病理學(xué)與病理生理學(xué)教研室王哲,、黃高昇教授課題組近期發(fā)現(xiàn)了一種新的腫瘤類型,,他們將它命名為“炎癥性單形性未分化肉瘤”(Inflammatory Monomorphic Undifferentiated Sarcoma)。從目前的資料來看,,該腫瘤常發(fā)生于年輕患者的骨和軟組織,,其特點(diǎn)表現(xiàn)為:(1)患者臨床表現(xiàn)為疼痛性腫物生長,腫物局部有紅,、腫,、熱、痛等化膿性炎癥的表現(xiàn),;同時(shí)患者有發(fā)熱,、白細(xì)胞升高的化膿性炎癥全身表現(xiàn);腫瘤局部可穿刺抽出膿液,,但膿液多種微生物培養(yǎng)均為陰性結(jié)果,,手術(shù)切除的腫瘤表面也可見膿性滲出物,多種抗生素治療無效,。(2)手術(shù)切除的腫瘤病理形態(tài)非常特殊,,腫瘤由單一的胞漿豐富的上皮樣細(xì)胞組成,胞漿嗜酸性或透明,,胞膜明顯,;泡狀腫瘤細(xì)胞核大、圓形或卵圓形,,染色質(zhì)開放,,具有巨大的嗜酸性核仁;腫瘤中可見大量的嗜中性粒細(xì)胞浸潤,,并有許多微膿腫形成,。(3)通過免疫組化和電鏡等技術(shù),未能發(fā)現(xiàn)腫瘤細(xì)胞特異的分化,。(4)腫瘤進(jìn)展非??欤L迅速,,早期發(fā)生局部復(fù)發(fā)和淋巴結(jié)轉(zhuǎn)移,,對(duì)多種化療方案均無反應(yīng),患者均在發(fā)病后4月內(nèi)死于廣泛轉(zhuǎn)移和嚴(yán)重并發(fā)癥,。該腫瘤具有獨(dú)特的臨床表現(xiàn),、病理學(xué)形態(tài)和預(yù)后特征,與目前已發(fā)現(xiàn)的任何一種已知腫瘤都不相同,,在世界衛(wèi)生組織的腫瘤病理學(xué)分類中沒有相似的腫瘤類型,,是一種全新的腫瘤類型,,。對(duì)于該腫瘤的報(bào)道近期發(fā)表在澳大利亞皇家病理學(xué)院的官方病理專業(yè)雜志《Pathology》,。炎癥性單形性未分化肉瘤是我校自己發(fā)現(xiàn)并命名的腫瘤類型,,具有自主知識(shí)產(chǎn)權(quán),是建國以來我國病理界學(xué)者首個(gè)自主發(fā)現(xiàn)和命名的新的腫瘤類型,。(生物谷Bioon.com)
生物谷推薦原文出處:
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.