Pulmonary hamartoma

肺错构瘤
  • 文章类型: Case Reports
    肺错构瘤(PH)很少见,但却是肺部最常见的良性肿瘤。它们生长缓慢,通常在生命的第六个十年中在胸部成像中偶然发现。大约10%的肺错构瘤是支气管内。很少,肺错构瘤可根据其大小和位置引起一系列肺部症状。我们介绍一例支气管内错构瘤导致气道阻塞和阻塞性肺炎复发的病例。
    Pulmonary hamartomas (PH) are rare but are the most common benign tumors found in the lungs. They are slow-growing and are usually found incidentally on chest imaging during the sixth decade of life. Approximately 10% of pulmonary hamartomas are endobronchial. Rarely, pulmonary hamartomas can cause a spectrum of pulmonary symptoms depending on their size and location. We present a case of endobronchial hamartoma causing airway obstruction and recurrent post-obstructive pneumonia.
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  • 文章类型: Case Reports
    背景:肺错构瘤是肺部良性病变。组织病理学,肺错构瘤由不同数量的间充质成分组成,包括软骨组织,成熟脂肪组织,纤维基质,平滑肌,并截留了呼吸道上皮。大多数肺错构瘤病例无症状,在成像过程中偶然发现。它们通常表现为界限清楚的病变,最大尺寸小于4厘米。超过8厘米的无症状巨大肺错构瘤很少见。
    方法:在当前的病例报告中,在心脏病检查中,偶然发现一名59岁女性的肺部肿块为12.0×9.5×7.5厘米。严重的,病变呈小叶状,白色至棕白色固体切面和小的囊性区域。微观上,有代表性的肿瘤切片显示软骨粘液样外观,周围有相对低细胞的基质和包裹的呼吸上皮。没有注意到显著的非典型性。没有注意到有丝分裂,根据Ki-67免疫组织化学,增殖指数非常低(<1%)。成熟的脂肪组织在许多领域很容易识别。组织形态学与肺错构瘤一致。将肉瘤靶向的基因融合组进一步应用于这种情况。在这种情况下,显微镜检查和肉瘤靶向基因融合组结果的联合评估排除了恶性肉瘤转化。纵隔和肺门淋巴结在组织学上是良性的。手术后,患者术后度过了一个平稳的时期.
    结论:巨大的肺错构瘤是罕见的;我们的病例是无症状患者的巨大错构瘤的一个例子。这个肿瘤的大小令人担忧。因此,为了正确诊断和排除共存的恶性肿瘤,需要对病变进行仔细和全面的检查。
    BACKGROUND: Pulmonary hamartomas are benign lung lesions. Histopathologically, pulmonary hamartoma is composed of varying amounts of mesenchymal elements, including chondroid tissue, mature adipose tissue, fibrous stroma, smooth muscle, and entrapped respiratory epithelium. Most pulmonary hamartoma cases are asymptomatic and found incidentally during imaging. They usually appear as well-circumscribed lesions with the largest dimension of less than 4 cm. Asymptomatic giant pulmonary hamartomas that more than 8 cm are rare.
    METHODS: In the current case report, a 12.0 × 9.5 × 7.5 cm lung mass was incidentally noticed in a 59-year-old female during a heart disease workup. Grossly, the lesion was lobulated with pearly white to tan-white solid cut surface and small cystic areas. Microscopically, representative tumor sections demonstrate a chondromyxoid appearance with relatively hypocellular stroma and entrapped respiratory epithelium at the periphery. No significant atypia is noted. No mitosis is noted, and the proliferative index is very low (< 1%) per Ki-67 immunohistochemistry. Mature adipose tissue is easily identifiable in many areas. Histomorphology is consistent with pulmonary hamartoma. A sarcoma-targeted gene fusion panel was further applied to this case. Combined evaluation of microscopic examination and sarcoma-targeted gene fusion panel results excluded malignant sarcomatous transformation in this case. The mediastinal and hilar lymph nodes are histologically benign. After surgery, the patient had an uneventful postoperative period.
    CONCLUSIONS: Giant pulmonary hamartoma is rare; our case is an example of a huge hamartoma in an asymptomatic patient. The size of this tumor is concerning. Thus, careful and comprehensive examination of the lesion is required for the correct diagnosis and to rule out co-existent malignancy.
