Computed tomography in pulmonary artery sarcoma: distinguishing features from pulmonary embolic disease

OBJECTIVE: The purpose of this study was to present the computed tomography (CT) findings of pulmonary artery sarcoma in 7 patients with a focus on the distinguishing features of pulmonary embolic disease. METHODS: For the 9 years from December 1993 to November 2002, we treated 7 patients with pathologically proven pulmonary artery sarcoma, and during the 2 years from December 2000 to November 2002, we treated 40 patients with acute (n = 33) or chronic (n = 7) pulmonary embolism. In these patients, pulmonary embolism was diagnosed from serial CT or clinical findings. Two chest radiologists, blinded to the diagnoses, independently reviewed the scans of all 47 patients in random order, and the so-documented CT features of sarcoma and pulmonary embolism were compared by using Fisher exact test or the generalized estimating equations test. RESULTS: The two most frequent CT findings of pulmonary artery sarcomas were a low-attenuation filling defect occupying the entire luminal diameter of the main (n = 1) or proximal (n = 6) pulmonary artery and an expansion of any segment of the pulmonary artery with extensive intraluminal filling defect, as observed in six (86%) of 7 patients. In contrast, the finding of a lesion occupying the entire luminal diameter at the level of proximal pulmonary arteries was absent in all 40 patients with pulmonary embolism (P < 0.0001) (kappa = 0.9111). Expansion of the pulmonary arteries was seen in one (3%) of 40 patients with pulmonary embolism (P < 0.0001) (kappa = 0.9108). Extraluminal extension was observed in 5 of 7 (71%) patients with sarcoma, but in no patient with an embolism (P < 0.0001) (kappa = 0.8773). CONCLUSION: CT can help differentiate pulmonary artery sarcoma from pulmonary embolism by indicating a low-attenuation filling defect occupying the entire luminal diameter of the proximal or main pulmonary artery, expansion of the involved arteries, or extraluminal tumor extension.     

Pulmonary artery sarcoma mimicking chronic thromboembolic disease: Computed tomography and magnetic resonance imaging findings

Purpose The diagnosis of the rare primary malignant tumors of the pulmonary arteries is often delayed as symptoms are nonspecific.Methods Computed tomography (CT) and magnetic resonance imaging (MRI) of three patients with occlusion of the right pulmonary artery, two with sarcoma and one with chronic thromboembolic disease were analyzed for discriminating diagnostic criteria.Results Criteria suggesting pulmonary artery sarcoma are inhomogeneous high or low attenuation (hemorrhage, necrosis), soft-tissue density in pulmonary arteries, vascular distension, enhancement after administration of gadopentetate dimeglumine. Criteria for chronic thromboembolic disease are homogeneous soft-tissue lesions, abrupt vascular narrowings and cutoffs, as well as regional parenchymal hyperdensities.Conclusion CT and MRI provide complementary findings suggesting advanced pulmonary artery sarcoma instead of chronic thromboembolism.     

原发性肺肉瘤的CT表现及鉴别诊断

目的 探讨原发性肺肉瘤(PPS)的CT特征表现.方法 回顾分析5例PPS患者的CT表现和临床病史、病理资料,结合相关文献进行分析总结.结果 肿块较大,最大径约9.8 cm,平均为6.3 cm.1例呈鹿角状,1例类圆形,3例不规则团块状;5例患者均未见毛刺征象;2例伴有纵隔淋巴结肿大;5例患者中病灶内均未见钙化;1例伴有同侧肺动脉侵犯形成肺动脉瘤征象,1例伴有同侧下肺动脉栓塞;5例增强后病灶均轻度强化;2例伴有同侧胸腔积液征象.5例患者周围骨质均未见骨质破坏征象.结论 PPS的CT表现具有相对特异性,确诊需要依靠病理检查.     

Rare causes of pulmonary hypertension: spectrum of radiological findings and review of the literature

Following a brief introduction covering the clinical signs and symptoms of pulmonary hypertension (PH), its most recent classification into six groups, and the computed tomography (CT) features common to all forms of PH, this paper illustrates the typical patterns that can be found on chest radiography and CT in rare causes of PH. We present and compare with the existing literature our personal series of cases of rare forms of PH, found in the following diseases: veno-occlusive disease, pulmonary capillary haemangiomatosis, non-thrombotic pulmonary embolism (tumour embolism and carcinomatous lymphangitis, talcosis, hydatid disease), pulmonary artery sarcoma, neurofibromatosis, sarcoidosis, and Langerhans cell histiocytosis. Rare forms of PH show low incidence and prevalence, and are, therefore, poorly recognised. Their diagnosis is a challenge for clinicians, pathologists, and radiologists, and any additional knowledge about the CT findings may help the diagnosis in the case of patients affected by PH of unknown origin.     

