软组织多形性透明变性血管扩张性肿瘤

软组织多形性透明变性血管扩张性肿瘤（pleomorphic hyalinizing angiectatic tumor of soft parts,PHAT）是一种新近认识的软组织肿瘤类型,此肿瘤由Smith等,于1996年首先报道。肿瘤界限较清楚,镜下显示3个特征性的形态学改变：扩张性的薄壁血管呈簇状分布;分布于扩张血管之间的多形性瘤细胞;血管壁、血管周围及瘤细胞之间伴有明显的透明变性。     

软组织多形性透明变性血管扩张性肿瘤

目的：探讨软组织多开矿性透明变性血管扩张性肿瘤的临床病理学特点。方法：对２例软组织多形性透明变性血管扩张性肿瘤进行光镜观察和免疫组化检测。结果：１例为５８岁女性,表现为颈部皮下缓慢性生长的肿块;另１例为３１岁男性,表现为右后腹膜内肿块。眼观,肿瘤境界均较清楚,镜检,显示以下三个特征性的形态学改变：（１）呈簇性分布扩张性的薄壁血管;（２）分布于扩张血管之间的多形性瘤细胞;（３）血管壁、血管周围及瘤细     

伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤的临床病理观察

目的 探讨伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤的病理学特点、诊断及鉴别诊断要点.方法 采用HE及免疫组织化学(EnVision法)方法 ,对7例分别发生于眼眶及眶外的伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤进行病理学分析.结果 2例为发生于眼眶的肿瘤,其中1例为复发病例;5例为眶外肿瘤.镜下特征:肿瘤境界清楚,由无明显异型性的圆形或梭形的肿瘤细胞与胶原混杂组成,富含血管,部分区域形成假血管腔隙样结构,多核巨细胞衬于腔隙的内壁或散布于间质中.免疫组织化学标记显示肿瘤细胞和巨细胞表达CD34.7例均行肿块切除术,术后随访4例,均无复发.结论 伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤是一种眼眶和眶外组织的中间型软组织肿瘤,局部切除多可治愈,组织学上需与巨细胞纤维母细胞瘤、软组织多形性透明变性血管扩张性肿瘤、血管瘤样型纤维组织细胞瘤相鉴别.     

软组织多形性玻璃样变血管扩张性肿瘤的临床病理学观察

目的探讨软组织多形性玻璃样变血管扩张性肿瘤(pleomorphic hyalinizing angiectatic tumor of soft parts,PHAT)的临床病理特点。方法回顾性分析6例PHAT的临床资料、组织学形态和免疫组化标记,并复习相关文献。结果6例均发生于成年人,男性和女性各占3例,平均年龄为44岁。肿瘤分别位于下肢(3例)、颈部(2例)和腹膜后(1例)。临床上多表现为无痛性肿块,术前病程为3个月~38年。大体上,肿瘤境界清楚,结节状,平均直径为4.3 cm。切面呈灰白、灰红或灰褐色,部分病例可见出血性囊腔。镜下,肿瘤内含有成簇分布的薄壁扩张血管,血管壁附有一层厚的玻璃样变物质(纤维素和胶原纤维),可向血管周围间质内延伸,血管之间为片状分布的多形性瘤细胞,核大深染,核内可见假包涵体,但核分裂象罕见。部分区域内瘤细胞呈梭形,排列成条束状,类似神经鞘瘤。免疫组化标记显示,瘤细胞主要表达vimentin,部分病例还表达CD34和CD99,而S-100蛋白、α-SMA、bcl-2、EMA和CD68均为阴性。6例均经手术切除,随访8个月~13年,无1例复发。结论PHAT是一种具有低度恶性潜能的软组...     

