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原发性骨恶性巨细胞瘤:三例罕见病例系列

Primary malignant giant cell tumor of bone: a series of three rare cases.

作者信息

Kapoor Sudhir K, Jain Vineet, Agrawal Mayank, Singh Sompal, Mandal A K

机构信息

Department of Orthopaedics and Pathology, Maulana Azad Medical College and LN Hospital, New Delhi, India.

出版信息

J Surg Orthop Adv. 2007 Summer;16(2):89-92.

Abstract

The purpose of this research was to study incidence; clinical, histological, and radiological features; and outcome of primary malignant giant cell tumor (PMGCT). The authors retrospectively reviewed all cases of giant cell tumor (GCT) in which a diagnosis of GCT was related to sarcoma treated in their department between 1997 and 2004. Three cases of PMGCT were found according to the criterion of Hutter and Dahlin. Histological and radiological records of all the three cases were reviewed. In these three cases of PMGCT, the initial clinical and radiological findings were the same as those for benign giant cell tumor. Wide excision of the tumor was performed in all three cases. In two cases, knee arthrodesis was performed, and in one case a custom-made total knee replacement was performed. PMGCT was diagnosed on initial biopsy in one patient, in the second patient it was diagnosed in the excised specimen, and in third case it was only diagnosed after local recurrence 6 months after initial treatment. All the patients died within 5 months of detection of recurrence and metastasis. PMGCT has a very poor prognosis. Histological examination is highly significant in such cases. Awareness about this entity, adequate biopsy, and sampling of specimen can aid in early diagnosis, which may improve the overall prognosis.

摘要

本研究的目的是探讨原发性恶性巨细胞瘤(PMGCT)的发病率、临床、组织学和放射学特征以及预后情况。作者回顾性分析了1997年至2004年间在其科室接受治疗的所有诊断为巨细胞瘤(GCT)且与肉瘤相关的病例。根据Hutter和Dahlin的标准,发现了3例PMGCT。对这3例病例的组织学和放射学记录进行了回顾。在这3例PMGCT中,最初的临床和放射学表现与良性巨细胞瘤相同。所有3例均进行了肿瘤广泛切除。2例行膝关节融合术,1例行定制全膝关节置换术。1例患者在初次活检时诊断为PMGCT,第2例在切除标本中诊断,第3例仅在初次治疗6个月后局部复发后才诊断。所有患者在复发和转移检测后5个月内死亡。PMGCT预后很差。组织学检查在这类病例中具有重要意义。认识到这一实体、进行充分的活检以及标本采样有助于早期诊断,这可能改善总体预后。

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