Cytohistological correlation,immunohistochemistry and Murine Double Minute Clone 2 amplification of pulmonary artery intimal sarcoma: A case report with review of literature

Pulmonary artery intimal sarcoma is a rare aggressive intraluminal tumor often misdiagnosed as acute or chronic pulmonary thromboembolism due to its clinical presentation and radiological findings. Thus early diagnosis is very crucial and may improve patient outcome. There is limited literature on diagnosis of pulmonary artery sarcoma by endobronchial ultrasound‐guided transbronchial needle aspiration (EBUS‐TBNA). Herein, we report a case of mass‐like lesion in the PA diagnosed on cytological material obtained by EBUS‐TBNA with rapid on‐site evaluation (ROSE). The aspirate showed pleomorphic malignant spindled cells arranged in loosely cohesive clusters. The intraluminal origin of PAIS was supported by radiographic findings. Subsequently, the patient received preoperative chemotherapy and underwent tumor resection with reconstruction. This report describes the cytomorphologic features of this rare intravascular tumor and demonstrates how limited cytological sample obtained from EBUS‐TBNA with ROSE can be triaged efficiently for ancillary studies like immunohistochemistry and MDM2 amplification, thus expediting the management.     

Primary intimal sarcoma of pulmonary valve and trunk with osteogenic sarcomatous elements. Report of a case considered to be pulmonary embolus.

A 49-year-old woman had a six-week history of increasing shortness of breath and fatigue. X-ray films and pulmonary scans showed multiple areas of emboli, especially in the right lung. Treatment with heparin was unsuccessful, and although a repeat scan showed only slight changes, the patient's condition deteriorated. Acute pulmonary edema and ventricular tachycardia preceded her death. Autopsy revealed a primary intimal sarcoma with osteogenic elements arising in the posterior leaflet of the pulmonary valve and obstructing the main pulmonary artery and its right branch.     

An unusual primary vascular tumor: intimal sarcoma of the pulmonary artery

Primary sarcomas of great vessels are rare and involve the aorta, pulmonary artery and inferior vena cava. The pathologic classification of these tumors can be made on the location of the sarcoma in relation to the vessel wall, luminal or mural. Luminal sarcomas are usually intimal sarcoma and mural sarcoma are most frequently leiomyosarcoma. The myofibroblastic or endothelial differentiation of these tumors is still debated. We report a case of intimal sarcoma of the pulmonary artery.     

人肺动脉干、右肺动脉和左肺动脉多层螺旋CT成像的基础研究

目的利用多层螺旋CT对人肺动脉干、右肺动脉和左肺动脉成像,测量评价肺动脉干、右肺动脉和左肺动脉。方法选取正常人340例,胸部多层螺旋CT增强扫描,将图像传至工作站进行三维重建,观察测量肺动脉干、右肺动脉和左肺动脉,然后对肺动脉干、右肺动脉和左肺动脉进行比较分析。结果①螺旋CT扫描成像测量的肺动脉的管径明显小于X线测量的肺动脉的管径,有统计学意义(P0.05);②男、女性别之间的肺动脉管径也有显著差异(P0.05);③不同年龄组别之间的肺动脉也有差异(P0.05);④右、左肺动脉之间比较也有显著性统计学意义(P0.01);⑤不同的重建方法各有其优越性,其中,MIP重建能较好的显示肺动脉干、右肺动脉左肺动脉,并且其测量值比其它重建方法测量较为准确。结论多层螺旋CT胸部增强扫描,通过工作站三维重建能较好的显示肺动脉干、右肺动脉和左肺动脉,并对其进行科学的评价,这种方法优于传统的尸体解剖方法,其数据可靠,此方法可以对活体人的器官进行大样本的形态学研究。     

