伴破骨细胞样巨细胞肾肿瘤2例及文献复习

目的 探讨伴破骨细胞样巨细胞肾肿瘤(RT-OGC)的临床病理特点。方法 观察2例RT-OGC的病理形态学改变和免疫组织化学检测，并结合文献进行讨论。结果 肾盂移行细胞乳头状癌型1例，破骨细胞样巨细胞与移行细胞癌相连接；肾肉瘤样癌型1例，破骨细胞样巨细胞在肉瘤样成分周围分布或与肉瘤样成分混杂。免疫表型：破骨细胞样巨细胞CD68、Vim、αl-ACT均阳性，CK、EMA均阴性。结论 RT-OGC是少见的肾恶性上皮性肿瘤的一个亚型，破骨细胞样巨细胞起源于间叶组织的单核巨噬细胞系统，是机体对肿瘤的反应，其组织形态要与癌肉瘤和骨巨细胞瘤鉴别。     

伴有破骨细胞样巨细胞的胰腺未分化癌1例报道及文献复习

目的：探讨伴有破骨细胞样巨细胞的胰腺未分化癌的临床病理特点.方法：观察1例伴有破骨细胞样巨细胞的胰腺未分化癌的形态学特征,并进行免疫组织化学染色.结果：伴有破骨细胞样巨细胞的胰腺未分化癌肿瘤主要有两种细胞组成,一种为单核细胞,分为组织细胞样单核细胞和梭形或多形性瘤细胞两型;另一种为多核巨细胞,分为非肿瘤性的破骨细胞样巨细胞和瘤巨细胞两型.免疫组织化学研究显示,这两种细胞Vimentin均阳性,均不表达cytokeratin（AE1/AE3）,CK5/6,CEA,CgA;其中破骨细胞样巨细胞、组织细胞样单核细胞CD45,CD68阳性,而瘤巨细胞和梭形瘤细胞阴性.结论：伴有破骨细胞样巨细胞的胰腺未分化癌是一种罕见恶性肿瘤,可能为胰腺未分化癌的一个亚型.诊断需与胰腺恶性纤维组织细胞瘤、转移的骨巨细胞瘤或黑色素瘤等鉴别.     

骨小细胞恶性肿瘤34例病理形态学研究

目的 研究骨小细胞恶性肿瘤(SCMT)的病理形态和免疫组化特点。方法 应用免疫组化SP法对34例SCMT进行组织学观察。结果 34例SCMT中22例为弥漫型非霍奇金恶性淋巴瘤，其中21例B细胞性，1例T细胞性；瘤组织表达CD45(LCA)、CD20(L26)或C1345RO(UCHL—1)。7例浆细胞肿瘤，其中5例为多发性骨髓瘤、2例为孤立性浆细胞瘤，表现为单一的不同分化程度的肿瘤性浆细胞；免疫组化示6例CD38( )。2例Ewing肉瘤显示排列密集、大小较一致的圆形细胞；肿瘤表达CD99和Vim。1例小细胞骨肉瘤，肿瘤由丰富密集的小细胞和网格状的骨样组织组成，瘤细胞Vim阳性。1例间叶性软骨肉瘤示富于血管的圆形或梭形细胞和透明软骨；瘤细胞表达Vim，软骨细胞表达S—100蛋白。1例小细胞癌示小细胞紧密片巢状排列，表达CK和EMA。结论 骨SCMT组织学类型各有不同的病理形态和免疫组化特征，结合临床和X线表现可作出正确的病理诊断。     

胰腺破骨细胞样巨细胞瘤1例并文献复习

目的探讨胰腺破骨细胞样巨细胞瘤(osteoclast-like giant cells of tumor,OGCT)的临床病理特征、免疫表型、治疗及预后。方法回顾性分析1例胰腺OGCT的临床病理特征和免疫表型,并复习相关文献。结果 OGCT患者CT示胰体尾部囊实性包块,伴广泛出血坏死,由多种形态的单核细胞和破骨细胞样多核巨细胞构成。免疫表型:瘤细胞vimentin、CD68均阳性,CKpan、CEA均阴性,Ki-67增殖指数5%～10%。结论 OGCT属于胰腺罕见肿瘤,病理形态类似骨的巨细胞瘤;治疗以手术完整切除为主,分析其临床病理特征、治疗及预后,有助于提高对该肿瘤的认识水平。     

