Pulmonary Artery Sarcoma: Diagnosis and Postoperative Follow-Up With Gadolinium-Diethylenetriamine Pentaacetic Acid-Enhanced Magnetic Resonance Imaging
1995; Elsevier BV; Volume: 70; Issue: 2 Linguagem: Inglês
10.4065/70.2.173
ISSN1942-5546
AutoresRichard B. Rafal, John N. Nichols, John A. Markísz,
Tópico(s)Vascular Anomalies and Treatments
ResumoPulmonary artery sarcomas are infrequently encountered and are often misdiagnosed before surgical exploration or autopsy. On clinical examination and conventional imaging studies, they are frequently mistaken for pulmonary emboli. To our knowledge, this case report is the first to describe the postoperative magnetic resonance imaging (MRI) appearance of residual disease of a pulmonary artery sarcoma and to assess the potential usefulness of MRI for monitoring such lesions postoperatively. Contrast-enhanced MRI, a relatively noninvasive method, seems well suited for postsurgical follow-up of pulmonary artery sarcomas. Pulmonary artery sarcomas are infrequently encountered and are often misdiagnosed before surgical exploration or autopsy. On clinical examination and conventional imaging studies, they are frequently mistaken for pulmonary emboli. To our knowledge, this case report is the first to describe the postoperative magnetic resonance imaging (MRI) appearance of residual disease of a pulmonary artery sarcoma and to assess the potential usefulness of MRI for monitoring such lesions postoperatively. Contrast-enhanced MRI, a relatively noninvasive method, seems well suited for postsurgical follow-up of pulmonary artery sarcomas. Pulmonary artery sarcomas almost always develop in the main pulmonary artery1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar and may extend into the pulmonary artery branches orproximally into the pulmonic valve, opposite pulmonary artery, or right ventricle.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar, 4Weinreb JC Davis SD Berkmen YM Isom W Naidich DP Pulmonary artery sarcoma: evaluation using Gd-DTPA.J Comput Assist Tomogr. 1990; 14: 647-649Crossref PubMed Scopus (53) Google Scholar When detected, about 50% of these tumors are confined intravascularly; the rest exhibit transmural extension into adjacent lung parenchyma, bronchi, or lymph nodes.5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar Distant metastatic disease can also occur.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar A 45-year-old woman experienced an episode of dyspnea on exertion followed by syncope and cyanosis 10 days before admission to a hospital to rule out myocardial infarction. Propranolol was prescribed after echocardiography was suggestive of idiopathic hypertrophic subaortic stenosis. After use of the medication had been discontinued, the symptoms recurred. A physical examination revealed a systolic murmur at the apex of the heart and along both upper sternal borders. Chest roentgenography showed cardiomegaly. Electrocardiography demonstrated precordial T-wave inversion; cardiac enzymes were normal. While hospitalized, the patient had increasing shortness of breath, and a lung scan showed bilateral ventilation-perfusion mismatches highly suggestive of pulmonary embolism. While receiving anticoagulant therapy, she experienced more dyspnea. Repeated echocardiography disclosed mild pulmonic valvular stenosis. Findings on a Doppler ultrasound examination of the lower extremities and pelvis were normal. Intravenous administration of contrast medium to enhance a computed tomographic (CT) scan revealed a filling defect in the main pulmonary artery and its branches, which was interpreted as thrombosis. Gated spin-echo T1-weighted axial magnetic resonance imaging (MRI) (Technicare; 0.6 T) obtained both before and after administration of gadolinium-diethylenetriamine pentaacetic acid (Magnevist, Berlex Laboratories, Wayne, New Jersey) demonstrated an inhomogeneously enhancing filling defect of the pulmonary artery and its branches (Fig. 1), consistent with a pulmonary artery tumor. Subsequently, the patient underwent thoracotomy and pulmonary thromboembolectomy, and a pulmonary artery mass was removed. (The mass was thought to have been completely excised.) Histologic examination, including immunocytochemical studies, revealed a Fibromyxoid subtype of leiomyosarcoma. A follow-up MRI scan (Signa, General Electric; 1.5 T) obtained with and without administration of gadolinium-diethylenetriamine pentaacetic acid disclosed the presence