Intimal sarcoma of the pulmonary artery: report of an autopsy case

Primary pulmonary artery sarcomas (PASs) are rare and lethal tumors. They are easily misdiagnosed as chronic pulmonary embolism, mediastinal mass or tumor emboli, which delay a proper treatment. Although the advanced technologies are now increasingly being used, their diagnosis is usually hard to establish preoperatively at the present time. We report here a case of a 68-year-old female with PAS with lung metastases, who firstly presented with symptoms of common cold and anemia. Although a PAS had been suspected, the final diagnosis of pulmonary intimal sarcoma was made only postoperatively by histological and immunohistochemical examination. The patient died 8 months after the operation because of tumor growth progression, despite adjuvant chemotherapy and radiation therapy. Although pulmonary intimal sarcomas are usually of poorly differentiated mesenchymal malignancy, most reported cases are immunohistochemically positive for vimentin, alpha-smooth muscle actin (SMA), and/or desmin, therefore resembling leiomyosarcomas. However, the diagnosis of leiomyosarcoma should not be made on the basis of immunostains in the absence of typical morphologic features, and PAS, like the present case, should be more appropriately classified as intimal sarcoma according to the new WHO Classification of Tumours of Soft Tissue and Bone published in 2002.     

Chronic expanding hematoma of the thorax

We report the first case in Korea of a chronic expanding hematoma, which presented as a huge mass in the pleural cavity. A 67-year-old woman exhibiting a slowly-expanding intrathoracic mass, as revealed by a chest radiograph, was admitted to our hospital. The patient had undergone a pneumonectomy 37 years earlier during treatment for pulmonary tuberculosis. Computed tomography revealed a huge mass in her right hemithorax. The differential diagnosis of this mass included chronic empyema combined with a malignancy, such as lymphoma or a soft tissue sarcoma. The tumor, which was classified as an encapsulated chronic hematoma, was removed surgically. Samples sent for histopathological and microbiological analysis revealed no evidence of neoplasia or infection. The patient was finally diagnosed with a chronic expanding hematoma of the thorax. This case is particularly rare due to the patient's development of a very large mass after undergoing treatment for tuberculosis more than 30 years earlier.     

螺旋CT成像对右肺上叶肺段与亚肺段动脉管径的评价

目的探讨多层螺旋CT成像对右肺上叶肺段和亚肺段动脉管径的评价。方法 240例行胸部CT增强扫描的正常成人,运用不同方法进行三维重建并测量右肺上叶肺段和亚肺段动脉管径,再按性别分2组、按年龄分3组(20～40岁、41～60岁、61～87岁)进行分析比较。结果①性别之间、不同年龄组之间的右肺上叶肺段和亚肺段动脉管径差异均具有统计学意义(P<0.05);②不同重建方法各有其优越性,MPR重建图像能较好显示肺段和亚肺段动脉,应用此法测量动脉管径较为准确。结论多层螺旋CT成像能较好评价右肺上叶肺段和亚肺段动脉管径,为临床诊断提供参考数据。     

Tumor-related thrombotic pulmonary microangiopathy: review of pathologic findings and pathophysiologic mechanisms

We report a middle-aged woman who died 2 days after presenting with dyspnea and severe pulmonary hypertension of unknown etiology. Her symptoms were highly suggestive of pulmonary embolism, but clinical evaluations for that disease yielded negative results. Autopsy revealed a Krukenberg tumor of the left ovary, representing metastatic gastric carcinoma from an occult primary lesion. Although the lungs exhibited no gross evidence of pulmonary emboli or neoplasia, microscopic examination revealed diffuse microscopic metastases in the pulmonary arterial vasculature. The pulmonary arteries exhibited fibrocellular intimal proliferation with smooth muscle colonization of the luminal neoplastic lesions and associated microthrombi. This disease entity, known as tumor-related thrombotic pulmonary microangiopathy, results in generalized microvascular obliteration and subsequent pulmonary hypertension. It is a rare condition that is distinct from ordinary pulmonary thromboembolism and primary pulmonary hypertension. Tumor-related thrombotic pulmonary microangiopathy should be considered diagnostically by the autopsy pathologist in cases of rapidly evolving pulmonary hypertension in a middle-aged or elderly individual, or respiratory failure of unknown cause, especially if there is a history of a visceral malignancy.     

A case of deep vein thrombosis and pulmonary thromboembolism after intravenous immunoglobulin therapy

Although high-dose intravenous immunoglobulin (IVIG) is generally considered a safe medication for various immune-mediated diseases, thrombotic events have been reported as a complication of the therapy. We report a case who developed thrombotic complications after receiving IVIG. A 56-yr-old woman with idiopathic thrombocytopenic purpura received IVIG at a dose of 400 mg/kg/day for five days. Three days after the administration of IVIG, the patient developed painful edema in the left leg. Lower extremity doppler ultrasound revealed deep vein thrombosis in the left leg. Chest computed tomography (CT) scan demonstrated a filling defect indicating thromboembolism of the right pulmonary artery. After three weeks of enoxaparin therapy, her symptoms and pulmonary embolism on CT improved. This case suggests clinicians should be cautious in the development of thromboembolism by administration of IVIG, especially in patients with thrombophilia.     