卵巢黏液性肿瘤伴肉瘤样附壁结节3例临床病理分析

目的 探讨卵巢黏液性囊性肿瘤伴肉瘤样附壁结节的临床表现、病理形态及免疫组化特点.方法 观察3例卵巢黏液性肿瘤伴肉瘤样附壁结节的临床资料、组织学形态、免疫组化特点,并对相关文献进行复习.结果 3例肉瘤样附壁结节主要由破骨样巨细胞、卵圆形的单核细胞以及多少不等的梭形细胞构成.可见核分裂象和病理性核分裂,部分区域细胞显示多形性,类似恶性纤维组织细胞瘤,但结节体积均较小且境界清楚,缺乏血管浸润.单核细胞和梭形细胞vimentin(+),desmin、CK(-);多核巨细胞CD68、vimentin(+),CK、desmin(-).结论 肉瘤样附壁结节是一种良性病变,卵巢黏液性肿瘤伴肉瘤样结节的生物学行为取决于黏液性肿瘤本身的性质.熟悉肉瘤样附壁结节的组织学特征和免疫表型可避免将其误诊为恶性肿瘤.     

胰腺黏液性囊性肿瘤伴浸润性巨细胞型间变性癌1例并文献复习

目的探讨胰腺黏液性囊性肿瘤(mucinous cystic neoplasm,MCN)伴浸润性巨细胞型胰腺间变性癌(anaplastic pancreatic carcinomas,ANPCs)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析1例胰腺MCN伴浸润性巨细胞型ANPCs临床病理资料,并复习相关文献。结果患者老年女性,发现胰周占位病变3年余,行胰体尾及脾脏切除术。镜下见肿瘤由分化较好的MCN及低分化的巨细胞型间变性癌构成,间变性癌区可见瘤巨细胞吞噬中性粒细胞现象。免疫表型:瘤细胞表达CK、CK7、CA199及MUC1,部分表达vimentin,不表达CD20、CD3及CD30。治疗后随访4个月尚未复发。结论胰腺MCN伴浸润性巨细胞型ANPCs罕见,属于胰腺少见的恶性上皮性肿瘤;其中MCN可能为间变性癌的中间过渡状态,最终转化为ANPCs。     

乳腺结节性筋膜炎1例及文献复习

目的探讨乳腺结节性筋膜炎的临床病理特征。方法对1例乳腺结节性筋膜炎的临床表现、组织形态和免疫表型进行研究,并文献复习。结果该例肿块3cm×2cm,边界不清,向周围乳腺及脂肪组织浸润。肿块主要由梭形细胞组成,其中含少量破骨样巨细胞。梭形细胞表达Vim、SMA和MSA,破骨样巨细胞表达CD68(KP-1),未发现CK阳性细胞。结论结节性筋膜炎很少复发,也不转移,组织学形态易与一些良恶性肿瘤混淆,需做免疫组化检测以明确其性质。     

乳腺上皮样型管周间质肉瘤病理特征及与叶状肿瘤比较观察

目的 探讨乳腺上皮样型管周间质肉瘤的临床病理特点及与叶状肿瘤的关系。方法 采用HE、特殊染色、免疫组化染色(CK，EMA，S-100蛋白，SMA，Vim，Des，MG，CD34，CD99，CD117，PR，HMB45)对1例乳腺上皮样型管周间质肉瘤与5例叶状肿瘤(良性、交界性各1例，恶性3例)做比较性观察。结果 乳腺管周间质肉瘤(上皮样型)有独特的镜下图像：①显著的多角形(上皮样)细胞绕导管或小管的上皮肌上皮层呈间质性增生，无叶状结构；②组织学模式有袖套状、花冠状、菊形团状、结节状、融合结节状和片状浸润；③瘤细胞形态有：多角形(大、小)、柱状和梭形。多角形细胞呈上皮样形态，异型明显，核分裂象多见(10～30个／10HPF，个别区域达6个／1HPF)，病理性核分裂象易见，在浸润灶内见肿瘤性坏死；④瘤细胞Vim弥漫阳性、EMA灶性阳性、CD99和CD117灶性弱阳性、CD34少数阳性，CK、SMA、S-100蛋白、Des、MG、PR、HMB45均阴性。5例叶状肿瘤均具备叶状结构、间质过度增生、细胞密集(异质性分布)、核分裂象2～10个／10HPF等诊断要素。在3例恶性叶状肿瘤中，2例有极少的上皮样袖套状病灶，2例有梭形细胞袖套状病灶。结论 乳腺上皮样型管周间质肉瘤是一种极罕见的恶性纤维上皮肿瘤亚型，它可能是恶性叶状肿瘤的最早期病变，也可能是一种独特的类型。     