肾脏上皮样血管平滑肌脂肪瘤的病理观察

目的对肾脏上皮样血管平滑肌脂肪瘤（epithelioid agiomyolipoma,EAML）的病理诊断、鉴别诊断和预后进行分析。方法2例肾脏EAML（其中1例为复发病例）,复习其临床资料,病理学检查包括常规病理学、免疫组织化学和超微结构,并进行随访。结果光镜下肿瘤均主要由具有多形性和不典型性的上皮样细胞组成,部分区域有明显的血管周上皮样排列;可见出血和坏死;并可见静脉内瘤栓;淋巴结内可见上皮样肿瘤细胞累及。免疫组织化学肿瘤细胞（包括淋巴结内肿瘤）HMB45、平滑肌肌动蛋白（SMA）、神经元特异性烯醇化酶（NSE）和波形蛋白弥漫阳性;S-100、melanpan和CD68散在阳性;而上皮细胞膜抗原（EMA）、AE1／AE3、CK7、CD117、肌肉特异性肌动蛋白（MSA）、结蛋白、白细胞共同抗原（LCA）、CD20、CIM5RO、CD30、CD15、嗜铬素（CgA）、突触素（Syn）、bcl-2、雌孕激素受体（ER、PR）和p53均为阴性。电镜检查可见一些肿瘤细胞内有黑色素小体样的致密颗粒、肌丝、密体,肿瘤细胞外可见不连续的基膜。2例患者手术后10个月状态良好,无肿瘤局部复发和转移征象。结论血管周上皮样排列、寻找经典血管平滑肌脂肪瘤的结构和肿瘤细胞表达HMIM5和SMA对于诊断和鉴别诊断至关重要。而细胞的不典型性、出血坏死和核分裂象可能只表明肿瘤的恶性潜能：淋巴结受累、肾静脉瘤栓不是恶性的诊断依据：只有远处转移才是恶性的证据。     

鼻腔鼻窦血管外皮瘤样肿瘤4例临床病理分析

目的探讨鼻腔鼻窦血管外皮瘤样肿瘤的临床病理学特征、诊断、鉴别诊断及免疫表型。方法回顾性分析4例鼻腔鼻窦血管外皮瘤样肿瘤的临床资料、病理学特征及免疫表型,并复习相关文献。结果 4例患者中男、女各2例;平均年龄62岁;3例发生于右鼻腔,1例发生于左鼻腔;镜下见肿瘤界限清楚,无包膜,由形态较一致的短梭形或卵圆形肿瘤细胞构成,呈席纹状或条束状排列,伴毛细血管增生和鹿角状血管壁透明变性。术后平均随访41个月,均未见复发和转移。免疫表型:4例均弥漫表达vimentin、SMA、β-catenin和Cyclin D1,Ki-67增殖指数3%～5%;其中2例弱表达BCL-2;1例弱表达CD99、CD117;desmin、CD34均不表达。结论鼻腔鼻窦血管外皮瘤样肿瘤属于中间性肿瘤,预后较好;结合病理学形态和免疫表型可明确诊断。     

子宫血管周上皮样细胞肿瘤临床病理观察

目的研究子宫血管周上皮样细胞肿瘤的病理学特征、诊断、鉴别诊断和生物学行为。方法对5例子宫血管周上皮样细胞肿瘤进行常规组织学和免疫组织化学（SP法）染色和观察,对患者进行随访,并复习相关文献。结果光镜下5例肿瘤均由透明或嗜酸细胞巢或宽窄不等的细胞索组成,间质有丰富的小血管和程度不等的透明变。免疫组织化学染色示5例瘤细胞均黑色素细胞标记阳性和程度不等的结蛋白和平滑肌肌动蛋白（SMA）阳性,CK和CD10阴性。5例患者现均存活。结论子宫血管周上皮样细胞肿瘤具有较特征性的组织病理及免疫组织化学特点,HMB45阳性对诊断有重要作用。该肿瘤分良性、恶性潜能不能确定和恶性三类,应与透明细胞癌和上皮样平滑肌肿瘤区别。     

后肾间质肿瘤的病理观察

目的观察后肾间质肿瘤的临床病理表现,探讨其有病理诊断价值的组织学特征。方法对2例后肾间质肿瘤进行HE染色及免疫组织化学染色（EnVision法）,并进行观察。结果后肾间质肿瘤为单侧性肾占位,肿瘤位于肾髓质,边界清,呈“扇贝形”与相邻肾组织连接。镜下主要表现为：肿瘤内可见单个的肾小球和肾小管,梭形或星形肿瘤细胞呈“葱皮样”围绕肾小管和肾血管排列,细胞分布不均匀,在低倍镜下形成“结节样”图像;大部分肿瘤中内陷的小血管壁发育不良。肿瘤细胞CD34阳性表达。结论近肾小管区细胞和血管组成的“结节样”图像和CD34阳性是具有诊断和鉴别诊断价值的组织学特征。     