PET-positive fibrous dysplasia--a potentially misleading incidental finding in a patient with intimal sarcoma of the pulmonary artery

Benign bone tumors can show an increased FDG uptake in FDG-PET/CT investigations. In the presented case, an incidentally detected PET-positive asymptomatic fibrous dysplasia was initially misinterpreted as a metastasis in a patient with intimal sarcoma of the pulmonary artery.     

Primary pulmonary malignant fibrous histiocytoma mimics pulmonary artery aneurysm with partial thrombosis: various radiologic evaluations

Primary pulmonary malignant fibrous histiocytoma (MFH) is very rare, so only a few imaging features have been reported. We report one case of rapidly growing primary pulmonary MFH mimicking a partially thrombosed pulmonary artery aneurysm and its radiologic findings, including multidetector row computed tomography (MDCT), conventional angiography, and fluorodeoxyglucose-positron emission tomography CT ([18F] FDG-PET/CT). On multi-phasic MDCT, this mass mimicked a pulmonary artery aneurysm with partial thrombosis. However, pulmonary artery aneurysm was excluded and suggested as a hypervascular parenchymal mass by subsequent conventional angiography. On [18F] FDG-PET/CT, it was a highly metabolic mass, showing a maximal standard uptake value (SUV) 12.1. Although primary pulmonary MFH is very rare and has no specific imaging findings, our experience might be helpful to differentiate a hypervascular pulmonary mass.     

The computed tomographic appearances in pulmonary artery atresia

Isolated pulmonary artery atresia is an uncommon congenital abnormality. Plain radiography and ventilation/perfusion lung scintigraphy may suggest the diagnosis but pulmonary angiography has traditionally been recommended for confirmation. The computed tomographic (CT) appearances of four patients with isolated atresia of a pulmonary artery are described. In each case the atretic segment of the abnormal vessel could be identified. Other CT findings included reduction of volume of the involved hemithorax, increased density of the affected lung, a small hilum, and an abnormal pulmonary vascular pattern thought to be due to the collateral systemic arterial supply to the intrapulmonary branches of the atretic pulmonary artery. The diagnosis of isolated pulmonary artery atresia can be made using dynamic, contrast enhanced CT without recourse to more invasive procedures.     

Pulmonary artery sarcoma detected on F-18 FDG PET/CT as origin of multiple spinal metastases

A 67-year-old man with back pain was diagnosed as having multiple spinal metastases on MRI. On CT scan, only a filling defect in the right pulmonary artery was observed and suspected as venous thromboembolism. On F-18 fluorodeoxyglucose (FDG) PET/CT, intense hypermetabolism was observed in the right pulmonary artery in addition to the metastatic spine lesions. Biopsy confirmed the lesion as a primary pulmonary artery sarcoma (PAS), and the spine lesions as metastases of PAS. Although PAS is rare and its bone metastasis presenting initial symptom is extremely rare, FDG PET/CT is an effective diagnostic modality for PAS, not only in discrimination from venous thromboembolism, but also in workup of metastatic origin.     

Pulmonary Metastases of Alveolar Soft-Part Sarcoma: CT Findings in Three Patients

Alveolar soft-part sarcoma is a rare soft tissue sarcoma of young adults with unknown histogenesis, and the organ most frequently involved in metastasis is the lung. We report the CT findings of three patients of pulmonary metastases of alveolar soft-part sarcoma, which manifested as clearly enhanced pulmonary nodules or masses. On enhanced scans, some of the masses were seen to contain dilated and tortuous intratumoral vessels.     

Primary pulmonary artery myxoma: a rare case

Pulmonary embolism is the most frequent diagnosis for a filling defect in the pulmonary artery, but a tumor in the arteriae pulmonalis should be contained in the differential diagnosis. Primary pulmonary artery myxoma is extremely rare, and only a few cases have been reported. The early diagnosis of this disease is difficult, but it is feasible with modern radiographic methods, which play an important role in the presentation of the origin and extension of the tumor. Here, we review one case with computed tomographic (CT) and pulmonary CT angiographic findings to emphasize the significance of the imaging method in its diagnosis.     