颅内纤维组织细胞瘤临床病理,免疫组化研究

本文对18例(包括复发病例共27例次)颅内纤维组织细胞瘤进行临床病理、免疫组化研究。所有病例光镜下均有典型或不典型轮辐状结构、组织细胞样和纤维母细胞样细胞、未分化细胞、黄色瘤细胞、多核瘤巨细胞等,并用电镜证实。27例次和颅外对照组5例均行免疫组化分析:α_1—AT、α_1—ACT、Lys、Vim、ConA、EMA、和GFAP,部分病例加做F8RAg、UEA、FN及S—100、CK、CEA,均采用ABC法。结果表明,α_1—AT、α_1—ACT、Lys(组织细胞及其肿瘤标记物)以及Vim、ConA免疫组化染色均呈不同程度阳性,α_1—AT、α_1—ACT、Vim、Con A表达较强、Lys较弱,其它标记物阴性,与对照组一致。本病发生率为0.45%,中青年男性多见,肿瘤多位于脑膜,容易复发,不易转移。     

胰腺实性-假乳头状瘤8例临床病理分析

目的 探讨胰腺实性-假乳头状瘤(SPT)的临床病理学及免疫组化特点。方法 8例SPT作HE、PAN、免疫组织化学(SP法)染色观察。结果 8例SPT中7例为女性，1例男性，年龄16～63岁，平均33．1岁。术后均无复发。肿瘤体积较大，平均直径7．9cm。有包膜，囊实性相间。镜检：肿瘤由乳头和囊实区混合组成，细胞形态一致，核圆或卵圆，异型不明显，核分裂象罕见。瘤细胞围绕纤维血管轴心形成特征性假乳头结构。2例侵犯胰腺组织。8例Vim、α1-AT、α1-ACT和NSE均阳性，4例Syn、CgA和AE1／AE3阳性，5例CD56阳性，5例PR阳性；Glu、Ins、Pol、EMA、p53、Ki-67、ER均阴性。结论 胰腺SPT好发年轻女性，具有独特的临床病理特点，手术切除治愈率高，应视为低度恶性肿瘤，免疫组化提示可能起源于胰腺多能干细胞。     

Giant cell tumor of the pancreas of mixed osteoclastic and pleomorphic cell type: evidence for a histogenetic relationship and mesenchymal differentiation

We describe a giant cell tumor of the pancreas composed of a mixture of osteoclastic and pleomorphic cell types. This rare tumor had a unique immunohistochemical profile. Both types of tumor giant cells stained for vimentin, alpha-1-antitrypsin, alpha-1-antichymotrypsin, synaptophysin, muscle actin, and neuron-specific enolase, but not for epithelial markers. Electron microscopy showed cells which resembled primitive fibroblasts and osteoclast with no epithelial features. These findings are most consistent with mesenchymal differentiation. The extensive homologies in immunohistochemical staining of both osteoclastic and pleomorphic giant cells in this case indicates that these cells are histogenetically related.     

Osteoclast-like giant cell tumor of the pancreas: an immunohistochemical and ultrastructural study

We report a rare case of osteoclast-like giant cell tumor of the pancreas in a 70-year-old Japanese woman. The tumor was composed of a proliferation of ovoid to spindle-shaped mononuclear cells admixed with osteoclast-like giant cells. The tumor cells were immunore-active for vimentin, ±₁-antitrypsin, and CD68. In ultrastructural examination, the giant cells resembled osteoclasts, and the mononuclear stromal cells had fibroblastic and histiocytic features. No elements of epithelial differentiation were found in this tumor. These findings suggest that this tumor had a derivation similar to giant cell tumor of bone.This study was presented at the 28th Annual Meeting of the Clinical Electron Microscopy Society of Japan, Osaka, October 17–19, 1996.     