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  • 文章类型: Case Reports
    背景:炎性横纹肌母细胞肿瘤是相对较新认识的恶性潜能较低的软组织肿瘤。这里,我们介绍了一例并发炎性横纹肌母细胞瘤(IRMT),肾上腺嗜铬细胞瘤,1型神经纤维瘤病(NF1)患者的肺错构瘤。据我们所知,这是第一次在文献中描述这种肿瘤。
    方法:一名已知NF1的20多岁女性患者被诊断患有炎性横纹肌母细胞瘤,嗜铬细胞瘤,和肺错构瘤在短时间内发生。发现IRMT具有近单倍体基因组,并显示出典型的免疫组织化学特征以及局灶性异常p53表达模式。
    结论:该病例报告加强了以下理论:肿瘤抑制因子NF1的缺陷在炎性横纹肌母细胞肿瘤的发病机制中起着核心作用,IRMT可能是1型神经纤维瘤病相关肿瘤的一部分。
    BACKGROUND: Inflammatory rhabdomyoblastic tumors are relatively recently recognized soft tissue tumors with a low malignant potential. Here, we present a case of concurrent inflammatory rhabdomyoblastic tumor (IRMT), adrenal pheochromocytoma, and pulmonary hamartoma in a patient with neurofibromatosis type 1 (NF1). To our knowledge, this is the first time that this constellation of tumors has been described in the literature.
    METHODS: A female patient in her late 20s with known NF1 was diagnosed with an inflammatory rhabdomyoblastic tumor, pheochromocytoma, and pulmonary hamartoma in a short succession. IRMT was found to harbor a near-haploid genome and displayed a typical immunohistochemical profile as well as a focal aberrant p53 expression pattern.
    CONCLUSIONS: This case report strengthens the theory that defects in the tumor suppressor NF1 play a central role in the pathogenesis of inflammatory rhabdomyoblastic tumors and that IRMT may be part of the spectrum of neurofibromatosis type 1 related tumors.
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  • 文章类型: Case Reports
    在一名52岁男子的左肺中发现了一个孤立的周围肺结节。它位于下叶,在胸部计算机断层扫描中测量主轴18.5厘米。获得了一个切开的核心活检和一个温和的增生,单态,观察到交织束中的梭形细胞。因此,随后进行了肿瘤的手术切除。宏观上,肿瘤表现为界限良好的结节,切面坚硬而发白。组织学上,肿瘤主要由温和的单形梭形细胞组成,以束状生长为主,其中涉及许多截留的正常呼吸上皮的管状和裂隙样空间。粘液样改变,还观察到基质透明化和分散的奇异单核细胞和多核细胞。基于临床形态学,免疫表型和分子特征,我们诊断为肺腺肌瘤样错构瘤恶性转化为肺平滑肌肉瘤。据我们所知,这是在已经罕见的肿瘤中首次描述的这种异常罕见的病例。
    A solitary peripheral lung nodule was found in the left lung of a 52-year-old man. It was located in the lower lobe and measured 18.5 cm of major axis on chest computed tomography. A tru-cut core biopsy was obtained and a proliferation of bland, monomorphic, spindle cells in interlacing fascicles was observed. Accordingly, a surgical resection of the neoplasm was subsequently carried out. Macroscopically, the tumor appeared as a well-circumscribed nodule with a firm and whitish cut surface. Histologically, the neoplasm was predominantly composed of bland and monomorphic spindle cells, with a predominantly fascicular growth pattern, in which many tubular and cleft-like spaces of entrapped normal respiratory epithelium were involved. Myxoid change, stromal hyalinization and scattered bizarre mononucleated and multinucleated cells were also observed. Based on clinico-morphological, immunophenotypical and molecular features, we made a diagnosis of malignant transformation of pulmonary adenoleiomyomatous hamartoma into pulmonary leiomyosarcoma. As far as we know, this is the first described case of this exceptionally rare occurrence in an already rare neoplasm.
