 ↑ 47.0 U/ml, AFP (-), CEA (-), CA199 (-), and CA724 (-). Non-contrast-enhanced CT scan of the abdomen showed that the spleen was significantly enlarged in volume and irregular in shape and had multiple nodules and clumpy low-density shadows, unclear boundaries, uneven density, and multiple necrotic areas. Enhanced CT showed diffuse uneven mild enhancement of the spleen, and the degree of enhancement increased with time. Multiple nodular low-density shadows were seen in the liver, which were slightly enhanced by the enhanced scan.18F-FDG PET/CT showed multiple nodular and massive lesions in the spleen with multiple necrotic areas. There were multiple nodular lesions in the liver, the level of FDG metabolism increased, the SUVmax of the spleen lesions was 9.0, and the SUVmax of the liver lesions was 5.6. The 18F-FDG PET/CT diagnosis was splenic malignancy with liver metastasis. Finally, after a multidisciplinary discussion, it was decided to perform laparoscopic total splenectomy and portal vein infusion chemotherapy. Pathological examination showed that the tumor cells were round, oval, or fusiform, with obvious atypia, arranged into a cable or anastomosed vascular lumen. The final diagnosis was
primary splenic angiosarcoma with massive necrosis. After surgery, the patient received antitumor combined therapy and died 5 months later.
UNASSIGNED: The incidence of PSA is very low, and its clinical and radiological manifestations lack specificity. 18F-FDG PET/CT imaging has a certain diagnostic value for PSA and significant utility in preoperative staging, guiding biopsy procedures, evaluating postoperative treatment response, and monitoring disease recurrence. PSA should be considered in the presence of a space-occupying lesion within the spleen that exhibits necrotic areas, shows progressive enhancement on contrast-enhanced scans, and demonstrates heterogeneous increases in FDG uptake.