CT findings of pulmonary hamartoma with special reference to epithelial-lined clefts and connection with pulmonary arteries

PURPOSE: The purpose of this study was to clarify the characteristic CT findings of pulmonary hamartoma. MATERIALS AND METHODS: The thin-section CT and multiplanar reformation images of 19 pulmonary hamartomas diagnosed by surgical resection were analyzed and correlated with the pathological findings. RESULTS: Most hamartomas presented lobulated nodules apart from pleura. There was no case in which fat density was recognized. Only one case was recognized as having calcification. Air density in the connection of the side or the inside was pointed out in 5 cases (26%). Air density reflected epithelial-lined cleft. The connection with the bronchus was recognized in 4 cases (21%), and the connection with the pulmonary artery branch was recognized in 10 cases (53%). However, the connection of the pulmonary artery and bronchus could not be pathologically confirmed in most cases. The connection with the pulmonary vein was not recognized in any of the cases. CONCLUSION: Air density in the connection of the side or the inside is characteristic of pulmonary hamartoma. Pulmonary artery branches connect beyond half of hamartomas. This finding suggests close relations in the bronchus along the artery. It is important that there is no connection of the pulmonary vein, to differentiate it from lung cancer.     

Osteosarcoma of the lumbosacral spine invading the central venous pathways,right-sided cardiac chambers,and pulmonary artery

We report an unusual case of lumbosacral osteogenic sarcoma with cauda equina syndrome and invasion into the central venous and cardiac system. A 41-year-old Hispanic man presented to the emergency department complaining of severe low back pain, cauda equina syndrome, bilateral lower extremity edema, and an extra heart sound on physical examination. CT of the lumbosacral spine done in the emergency department demonstrated a sclerotic lesion in the sacrum with cortical destruction, extension into the spinal canal and a bulky soft tissue mass containing calcifications. Supplemental MRI demonstrated marrow replacement of L4, L5, and the sacrum, soft tissue extension of the tumor, and invasion iliac veins extending into the IVC; however, the full extent of the intravascular tumor was not seen on this examination. Surgical laminectomy and biopsy of the spinal tumor provided the diagnosis of osteogenic sarcoma. A transthoracic echocardiogram was performed while the patient was recovering due to nonsustained ventricular tachycardia, which showed an echogenic mass within the right atrium and ventricle. CT pulmonary angiogram confirmed the echocardiogram showing a tumor extending through the pulmonary valve into the main pulmonary artery. The patient underwent en bloc resection of the tumor from the venous and cardiac systems. Histologic examination of the tumor confirmed osteogenic sarcoma. While vertebral osteogenic sarcoma is uncommon, invasion of the spinal canal is common in these tumors. However, tumor extending into the central venous and cardiac system is rare. The previously reported cases of central venous and cardiac involvement have been related to distant metastases or primary cardiac osteosarcomas. There is only one other reported case of direct extension into the venous system by an iliac bone osteosarcoma in an adolescent; however, the tumor did not extend into the pulmonary circulation.     

64层螺旋CT心胸联合造影在急性胸痛病因鉴别诊断中的价值

目的 探讨64层螺旋CT在急性胸痛病因鉴别诊断中的临床价值。方法 对36例临床急性胸痛的患者行64层螺旋CT心胸联合血管造影检查。对所有扫描原始数据用冠状、矢状面多平面重组（MPR）、曲面重组（CPR）、容积再现（VR）和最大密度投影（MIP）等方法行冠状动脉、肺动脉和胸主动脉成像。由2名有经验的放射科医生对所有图像进行观察，对疾病进行诊断，其中16例患者同时行选择性血管造影检查。结果 所有行64层螺旋CT心胸联合血管造影的患者1次检查均可清晰显示双侧肺动脉、冠状动脉主干及其主要分支以及胸主动脉，同时均可进行纵隔及肺窗重组，对胸部疾病进行诊断。显示冠状动脉狭窄10例，急性肺动脉栓塞14例，主动脉夹层6例（其中1例同时伴有冠状动脉右支急性血栓形成）；并诊断气胸1例、缩窄性心包炎1例；4例未见明显异常。结论 64层螺旋CT1次心胸联合血管造影检查能清晰地显示冠状动脉、肺动脉及主动脉，对其病变作出正确诊断，还能清晰地显示胸部疾病，是急性胸痛病因诊断无创、可靠的检查方法。     

A case of pulmonary synovial sarcoma diagnosed with detection of chimera gene: imaging findings

Pulmonary synovial sarcoma is a rare disease, and reports detailing clinicians' radiological findings are few. We report a case of a primary pulmonary synovial sarcoma in a 68-year-old woman. Chest CT revealed a well-defined and homogeneous oval mass measuring 3x2.5 cm in the left lower lobe in contact with the visceral pleura. No pleural effusion was evident. No calcification or fat component was detected. The tumor showed homogeneous hypointensity on both T1- and T2-weighted MR imaging. In this case, a lung metastasis could be excluded with fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET), and the final diagnosis was histopathologically confirmed by the chimeric gene detection.     