Combined hepatocellular carcinoma and osteoclast-like giant cell tumor of the liver: Possible clue to histogenesis

Hepatic giant cell tumor is extremely rare, and only five cases have been reported of overt hepatocellular carcinoma, thus its histogenesis is controversial. Herein is reported a case of simultaneous hepatocellular carcinoma and osteoclast-like giant cell tumor in a single tumor. A liver tumor was found in a 74-year-old woman. Histologically the tumor consisted of two distinct components: mononuclear and multinuclear giant cells with osteoclastic giant cells, and a conventional hepatocellular carcinoma. The boundary between the two components showed transitional features. Immunohistochemistry showed that the osteoclast-like giant cells were CD68 and vimentin positive, but cytokeratin and AFP negative, while spindle-shaped cells were positive only for vimentin. In a portion of the hepatocellular carcinoma the cells were cytokeratin-8 and AFP positive. Ki-67 positivity was 10% for the hepatocellular carcinoma, 60% for the spindle-shaped cells, and 0% for the giant cells. It is possible that the tumor might have had a hepatocellular carcinoma origin, given the more highly proliferative sarcomatous changes and reactive osteoclast-like cells. This case provides a clue to the histogenesis of hepatic giant cell tumors.     

Osteoclast-like giant cell tumor of the pancreas: immunophenotypic similarity to giant cell tumor of bone

An immunophenotype was performed on an osteoclast-like giant cell tumor of the pancreas using a panel of antibodies to epithelial and leukocyte antigens. Several antibodies to cytokeratin and carcinoembryonic antigen were negative in the tumor. Osteoclast-like cells were positive for CD4, CD13, CD45, CD68, CD71, and vimentin, but negative for lysozyme and HLA-DR. Mononuclear tumor cells were positive for CD4, CD11c, CD13, CD14, CD45, CD68, CD71, HLA-DR, and vimentin, but negative for lysozyme. The phenotype is similar to that previously described for giant cell tumor of bone. The osteoclast-like cell phenotype is also similar to that reported for normal osteoclasts. The findings support a nonepithelial origin for osteoclast-like giant cell tumor of the pancreas, and suggest a derivation similar to giant cell tumor of bone.     

Ductal adenocarcinoma of the pancreas with huge cystic degeneration: a lesion to be distinguished from pseudocyst and mucinous cystadenocarcinoma

Cystic neoplasms of the pancreas are rare and often mistaken for pseudocyst by imaging studies and macroscopic examination. We describe an unusual tumor of the pancreas composed of a mural nodule of anaplastic carcinoma arising from a huge ductal adenocarcinoma undergoing cystic degeneration. The cyst measured 27 x 13 x 4 cm. Light microscopy showed that the cyst was partly lined by a single layer of cuboidal to columnar tumor cells with focal mucin production and was surrounded by hyalinized connective tissue. Most lining epithelial cells were absent owing to extensive degenerative process. Immunohistochemical studies showed positive staining of cytokeratin and vimentin for pleomorphic giant tumor cells, which were negative for leukocyte common antigen (CD45), KP-1 (CD68), epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The ductal adenocarcinoma stained strongly positive for cytokeratin and EMA, and negative for vimentin, CD45, CD68, and CEA. The clinical course of the current case was extremely poor and the prognosis resembled that of an anaplastic carcinoma. Therefore, we like to emphasize the importance of complete excision and extensive sampling of any cystic neoplasms in the pancreas including those with large cystic component to avoid missing the malignant elements.     

Undifferentiated pancreatic carcinoma with osteoclast-like giant cells: Report of a case with osteochondroid differentiation

We report a case of an undifferentiated pancreatic carcinoma with osteoclast-like giant cells with focal osteochondroid differentiation in a 66-year-old man, who presented with painless jaundice, pruritis, and weight loss. Imaging studies revealed an inhomogeneous mass in the head of the pancreas. A pylorus preserving pancreaticoduodenectomy was performed. The resection specimen revealed a 9.5×4.2×3.2 cm³ solid neoplasm in the pancreatic head with direct extension into duodenum and common bile duct. Microscopy showed a cellular neoplasm composed of pleomorphic mononuclear cells (pancytokeratin, and EMA-positive; LCA, and CD68 negative) and osteoclast-like multinucleated giant cells (vimentin, LCA, and CD68-positive; pancytokeratin, and EMA-negative) consistent with OGTP. The tumor contained a focal area of osteochondroid differentiation. Evidence supports that the tumor giant cells are non-neoplastic and of histiocytic origin. Osteochondroid differentiation within undifferentiated carcinoma is unusual; its presence might suggest a sarcoma diagnosis on biopsy material.     

