以肠梗阻为表现的原发性回肠髓样肉瘤:一份病例报告。

IF 0.7 Q4 SURGERY
Hitoshi Minagi, Nobuhiko Kanaya, Yoshitaka Kondo, Yoshihiko Kakiuchi, Shinji Kuroda, Ryohei Shoji, Hajime Kashima, Yuki Matsumi, Satoru Kikuchi, Kunitoshi Shigeyasu, Fuminori Teraishi, Shunsuke Kagawa, Toshiyoshi Fujiwara
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引用次数: 0

摘要

背景:髓样肉瘤(MS)是由髓样胚块或未成熟髓样细胞构成的髓外肿瘤。它经常与急性髓系白血病(AML)同时发生,但也可在无白血病的患者中出现异常表现。在此,我们介绍一例罕见的原发性多发性骨髓瘤病例,该病起源于小肠,但无骨髓受累的证据:一名 33 岁女性,无相关病史,因反复腹痛入住我院。计算机断层扫描(CT)显示回肠壁增厚导致肠梗阻,怀疑为回肠肿瘤。最初怀疑是异位子宫内膜异位症,因为腹痛与月经周期有关,而且在随访的 CT 扫描中也观察到了变化。双气囊内镜检查未能发现病变。尽管进行了保守治疗,但梗阻仍然存在,于是进行了腹腔镜回肠部分切除术,结果发现回肠和肠系膜广泛受累。此外,切除的回肠系膜极度增厚。手术标本的组织病理学和免疫组化检查显示回肠多发性硬化。出院后进行的骨髓穿刺检查显示白血病细胞学结果为阴性,最终诊断为原发性回肠多发性硬化症。她的术后恢复顺利,目前正在另一家医院接受针对急性髓细胞白血病的全身化疗:结论:尽管小肠多发性硬化非常罕见,而且术前可能不会考虑,但与其他小肠恶性肿瘤相似的手术治疗可确保术后诊断正确并进行适当的化疗。考虑到化疗的潜在需求,确保手术安全以迅速启动化疗至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Primary ileal myeloid sarcoma presenting with bowel obstruction: a case report.

Background: Myeloid sarcoma (MS) is an extramedullary tumor constituted by myeloid blasts or immature myeloid cells. It frequently occurs in conjunction with acute myeloid leukemia (AML); however, it can exceptionally manifest in patients without leukemia. Here, we present a rare case of primary MS originating in the small bowel without evidence of bone marrow involvement.

Case representation: A 33 year-old female with no relevant medical history was admitted to our hospital with recurrent abdominal pain. Computed tomography (CT) revealed bowel obstruction due to thickening of the ileum wall, which was suspected to be an ileal tumor. Initially, ectopic endometriosis was suspected because of abdominal pain associated with the menstrual cycle and changes observed on a follow-up CT scan. The lesion could not be detected by double-balloon endoscopy. Despite conservative treatment, the obstruction persisted, and laparoscopic partial ileal resection was performed, which revealed extensive involvement of the ileum and mesentery. Additionally, the mesentery of the resected ileum was extremely thickened. Histopathological and immunohistochemical examinations of the surgical specimen indicated ileal MS. Bone marrow aspiration after discharge was negative for cytological findings of leukemia, leading to a final diagnosis of primary ileal MS. Her postoperative course was uneventful, and she is currently undergoing systemic chemotherapy tailored to AML at another hospital.

Conclusions: Even though MS of the small bowel is rare and may not be considered preoperatively, similar surgical treatment to that of other small bowel malignancies can ensure proper postoperative diagnosis and appropriate chemotherapy. Given the potential need for chemotherapy, ensuring surgical safety that allows for its rapid initiation is critical.

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