肺硬化性血管瘤临床与影像学特征分析

目的探讨肺硬化性血管瘤的影像学特点,提高对本病的认识。方法对我院6例经手术病理证实为肺硬化性血管瘤患者的临床与影像学资料进行分析。结果6例患者中1例无明显症状,其余5例均有不同程度的咳嗽、咳痰、胸闷、胸痛等症状;所有患者查体均未见明显阳性体征。6例共7个病灶,直径均值为3.86cm,其中4个位于右侧肺内,6个位于肺野中内带,1例为同侧肺内多发性病灶。结论肺硬化性血管瘤是一种较少发生于肺内的良性上皮性肿瘤,影像学表现具备一般肺内良性肿瘤的特点。全面分析本病的影像学表现,结合临床特点,有益于提高术前诊断的准确率。     

Sclerosing hemangioma of the lung with cystic appearance

A case of cystic sclerosing hemangioma of the lung is reported. A chest X-ray of a 55-year-old woman who had been suffering from a cough with sputum for several months revealed an abnormal nodular shadow. A chest CT scan revealed a solitary tumor with cystic appearance occupying S7 of the right lung and the inferior pulmonary ligament. Radiological differential diagnosis for the lesion included bronchogenic cyst, cystic Schwannoma, pulmonary necrotic carcinoid, and lung carcinoma. Right lower lobectomy was carried out and the lesion was pathologically diagnosed as sclerosing hemangioma of the lung with cystic features, expanding into the pulmonary ligament. Differential diagnosis of the cystic lesion of the lung should include cystic sclerosing hemangioma as observed in this case.     

Clear cell tumor of the lung

Clear cell tumor of the lung is a rare benign tumor. We report herein a case of clear cell tumor of the lung. A 45 year-old woman with a round mass lesion of approximately 2 cm diameter on chest X-ray underwent a thoracotomy. Pathologic examination revealed sheets of large round or polygonal cells with clear cytoplasm and immunoreactive positivity for HMB-45 and vimentin. Investigation with abdominal CT scans showed no evidence of renal disease, and the tumor was diagnosed as clear cell tumor of the lung.     

Imprint cytologic features of pulmonary sclerosing hemangioma: comparison with well-differentiated papillary adenocarcinoma

BACKGROUND: Sclerosing hemangiomas (SH) of the lung are uncommon tumors and are thought to be benign. However, histogenesis of these tumors has not yet been characterized adequately. Moreover, there are few reports dealing with their cytologic features, and it is generally considered difficult to make accurate diagnoses of sclerosing hemangiomas that have a predominantly papillary pattern.METHODS: Cytologic features were analyzed for 15 sclerosing hemangiomas, and cytologic features of sclerosing hemangioma were compared with features of 22 cases of well-differentiated papillary adenocarcinoma classified as pathologic Stage 1A.RESULTS: Blood and round cells were observed more frequently in SH than in adenocarcinomas (P < 0.05), whereas necrosis was seen more frequently in adenocarcinomas than in SH (P < 0.05). The presence of nucleoli, nuclear indentations, irregularities of nuclear margins, nuclear polymorphisms, and high nuclear-cytoplasmic (NC) ratios of tumor cells were observed less frequently in SH. Polynuclear (having three or more nuclei) tumor cells were observed only in adenocarcinoma cases. In morphometric studies, the nuclear areas, cytoplasmic areas, NC ratios, long axes of nuclei, short axes of nuclei, and nuclear rotundity ratios were significantly higher in adenocarcinoma cells than in SH cells (P < 0.05).CONCLUSIONS:The presence of polymorphous cells and tumor cells with bland nuclei are characteristic cytologic findings associated with sclerosing hemangioma. It is possible to make accurate diagnoses for SH cases preoperatively by careful cytologic characterization.     

Sclerosing hemangioma of the lung. Immunohistochemical characterization of its origin as related to surfactant apoprotein

Lung tissues from 13 patients with pulmonary sclerosing hemangioma were studied with antibody against surfactant apoprotein, Factor VIII-related antigen, or lysozyme. Surfactant apoprotein was detected in the cytoplasm of the cells lining cystic spaces and papillary projections. Surfactant apoprotein was found in a small number of stromal cells with abundant eosinophilic or clear cytoplasm and round to oval nuclei, which were characteristic in pulmonary sclerosing hemangioma as the main component. Surfactant apoprotein was also found in the stromal cells with small, dark nuclei similar to the lining cells. The lining and stromal cells contained neither Factor VIII-related antigen nor lysozyme. Our demonstration of surfactant apoprotein in these cells provides further support for the idea that pulmonary sclerosing hemangioma primarily consists of epithelial cells with differentiation to type II pneumocytes, as was deduced from ultrastructural investigations.     