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  • 文章类型: Letter
    暂无摘要。
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  • 文章类型: Case Reports
    随着低剂量胸部计算机断层扫描(CT)的广泛应用,越来越多的人发现肺结节。常见的肺部疾病,如肺腺癌,肺鳞状细胞癌,结核病通常可通过影像学检查诊断。然而,当多种类型的肺癌与其他良性肿瘤合并时,如何做出准确的诊断?在这份报告中,我们介绍了一例罕见的同时发生肺腺癌的患者,肺鳞状细胞癌,肺结核,和肺错构瘤,以前没有报道过。这名患者在吉林大学第二医院胸外科接受了手术干预,目前已完全康复出院。患者术前正电子发射断层扫描(PET-CT)结果与术后病理诊断不一致。影像学检查结果不典型,病理诊断异常罕见。我们分享这个病例报告有助于临床经验的积累。
    With the widespread use of low-dose chest Computed Tomography (CT), lung nodules are being increasingly detected. Common pulmonary conditions such as lung adenocarcinoma, lung squamous cell carcinoma, and tuberculosis are typically diagnosable through imaging examinations. Nevertheless, when multiple types of lung cancer are combined with other benign tumors, how can an accurate diagnosis be made? In this report, we present a rare case of a patient with the simultaneous occurrence of lung adenocarcinoma, lung squamous cell carcinoma, pulmonary tuberculosis, and pulmonary hamartoma, which has not been previously reported. This patient underwent surgical intervention in the Department of Thoracic Surgery at the Second Hospital of Jilin University and has now fully recovered and been discharged. The patient\'s preoperative positron emission tomography-CT(PET-CT)results did not align with the postoperative pathological diagnosis. The imaging findings were atypical, and the pathological diagnosis was exceptionally rare. We share this case report to contribute to the accumulation of clinical experience.
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  • 文章类型: Journal Article
    目的:肺胎盘移位(PT)是一种良性病变,可通过切除术治愈,表现为异常的特殊形态变化,包括肺错构瘤中的胎盘样大疱性变化。在这项回顾性研究中,我们的目的是检查肺错构瘤的组织病理学特征,为了评估不同的组织学成分,尤其是PT,并探讨PT模式的重要性及其与其他临床病理特征的关系。
    方法:从2001年至2021年的记录中招募35例肺错构瘤,根据PT的存在分为两组,病理检查为PT(-)和PT(+)。
    结果:所有患者中77.1%为男性。两组在年龄方面无显著差异,性别,合并症,存在的症状,肿瘤定位,放射学检查结果(P>0.05)。28例(80%)患者全部切除肺错构瘤。其中5例(17.9%)切除材料中存在不同程度的PT成分,在5%至80%之间,都是男性患者。在15名PT(-)和5名PT()患者中进行了冰冻切片检查,但在任何PT()患者中均未达到冰冻切片的诊断。两组材料中大部分为软骨样成分(52.22±29.7%)(P<0.05)。
    结论:胎盘乳头状突起是与肺错构瘤相关的可用模式,特别是在冰冻切片中观察到的这些突起对于识别错构瘤的PT模式非常关键,因为它们会导致恶性肿瘤鉴别诊断的混淆。
    OBJECTIVE: Pulmonary placental transmogrification (PT) is a benign lesion curable by resection, represented by an unusual peculiar morphological variation including placentoid bullous change in the pulmonary hamartoma. In this retrospective study, we aimed to examine the histopathological features of pulmonary hamartomas in lung, to evaluate the different histological components, especially PT, and to investigate importance of PT pattern and its relationship with other clinicopathological features.
    METHODS: Thirty-five cases of pulmonary hamartomas were recruited from the records between 2001 and 2021, divided into two groups according to presence of PT, as PT (-) and PT (+) in pathological examination.
    RESULTS: 77.1% of all patients were male. There was no significant difference between the two groups in terms of age, sex, comorbidity, presence of symptoms, tumor localization, and radiological findings (P > 0.05). Pulmonary hamartomas were resected totally from 28 patients (80%). Five of these patients (17.9%) had PT components in resection materials with varying degree between 5 and 80%, and all were from male patients. Examination with frozen sections were performed in 15 PT (-) and 5 PT (+) patients but diagnosis with frozen sections was not achieved in any of PT (+) patients. Most of materials included chondroid components (52.22 ± 29.7%) in both groups (P < 0.05).
    CONCLUSIONS: The placental papillary projections are available patterns associated with a pulmonary hamartoma and these projections observed especially in frozen sections are very crucial to recognize PT pattern in hamartomas, as they can result in confusions in differential diagnosis of malignities.
