PDF(Pubmed)
Abstract：
Sclerosing angiomatoid nodular transformation (SANT) is a benign vascular lesion of the spleen with uncertain etiology. It predominantly affects women between the ages of 30 and 60 years. Clinically, it is asymptomatic or can cause abdominal pain, but usually discovered incidentally on imaging, which can identify a mass but may not provide a definitive diagnosis. In uncertain vascular lesions, there is always a risk of spontaneous rupture of large vessels and the potential for spreading malignancy. Hence, the final diagnosis is rendered on microscopy after splenectomy. A middle-aged female came to the clinic complaining of abdominal pain. Radiology showed a solid splenic mass and the patient underwent splenectomy. Gross examination showed a 3 cm white firm mass with focal hemorrhage. Microscopy revealed multiple nodules of variable sizes surrounded by fibrosclerotic stroma. The nodules showed round to slit-like vascular spaces with numerous red blood cells. The internodular stroma consisted of dense fibrous tissue with scattered plump myofibroblasts and lymphoplasmacytic inflammatory cells. These distinctive features lead to the diagnosis of SANT. SANT possesses characteristic histologic features with distinctive immunohistochemistry (IHC). IHC reveals three different types of vessels within the nodules as follows: (1) small veins (CD34-, CD31+, CD8-), (2) sinusoids (CD34-, CD31+, CD8+), and (3) capillaries (CD34+, CD31+, CD8-). All three types of vessels are negative for CD21/CD35 and CD68. Hemangioma and littoral cell angioma are two frequent vascular tumors in the spleen that should be considered differential diagnoses. Both lesions lack the microscopic features of SANT and have only a single type of vessel. The vessels in hemangioma are (CD31+, CD34+, CD8-), while in littoral cell angioma they are (CD31+, CD34-, CD8-, CD21+, CD68+). There are no specific clinical or radiologic findings for SANT. It is important to recognize these characteristic features and to differentiate them from other benign and malignant lesions, such as angiosarcoma. A thorough histopathologic examination and IHC are helpful in making the correct diagnosis.
摘要：
硬化性血管瘤样结节性转化（SANT）是脾脏的良性血管病变，病因不确定。它主要影响30至60岁的女性。临床上,它无症状或可引起腹痛，但通常是在成像时偶然发现的，可以识别肿块，但可能无法提供明确的诊断。在不确定的血管病变中，总存在大血管自发破裂的风险和恶性肿瘤扩散的可能性.因此，最终诊断是在脾切除术后的显微镜下做出的。一位中年女性来到诊所，抱怨腹痛。放射学显示脾脏实性肿块，患者接受了脾切除术。大体检查显示3厘米白色硬肿块，局灶性出血。显微镜检查显示多个大小可变的结节被纤维硬化基质包围。结节显示圆形至狭缝状血管间隙，有许多红细胞。结节间基质由致密的纤维组织和分散的丰满的肌成纤维细胞和淋巴浆细胞炎性细胞组成。这些独特的特征导致SANT的诊断。SANT具有特征性的组织学特征，具有独特的免疫组织化学（IHC）。IHC显示结节内三种不同类型的血管如下：（1）小静脉（CD34-，CD31+,CD8-),(2)正弦曲线(CD34-,CD31+,CD8+)，和（3）毛细血管（CD34+，CD31+,CD8-）。所有三种类型的血管都是CD21/CD35和CD68阴性的。血管瘤和沿岸细胞血管瘤是脾脏中两种常见的血管肿瘤，应考虑进行鉴别诊断。两种病变均缺乏SANT的微观特征，并且仅具有单一类型的血管。血管瘤中的血管是（CD31+，CD34+,CD8-),而在沿岸细胞血管瘤中，它们是(CD31+，CD34-,CD8-,CD21+,CD68+）。SANT没有特定的临床或放射学发现。重要的是要认识到这些特征，并将它们与其他良性和恶性病变区分开来，如血管肉瘤。彻底的组织病理学检查和IHC有助于做出正确的诊断。