20例肺硬化性血管瘤临床病理分析

目的寻找肺硬化性血管瘤(Pulmonary sclerosing hemangioma,PSH)的诊断和鉴别诊断方法。方法  回顾性分析20例PSH临床症状、CT和冰冻切片诊断、组织学及免疫组织化学染色特点。结果  女性18例,平均年龄55岁。右肺11例,双肺1例,左肺8例。CT检查19例表现为孤立性肺结节,1例双肺分别见有结节;CT诊断肺癌17例,食管癌伴有肺转移2例,错构瘤1例。有10例进行术中冰冻检查, 2例诊断为类癌,2例细支气管肺泡癌,1例描述性诊断PSH,5例炎性假瘤等良性病变。常规切片中同时具有实性区、乳头区、硬化区、血管瘤样区4种结构的11例,并可见圆形和立方形两种肿瘤细胞, HE切片诊断PSH。9例结合免疫组织化学染色,中央圆形细胞EMA、TTF-1、Vimentin弥漫强阳性,AE1/AE3阴性,被覆立方细胞AE1/AE3和TTF-1阳性,而Vimentin阴性等做出诊断。结论  PSH是常发生于中老年女性的少见肿瘤,多表现为孤立性肺结节,CT和术中冰冻切片检查均易误诊,前者极易误诊为肺癌,后者虽然在判断性质方面较前者准确性高,但是多数PSH仍然很难确诊,术后常规切片辅以免疫组织化学检查是诊断和鉴别诊断的有效方法。     

Ultrastructural study of a postinflammatory "tumor" of the lung.

A typical postinflammatory "tumor" of the lung was resected from an asymptomatic 22-year-old white woman. Light and electron microscopic studies showed the lesion to be composed of metaplastic pneumonocytes, proliferated stroma, and chronic inflammatory cells. These findings confirm the inflammatory nature of the lesion and serve to distinguish this lesion from the neoplastic pulmonary sclerosing hemangioma.     

Sclerosing hemangioma of the lung: an ultrastructural study.

A well-circumscribed nodular mass was excised from the lingula of the left lung of a 40-year-old-female. The lesion was discovered on routine chest x-ray examination and corresponded to a typical "sclerosing hemangioma" described by Liebow and Hubbell. Electron microscopic study revealed unequivocal epithelial cells in both the irregular spaces and the solid areas of the tumor; some of these cells were identical to granular pneumonocytes. The true vascular component was sparsely distributed. We concluded that "sclerosing hemangioma" was a misnomer because we were dealing with an essentially epithelial lesion.     

甲状腺转录因子-1在肺硬化性血管瘤组织起源中的应用

目的 探讨甲状腺转录因子-1（TTF-1）对肺硬化性血管瘤（PSH）组织起源的意义。方法 收集36例肺硬化性血管瘤患者的临床病理资料。并对手术标本进行多种抗体免疫组化染色,抗体包括TTF-1,SpA,CK,EMA,F-Ⅷ,CD34,calretinin,HBME,synaptophysin,chromogranin,actin,S-100等。结果 36例肺硬化性血管瘤患者,未发现多发及转移病例,形态学上PSH表现为表面细胞（SC）和圆形细胞（RC）两种瘤细胞成分,免疫组化显示两种瘤细胞几乎90%以上均表达TTF-1及EMA;SC可表达CK和SpA,而RC几乎不表达CK和SpA,少数几例PSH中的RC呈散在灶状表达SpA或synaptophysin或S-100,其余间皮源性,血管源性,神经内分泌源性及肌上皮源性抗体在两种细胞中均呈阴性染色结果。结论 PSH实际上是来源于肺泡上皮的良性肿瘤,应称之为良性肺泡上皮细胞瘤,其中表面上皮相对成熟,圆形上皮属于原始肺泡上皮,且可向其他上皮方向分化。     