腹腔上皮样炎性肌纤维母细胞肉瘤临床病理分析并文献复习

目的：通过对上皮样炎性肌纤维母细胞肉瘤(epithelioid inflammatory myofibroblastic sarcoma, EIMS)的临床病理学特征的分析,提高对其诊断认识能力,减少误诊。方法：分析3例EIMS的临床病理特征并复习相关文献。结果：术后镜下示：肿瘤细胞弥漫分布,呈圆形、上皮样,胞浆丰富、嗜酸性、核偏位,核仁明显,部分可见梭形细胞,背景显著黏液变性伴突出的炎症细胞浸润,以中性粒细胞为主。免疫表型：肿瘤细胞表达ALK、Desmin、CD30、EMA、INI-1;部分表达：CK和SMA;不表达LCA、CD15、MyoD1、S-100、HMB-45、CD20、CD3、CD34、CD117和DOG-1。FISH检测显示3例均有ALK基因相关易位。结论：EIMS是高度恶性肿瘤,发生率比较低,细胞学形态特征、免疫组织化学特点及分子特征对本病的诊断具有重要价值。     

腹膜后恶性血管周上皮样细胞肿瘤的临床病理分析

目的:探讨腹膜后血管周上皮样细胞肿瘤(perivascular epithelioid cell neoplasms,PEComas)的临床和病理学特点,提高对PEComas的认识和诊断水平。方法:对1例腹膜后恶性PEComas进行临床病理分析及免疫组织化学研究,并复习相关文献。结果:肿瘤组织主要由上皮样细胞构成,胞质丰富、透明或嗜酸性颗粒状,间质富于血管。免疫组织化学显示:肿瘤细胞平滑肌肌动蛋白、HMB45,melan A及Desmin阳性,上皮膜抗原和vimentin局灶阳性,CgA,Syn,S-100,CD117,CD34,细胞角蛋白,calretinin及inhibin均阴性。结论:腹膜后PEComas是一种非常罕见的肿瘤,具有独特的组织学和免疫组织化学特征,应与腹膜后其他上皮样细胞肿瘤进行鉴别。     

Pleomorphic hyalinizing angiectatic tumor of soft parts: Case Report with unusual ganglion-like cells and review of the literature

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a recently described, non-metastasizing tumor of uncertain lineage. This tumor distributes equally between the genders and has a predilection for the subcutaneous soft tissue, particularly in lower extremity, other locations are rare. Based on the recent literature, PHAT is suspected to encompass the morphological spectrum with other tumors such as myxoinflammatory fibroblastic sarcoma (MIFS) and hemosiderotic fibrolipomatous tumor (HFLT), although cytogenetic data remain inconsistent. We report a case of PHAT that arose in the upper arm with unusual morphology which showed ganglion-like cells similar to Reed-Sternberg-like cells found in MIFS. The tumor had strong immunohistochemical expression of CD34, CD99, and was negative for S-100. The ganglion-like cells were positive for both CD34 and CD68 but negative for CD30. The translocation between chromosome 1 and 10, a frequent finding of MIFS and HFLT, was not identified by FISH excluding the possibility of hybrid PHAT and MIFS. We conclude FISH can be a potential useful tool to separate PHAT with atypical morphology from hybrid tumor in doubted cases. Due to the rarity of PHAT and lack of consistent pathogenetic signatures, more cases and further studies will be needed to elucidate the pathogenesis and nature of this tumor.     

Pleomorphic hyalinizing angiectatic tumor of soft parts

A case of recently descrlbed pleomorphic hyaliniring anglectatic tumor (PHAT) of soft parts Is reported. The subcutaneous solid tumor arising In the axils of a 58-year-old man was histologically characterized by sheets of mitotic ally Inactive oval and pleomorphic cells, Itranuclear cytoplasmic Inclusions, and clusters of ectatic vessels with perivascular hyalinization. Mono- and multinucleated giant cells were also present. A hemanglopericytoma-like pattern of vascularity, pseudovascular spaces, stromal collagen with degenerative changes, and Immunoreactivity for CD34 were observed. Since these features were very similar to those of solitary fibrous tumors of various sites and newly categorized giant cell angiofibroma, it Is considered that PHAT, solitary fibrous tumor, and giant cell angiofibroma may be In the same family of tumor. The tumor was diploid with a low S-phase fraction. The patient was well with no evidence of disease for 23 months.     