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  • 文章类型: Journal Article
    目的:基于计算机断层扫描(CT)的肺错构瘤(PH)诊断是一个挑战,尤其是具有不典型影像学特征的患者。本研究旨在总结18F-Fluoro-D-葡萄糖正电子发射断层扫描-计算机断层扫描(18F-FDGPET-CT)在PH诊断中的影像学特征,探讨PET-CT在PH诊断中的应用价值。
    方法:回顾性分析文献中诊断为PH并接受PET-CT检查的患者,这些是科克伦图书馆的出版物,PubMed,摘录医学数据库(EMBASE),中国国家知识基础设施(CNKI)和万方数据库,从2008年到2022年6月。收集的其他20例均为我院2008年至2022年6月的患者。患者的症状,胸部CT的影像学特征,PET-CT特征,分析PET-CT的原因及并发症。
    结果:在这项回顾性研究中,共有216例患者被诊断为PH,并接受了PET-CT检查。其中20例为我院2008年1月至2022年6月的患者。其他病例是从文献中收集的。大多数PH病变的平均直径为1.7±1.0cm。PH病变的平均最大标准化摄取值(SUVmax)为1.2±1.1。它们的大多数SUVmax低于国际公认的截止值(SUVmax=2.5)。PET-CT对PH的诊断优于CT,但对PH病变的CT诊断与PET-CT诊断之间存在相关性。为了绘制接收器工作特性(ROC),我们选择了29例PH病变SUVmax值明确的患者,收集29例SUVmax值明确的肺癌患者作为对照组。ROC曲线分析显示,SUVmax的曲线下面积(AUC)为0.899,最佳诊断阈值为SUVmax>2.65。PET-CT可以通过在本研究中应用2.65的SUVmax作为截止值,以89.66%的灵敏度区分PH和恶性病变。
    结论:PET-CT可能是诊断PH的有用工具,显示出比CT更好的诊断灵敏度。但PET-CT不能作为单一的诊断手段,应结合其他方法和患者病史做出最正确的诊断。
    The diagnosis of pulmonary hamartoma (PH) based on computed tomography (CT) is a challenge, especially in patients with atypical imaging characteristics. This study was aimed at summarizing the imaging characteristic of 18F-Fluoro-D-glucose positron emission tomography-computed tomography (18F-FDG PET-CT) in PH and exploring the application value of PET-CT in the diagnosis of PH.
    Patients diagnosed with PH who had undergone PET-CT from literature pertaining were retrospectively analyzed, which were cases of publications from the Cochrane Library, PubMed, Excerpta Medica Database (EMBASE), China National Knowledge Infrastructure (CNKI) and Wanfang databases, from 2008 to June 2022. The other 20 cases of the collection were patients from our hospital from 2008 to June 2022. Patients\' symptoms, imaging characteristics of chest CT, PET-CT characteristics, the reason for PET-CT and the complications were analyzed.
    In this retrospective study, a total of 216 patients were diagnosed with PH and had been examined by PET-CT. 20 of the cases were patients of our hospital from January 2008 to June 2022. The other cases were collected from the literature. The mean diameter of most PH lesions is 1.7 ± 1.0 cm. The mean maximum standardized uptake value (SUVmax) of the PH lesions was 1.2 ± 1.1. Most of their SUVmax were lower than internationally recognized cut-off value (SUVmax = 2.5). PET-CT was superior to CT in the diagnosis of PH but there was a correlation of between CT diagnosis and PET-CT diagnosis for the PH lesions. In order to draw the Receiver operating characteristic (ROC), we selected 29 patients with a clear SUVmax value of their PH lesion, and 29 lung cancer patients with clear SUVmax value in our hospital were collected as a control group. ROC curve analysis showed that the area under curve (AUC) of SUVmax was 0.899, and the optimal diagnostic threshold was SUVmax > 2.65. PET-CT could distinguish PH from malignant lesions with a sensitivity of 89.66% by applying a SUVmax of 2.65 as a cut-off in this study.
    PET-CT might be a useful tool to diagnose PH, which shows a better diagnostic sensitivity than CT. But PET-CT can not be used as a single diagnostic approach, which should be combined with other methods and the patients\' history to make the most correct diagnosis.
