肺淋巴管肌瘤病的临床病理分析

目的 探讨肺淋巴管肌瘤病（pulmonary lymphangioleiomyomatosis,PLAM)的发病特点、临床病理特征及预后.方法 对7例PLAM进行临床资料分析、组织形态观察及免疫组化研究,并复习相关文献.结果 7例均为女性,临床表现为进行性呼吸困难、自发性气胸,X线及CT示双肺弥漫性、多发性小囊肿,镜下见异常平滑肌样细胞围绕支气管、血管和淋巴管分布,并向周围延伸;HMB-45、SMA、ER和PR可均阳性.结论 PLAM是一种罕见的肺疾病,以平滑肌细胞样的异常增生及浸润为特点,根据临床和组织形态特点,结合免疫表型可确诊,HMB-45对该病具有诊断意义.     

肺淋巴管平滑肌瘤病临床病理学特征

目的探讨肺淋巴管平滑肌瘤病(pulmonarylymphangioleiomyomatosis,PLAM)临床和病理特征。方法对3例PLAM患者的临床资料、HE及免疫组化染色结果进行分析,并结合文献进行复习。结果PLAM是一种持续发展的弥漫性肺疾病。只发生在女性,特别是绝经前妇女。临床表现为反复发作的自发性气胸、活动后呼吸困难、咯血和乳糜胸等。高分辨率CT(HRCT)可见两肺弥漫性分布的薄壁小囊状改变。组织学特点为肺淋巴管、小气道、小血管的管壁及其周围的平滑肌细胞弥漫性异常增生。免疫组化结果显示3例均表达HMB-45、SMA、actin、MMP-2、desmin,2例表达PR、ER。经随访,有2例死亡。结论育龄期妇女发生渐进性呼吸困难,并反复出现气胸,胸部HRCT示两肺弥漫性分布薄壁囊状改变,临床上应考虑到PLAM的可能,最好能行肺组织活检明确诊断。PLAM在影像学与病理组织学上有特征性表现,免疫组化HMB-45阳性具特异性,预后较差。     

淋巴管平滑肌瘤病2例临床病理学分析

目的探讨淋巴管平滑肌瘤病(lymphangioleiomyoma-tosis,LAM)临床、影像、病理特征及预后特点。方法对发生于纵隔及合并肺癌的2例LAM的临床资料、组织学及免疫表型进行分析,并复习相关文献。结果 LAM发病原因尚不明确,好发于育龄期女性。病变的发生与淋巴管和血管密切相关,不同发病部位临床症状各不相同。显微镜下示淋巴管、血管壁周围可见不同成熟度的平滑肌细胞弥漫性异常增生,肿瘤细胞可呈梭形或上皮样。免疫表型:梭形肿瘤细胞desmin和SMA阳性,HMB-45和MELAN-A阴性。结论LAM是一种罕见的慢性、进行性恶化的肿瘤性疾病,常累及全身多个系统,肺是主要累及的器官。依据临床症状、影像学和组织学特点再结合免疫表型可明确诊断。     

心包原发性恶性间皮瘤的临床病理分析

目的：探讨心包原发性恶性间皮瘤的临床病理特征。诊断与鉴别诊断要点。方法：对4例心包原发性恶性间皮瘤进行临床病理分析。光镜及免疫组化染色观察并复习有关文献。结果：男3例,女1例,平均年龄42岁,3例呈局限型,1例为弥漫浸润型。组织学上可表现为肉瘤样梭形细胞型,上皮样型及双相型,免疫组化染色显示肉瘤样梭形细胞表CK、vimentin;上皮样型瘤细胞表达HBME1、CK。结论：原发于心包的恶性间皮瘤罕见,预后极差。临床常被误诊,其组织形态亦复杂多样,应注意与心包的良性增生性病变,心包转移性腺癌和梭形细胞肿瘤等相鉴别。     