肺硬化性血管瘤临床病理分析

 目的 探讨肺硬化性血管瘤（SHL）的临床病理特点。方法 对11例肺硬化性血管瘤进行光镜和免疫组化观察并结合文献进行分析。结果 SHL主要由圆形细胞和表面立方细胞二种细胞类型组成。组织结构多样，主要表现为实性区、乳头区、血管瘤样区和硬化区等四种结构。免疫组化标记二种细胞共同表达TTF-1和EMA。 结论 SHL为起源于原始的呼吸道上皮细胞的肿瘤，实性的圆形细胞和表面的立方细胞均为肿瘤细胞。形态应与炎性假瘤、肺Ⅱ型细胞乳头状瘤、肺泡腺瘤、典型类癌鉴别。部分SHL有侵袭性生长的倾向，外科手术治疗宜行肺叶切除术而非肿块剥离术。     

p53蛋白表达和基因突变在所谓肺硬化性血管瘤组织中的意义

背景与目的所谓肺硬化性血管瘤（so-called pulmonary sclerosing hemangioma，PSH）是一种至今不能肯定其来源及性质的少见肺肿瘤。虽然目前普遍认为PSH是一种良性肿瘤，但却发现有少数PSH可发生浸润和转移。p53蛋白表达和基因突变是反映肿瘤生物学行为的有用指标。本研究的目的是检测PSH组织中p53基因突变及p53蛋白的表达情况及其意义。方法应用免疫组化S-P法、激光捕获显微切割（laser capture microdissection，LCM）技术、单链构象多态性（single-stranded conformation polymorphism，SSCP）及DNA测序分析方法（5～8外显子）检测19例PSH中多角形细胞和表面立方细胞的p53蛋白表达和基因突变情况。结果19例PSH组织中p53蛋白阳性表达率为21．1％（4／19），SSCP及测序分析检测p53基因突变率分别为26．3％（5／19）和42．1％（8／19）。p53蛋白阳性表达的4例PSH组织，测序分析显示3例标本有突变发生，2例为错义突变，1例同时发生错义突变和移码突变；而p53蛋白阴性表达的15例PSH组织中，5例标本经测序证实亦发生了突变，4例为移码突变，1例为错义突变。在有突变的8例PSH组织中，单一多角形细胞突变5例，单一立方细胞突变2例，多角形细胞和立方细胞同时突变1例。结论PSH组织p53蛋白阳性表达并不能完全反映p53基因突变的真实情况；p53基因突变或蛋白表达异常均可发生在PSH组织中的两种不同的细胞内；p53基因的高突变率提示PSH可能具有潜在的恶性生物学行为。     

24例肺硬化性血管瘤的诊疗分析

背景与目的:肺硬化性血管瘤是一种少见的肺部良性肿瘤,本研究拟探讨其临床特点、诊断、治疗和预后情况,以进一步提高对该病的认识。方法:收集1999年1月至2007年7月中山大学肿瘤防治中心收治的24例肺硬化性血管瘤的临床资料,回顾性分析其临床特点、诊断、治疗及预后情况。结果:本组24例经石蜡病理切片证实为肺硬化性血管瘤的患者中,男性2例(8.3%),女性22例(91.7%);发病年龄21～76岁,中位年龄54.5岁;10例(41.7%)患者系体检发现,14例(58.3%)因咳嗽、咳血丝痰、胸痛、胸闷或气促等症状而就诊;影像学检查表现多为肺内孤立、边界清晰、密度均匀、圆形或类圆形的结节影,均未见点状钙化及空气新月征;全组病例均接受外科治疗,8例(33.3%)行肺叶切除术,13例(54.2%)行肺楔形切除术,2例(8.3%)行肿瘤摘除术,1例(4.2%)行肺段切除术,无手术相关并发症及手术死亡;术后随访,无复发转移。结论:肺硬化性血管瘤临床症状和影像学表现无特异性,术前诊断困难,确诊依靠病理,外科手术是有效治疗方法,可考虑肺叶切除或局限性切除,系统淋巴结清扫不推荐作为常规。     

Pulmonary sclerosing hemangioma of the lung. A type II pneumocytoma by immunohistochemical and immunoelectron microscopic studies

