富于巨细胞性骨肉瘤(GCRO)在临床病理工作中比较难遇到，由于含有大量巨细胞，所以很像是骨巨细胞瘤。 

GCRO的主要特点是：

1）影像学：干骺端溶骨的改变，局部伴有新骨形成；

2）组织学：非典型性肿瘤细胞，含有肿瘤性骨样组织，大量多核巨细胞。

这些特征是诊断与鉴别诊断的关键因素。

Radiol Med. 2013 May 28. [Epub ahead of print]

Giant cell-rich osteosarcoma in long bones: clinical, radiological and pathological features.

Wang CS, Yin QH, Liao JS, Lou JH, Ding XY, Zhu YB.

Source

Department of Radiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, No.197, Ruijin 2nd Road, Shanghai, 200025, China.

Abstract

PURPOSE:

The purpose of this study was to review the clinical presentation, imaging, pathology and outcome of patients withgiant cell-rich osteosarcoma (GCRO) of long bones.

MATERIALS AND METHODS:

Radiography (n=9), magnetic resonance imaging (MRI) (n=6), computed tomography (CT) (n=3) and clinical course of nine patients (five males and four females; mean age, 26 years) with pathologically confirmed GCRO were retrospectively reviewed. Specific imaging findings, including size, eccentricity, ossification, lysis, cystic change, expansile growth, periosteal reaction, cortical destruction, soft tissue extension and joint involvement were documented.

RESULTS:

Presenting symptoms were pain in six patients and pain and palpable mass in three. An ill-defined margin surrounding a predominantly osteolytic lesion was detected at the proximal tibia (n=7) or femur (n=2) on imaging studies. Seven cases showed limited ossification. Three cases had tumours in the metaphysis and six in the metaepiphysis. The average maximum tumour dimension was 4.7 cm×5.2 cm×7.8 cm. Microscopically, tumours were composed of atypical cells with scanty osteoid formation and multinucleated giant cells. All patients received chemotherapy, and surgery was performed in eight patients. Three patients were dead and six were alive at the last follow-up.

CONCLUSIONS:

GCRO is a rarer variant that has very close resemblance to giant cell tumour. Patients usually present nonspecific symptoms of pain and palpable mass. It usually shows an osteolytic lesion with locally spared new bone formation in the metaphysis and/or metaepiphysis on imaging. Histologically, the atypical tumour cells with osteoid formation and multinucleatedgiant cells are the key factor in the diagnosis and differential diagnosis.

 本例富于巨细胞性髓内高分化骨肉瘤，是不多见的，鉴别巨细胞瘤，二者鉴别还是很难的啊。

一要结合影像学；

二要结合临床；

三要结合大体观与仔细看组织切片；

四要结合IHC的特殊性，即P63阳性细胞如此丰富。

最后病理诊断：

（左胫骨近端）：富于巨细胞性髓内高分化骨肉瘤，肿瘤局部侵犯骨皮质。

鉴别诊断

o      骨巨细胞瘤

o      软骨母细胞瘤

o      软骨粘液样纤维瘤

o      髓内高分化骨肉瘤

瘤段截除组织IHC

o      Ki-67(5%+) 、P63(+)

o       S-100(软骨成份＋)

进一步HE和IHC图片。

我们的许多网友是在上海市第六人民医院进修的，请发言。谢谢！

在看看截骨的肿瘤组织切片。

让我们来看看手术截骨的标本。

瘤段截除标本大体检查：截除胫骨近端长9cm，剖面于髓腔内见灰白色肿块，一侧紧靠胫骨平台关节面，距另一侧切缘3cm，肿瘤范围约为5*4.5cm，质地中等。

 图10组织学，见上方中间处有肿瘤骨样组织。

请仔细阅读影像学图片、组织学图片，结合IHC标记结果。

请发言。谢谢！

主要IHC图片

活检组织IHC标记

o      阳性：P63（+）、Ki-67（5%+）、p53（弱+）、PGM-1（组织细胞+）

o      阴性：S-100（-）

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请先看看影像学和组织学特点，您将诊断什么？