肝脏血管平滑肌脂肪瘤9例临床病理分析

目的探讨肝脏血管平滑肌脂肪瘤(hepatic angiomyolipoma,HAML)的临床病理特点、诊断及鉴别诊断。方法对9例HAML进行临床病理学观察与免疫组化标记及复习有关文献。结果 9例HAML年龄28～56岁,平均44.2岁,均无结节硬化症。术前影像诊断多数为肝癌。眼观:肿瘤直径1.2～14 cm。肝左叶4例、肝尾状叶3例和肝右叶2例。肿瘤境界清楚,无包膜,质地脆、易碎,可见出血、坏死。镜检:瘤组织由上皮样细胞、平滑肌样细胞、脂肪组织和厚壁血管组成。上皮样细胞体积大或巨大,呈多边形,胞质丰富,嗜酸性或空泡状,核仁明显,可见核内包涵体,偶见多核巨细胞。平滑肌样细胞呈梭形,9例中有4例几乎缺如脂肪组织。瘤细胞弥漫性或巢团状排列。瘤组织出血、坏死明显,呈浸润性边缘。免疫组化标记:瘤细胞HMB-45及Melan-A阳性,SMA和vimentin散在阳性,CK、EMA、AFP均阴性。结论 HAML是一种罕见的间叶组织源性肿瘤,多见于女性,组织学上以单一方向分化的大上皮样细胞为主,瘤细胞较为特异的表达HMB-45及Melan-A。免疫组化标记可与其它肝脏肿瘤鉴别。     

上皮样型恶性周围神经鞘瘤

我们拟探讨上皮样型恶性周围神经鞘瘤形态学特征与预后的关系。１２例经光镜和免疫组化观察，其中７例作了电镜观察。组织学可分为纯上皮样型（５例）；混合型、伴有梭形细胞区（５例）和骨化型（２例）。１２例均呈Ｓ－１００蛋白阳性表达，并显示多种细胞骨架异质性，包括（１）神经细胞分化：表现为ＮＳＥ和神经微丝阳性反应，节细胞分化；（２）上皮性分化：角蛋白阳性反应，电镜见有桥粒和张力微丝束；（３）间叶性分化：波形蛋白阳性、骨化生和横纹肌样表型。该瘤的异质性与侵袭性同步，异质性明显的３例均因肺转移死于就诊后７个月至４年。     

肾脏黏液性管状和梭形细胞癌临床病理学观察

目的 探讨肾脏黏液性管状和梭形细胞癌的临床病理学特征、免疫表型及鉴别诊断。方法 应用常规病理、免疫组化方法观察1例肾脏黏液性管状和梭形细胞癌并复习相关文献。结果 肿物与周围肾组织分界清楚，组织学特点是排列呈管状的上皮样细胞，片状的梭形细胞和黏液性间质，无明显核的异型性，缺乏坏死。免疫组化：CK、EMA、vimentin阳性，SMA、desmin、S-100蛋白、HMB45等阴性。结论 肾脏黏液性管状和梭形细胞癌是WHO新确定的一类罕见的低度恶性肾上皮性肿瘤，预后较好，要与后肾腺瘤、肉瘤样癌和集合管癌等疾病相鉴别。     

上皮样血管平滑肌脂肪瘤6例临床病理分析

目的 探讨上皮样血管平滑肌脂肪瘤(epithelioid angiomyolipoma,EAML)的临床病理特点、免疫表型,诊断及鉴别诊断.方法 对5例肾脏和1例肝脏上皮样血管平滑肌脂肪瘤进行病理形态观察及免疫组织化学检测.结果 6例上皮样血管平滑肌脂肪瘤形态相似,肿瘤细胞多呈巢状和片状排列,围绕血管呈袖套状,瘤细胞体积大,形态单一,多边形或梭形,胞质丰富,嗜酸性颗粒状,细胞核较大,核仁明显呈空泡状.免疫组化显示肿瘤细胞HMB45、vimentin 和平滑肌特异性抗原(SMA,MSA)弥漫阳性;而EMA、CK(AE1/AE3)呈阴性.结论 上皮样血管平滑肌脂肪瘤是一种具有恶性潜能的间叶源性肿瘤,形态学上常容易误诊,免疫组化对鉴别诊断有重要意义.     

Ⅰ型胸膜肺母细胞瘤1例报道并文献复习

目的 探讨Ⅰ型胸膜肺母细胞瘤的临床病理特征.方法 大体及镜下观察,结合sP法免疫组化染色并文献复习.结果 患儿,女,3岁11月,临床主要表现为左肺囊性肿物.组织学特征:大体表现为含气的单纯囊肿,囊肿内可见细分隔;镜检囊壁被覆良性上皮,其下为原始间叶细胞增生,可见明显的横纹肌母细胞分化,部分梭形细胞呈纤维肉瘤样改变,其余囊壁及细间隔主要为纤维结缔组织,部分被覆呼吸道上皮,可见平滑肌,灶性钙化,及多核巨细胞反应等良性改变.免疫组化染色原始间叶细胞desmin及myogenin阳性.结论 Ⅰ型胸膜肺母细胞瘤形态学上貌似良性,预后较好,难与其他先天性良性肺囊肿区分,故应仔细观察多取材,镜下寻找囊壁内原始间叶成分,从先天性肺囊肿中分辨出这一罕见儿童恶性肿瘤具有重要临床意义.     