RUNX‐1 and CD44 as markers of resident stem cell derivation in undifferentiated intimal sarcoma of pulmonary artery

Vasuri F, Resta L, Fittipaldi S, Malvi D & Pasquinelli G
(2012) Histopathology  61, 737–743 RUNX‐1 and CD44 as markers of resident stem cell derivation in undifferentiated intimal sarcoma of pulmonary artery Aims: To report a rare case of undifferentiated intimal sarcoma (UIS) of the pulmonary artery in a 44‐year‐old woman, and to study it by electron microscopy and a novel immunohistochemical (IHC) panel that recognizes markers of endothelial and haematopoietic stemness, in order to extend current knowledge about the histogenesis of this rare neoplasm. Methods and results: Immunohistochemical reactions for CD31, CD34, α‐smooth muscle actin (α‐SMA), caldesmon, calponin, actin, desmin, epithelial membrane antigen, WT1, CD117, Ki67, nestin, RUNX‐1, platelet‐derived growth factor, NG2, CD44, CD90 and CD105 were performed manually or automatically. Neoplastic cells were negative for CD31 and CD34, but positive for calponin, nestin, WT1, CD44, and RUNX‐1. Electron microscopy was performed after osmium tetroxide fixation and staining with lead citrate and uranyl acetate. Ultrastructurally, tumour cells had slightly irregular nuclei, cisternae of rough endoplasmic reticulum, and punctate intercellular junctions. Conclusions: We report a case of pulmonary artery UIS expressing previously unreported markers, i.e. RUNX‐1, nestin, WT1, and CD44, that are commonly seen in different stages of the vascular differentiation hierarchy. These findings, together with the negativity for mature endothelial and smooth muscle markers, raise the question of whether this neoplasm may derive from a vessel wall‐resident stem cell, such as the haemangioblast or an embryonic‐like stem cell.     

Intimal-type primary sarcoma of the aorta

We report an intimal sarcoma presenting as an aortic aneurysm. A 68-year-old man suffered from chest pain and speech disturbance. Computed tomography showed a sacciform aneurysm of the aorta, which was resected, revealing a polypoid tumour measuring 1.5x2x2.5 cm projecting into the lumen. This proved to be a poorly differentiated high-grade sarcoma having morphological, immunophenotypic and ultrastructural features consistent with rhabdomyosarcomatous differentiation. Primary sarcomas of the aorta are extremely rare. Many cases have been diagnosed as "intimal" on the basis of their site of origin, and they are not easy to classify from their histological pattern. Electron microscopy and the use of a more comprehensive panel of immunohistochemical markers should be applied in the histological classification of"intimal" sarcoma.     

Unusual cause of acute right ventricular dysfunction: rapid progression of superior vena cava aneurysm complicated by thrombosis and pulmonary thromboembolism

Aneurysms of the major thoracic veins are rare. They are usually asymptomatic and thus treated conservatively. We report an extremely rare case of rapidly progressing superior vena cava (SVC) aneurysm complicated by thrombosis and acute pulmonary thromboembolism (PTE) with right ventricular dysfunction. Thrombolytic therapy for hemodynamically significant acute PTE was harmful to the patient in the present case, because it induced further thrombosis and mobilization of the thrombi within the aneurysm, subsequently causing de novo PTE. Surgical aneurysmectomy combined with pulmonary artery embolectomy would be a treatment of choice in patients with SVC aneurysm complicated by acute PTE.     

Right pulmonary artery sarcoma

A 57-year-old man presented with cough, chest pain and dyspnea. Peripheral lung opacities detected radiologically on admission were surgically resected and histologically confirmed as pulmonary infarcts. Subsequent radiological examinations revealed a stricture of the right pulmonary artery with evidence of pulmonary hypertension. The patient was commenced on anticoagulant therapy, which resulted in some clinical improvement. A hilar mass detected later was resected together with the right lung. Pathological examination of the hilar mass revealed a tumour within the lumen of the pulmonary artery causing almost total occlusion of the artery. Histology showed angiosarcomatous and osteosarcomatous areas. The patient is well and disease-free 9 months following resection.     

