病理学与病理生理学教研室王哲、黄高昇教授课题组近期发现了一种新的肿瘤类型,他们将它命名为“炎症性单形性未分化肉瘤”(Inflammatory Monomorphic Undifferentiated Sarcoma)。从目前的资料来看,该肿瘤常发生于年轻患者的骨和软组织,其特点表现为:(1)患者临床表现为疼痛性肿物生长,肿物局部有红、肿、热、痛等化脓性炎症的表现;同时患者有发热、白细胞升高的化脓性炎症全身表现;肿瘤局部可穿刺抽出脓液,但脓液多种微生物培养均为阴性结果,手术切除的肿瘤表面也可见脓性渗出物,多种抗生素治疗无效。(2)手术切除的肿瘤病理形态非常特殊,肿瘤由单一的胞浆丰富的上皮样细胞组成,胞浆嗜酸性或透明,胞膜明显;泡状肿瘤细胞核大、圆形或卵圆形,染色质开放,具有巨大的嗜酸性核仁;肿瘤中可见大量的嗜中性粒细胞浸润,并有许多微脓肿形成。(3)通过免疫组化和电镜等技术,未能发现肿瘤细胞特异的分化。(4)肿瘤进展非常快,生长迅速,早期发生局部复发和淋巴结转移,对多种化疗方案均无反应,患者均在发病后4月内死于广泛转移和严重并发症。该肿瘤具有独特的临床表现、病理学形态和预后特征,与目前已发现的任何一种已知肿瘤都不相同,在世界卫生组织的肿瘤病理学分类中没有相似的肿瘤类型,是一种全新的肿瘤类型,。对于该肿瘤的报道近期发表在澳大利亚皇家病理学院的官方病理专业杂志《Pathology》。炎症性单形性未分化肉瘤是我校自己发现并命名的肿瘤类型,具有自主知识产权,是建国以来我国病理界学者首个自主发现和命名的新的肿瘤类型。(生物谷Bioon.com)
生物谷推荐原文出处:
Pathology doi: 10.1097/PAT.0b013e328340c1f4
Inflammatory monomorphic undifferentiated sarcoma with distinct clinical and pathological features: a ‘new’ entity?
Wang, Zhe*,||; Huang, Gaosheng*,||; Yan, Qingguo*; Wang, Lu*; Zhu, Jin*; Lu, Yang*; Li, Peifeng*; Cheng, Hong*; Ma, M Joe?; Walker, Bruce F?; Allen, Philip W§
Abstract
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.