Three cases of pulmonary sclerosing hemangioma were studied by immunohistochemical and immunoelectron microscopic methods using a panel of antibodies. Six cases of adenocarcinoma of the lung, three cases of normal mesothelium, and three cases of mesothelioma were used as controls. The cytoplasm of some of the sclerosing hemangioma tumor cells was positive for the anti-lung surfactant apoprotein monoclonal antibody (PE-10). These cells were the pale cells of the solid areas, the cells covering the papillary projections, and the cells lining the cleft-like spaces. These cells also were positive for conventional epithelial cell markers. Some cells also were positive for vimentin. Electron microscopic study showed that the predominant cell was a poorly differentiated pneumocyte. Immunoelectron microscopic study also demonstrated that PE-10 existed in the rough endoplasmic reticulum of some of the cells in the solid areas, in the same way as normal type II pneumocytes. We concluded that the sclerosing hemangioma is an epithelial tumor with differentiation towards type II pneumocytes.     

Sclerosing hemangioma of the lung. Immunohistochemical demonstration of mesenchymal origin using antibodies to tissue-specific intermediate filaments

A case of pulmonary sclerosing hemangioma of the lung was studied by light microscopy and indirect immunofluorescence using tissue-specific antibodies against intermediate filament subunits. All the tumor cells stained positively and exclusively with antivimentin antibodies thus indicating their mesenchymal origin. In addition, positive staining with cytokeratin antibodies was observed in cells lining cystic spaces and elongated slit-like spaces were occasionally encountered throughout the tumor, disclosing residual epithelial elements. Using brightfield microscopy, the keratin-positive areas were identified as distorted alveolar spaces lined by hyperplastic respiratory epithelium entrapped within the tumor. It is proposed that these entrapped epithelial elements may account for the conflicting results obtained by different investigators in previous attempts to determine the histogenesis of this tumor.     

Intrathoracic Hodgkin's disease. A case presentation with multiple pulmonary nodules in the absence of mediastinal or hilar node disease

Patients with Hodgkin's disease (HD) most commonly present with peripheral lymph node enlargement. The finding of pulmonary parenchymal involvement is usually associated with hilar and/or mediastinal adenopathy; such instances are felt to represent spread to the lung by contiguity. Noncontiguous spread to peripheral lung parenchyma in the absence of hilar or mediastinal node involvement has rarely been reported. This is the first documented case report in a 32-year-old woman with nodular sclerosing HD. The unusual clinical presentation in this patient was associated with the histologic detection of vascular invasion in the lymph node and open-lung biopsy specimens.     

Estrogen Receptor- and Progesterone Receptor-Positive Diffuse Sclerosing Variant of Papillary Thyroid Carcinoma: A Case Report

The diffuse sclerosing variant of papillary thyroid carcinoma (DSV-PTC) is a relatively rare tumor. We herein report the case of young woman with DSV-PTC who developed cervical lymph node recurrence 7 years after the initial surgery. A 15-year-old female patient with no medical or family history of thyroid tumors developed a thyroid neoplasm in the right lobe. Right thyroidectomy and regional lymphadenectomy were performed, and the tumor was diagnosed as DSV-PTC. She was followed up as an outpatient. Seven years after the surgery, cervical lymph node recurrence developed. On microscopic examination, the thyroid tumor showed a papillary growth pattern with numerous psammoma bodies and distinct fibrosis. Immunohistochemically, the tumor cells were estrogen receptor and progesterone receptor positive with reduced membranous expression of E-cadherin and were intermingled with S-100-positive dendritic/Langerhans cells. DSV-PTC is characterized by a strong tendency for invasion and metastasis. Thus, accurate diagnosis is clinically important, and a morphological and immunohistochemical understanding of DSV-PTC is necessary.Key words: Diffuse sclerosing variant of papillary thyroid carcinoma, Estrogen receptor, Progesterone receptor, E-cadherin, S-100, Immunohistochemistry     

Plasma cell granuloma of the lung (inflammatory pseudotumor)

A case of plasma cell granuloma (PCG) of the lung in a 54-year old man is reported. PCG is a rare benign lesion that usually presents as a solitary nodule in the lung (coin lesion) at routine X-ray examination. Microscopically it consists of a granulomatous tissue where the major components are mature plasma cells. The immunohistochemical demonstration of polyclonality of plasma cells, excluding the diagnosis of plasmacytoma, confirms the inflammatory pseudotumoral nature of this lesion, although the etiology remains obscure. The presence of lymphocytes, histiocytes, macrophages, blood vessels with prominent endothelial cells and peripheral sclero-hyalinized connective tissue may pose problems in the differential diagnosis with sclerosing hemangioma, pseudolymphoma, nodular amyloidosis, pulmonary hyalinizing granuloma, chronic abscess and neoplasms of true histiocytic origin. The term inflammatory pseudotumor is preferable in describing this type of lesion.     