Pulmonary artery intimal sarcoma: the role of <Superscript>18</Superscript>F-fluorodeoxyglucose positron emission tomography in monitoring response to treatment

We report the case of 58-year-old man with pulmonary artery intimal sarcoma. He initially presented with cough, right-sided chest pain, and shortness of breath. Although the diagnosis of pulmonary embolism had been considered, chest radiograph and pulmonary perfusion scintigraphy showed a mass in the right hilum and no perfusion in the right lung. ¹⁸F-fluorodeoxyglucose positron emission computed tomography (FDGPET) showed increased FDG uptake in the mass obstructing the right pulmonary artery. Fine-needle biopsy revealed a pathological diagnosis of pulmonary artery intimal sarcoma. The patient was successfully treated with radiotherapy and adjuvant chemotherapy. FDG-PET was used for monitoring the response to therapy.     

MR and CT findings in pulmonary artery sarcoma

Pulmonary artery sarcomas are a rare and frequently forgotten cause of pulmonary artery occlusion. We present a case report that details magnetic resonance and new CT findings, which may help establish early diagnosis of this infrequently encountered tumor.     

Behcet病的胸部CT表现

目的 分析Behcet病的胸部CT表现,评价其在诊断中的作用.方法 回顾性分析13例有胸部病变的Behcet病的临床病历记录及CT影像资料,所有患者的诊断根据Behcet病国际研究小组诊断标准.结果 13例中3例表现为胸膜下斑片状实变影,3例为双肺弥漫磨玻璃密度影,1例左下叶塌陷伴有右下叶结节影,1例胸膜下孤立结节影,2例双侧少量胸水,2例伴有纵隔淋巴结增大,1例在治疗过程中CT表现为双肺弥漫性粟粒状结节.13例中8例有胸部血管病变,2例为上腔静脉栓塞;1例左锁骨下动脉瘤;1例左下肺动脉瘤伴附壁血栓;1例双下肺动脉瘤及左侧基底动脉瘤;3例右下肺动脉栓塞,其中2例伴有左肺动脉闭塞,1例伴有左侧基底动脉分支动脉瘤.结论 Behcet病的胸部CT表现多种多样,增强CT可显示胸部血管病变如上腔静脉或肺动脉栓塞、肺动脉瘤等,有助于对病变的评价.     

Primary synovial sarcoma of the sternum: computed tomography and magnetic resonance imaging findings

We report the computed tomography (CT) and magnetic resonance imaging (MRI) findings of a rare case of synovial sarcoma of the sternum in an 86-year-old man. CT demonstrated an inhomogenously enhanced soft-tissue-density mass of the sternum that destroyed bone cortex and protruded anteriorly. On MRI, the tumor showed a multinodular mass with internal septation and heterogeneous enhancement. These CT and MRI findings were nonspecific, but were similar to those of soft tissue synovial sarcomas. The tumor was more clearly demarcated by MRI than CT. This is the first report concerning the CT and MRI findings of synovial sarcoma of the sternum. Synovial sarcoma should be added to the gamut of primary malignant neoplasms of the sternum.     

Primary Ewing's sarcoma of the paranasal sinus with intracranial extension: imaging features

Extraskeletal Ewing's sarcoma is often described as a tumour involving the soft tissues of the lower extremities and the paravertebral region. Involvement of the paranasal sinus is a very rare entity. We present a case of primary Ewing's sarcoma of the paranasal sinus extending into the middle cranial fossa in a 40-year-old female. CT and MRI findings of this unusual case are discussed. To our knowledge, this case is the first to be reported with CT and MRI documentation.     

Primary leiomyosarcoma of the pulmonary artery: a diagnostic dilemma

Primary leiomyosarcoma of the pulmonary artery is a rare malignancy arising from the multipotential mesenchymal cell of the intima of the pulmonary artery. Due to its rarity and nonspecific clinical symptoms, the correct diagnosis and proper management are often delayed. Furthermore, it is frequently misdiagnosed as pulmonary embolism, mediastinal mass, pulmonary stenosis and lung cancer. Therefore, it is important to consider primary leiomyosarcoma of the pulmonary artery a possibility when a persistent filling defect is present in the pulmonary artery and there is no response to optimal anticoagulation treatment. Radiologic findings such as a unilateral mass continuously filling the pulmonary artery, inhomogenous enhancement, vascular distension, extravascular invasion into adjacent structure or uptake in the area of tumor on the FDG-PET can be helpful when differentiating pulmonary artery sarcoma (PAS) from chronic thromboembolism.