of substantial residual tumor (Fig. 2). Later, during cardiopulmonary bypass, the pulmonary artery was replaced with a graft. Subsequent MRI (1.5 T) (Fig. 3) showed no evidence of residual tumor.Fig. 2Gadolinium-enhanced magnetic resonance study after initial operation. Coronal (A) and axial (B) views (repetition time, 1,000 ms; echo time, 20 ms) show that intraluminal-enhancing tumor (arrows), although decreased, is still present.View Large Image Figure ViewerDownload (PPT)Fig. 3Magnetic resonance images after second operation. A, Axial T2-weighted (repetition time, 1,714 ms; echo time, 70 ms) image. B, Coronal contrast-enhanced (repetition time, 1,000 ms; echo rime, 20 ms) image. No residual mass or abnormal enhancement is evident in main pulmonary artery (arrows).View Large Image Figure ViewerDownload (PPT) Pulmonary artery sarcomas are rare; only a few more than 100 cases have been reported.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 7Sato H Matsuda H Hatakeyama T Okada M Nakamura K Primary pulmonary artery sarcoma: modern diagnostic imaging methods and successful surgical treatment.J Cardiovasc Surg. 1992; 33: 202-203PubMed Google Scholar Most studies have shown at least a slight female preponderance.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar The mean age at manifestation is 52 years.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar A high index of suspicion is necessary in order to make the diagnosis before an operation or autopsy because symptoms arc insidious and nonspecific.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 4Weinreb JC Davis SD Berkmen YM Isom W Naidich DP Pulmonary artery sarcoma: evaluation using Gd-DTPA.J Comput Assist Tomogr. 1990; 14: 647-649Crossref PubMed Scopus (53) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar Hence, diagnosis is difficult and often delayed. Frequent symptoms are dyspnea, chest pain, cough, and syncope. 1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar, 8Iversen S Hake U Schmiedt W Jakob H Ramp U Gabbert H et al.Resection of central primary pulmonary artery sarcoma.Eur J Cardiothorac Surg. 1991; 5: 603-607Crossref PubMed Scopus (14) Google Scholar Common entities such as pulmonary thromboembolism, right-sided heart failure, and lung cancer may manifest in a clinically similar manner and are often incorrectly suspected.3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar Normal findings on a venogram or Doppler ultrasound examination of the venae cavae and deep pelvic veins can help exclude venous thrombosis as an underlying cause. In addition, a chronic course, without episodes of acute shortness of breath, is highly suggestive of a neoplasm,5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar as is an increased erythrocyte sedimentation rate.8Iversen S Hake U Schmiedt W Jakob H Ramp U Gabbert H et al.Resection of central primary pulmonary artery sarcoma.Eur J Cardiothorac Surg. 1991; 5: 603-607Crossref PubMed Scopus (14) Google Scholar Early suspicion and detection may improve the uniformly poor prognosis; however, most routine diagnostic studies yield nonspecific findings. Chest roentgenographic findings may be interpreted as normap1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar but more often reveal a pulmonary nodule (or nodules),1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 4Weinreb JC Davis SD Berkmen YM Isom W Naidich DP Pulmonary artery sarcoma: evaluation using Gd-DTPA.J Comput Assist Tomogr. 1990; 14: 647-649Crossref PubMed Scopus (53) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar pulmonary artery enlargernent,1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar decreased pulmonary vascularity,1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 4Weinreb JC Davis SD Berkmen YM Isom W Naidich DP Pulmonary artery sarcoma: evaluation using Gd-DTPA.J Comput Assist Tomogr. 1990; 14: 647-649Crossref PubMed Scopus (53) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar cardiomegaly,1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar and a hilar mass or an infiltrate (or both).2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 4Weinreb JC Davis SD Berkmen YM Isom W Naidich DP Pulmonary artery sarcoma: evaluation using Gd-DTPA.J Comput Assist Tomogr. 