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  • 文章类型: Case Reports
    错构瘤是最常见的肺部良性肿瘤,但是它的气管形式仍然异常。咳嗽,呼吸困难,咯血,和胸痛都是气管错构瘤的可能症状。非特异性症状也可能导致延迟诊断,虽然治疗的选择取决于病变的大小和位置,保守治疗仍强烈建议。本报告介绍了一名57岁的男性,他因吸气性呼吸困难来到我们部门。临床放射学数据和支气管镜检查显示为脂肪性错构瘤类型的良性气管肿瘤。患者行硬质支气管镜肿瘤切除术,临床效果满意。
    Hamartoma is the most frequently observed benign lung tumor, but its tracheal form is still exceptionally encountered. Cough, dyspnea, hemoptysis, and chest pain are all possible symptoms of tracheal hamartoma. The non-specific symptoms may also lead to a delayed diagnosis, and while the choice of treatment varies depending on the size and location of the lesion, conservative treatments remain strongly recommended. This report presents the case of a 57-year-old male who presented to our department with inspiratory dyspnea. Clinico-radiological data and bronchoscopy revealed a benign tracheal tumor of the lipomatous hamartoma type. The patient underwent a tumor resection by rigid bronchoscopy with satisfactory clinical results.
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  • 文章类型: Journal Article
    目的:错构瘤是肺部常见的良性肿瘤。很少,肺癌符合可能发生在诊断时或随访期间。
    方法:在2016年至2019年之间,对38例接受外科手术并诊断为肺错构瘤的患者的临床病理特征进行了回顾性评估。根据年龄对病例进行分析,性别,放射学发现,结节的定位,手术方法,和肺癌的巧合。
    结果:平均年龄为50.2±11.1(范围28-76岁)。男性23例(60.5%),女性15例(39.5%)。平均大小为2.7±1.8(范围为0.8-10厘米)。在28名患者中,错构瘤直径<3厘米(73.6%)。18例错构瘤位于上叶(47.4%)。只有6例(15.8%)位于肺中央部分。多结节10例(26.3%)。在4例(10.5%)中,肺癌和错构瘤在诊断时同时出现。电视胸腔镜手术(VATS)29例(76.3%)。作为一种手术方法,4例(10.5%)进行了眼球摘除,楔形切除28例(73.7%),肺叶切除6例(15.8%)。早期随访未出现术后死亡。
    结论:肺错构瘤通常表现为具有良性放射学表现的孤立性肺结节。VATS楔形切除术是一种可以安全地用于诊断和治疗的方法。错构瘤在诊断或随访时可能与肺癌有关,因此,应该记住,在诊断为错构瘤的患者中看到的不同结节可能与肺癌有关。
    OBJECTIVE: Hamartomas are common benign tumors of the lung. Rarely, lung cancer coincidence may occur at the time of diagnosis or in the follow-up period.
    METHODS: Between 2016 and 2019, 38 patients who underwent a surgical procedure and diagnosed with lung hamartoma were retrospectively evaluated regarding clinicopathological features. Cases were analyzed according to age, sex, radiological findings, localization of nodules, surgical methods, and the coincidence of lung cancer.
    RESULTS: The mean age was 50.2±11.1 (range 28-76 years). There were 23 male (60.5%) and 15 female (39.5%) patients. Mean size was 2.7±1.8 (range 0.8-10 cm). In 28 patients, hamartoma was <3 cm in diameter (73.6%). Eighteen hamartomas were localized in the upper lobe (47.4%). Only 6 cases (15.8%) were localized at the central part of the lung. Multiple nodules were reported in 10 cases (26.3%). In 4 cases (10.5%), lung carcinoma and hamartoma were seen together at the time of diagnosis. Video-assisted thoracoscopic surgery (VATS) has been performed in 29 cases (76.3%). As a surgical method, enucleation was performed in 4 cases (10.5%), wedge resection in 28 cases (73.7%), and lobectomy in 6 cases (15.8%). No post-operative mortality appeared in the early follow-up.
    CONCLUSIONS: Pulmonary hamartomas are usually present as solitary pulmonary nodules with benign radiological findings. VATS wedge resection is a method that can be used safely in diagnosis and treatment. Hamartomas may be associated with lung cancer at the time of diagnosis or follow-up, so it should be kept in mind that a different nodule seen in patients diagnosed with hamartoma may be associated with lung cancer.
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