Pleomorphic hyalinizing angiectatic tumor with a sarcomatous component recurring as high-grade myxofibrosarcoma

Pleomorphic hyalinizing angiectatic tumor (PHAT) of the soft tissue is a rare distinctive tumor listed as a benign neoplasm in the new World Health Organization classification. It may recur and most reported recurrent tumors retained the typical morphological appearance of PHAT; rare tumors recurred with the appearance of a sarcoma. Reported herein is an additional example of recurrent PHAT, but in contrast to the previously described cases the present tumor morphologically qualified as a sarcoma from the very beginning; it recurred as a high-grade myxofibrosarcoma. A 76-year-old woman presented with a solitary subcutaneous tumor in the axilla that was surgically removed. Seven months later, the patient experienced a local recurrence. Microscopically, the typical features of PHAT were identified in the initial lesion, namely hyalinized, fibrin-containing vessels and pleomorphic stromal cells; there were areas of hemorrhage and necrosis. Additionally, peripherally located areas of the tumor manifested highly pleomorphic cells with frequent atypical mitoses, producing a sarcomatous appearance. The mitotic index in the sarcomatous part was 1/10 high-power fields (HPF). Hyalinized, fibrin-containing vessels were absent in these sarcomatous areas, and the stroma was myxoid. The recurrent lesion was composed of large highly pleomorphic oval, round, spindled or bizarre cells with a high mitotic rate, ranging from 3/10 HPF to 7/10 HPF. The neoplastic cells were arranged haphazardly in a myxoid matrix. Hyalinized, fibrin-containing vessels typical for PHAT were absent. PHAT may be more aggressive than previously thought, and PHAT may encompass a morphological spectrum of the lesion ranging from benign to malignant.     

Pleomorphic hyalinizing angiectatic tumor of the male breast: a heretofore unreported occurrence

Pleomorphic hyalinizing angiectatic tumor (PHAT) is considered an unusual mesenchymal tumor of intermediate malignant potential that is distinguished by a prominent angiectatic vasculature with clusters of thin-walled, fibrin-lined vessels surrounded by sheets of spindled and pleomorphic cells. This lesion occurs mostly in the subcutaneous soft tissues of the lower extremities. In this paper, the authors report the first case of PHAT involving the breast parenchyma of a 75-year-old man. The patient was referred to the hospital with a history of a painless left breast mass that had been growing for several years. The lesion did not recur after surgery. The tumor exhibited histopathologic and immunohistochemical features identical to those of a PHAT of other sites. The purpose of this report is to add a rare tumor to the differential diagnosis of mammary spindle cell neoplasms.     

Pleomorphic hyalinizing angiectatic tumor: cytomorphologic features on fine-needle aspiration

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare low-grade neoplasm occurring predominantly in the subcutaneous tissue of the lower extremity, which is characterized histologically by intermixed bland spindle cells and larger pleomorphic cells, ectatic blood vessels with fibrin, and prominent hyalinization. Here we describe the cytologic features of PHAT seen on fine-needle aspiration of a right calf mass in a 46-year-old man. The smears were cellular, composed predominantly of bland spindle cells dispersed singly as well as in tissue fragments and characterized by open chromatin and small nucleoli. A second population of pleomorphic spindle cells with coarse chromatin, prominent nucleoli, and occasional intranuclear pseudoinclusions was also present. Many of the pleomorphic cells surrounded hyalizined vessels. No mitotic figures were identified. The cytologic diagnosis was a "spindle cell lesion, NOS" with the differential diagnosis including benign lesions and low-grade neoplasms, including PHAT and schwannoma.     