1990; 14: 647-649Crossref PubMed Scopus (53) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar The findings, if any, depend on tumor size, growth pattern, and degree of transmural spread into the surrounding lung.5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar CT may reveal the presence of a polypoid intraluminal soft tissue mass extending into one or both of the pulmonary arteries, as well as any associated luminal expansion, hilar mass, or pulmonary nodules (or some combination of these findings).1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar Intravenous administration of contrast medium will outline a filling defect in the affected pulmonary artery. This nonenhancing defect may be indistinguishable from pulmonary artery occlusion due to thrombus unless other signs of malignant involvement (for example, pulmonary nodules or lymphadenopathy) are present.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar Echocardiography rarely shows an echogenic tumor in the pulmonary artery (or arteries).1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 7Sato H Matsuda H Hatakeyama T Okada M Nakamura K Primary pulmonary artery sarcoma: modern diagnostic imaging methods and successful surgical treatment.J Cardiovasc Surg. 1992; 33: 202-203PubMed Google Scholar The usefulness of transesophageal echocardiography in such cases has not yet been described in the literature. Bronchoscopy demonstrates only the late findings that occur after the neoplasm has invaded the wall of the bronchus.3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar Lung scans usually show multiple perfusion defects indistinguishable from thromboembolic disease;1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar absent perfusion of the affected lung is less common.2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar Unlike the changes noted with most pulmonary emboli, nuclear scan abnormalities of pulmonary artery sarcomas tend to remain unchanged on serial examinations.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar Occasionally, angiography can distinguish a filling defect due to a sarcoma from that caused by thrombus. The tumor tends to be lobulated and polypoid and may show a characteristic to-and-fro motion during the cardiac cycle.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 4Weinreb JC Davis SD Berkmen YM Isom W Naidich DP Pulmonary artery sarcoma: evaluation using Gd-DTPA.J Comput Assist Tomogr. 1990; 14: 647-649Crossref PubMed Scopus (53) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar Although the use of MRI in the preoperative diagnosis of a pulmonary artery sarcoma has been described,3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar, 4Weinreb JC Davis SD Berkmen YM Isom W Naidich DP Pulmonary artery sarcoma: evaluation using Gd-DTPA.J Comput Assist Tomogr. 1990; 14: 647-649Crossref PubMed Scopus (53) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar, 7Sato H Matsuda H Hatakeyama T Okada M Nakamura K Primary pulmonary artery sarcoma: modern diagnostic imaging methods and successful surgical treatment.J Cardiovasc Surg. 1992; 33: 202-203PubMed Google Scholar the current case report is the first to demonstrate the potential benefits of MRI in assessing the results of surgical treatment. On T1-weighted images, the appearance of a pulmonary artery sarcoma is that of an intraluminal mass of intermediate signal intensity; T2 weighting may cause a mild increase in the signal of the mass.3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar, 4Weinreb JC Davis SD Berkmen YM Isom W Naidich DP Pulmonary artery sarcoma: evaluation using Gd-DTPA.J Comput Assist Tomogr. 1990; 14: 647-649Crossref PubMed Scopus (53) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar Associated findings such as luminal expansion, extraluminal extension, or metastatic disease can also be detected on MRI.3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar Administration of gadolinium enhances a lesion that is indicative of tumor.4Weinreb JC Davis SD Berkmen YM Isom W Naidich DP Pulmonary artery sarcoma: evaluation using Gd-DTPA.J Comput Assist Tomogr. 1990; 14: 647-649Crossref PubMed Scopus (53) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar, 7Sato H Matsuda H Hatakeyama T Okada M Nakamura K Primary pulmonary artery sarcoma: modern diagnostic imaging methods and successful surgical treatment.J Cardiovasc Surg. 1992; 33: 202-203PubMed Google Scholar This enhancement gives MRI an advantage in the diagnosis and follow-up because a nonvascularized intraluminal thrombus, often confused clinically with a pulmonary artery sarcoma, should not enhance after injection of gadolinium.4Weinreb JC Davis SD Berkmen YM Isom W Naidich DP Pulmonary artery sarcoma: evaluation using Gd-DTPA.J Comput Assist Tomogr. 