Clinicopathological analysis of myeloid sarcoma with megakaryocytic differentiation
Clinicopathological analysis of myeloid sarcoma with megakaryocytic differentiation
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DOI：
10.1016/j.pathol.2021.08.015
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发表时间：
2022-05-26
期刊：
影响因子：
4.5
通讯作者：
Ohshima, Koichi
中科院分区：
文献类型：
--
作者：
Nagamine, Michiko;Miyoshi, Hiroaki;Ohshima, Koichi
髓样肉瘤（MS）定义为发生在骨髓以外的解剖部位的由髓样母细胞组成的肿瘤肿块。多发性硬化伴巨核细胞分化（MS with megakaryocytic differentiation，MSMgk）是一种罕见的恶性肿瘤，其临床病理特征尚不清楚，我们回顾了11例由表达CD 41的未成熟非淋巴造血细胞组成的髓外肿块形成恶性肿瘤，其中7例男性，4例女性（男女比例为1.75：1）。诊断时的平均和中位年龄分别为50岁和62岁，范围为2至78岁。髓外肿块单发3例（27%），多发8例（73%）。肿瘤部位为淋巴结6例，皮下3例，肌内1例，骨1例。11例患者中有7例（64%）有骨髓增生异常综合征（MDS）或骨髓增生性肿瘤（MPN）病史。3例患者（27%）在急性髓性白血病缓解期间发生MS，1例患者在其他部位复发MS。有4例病例的随访数据可用。肿瘤细胞CD 41、CD 33、CD 34、MPO和CD 68阳性分别为11例（100%）、3例（27%）、7例（64%）、4例（36%）和7例（64%）。2例成功进行了细胞遗传学分析。复杂但不一致的异常是明显的。与无巨核细胞分化的MS病例相比，MSMgk的生存期明显缩短（p=0.0033）。与无巨核细胞分化的MS相比，MSMgk更可能遵循MDS/MPN，涉及多个部位，并与较差的结局相关。更详细的研究，包括基因组或基因表达分析，可以证实MSMgk的特征。
Myeloid sarcoma (MS) is defined as a tumour mass consisting of myeloid blasts that occurs at an anatomical site other than bone marrow. MS with megakaryocytic differentiation (MSmgk) is extremely rare and its clinicopathological features have not been well described.We reviewed 11 cases in 11 patients of extramedullary mass-forming malignant tumours composed of immature non-lymphoid haematopoietic cells expressing CD41 with or without concurrent bone marrow lesions.The patients consisted of seven men and four women (1.75:1 male-to-female ratio). The mean and median ages at diagnosis were 50 and 62 years, respectively, ranging from 2 to 78 years. Extramedullary mass lesions were solitary in three cases (27%) and multiple in eight cases (73%). Tumour locations were lymph nodes (6 cases), subcutaneous tissue (3 cases), intramuscular (1 case), and bone (1 case). Seven of the 11 patients (64%) had a history of myelodysplastic syndrome (MDS) or myeloproliferative neoplasm (MPN). Three patients (27%) developed MS during remissions of acute myelogenous leukaemia, and one patient had a recurrence of MS at other sites. Follow-up data were available for four cases. Tumour cells were positive for CD41, CD33, CD34, MPO, and CD68 in 11 (100%), three (27%), seven (64%), four (36%), and seven (64%) cases, respectively. Cytogenetic analysis was successfully performed in two cases. Complex but inconsistent abnormalities were evident. When compared with cases of MS without megakaryocytic differentiation, the survival of MSmgk was significantly shorter (p=0.0033).Compared to MS without megakaryocytic differentiation, MSmgk is more likely to follow MDS/MPN, to involve multiple sites, and to be associated with poorer outcomes. More detailed studies, including genomic or gene expression analyses, could confirm the characteristics of MSmgk.