乳腺纤维瘤病样梭形细胞癌临床病理分析

目的 探讨乳腺纤维瘤病样梭形细胞癌（fibromatosis-like spindle cell carcinoma,FLSCC）临床病理特征。方法 对3例FLSCC病例进行光镜观察和免疫组化染色[CK、CK（34βE12）、vimentin、SMA、ER、PR、Ki-67、c-erbB-2]。结果 3例均为女性,年龄分别为47、53、56岁,均可触及乳腺肿块。肿瘤境界清楚,但镜下边缘呈浸润性。肿瘤主要是梭形细胞、多边形细胞、少量的管状腺体及鳞上皮巢混合,间质纤维明显增生伴胶原化,细胞成束状排列或散在分布,似纤维瘤病样改变。梭形细胞分化良好,异型性不明显,部分区域细胞较丰富,其间聚集的上皮簇或片状多边形细胞核有轻度异型,可见少数核分裂象。多边形细胞与梭形细胞有移行。病变中亦可见淋巴细胞、浆细胞聚集浸润。上皮细胞、多边形细胞及部分梭形细胞CK（34βE12）、CK（AE1／AE3）阳性,CK阴性的梭形细胞表达vimentin、SMA。3例均行肿块切除,其中1例,术后4个月复发,再行乳腺根治术。结论 乳腺（纤维瘤病样）梭形细胞癌是一种少见的、低度恶性肿瘤,诊断需依赖免疫组化标记并与乳腺其它梭形细胞肿瘤相鉴别。     

肺淋巴管平滑肌瘤病临床病理学观察

目的 探讨肺淋巴管平滑肌瘤病临床、病理特征。方法 对5例肺淋巴管平滑肌瘤病临床资料进行收集分析,HE切片观察,采用免疫组织化学(SP法)检测平滑肌肌动蛋白(SMA)、HMB45、基质金属蛋白酶(MMP)2、孕激素受体(PR)、雌激素受体(ER),并进行文献复习。结果 肺淋巴管平滑肌瘤病是原因不明的肺部疾病,只发生在女性,特别是绝经前妇女。临床表现为呼吸困难,咯血,气胸和乳糜胸等。病理学检查显示不同成熟度平滑肌细胞在细支气管壁、肺泡壁、淋巴管壁和血管壁周围增生,肺实质呈囊性变。增生的平滑肌细胞免疫组织化学5例SMA、HMB45、MMP2均阳性;1例的ER和PR均阳性,1例仅ER阳性,1例仅PR阳性,1例的ER和PR均阴性。结论 育龄期妇女如反复出现自发性气胸、咯血、活动后呼吸困难应考虑肺淋巴管平滑肌瘤病的可能,病理检查可确定肺淋巴管平滑肌瘤病的诊断。     

Fibrosarcoma mimicking plasmacytoma or carcinoma: an ultrastructural study of 4 cases

Because the fibroblast has a remarkable capability of phenotypic modulations, reflected in both morphologic and immunohistochemical (IHC) changes, ultrastructural studiesare mandatory to identify the variants of fibroblasts. Myofibroblasts or histiofibroblasts are such examples, demonstrating chimeric ultrastructural features of fibroblastic cells in common with smooth muscle cells or with histiocytes, respectively. The presence of epithelioid fibroblastic cells sharing morphologic features with epithelial or plasma cells has not been yet characterized. The authors identified 4 cases of fibrosarcomas (FS) characterized by an unusual phenotype and associated with peculiar ultrastructural findings. The electron microscopic (EM) findings were correlated with the histologic appearance and immunoprofile. All tumors were located in the extremities, 3 in soft tissues and 1 in the bone. By light microscopy 2 cases were composed predominantly by round uniform cells with a striking plasmacytoid appearance. One case mimicked carcinoma, composed predominantly by epithelioid cells and scattered giant tumor cells. The fourth case showed a mixture of plasmacytoid-like and epithelioid cells. By IHC, tumor cells were positive for vimentin and in 2 cases also for epithelial membrane antigen. Kappa/lambda light chain and cytokeratins markers were negative. By EM all 4 tumors showed in addition to classic features of fibroblasts, unusual epithelial-type features, such as secretory granules of "neurosecretory-type" (3 cases), rudimentary cell junctions (3 cases), microvilli (2 cases), and lumen-like structures (1 case). One plasmacytoid-type tumor showed finely granular extracellular deposits. The study describe 4 examples of fibrosarcomas with unusual features at the ultrastructural level, which are associated microscopically with a peculiar phenotype, mimicking plasmacytoma or carcinoma. These findings broaden the spectrum of fibroblastic cell variants in neoplasia.     

