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多形性透明变性血管扩张性肿瘤:1例罕见病例报告及鉴别诊断讨论

Pleomorphic Hyalinizing Angiectatic Tumour: A Rare Case Report and Discussion of Differential Diagnosis.

作者信息

Chalmeti Ambica, Arakeri Surekha U, Javalgi Anita P, Goyal Shefali

机构信息

Postgraduate Student, Department of Pathology, BLDEU's Shri B.M. Patil Medical College, Hospital and Research Centre, Vijayapur, Karnataka, India.

Professor, Department of Pathology, BLDEU's Shri B.M. Patil Medical College, Hospital and Research Centre, Vijayapur, Karnataka, India.

出版信息

J Clin Diagn Res. 2017 Aug;11(8):ED15-ED16. doi: 10.7860/JCDR/2017/27396.10405. Epub 2017 Aug 1.

Abstract

Pleomorphic Hyalinizing Angiectatic Tumour (PHAT) is one of the rare soft tissue tumour which is non-metastasizing. The origin of this tumour is yet uncertain. It occurs in adults as a slow growing subcutaneous mass mimicking clinically and histologically to various benign and malignant soft tissue tumours such as schwannoma, haemangioma and malignant fibrous histiocytoma. The microscopic features of this tumour include clusters of ectatic, fibrin containing, hyalinized blood vessels with pleomorphic and spindle shaped tumour cells showing intranuclear inclusions, stromal haemosiderin pigment and a variable inflammatory infiltrate. Despite marked pleomorphism, the lesion behaves as a low grade neoplasm, with frequent recurrences, but no metastases. The incidence of this tumour is very rare with less than 100 cases being published. Hence, awareness of this entity is must for proper management of the patient and to avoid misdiagnosis of the lesion. We report a case of pleomorphic hyalinizing angiectatic tumour in a 50-year-old man who presented with a slow growing mass in the left calf region since two years.

摘要

多形性透明变性血管扩张性肿瘤(PHAT)是一种罕见的非转移性软组织肿瘤。该肿瘤的起源尚不确定。它发生于成年人,表现为生长缓慢的皮下肿块,在临床和组织学上与多种良性和恶性软组织肿瘤相似,如神经鞘瘤、血管瘤和恶性纤维组织细胞瘤。该肿瘤的微观特征包括扩张的、含纤维蛋白的、透明变性的血管簇,伴有多形性和梭形肿瘤细胞,可见核内包涵体、间质含铁血黄素色素以及不同程度的炎症浸润。尽管具有明显的多形性,但该病变表现为低度肿瘤,常有复发,但无转移。该肿瘤的发病率非常低,发表的病例不到100例。因此,认识这一实体对于正确治疗患者和避免病变的误诊至关重要。我们报告一例50岁男性的多形性透明变性血管扩张性肿瘤,该患者自两年前起左小腿区域出现一个生长缓慢的肿块。

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