Pleomorphic hyalinizing angiectatic tumor of renal hilum

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a recently described rare entity. The tumor histogenesis is proposed to be of primitive undifferentiated mesenchyme. The tumor has a predilection for the subcutaneous soft tissue especially of lower extremity, although other locations have been well documented. We report a case of PHAT arising in the hilum of the kidney, clinically mimicking an infiltrating malignant neoplasm of renal pelvis. The tumor was discovered during workup for unrelated gastrointestinal tract symptoms. Because of the location of the lesion, excision of mass and radical nephrectomy were performed. The tumor had strong immunohistochemical expression of vimentin, CD34, CD99, and vascular endothelial growth factor (VEGF). No additional lesions were documented during 3 years of follow-up. This is consistent with the current thinking that PHAT is a benign neoplasm with increased incidence of recurrence. We document the unique retroperitoneal location of this rare tumor and suggest that PHAT should be considered among the list of unusual lesions at this site.     

Pleomorphic hyalinizing angiectatic tumor revisited: all tumors manifest typical morphologic features of myxoinflammatory fibroblastic sarcoma,further suggesting 2 morphologic variants of a single entity

We describe 9 cases of pleomorphic hyalinizing angiectatic tumor (PHAT). Recently described TGFBR3 and MGEA5 gene rearrangements in these tumors have confirmed the long-hypothesized link between PHAT and another soft tissue entity, the myxoinflammatory fibroblastic sarcoma (MIFS). Myxoinflammatory fibroblastic sarcoma and PHAT share the same translocation and in addition have a very similar clinical presentation. However, to our best knowledge, no study has ever addressed the striking morphologic similarities between MIFS and PHAT. Our findings based on histological criteria suggest that most, if not all, tumors diagnosed as PHAT might, in fact, represent examples of MIFS that, in addition to a conventional MIFS morphology, manifest aberrant angiectatic hyalinized vessels.     

Pleomorphic hyalinizing angiectatic tumor of soft parts: a case report and literature review

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare, recently recognized neoplasm occurring predominantly in the subcutaneous tissue of the lower limbs of adults. We report a case of PHAT in an 83-year-old woman who presented with a 5.0 x 5.0 x 2.0 cm mass in the soft part of her left thigh. Histologically, the tumor was well circumscribed by a thin fibrous capsule and predominantly composed of fusiform cells with eosinophilic cytoplasm and round-to-oval or pleomorphic nuclei. The tumor cells resembled those of malignant fibrous histiocytoma, but differed from them by less prominent mitotic figures. Immunohistochemically, the tumor cells were diffusely and strongly positive for CD34; partially positive for vimentin and CD99 (MIC-2); and negative for epithelial and non-epithelial markers. Ultrastructurally, the tumor cells had pleomorphic cytoplasm and nucleus. Intermediate-sized cytoplasmic filaments were observed in a few tumor cells, but neurosecretory-type granule-like intracytoplasmic organelles were not seen. These findings suggest that this tumor is derived from stromal fibroblast, such as solitary fibrous tumors or giant cell angiofibroma.     

Pleomorphic hyalinizing angiectatic tumor

Authors present an additional case report of pleomorphic hyalinizing angiectatic tumor (PHAT), only recently recognized entity amongst the soft tissue tumors. 63-year-old man underwent surgery for subcutaneous tumor growing on the forearm. Grossly, ovoid well demarcated tumor 4.0 x 3.5 x 2.5 cm was removed. Histologically, hypovascular areas of spindle-cells arranged in sheets and fascicles resembling spindle-cell sarcoma and areas rich on ectatic vessels with fibrin deposits or thickened hyalinized walls were present. Some cells contained intranuclear cytoplasmatic inclusions, lobulated nuclei with or without prominent nucleoli or multiple nuclei. Mitoses were less than 1 per 30 HPF, Ki-67 nuclear positivity was observed in 2.6% of tumor cells. Immunohistochemically, cells were positive with vimentin, but negative for CD34, S100, desmin, smooth-muscle actin, EMA, VWF, CD99, bcl-2 and cytokeratins. The patient did well, without recurrent tumor after 13 months follow-up. Spectrum of tumors including solitary fibrous tumor, giant-cell angiofibroma, neurilemmoma, malignant fibrous histiocytoma and acral myxoinflammatory tumor with atypical bizarre giant cells is discussed in differential diagnosis.