Department of Internal Medicine, Allegheny Health Network, Pittsburgh, PA, U.S.A.;
Department of Pathology, Allegheny Health Network, Pittsburgh, PA, U.S.A.
Anticancer Res. 2022 Jul;42(7):3595-3599. doi: 10.21873/anticanres.15846.
Myeloid Sarcoma (MS) are tumors containing myeloid blasts occurring in a location other than the bone marrow, including lymph nodes, skin, and soft tissues. MS presenting as polyserositis however is very rare, with only a few cases in the literature.
A 20-year-old male presented with cough, shortness of breath and was found to have left upper lobe consolidation, left pleural effusion, pericardial effusion, and a large anterior mediastinal mass. A transthoracic echocardiogram showed pericardial effusion with tamponade physiology. He underwent emergent pericardiocentesis and thoracentesis. The fluid studies showed flow cytometry findings consistent with MS/ acute myeloid leukemia (AML) phenotype. A bone marrow aspirate and biopsy were unremarkable and showed no immunophenotypic findings diagnostic of acute leukemia or a lymphoproliferative disorder. Cytogenetics was negative for AML abnormalities per FISH analysis. Videoassisted thoracoscopy surgery (VATS) with biopsy of the mediastinal mass, pericardium, and left upper lobe of the lung was consistent with MS. He was treated with induction cytarabine and idarubicin, and a follow up PET-CT scan showed complete remission. He is currently day 200 + post stem cell transplant with no evidence of disease recurrence.
To the best of our knowledge, this is the first case of isolated myeloid sarcoma presenting as polyserositis, without prior leukemia/ bone marrow involvement. Hence, fluid studies should involve cytometry analysis and MS should be entertained as a differential for polyserositis, even without a history of prior leukemia. Timely diagnosis can expedite aggressive chemotherapy required for a potentially life-threatening disease.
骨髓肉瘤（MS）是指除骨髓以外的其他部位出现髓系原始细胞的肿瘤，包括淋巴结、皮肤和软组织。然而，以多发性浆膜炎为表现的 MS 非常罕见，文献中仅有少数病例报道。
一名 20 岁男性因咳嗽、呼吸急促就诊，胸部 CT 检查发现左肺上叶实变、左侧胸腔积液、心包积液和巨大前纵隔肿块。经胸超声心动图显示有心包填塞生理改变的心包积液。患者接受了紧急心包穿刺和胸腔穿刺。液体研究显示流式细胞术检查结果与 MS/急性髓系白血病（AML）表型一致。骨髓抽吸和活检未见异常，无急性白血病或淋巴增生性疾病的免疫表型表现。FISH 分析显示核型正常，无 AML 异常。纵隔肿块、心包和左肺上叶的电视辅助胸腔镜手术（VATS）活检与 MS 一致。患者接受了阿糖胞苷和伊达比星诱导化疗，随后的 PET-CT 扫描显示完全缓解。目前患者行干细胞移植后第 200 天+，无疾病复发迹象。
据我们所知，这是首例孤立性骨髓肉瘤以多发性浆膜炎为表现、无白血病/骨髓受累的病例。因此，液体研究应包括细胞术分析，即使没有先前白血病的病史，也应考虑 MS 作为多发性浆膜炎的鉴别诊断。及时诊断可以加快潜在危及生命疾病所需的强化化疗。
