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硬化性血管脂肪瘤样结节性转化(SANT):一种罕见的脾脏肿瘤和贫血的不常见病因。

Sclerosing Angiomatoid Nodular Transformation (SANT): A Rare Splenic Tumor and Unusual Cause of Anemia.

机构信息

Upper Gastrointestinal Surgery, Lyell Mcewin Hospital, Adelaide, South Australia, Australia.

出版信息

Am J Case Rep. 2021 Nov 13;22:e933598. doi: 10.12659/AJCR.933598.

Abstract

BACKGROUND Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign disease of the splenic red pulp of unknown etiology. Definite diagnosis is made on histopathology alone as it has no diagnostic radiologic characteristics. CASE REPORT We present a case of a large incidental splenic mass in a middle-aged man, whose refractory anemia resolved with splenectomy. Our initial imaging studies failed to differentiate this lesion from other splenic lesions like hamartoma and haemangioma. To the best of our knowledge, no SANT cases in the literature have been investigated with red cell scan, as performed in our patient, nor has any case had resolved anemia after treatment. Splenectomy was offered as malignancy could not be safely excluded. Histopathology confirmed the diagnosis of SANT. The patient made good recovery and had no signs of recurrence 2 years later. CONCLUSIONS SANT of the spleen is a rare condition that can mimic many other splenic tumors and poses diagnostic challenge when histopathology is unavailable. Our case adds to the number of SANT reported in literature in the hope of elucidating the pathophysiology of this rare condition.

摘要

背景

脾脏硬化性血管样结节性转化(SANT)是一种罕见的脾脏红髓良性疾病,病因不明。由于其无诊断性影像学特征,故仅依靠组织病理学才能明确诊断。

病例报告

我们报告了 1 例中年男性偶然发现的巨大脾脏肿块,脾切除术使他的难治性贫血得到缓解。我们的初始影像学研究未能将该病变与其他脾脏病变(如错构瘤和血管瘤)区分开来。据我们所知,在文献中,没有 SANT 病例接受过红细胞扫描检查,而我们的患者进行了此项检查,也没有任何病例在治疗后贫血得到缓解。由于不能安全排除恶性肿瘤,因此建议进行脾切除术。组织病理学证实了 SANT 的诊断。患者恢复良好,2 年后无复发迹象。

结论

脾脏 SANT 是一种罕见疾病,可模拟许多其他脾脏肿瘤,在组织病理学不可用时,诊断具有挑战性。我们的病例增加了文献中报告的 SANT 数量,希望能阐明这种罕见疾病的病理生理学。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bf12/8601013/b98f18f20863/amjcaserep-22-e933598-g001.jpg

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