Fibrosarcoma Mimicking Plasmacytoma or Carcinoma: An Ultrastructural Study of 4 Cases

Because the fibroblast has a remarkable capability of phenotypic modulations, reflected in both morphologic and immunohistochemical (IHC) changes, ultrastructural studiesare mandatory to identify the variants of fibroblasts. Myofibroblasts or histiofibroblasts are such examples, demonstrating chimeric ultrastructural features of fibroblastic cells in common with smooth muscle cells or with histiocytes, respectively. The presence of epithelioid fibroblastic cells sharing morphologic features with epithelial or plasma cells has not been yet characterized. The authors identified 4 cases of fibrosarcomas (FS) characterized by an unusual phenotype and associated with peculiar ultrastructural findings. The electron microscopic (EM) findings were correlated with the histologic appearance and immunoprofile. All tumors were located in the extremities, 3 in soft tissues and 1 in the bone. By light microscopy 2 cases were composed predominantly by round uniform cells with a striking plasmacytoid appearance. One case mimicked carcinoma, composed predominantly by epithelioid cells and scattered giant tumor cells. The fourth case showed a mixture of plasmacytoid-like and epithelioid cells. By IHC, tumor cells were positive for vimentin and in 2 cases also for epithelial membrane antigen. Kappa/lambda light chain and cytokeratins markers were negative. By EM all 4 tumors showed in addition to classic features of fibroblasts, unusual epithelial-type features, such as secretory granules of "neurosecretory-type" (3 cases), rudimentary cell junctions (3 cases), microvilli (2 cases), and lumen-like structures (1 case). One plasmacytoid-type tumor showed finely granular extracellular deposits. The study describe 4 examples of fibrosarcomas with unusual features at the ultrastructural level, which are associated microscopically with a peculiar phenotype, mimicking plasmacytoma or carcinoma. These findings broaden the spectrum of fibroblastic cell variants in neoplasia.     

Striated muscle cells in non-neoplastic lung tissue: a clinicopathologic study

This study was performed to study the prevalence, origin, and clinical implication of striated muscle cells in congenital non-neoplastic lung abnormalities. Five cases of striated muscle cell proliferation within congenital non-neoplastic pulmonary abnormalities were identified from a series of 31 (16%) resected specimens obtained at King's College Hospital, London, during the period 1992 to 1998. Lung tissue was also obtained from 48 normal human fetuses and serial sections stained for the presence of striated muscle. A histologic and immunohistochemical study of the clinical cases and the fetal material was performed by using phosphotungstic acid hematoxylin staining and immunostaining for myoglobin and desmin. Striated muscle cells were identified either as a diffuse or a focal proliferation within the lung interstitia of five infants. The congenital lung anomalies were intra-abdominal pulmonary sequestration associated with congenital cystic adenomatoid malformation (CCAM), intrathoracic sequestration again with features of CCAM, an intrathoracic sequestration associated with a congenital diaphragmatic hernia, and 2 Stocker type II intrathoracic CCAMs. Striated muscle cells were not identified in any section of lung tissue derived from the fetal series. Striated muscle cells proliferation in non-neoplastic lung tissue is more common than usually reported. Although the exact origin of such cells is speculative, because it is always detected within pulmonary anomalies, a wide morphogenetic error is likely. The clinical implication of its presence has to be further defined. HUM PATHOL 31:1477-1481.     

Clinicopathologic features of the mixed epithelial and mesenchymal type metaplastic breast carcinoma with myoepithelial differentiation in a subset of six cases

Metaplastic carcinoma (MC) of the breast, consisting of epithelial and mixed epithelial-mesenchymal tumors, are extremely rare human neoplasms. They are mostly detected between the 5th and 7th decade and have an unfavorable prognosis. Therefore, it is of utmost important to find out the behavior and also the immunohistochemical (IHC) profile of these tumors. In the current study, the aim was to examine 6 cases of MC with detailed clinico-pathological variables of cancer, follow-up and IHC profile of several antigens. The following immunohistochemical markers were used: MNF116, vimentin, CD10, smooth muscle actin (SMA), estrogen/progesterone receptors and HER-2/neu. The mean age was 39.1 the mean size was 3.3 cm. 83% of the cases had spindle cell sarcoma-like areas. Two of six cases also had a chondrosarcoma-like component. The epithelial component was invasive ductal carcinoma in all. MNF116, vimentin, CD10, and SMA expressions were as follows: mesenchymal cells: 33%, 100%, 50%, 83%, epithelial cells: 100%, 50%, 33%, 0%. All were triple negative. 66.6% presented with the axillary lymph node metastases. The mean follow-up period was 51 months, 50% died of the disease. Two had distant metastases to the lung. Our series which only included mixed epithelial-mesenchymal type metaplastic carcinoma of the breast showed myoepithelial differentiation with a worse prognosis.     

