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脾脏硬化性血管瘤样结节性转化伴血小板减少症

Sclerosing angiomatoid nodular transformation of the spleen with thrombocytopenia.

作者信息

Kuroiwa Hidemichi, Ueno Masaki, Hirokawa Fumitoshi, Shigekawa Yoshinobu, Kimura Masamichi, Tabata Hirotaka, Deguchi Masaaki, Hoshida Yoshihiko, Nakamori Mikihito

机构信息

Department of Surgery, National Hospital Organization Osaka Minami Medical Center, 2-1 Kidohigashimachi, Kawachinagano-shi, Osaka, 586-8521, Japan.

Department of Surgery, Wakayama Medical University Hospital, Wakayama, Japan.

出版信息

Clin J Gastroenterol. 2025 Jul 13. doi: 10.1007/s12328-025-02174-3.

DOI:10.1007/s12328-025-02174-3
PMID:40652440
Abstract

Sclerosing angiomatoid nodular transformation (SANT) is a rare benign vascular disease of the spleen. We report the case of a patient with SANT with thrombocytopenia. To achieve recovery from thrombocytopenia, resection of this splenic tumor was required. The patient was a 74-year-old woman who was admitted to our hospital due to gradually decreasing platelet counts. Contrast-enhanced computed tomography showed a 66 × 62 mm solid tumor in the spleen. Regarding thrombocytopenia, drug-induced or hematologic disease was ruled out, and no other possible causes were identified besides this splenic tumor. She underwent laparoscopic splenectomy for diagnosis and improvement of thrombocytopenia. This splenic tumor macroscopically resembled an angiomatoid tumor. To distinguish the characteristics of intratumoral vascular components, immunohistochemical staining including CD34, CD8, CD31, and alpha-smooth muscle actin (SMA) was performed and finally this tumor was pathologically diagnosed as SANT. The patient's platelet counts returned to normal range after surgery. If a splenic tumor with thrombocytopenia is identified, SANT might be a differential diagnosis, although it is a rare benign vascular disease of the spleen and difficult to diagnose. For definitive diagnosis, extraction of the splenic specimen may be required.

摘要

硬化性血管瘤样结节性转化(SANT)是一种罕见的脾脏良性血管疾病。我们报告一例患有SANT并伴有血小板减少症的患者。为使血小板减少症得以恢复,需要切除该脾脏肿瘤。患者为一名74岁女性,因血小板计数逐渐下降入住我院。增强计算机断层扫描显示脾脏有一个66×62毫米的实性肿瘤。关于血小板减少症,排除了药物性或血液系统疾病,除了这个脾脏肿瘤外未发现其他可能原因。她接受了腹腔镜脾切除术以进行诊断并改善血小板减少症。这个脾脏肿瘤在宏观上类似于血管瘤样肿瘤。为区分肿瘤内血管成分的特征,进行了包括CD34、CD8、CD31和α平滑肌肌动蛋白(SMA)在内的免疫组织化学染色,最终该肿瘤经病理诊断为SANT。患者术后血小板计数恢复到正常范围。如果发现伴有血小板减少症的脾脏肿瘤,SANT可能是一种鉴别诊断,尽管它是一种罕见的脾脏良性血管疾病且难以诊断。为明确诊断,可能需要获取脾脏标本。

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本文引用的文献

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