Inflammatory monomorphic undifferentiated sarcoma with distinct clinical and pathological features: a 'new' entity?
Inflammatory monomorphic undifferentiated sarcoma with distinct clinical and pathological features: a 'new' entity?
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DOI：
10.1097/pat.0b013e328340c1f4
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发表时间：
2011-01-01
期刊：
影响因子：
4.5
通讯作者：
Allen, Philip W.
中科院分区：
文献类型：
--
作者：
Wang, Zhe;Huang, Gaosheng;Allen, Philip W.
目的：描述两例高度侵袭性的、显然是“新的”和罕见的儿童期和成人早期软组织和骨恶性肿瘤的患者，我们将其命名为炎性单形性未分化肉瘤。两个组织学上相同的肿瘤位于肱骨近端和前胸壁的男性，年龄分别为6岁和31岁，表现为孤立的、疼痛的、压痛的、坏死的肿块，伴有发热，白细胞增多和阴性微生物培养。广泛坏死的切除肿瘤由大的单形上皮样细胞组成，细胞核呈囊泡状，嗜酸性核仁突出，大量嗜酸性细胞质被大量中性粒细胞和嗜酸性粒细胞包围，形成无菌微囊。免疫组织化学和超微结构研究显示没有特异性分化。这两种肿瘤是非常积极的，早期局部复发，转移到区域和远处的淋巴结和内脏，并没有反应，几个不同的化疗方案。结论：仔细审查的文献使我们相信，炎性单形未分化肉瘤可能代表一种罕见的和独特的临床病理实体，似乎没有以前描述过。
Aim: To describe two patients with a highly aggressive, apparently 'new' and rare soft tissue and bone malignancy of childhood and early adult life that we have named inflammatory monomorphic undifferentiated sarcoma.Methods and results: Two histologically identical tumours located in the proximal humerus and the anterior chest wall of males aged 6 and 31 presented as solitary, painful, tender, necrotising, masses, associated with fever, leukocytosis and negative microbiological cultures. The extensively necrotic resected tumours consisted of large, monomorphic epithelioid cells with vesicular nuclei, prominent eosinophilic nucleoli, and abundant eosinophilic cytoplasm surrounded by numerous neutrophils and eosinophils which formed sterile microabscesses. Immunohistochemical and ultrastructural studies revealed no specific differentiation. Both tumours were very aggressive, with early local recurrence, metastasis to regional and distant lymph nodes and viscera, and no response to several different chemotherapeutic regimens.Conclusion: A careful review of the literature led us to believe that inflammatory monomorphic undifferentiated sarcoma may represent a rare and distinct clinicopathological entity that does not appear to have been previously described.