Myofibroblasts proliferation of idiopathic and collagen vascular disorders associated nonspecific interstitial pneumonia

Nonspecific interstitial pneumonia (NSIP) has been recognized as a separate histological classification of interstitial lung disease. Similar features are found not only in idiopathic NSIP, but also in NSIP associated with collagen vascular disorder (CVD-NSIP). We examined the clinical symptoms, laboratory findings, and prognosis of 13 cases of idiopathic NSIP and 11 cases of CVD-NSIP. Immunohistochemical staining was performed using the streptavidin/biotin/peroxidase method with anti-alpha-smooth muscle actin antibody. No differences in the distribution of clinical features, laboratory findings, and prognosis were observed between idiopathic NSIP and CVD-NSIP. In immunohistochemical staining of the fibrosing areas, myofibroblasts were observed in 7 of 13 idiopathic NSIP cases, but in 10 of 11 CVD-NSIP cases. With regards to intra-alveolar organization, myofibroblasts were observed in all 10 CVD-NSIP cases, but they were observed in only 2 of 9 idiopathic NSIP cases. We found a significantly higher myofibroblast proliferation in the intra-alveolar organization of CVD-NSIP compared to idiopathic NSIP. Clinically, idiopathic NSIP and CVD-NSIP are similar, but are pathologically different.     

Spindle cell carcinoids of the lung with paraganglioid features: a reappraisal of their histogenetic origin from paraganglia using immunohistochemical and electronmicroscopic techniques

Five cases of spindle cell carcinoids of the lung were analyzed by immunohistochemical and ultrastructural technique. They were found to be biphasic tumors composed of the major component of neuroendocrine cells (chief cells) and a minor component of dendritic cells (supporting cells). The chief cells displayed positivity for neuroendocrine phenotypic antigenic markers: neuron specific enolase (NSE), chromogranin A, and synaptophysin. They contained varying numbers of dense-core granules by electron microscopy. In addition, the chief cells expressed cytoplasmic positivity for cytokeratins. The supporting cells were dendritic in appearance and displayed strong positivity for S-100 protein in all cases. Glial fibrillary acidic protein was positive in two cases. On electron microscopy, the supporting cells were agranular and found along the external lamina surrounding the nests of tumor cells. In two cases, rare ganglion cell-like cells were present. The histomorphologic, immunohistochemical, and ultrastructural features were contrastingly different from the classical pulmonary carcinoid and rather resembled gangliocytic paragangliomas arising from small intestine and spine. It is proposed that pulmonary carcinoids with biphasic features are better designated as gangliocytic paragangliomas of the lung rather than paraganglioid carcinoids.     

Pulmonary adenocarcinomas: classification and reporting

Pulmonary adenocarcinoma is the most common, and the most diverse form of primary lung carcinoma. The histological complexity of these tumours poses problems for pathologists. The current WHO classification of pulmonary adenocarcinoma does not adequately address a number of clinically relevant, biological factors. The accurate diagnosis of adenocarcinoma on small biopsy specimens, accounting for most diagnoses of this disease, is challenged by the absence of tumour architecture in most samples. Tumours showing a pure bronchioloalveolar (BAC) pattern are now best regarded as adenocarcinoma- in-situ ; yet invasive adenocarcinomas may also show elements with the BAC pattern, dictating a better prognosis but biologically not necessarily in-situ disease. Multifocal BAC-pattern adenocarcinoma still poses considerable conceptual and diagnostic problems. In small tumours the papillary pattern, especially when micropapillary, confers a poor prognosis but this is not reflected in larger tumours. In early tumours of predominantly BAC ( in-situ ) pattern, the identification of invasion is particularly difficult, yet minor degrees of infiltration seem not to degrade prognosis. It may therefore be possible to define a minimally invasive category of adenocarcinoma. Consequently, there are a number of issues to consider when reporting this tumour type, depending on the nature of the diagnostic specimen. The rapid emergence of chemotherapeutic agents with histology-specific efficacy will increase the need for more accurate and specific diagnosis of adenocarcinoma on small samples. Immunohistochemistry may help suggest this diagnosis when the features are non-specific but immunohistochemical findings are not diagnostic of this form of lung cancer. The emerging clinical and prognostic relevance of a number of histological features in these complex tumours strengthens the argument in favour of including quantitative detail of pattern sub-types in reports on resected tumours.