Hyalinizing spindle cell tumor with giant rosettes with pulmonary metastasis after a long hiatus: a case report

Hyalinizing spindle cell tumor with giant rosettes (HSCTGR) is a recently described tumor, which is regarded as an unusual variant of low-grade fibromyxoid sarcoma. Proof of a metastatic potential was lacking. The patient in the report was a 35-yr-old woman who showed multiple bilateral pulmonary nodules with massive pleural effusion in the right side. She had a history of a mass excision in the right thigh 11 yrs ago at another hospital, which was reported as a "leiomyoma". Two years before this presentation, the patient received a routine chest radiograph which demonstrated bilateral multiple pulmonary nodules. A lobectomy of the left upper lung was performed. The histological findings revealed a well-circumscribed nodule that was characterized by a spindle-shaped fibrous to hyalinized stroma with criss-crossing short fascicles and giant collagen rosettes surrounded by a rim of spindle-shaped cells. Electron microscopy confirmed the fibroblastic nature of the tumor. This case, in addition to at least two other cases reported in the literature, demonstrates that the HSCTGR is a malignant neoplasm with the capacity to metastasize after a long hiatus.     

Isolated Petroclival Craniopharyngioma with Aggressive Skull Base Destruction

We report a rare case of petroclival craniopharyngioma with no connection to the sellar or suprasellar region. MRI and CT images revealed a homogenously enhancing retroclival solid mass with aggressive skull base destruction, mimicking chordoma or aggressive sarcoma. However, there was no calcification or cystic change found in the mass. Here, we report the clinical features and radiographic investigation of this uncommon craniopharyngioma arising primarily in the petroclival region.     

Resolution of pulmonary multiplanar reconstruction images from 0.5-mm theoretical isotropic data: a fundamental study using an inflated and fixed lung specimen

The aim of the present study was to define the resolution of multiplanar reconstruction (MPR) of the lung from "theoretical isotropic data." Using inflated and fixed lung specimens of the pig placed in the chest wall phantom, 0.5-mm isotropic data were obtained with 2 different helical pitches: 1:7 (high-quality mode) or 1:13, (high-speed mode), and 2 different tube currents: 250 mAs (high-tube-current mode) or 100 mAs (low-tube-current mode), with or without overlapping reconstruction. MPRs were created from these axial data. The diameter of the smallest visible pulmonary artery and bronchi of these CT images were measured on the corresponding slices of the specimen. The high-speed and low-tube-current mode significantly degraded the image quality due to increased noise. The smallest visible pulmonary artery and bronchus resolved on MPRs from axial-spiral data with 0.5-mm collimation were approximately 100 micrometer and 1,000 micrometer in diameter, respectively. In conclusion, helical pitch and tube current influence the resolution of MPR of the lung.     

肺动脉内膜肉瘤临床病理学观察

目的观察肺动脉内膜肉瘤的临床病理特征,了解其基因改变情况。方法分析中日友好医院2017年1月至2020年1月间行肺动脉内膜剥除术病例60例,收集其中5例肺动脉内膜肉瘤患者临床资料,采用HE染色、免疫组织化学染色及荧光原位杂交进行病理形态学观察,通过RNA测序方法分析肿瘤融合基因改变。结果肺动脉内膜肉瘤检出率为8.3%(5/60),中位年龄49岁,女性多于男性,临床表现不特异。肿瘤由异型梭形细胞或上皮样细胞组成,可出现骨肉瘤及平滑肌肉瘤分化。肿瘤细胞可表达血小板源性生长因子受体α、细胞周期蛋白依赖性激酶4(CDK4)及MDM2,并伴有MDM2、CDK4及表皮生长因子受体基因共扩增。RNA测序检出多种框内融合基因改变。结论肺动脉内膜肉瘤是一类罕见、高度异质性并伴有多基因复杂改变的低~未分化肉瘤,治疗效果不佳,预后差。     