Osteoclast-like giant cell tumour of the pancreas presenting as a pseudocyst-like lesion

 A 57-year-old male patient presented with a cystic lesion in the tail of the pancreas, which was considered to be a pseudocyst. He was treated by cystojejunostomy but one year later a tumour was found to have invaded the stomach and jejunum. This was an osteoclast-like giant cell tumour containing a small area of typical ductal adenocarcinoma. Immunohistochemical staining revealed that the pleomorphic tumour cells were positive for cytokeratin, epithelial membrane antigen, vimentin and the proliferation marker MIB-1. The osteoclast-like giant cells and some small histiocytic cells stained for leukocyte common antigen and histiocytic markers and were negative for MIB-1. At autopsy, tumour rests were found in the pancreas but there were no metastases. Osteoclast-like giant cell tumours of the pancreas may present as cystic lesions and should be included in the differential diagnosis of pseudocysts. Received: 19 December 1996 / Accepted: 20 March 1997     

Giant-cell containing neoplasms of the pancreas: an aspiration cytology study

Giant-cell containing neoplasms of the pancreas are rare with few reports documenting their cytologic appearance. Giant-cell containing neoplasms of the pancreas have been divided into two subtypes corresponding to the osteoclastic giant-cell tumor of the pancreas and the pleomorphic giant-cell carcinoma of the pancreas. Despite the better prognosis reported in some series for osteoclastic giant-cell tumors, the most recent edition of the World Health Organization classification lumps the two entities into a single category designated as undifferentiated carcinoma with osteoclast-like giant cells. Smears obtained from osteoclastic giant-cell tumors show numerous giant-cells with clustered overlapping, bland appearing nuclei containing prominent nucleoli consistent with an osteoclast-type multinucleated giant-cell. These neoplasms contain a second population of mononuclear cells showing more marked nuclear atypia. Pleomorphic giant-cell carcinomas are characterized by anaplastic giant-cells displaying marked nuclear pleomorphism. The mononuclear component is also pleomorphic with markedly atypical epithelioid and spindle shaped cells. In three reported cases, a tumor contained a mixture of the two cell patterns. Thus, undifferentiated carcinoma with osteoclast-like giant cells and pleomorphic giant cell carcinoma may represent a morphologic spectrum with pure osteoclast-like giant-cell tumors at one end and pleomorphic giant-cell carcinoma at the other. Fine-needle aspiration specimens from pure osteoclast-like giant-cell tumors will contain a population of bland multinucleated osteoclastic-like giant-cells that differ markedly from the anaplastic giant-cells of pleomorphic giant-cell carcinoma. The difference in the appearance of the giant-cells aids in distinction of the two neoplasms. When in pure form, the two neoplasms may follow different clinical courses.     

Plexiform fibrohistiocytic tumor. Report of a case involving preoperative aspiration cytology and immunohistochemical and ultrastructural analysis of surgical specimens.

A typical case of plexiform fibrohistiocytic tumor (Enzinger and Zhang) occurring in the skin and subcutis of the abdominal wall in a 7-year-old girl is reported. Preoperative fine-needle aspiration cytology revealed a benign lesion with fibroblastic-histiocytic features which also contained bi- and multinucleated giant cells. The surgical specimen showed a tumor with multiple small nodules within fibrous septa; these nodules were composed of spindle cells and epithelioid cells and contained scattered multinucleated osteoclast-like cells. The tumor cells showed ultrastructural and immunohistochemical features of myofibroblasts and histiocyte-like cells. Thus, there was an abundance of lysosomes, prominent filopodia and bundles of thin cytofilaments along the cytoplasmic border, as well as immunoreactivity for alpha-smooth-muscle-specific actin, alpha-1-antitrypsin and alpha-1-antichymotrypsin. Ultrastructurally there were tumor cells exhibiting features of histiocytes which also contained bundles of actin of smooth muscle type. The presented case of plexiform fibrohistiocytic tumor appears to be composed of a rather peculiar cell form, somewhere between myofibroblasts and histiocytes.