Ki-67与p53在肺硬化性血管瘤中的表达及其临床意义

 【摘要】　目的　分析Ki-67与p53蛋白在肺硬化性血管瘤（PSH）表面被覆细胞和间质多边形细胞中的表达以及Ki-67、p53蛋白与PSH的生物学行为的关系。方法　随机数字表法抽取21例PSH患者手术切除标本,应用免疫组织化学双标法检测21例标本Ki-67和CK8／18蛋白表达,免疫双荧光组织化学染色技术检测9例p53和AE1／AE蛋白的表达。结果　CK8／18和AE1／AE3定位于细胞膜,表达于表面被覆细胞; Ki-67与p53定位于细胞核,在两种细胞均见表达。其中Ki-67在表面被覆细胞中表达阳性率＜1 %,在间质多边形细胞中阳性率为1 %～10 %;p53主要表达于间质多边形细胞（单信号6例）,仅个别被覆细胞阳性（双信号3例）。结论　PSH中的间质多边形细胞和表面被覆细胞具有形态和免疫表型的差异,间质多边形细胞的增殖指数和基因突变均高于被覆细胞,PSH的生物学行为可能主要决定于间质多边形细胞。     

Carcinoid tumorlets of the lung with metastasis to a peribronchial lymph node. Report of a case and review of the literature

A case of a rare entity, pulmonary tumorlets that metastasized to a peribronchial lymph node, is reported. The patient, a 38-year-old man, underwent a right pneumonectomy for end-stage lung disease caused by bronchiectasis, chronic bronchitis, and pulmonary fibrosis. No tumors were detected on radiologic or on gross examination of the lung. Microscopically, multiple tumorlets were identified in the fibrotic pulmonary parenchyma. Five peribronchial lymph nodes were found and appeared grossly normal. A microscopic focus of metastatic tumor, histologically identical to the pulmonary tumorlets, was discovered in one of these nodes. This metastasis was identified only because a diligent search for peribronchial lymph nodes was undertaken, and because sections of each node were obtained. Pathologists usually do not extensively examine peribronchial lymph nodes in cases of chronic inflammatory disease of the lung, even when small tumorlets are discovered as incidental findings, because it is presumed that the tumorlets have not metastasized. As shown by our case, this presumption is not always correct. Pulmonary tumorlets may metastasize to peribronchial lymph nodes more frequently than has been previously recognized.     

Prognostic variability among nonsmall cell lung cancer patients with pathologic N1 lymph node involvement. Epidemiological figures with strong clinical implications

BACKGROUND: Patients who have nonsmall cell lung cancer with N1 lymph node status are an intermediate group of patients who have a variable prognosis. Differences in lymph node level (hilar or pulmonary lymph nodes) may influence patient survival. The authors retrospectively analyzed the factors that influenced prognosis, including the level of N1 lymph node involvement. METHODS: The authors used the Tuscan Cancer Registry archives to retrieve records on 2523 patients who had lung tumors diagnosed during the period from 1996 and 1998 in the provinces of Florence and Prato, central Italy. To analyze the survival of patients according to the level of lymph node involvement, the prognoses of patients with nonsmall cell lung cancer who had N1 lymph node status were compared in a population-based case series. Among 112 patients with pathologic N1 status, the following variables were analyzed for their influence on postoperative survival: gender, age, cell type, pathologic tumor status, the number of metastatic lymph nodes, the level of metastatic lymph nodes (hilar or pulmonary), and the type of surgical resection. RESULTS: The 5-year survival rates for patients who had involvement of pulmonary and hilar lymph nodes were 41.2% and 21.8%, respectively (P =.005). A Cox proportional hazards model analysis indicated that the presence of hilar lymph node involvement was an independent prognostic factor. CONCLUSIONS: N1 pathologic lymph node status was identified in a combination of subgroups with different prognoses, and the presence of hilar lymph node disease had prognostic significance. This difference in survival may lead to the use of different therapies for these subgroups of patients with pathologic N1 non-small cell lung cancer.