1990; 14: 647-649Crossref PubMed Scopus (53) Google Scholar In our patient, gadolinium enhancement was also useful for detecting unsuspected residual tumor postoperatively. The prognosis associated with pulmonary artery sarcomas has been dismal.7Sato H Matsuda H Hatakeyama T Okada M Nakamura K Primary pulmonary artery sarcoma: modern diagnostic imaging methods and successful surgical treatment.J Cardiovasc Surg. 1992; 33: 202-203PubMed Google Scholar Because of the insidious nature of associated symptoms, these neoplasms are often well advanced before they manifest clinically.2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 8Iversen S Hake U Schmiedt W Jakob H Ramp U Gabbert H et al.Resection of central primary pulmonary artery sarcoma.Eur J Cardiothorac Surg. 1991; 5: 603-607Crossref PubMed Scopus (14) Google Scholar On average, the survival time after the onset of symptoms is only 12 months.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar Therapeutic success depends on early diagnosis and management,7Sato H Matsuda H Hatakeyama T Okada M Nakamura K Primary pulmonary artery sarcoma: modern diagnostic imaging methods and successful surgical treatment.J Cardiovasc Surg. 1992; 33: 202-203PubMed Google Scholar but only a few patients have experienced long-term survival.2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar Local surgical resection,1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar homograft insertion,2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar irradiation,1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar and chemotherapy1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 2Head HD Flam MS John MJ Lipnik SS Slater DL Stewart RD Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy.Ann Thorac Surg. 1992; 53: 332-334Abstract Full Text PDF PubMed Scopus (56) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar have all been used; long-term success has been minimal. Two new therapeutic techniques have been attempted for pulmonary artery sarcomas. Heart-lung transplantation is potentially curative in patients with disease confined to the pulmonary arteries.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar Similarly, radiotherapy used in conjunction with a radiosensitizer showed a slight decrease in the size of an advanced tumor in one reported case.3Smith WS Lesar MS Travis WD Lubbers P Sen RP Ginsberg AM et al.MR and CT findings in pulmonary artery sarcoma.J Comput Assist Tomogr. 1989; 13: 906-909Crossref PubMed Scopus (38) Google Scholar Long-term therapeutic success, however, necessitates early diagnosis, which has been elusive. We believe that, if awareness of this tumor is increased, contrast-enhanced MRI will have an important role in identifying candidates in whom treatment would provide a cure or long-term palliation and in monitoring the results of such treatment. MRI seems especially helpful in detecting recurrent (or residual) disease, which can be a problem after therapy.1Britlon PD Primary pulmonary artery sarcoma—a report of two cases, with special emphasis on the diagnostic problems.Clin Radiol. 1990; 41: 92-94Abstract Full Text PDF PubMed Scopus (31) Google Scholar, 5Bressler EL Nelson JM Primary pulmonary artery sarcoma: diagnosis with CT, MR imaging, and transthoracic needle biopsy.AJR Am J Roentgenol. 1992; 159: 702-704Crossref PubMed Scopus (47) Google Scholar, 6Baker PB Goodwin RA Pulmonary artery sarcomas: a review and report of a case.Arch Pathol Lab Med. 1985; 109: 35-39PubMed Google Scholar Although pulmonary artery sarcomas are rare, they should be considered in the previously noted nonspecific settings. Clinical findings that favor the diagnosis are a lengthy clinical course, symptoms unresponsive to anticoagulant therapy, and an increased erythrocyte sedimentation rate. Imaging findings suggestive of a diagnosis include an unchanging lesion on serial radionuclide lung scans, negative results on a Doppler ultrasound study of the legs and deep pelvic veins, and an enhancing mass on MRI. Increased clinical awareness of this entity and use of contrast-enhanced MRI can lead to early diagnosis. Contrast-enhanced MRI is a useful and relatively noninvasive method for preoperative diagnosis and postsurgical follow-up of these rare tumors.
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