Instillation of Amphotericin B by bronchoscopy combined with systemic voriconazole in advanced non-small cell lung cancer patients with chronic cavitary pulmonary aspergillosis: A case series and literature review

Although the treatment of aspergillosis has been studied for years, the optimal nonsurgical treatment of chronic cavitary pulmonary aspergillosis (CCPA) remains unsatisfactory, especially in lung cancer. We report two advanced non-small cell lung cancer (NSCLC) patients who recovered from CCPA following instillation of Amphotericin B (AmB) by bronchoscopy combined with systemic voriconazole. The first patient was diagnosed with lung adenocarcinoma after right upper lobe resection and was treated with anaplastic lymphoma kinase-targeted therapy. Chest computed tomography (CT) revealed a right pulmonary cavity containing solid materials. The second patient was diagnosed with squamous cell carcinoma and received immunotherapy following surgery, chemotherapy, and radiotherapy. Chest CT tomography revealed a mass in the right lung cavity. Both patients' cultures and next-generation sequencing of their bronchoalveolar lavage (BAL) samples revealed presence of Aspergillus fumigatus. In addition, the galactomannan test of both patients BAL samples was positive. Systemic voriconazole was prescribed based on in vitro susceptibility testing. The chest images and clinical symptoms of both patients did not improve after one month of voriconazole therapy within the therapeutic blood concentration. Considering the low local concentrations of antifungals against CCPA, AmB instillation by bronchoscopy combined with systemic voriconazole was utilized. The chest CT images and clinical symptoms of both patients markedly improved in the following third month. Instillation of AmB combined with systemic voriconazole may be a promising treatment option for NSCLC patients with CCPA who fail voriconazole monotherapy.     

CT三维重建辅助达芬奇机器人解剖性肺段切除术的临床应用

目的 探讨CT三维重建技术辅助达芬奇机器人解剖性肺段切除的可行性及临床效果。方法 回顾性分析。纳入2017年5月-2018年9月上海交通大学医学院附属瑞金医院胸外科接受达芬奇机器人肺段切除术的肺结节患者47例, 其中男16例、女31例,年龄(53.15±12.19)岁,均在术前依据CT影像资料完成肺三维重建,肺部结节最大径为(8.04±2.96)mm。术者依据重建影像设计并进行解剖性肺段切除术,收集分析患者一般临床特征及围手术期资料。结果 术前CT三维重建可清晰显示肺部动、静脉和支气管的空间位置关系,可准确显示肺结节的位置和肺段边界。所有患者均根据术前CT三维重建影像设计并完成达芬奇机器人解剖性肺段切除术,其中40例肺段切除术和7例肺亚段切除术;手术中解剖观察与术前CT三维重建对比,患者肺血管、支气管位置及分支的显示均吻合,手术均顺利完成,无中转开胸。手术时间(129.68±43.14)min,术中出血(104.36±76.31)mL,术后胸腔引流量(748.30±444.63)mL,术后拔管时间(2.89±1.73)d,术后住院时间(4.38±1.78)d,术后并发症发生率为8.5%(4/47)。术后病理结果显示:恶性肿瘤42例,良性病变5例。42例恶性肿瘤患者切缘均为阴性,淋巴结清扫个数为(7.29±5.62)枚,淋巴结清扫站数为(5.09±1.75)站。术后随访3个月,无一例患者肿瘤复发或死亡。结论 CT三维重建辅助达芬奇机器人解剖性肺段切除术是安全有效的。     

Thymic adenocarcinoma associated with thymic cyst: a case report and review of literature

Thymic adenocarcinoma of the mediastinum is extremely rare. Only 12 cases adenocarcinoma associated with a thymic cyst have been reported in the literature. In this report, we describe a case of a thymic adenocarcinoma associated with thymic cyst. A 70-year-old man, presented with two months of chest tightness, breath shortness and chest pain. The contrast enhanced CT scan of chest revealed a round, cystic mass in right anterior mediastinum. Microscopic examination revealed that the lining consisted of flat to cuboidal and columnar epithelium and adenocarcinoma infiltrated into the thymic tissue and the soft tissue around the wall of cyst. Based on clinical history, imaging studies, pathological findings and absence of extramediastinal tumor, a diagnosis of thymic adenocarcinoma associated with thymic cyst was established. The patient was alive without any sign of recurrence 7 months after the operation.