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Aggressive angiomyxoma (AAM) is a rare benign tumor that arises from connective tissue, prominently located in the vulva, vagina, perineum, and pelvis and is mainly found in women aged about 20-40 years old. Giant intraabdominal tumors have rarely been described. These tumors develop slowly over time and are often difficult to diagnose due to various clinical findings, especially in the early stages. Even though surgery is the primary treatment method, the possibility of complete resection is sometimes limited because the tumor tends to infiltrate nearby structures, leading to local recurrence. Only about 10% of AAM cases can be accurately diagnosed before treatment, which causes ineffective outcomes. This article demonstrates a case of giant intra-abdominal AAM precisely diagnosed by suspicious signs on CT and MRI scans before starting treatment.

Background: The incidence of atypical ductal hyperplasia (ADH) is increasing due to mass screening. Because of the underestimation risk of malignancy, the management remains controversial. Our goal was to analyze clinicopathologic features of patients with ADH diagnosed on directional vacuum-assisted biopsy (DVAB). The objectives of this continuous retrospective study were to evaluate the underestimation rate of malignancy and to identify predictors of upgrade to carcinoma. Methods: Between 2003 and 2010, 3159 patients underwent stereotactic DVAB in our institute. We retrospectively evaluate clinical, mammographic and pathological features of 298 cases of ADH who underwent surgical excision in our center (93.1%). Patients with concurrent history of breast cancer, intraductal carcinoma (DCIS) associated, or with no follow-up or surgical excision on place were excluded. Histological scar of macrobiopsy was systematically searched in surgical specimens. A pathologic upgrade was defined by presence of invasive cancer or DCIS on surgical specimen. Statistical tests used were the chi-square or Fisher's exact test. Results: Among the 298 studied DVAB, 224 ADH were isolated (75.2%), 46 associated to flat epithelial atypia (15.4%) and 28 to lobular neoplasia (9.4%). 98.4% patients presented microcalcifications at diagnosis. In 52 cases, lesions were upgraded to DCIS (n = 38) or invasive cancer (n = 14). The underestimated rate was 17.5%. In 67.3% cases, upgrade lesions were low or intermediate grade DCIS. Only the history of contralateral breast cancer was significantly correlated with the underestimated rate (p = 0.04). There was no statistical difference between these 52 cases and the 246 others for: family history, size of calcification, sampling number, histological lesion, and quality of calcifications removal. Conclusions: In this study, DVAB may not be considered a therapeutic procedure in case of ADH, even in the case of complete removal of microcalcifications. It is still challenging to identify a subgroup of ADH cases with a low upgrade rate. [ABSTRACT FROM AUTHOR]

Purpose: While 18 F-FDG PET/CT (FDG-PET/CT) is consensual for clinical stage ≥ IIB breast cancers (BC), its benefit for stage I or IIA HER2+ or triple-negative breast cancer (TNBC) patients lacks sufficient evidence. We reported a single-institution, retrospective study evaluating FDG-PET/CT impact on patient management and staging for stage I or IIA HER2+ or Triple-Negative BC., Methods: Patients who underwent FDG-PET/CT staging before any treatment between January 2015 and December 2020 at Oscar Lambret Center were included., Exclusions: patients with symptoms or conventional imaging suggestive of metastatic dissemination, or with prior malignancies. Initial stage was determined from mammography, breast ultrasound, breast MRI, and clinical examination. Staging and therapeutic impact based on FDG-PET/CT findings collected, including intra- (modification of dose/site/strategy in a type of management previously indicated) and inter-modality (modification of planned treatment strategy) changes., Results: The cohort included 287 female patients with clinical stage I or IIA, HER2+ , or TNBC. Therapeutic impact observed for 18% of patients (n = 52), with 2% (n = 7) undergoing inter-modality change with omission of planned surgery. The impact on patient management was higher for stage IIA patients (20%, 47/237) than for stage I patients (10%, 5/50). Among stage IIA disease, changes in management were more important for T2N0 patients (22%, 44/205) than for T1N1 patients (9%, 3/32). While not statistically significant, trends suggest usefulness of FDG-PET/CT for T2N0 patients., Conclusion: Considering substantial therapeutic implications, our study suggests the usefulness of FDG-PET/CT for patients with stage IIA, HER2-positive, or Triple-Negative BC with tumor size > 2 cm (T2N0)., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Synovial sarcoma (SS) is an uncommon malignant tumor, ranking third in prevalence within the soft tissue sarcomas group. The vast majority of synovial sarcomas are present in the extremities, with only 15% developing in the retroperitoneal space. Retroperitoneal synovial sarcoma (RSS) is an infrequent case of SS, with only about 20 cases reported in the literature. Diagnosing RSS before treatment remains challenging because of its nonspecific clinical symptoms. The disease is often detected at a later stage, leading to additional damage to other organs as well as complicated and ineffective treatment. Consequently, the 5-year survival rate is only 20%-29%. This report introduces a case of RSS in a 19-year-old male patient with imaging characteristics on computed tomography (CT) and magnetic resonance (MR)., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Background: The choice between different diffusion-weighted imaging (DWI) techniques is difficult as each comes with tradeoffs for efficient clinical routine imaging and apparent diffusion coefficient (ADC) accuracy., Purpose: To quantify signal-to-noise-ratio (SNR) efficiency, ADC accuracy, artifacts, and distortions for different DWI acquisition techniques, coils, and scanners., Study Type: Phantom, in vivo intraindividual biomarker accuracy between DWI techniques and independent ratings., Population/phantoms: NIST diffusion phantom. 51 Patients: 40 with prostate cancer and 11 with head-and-neck cancer at 1.5 T FIELD STRENGTH/SEQUENCE: Echo planar imaging (EPI): 1.5 T and 3 T Siemens; 3 T Philips. Distortion-reducing: RESOLVE (1.5 and 3 T Siemens); Turbo Spin Echo (TSE)-SPLICE (3 T Philips). Small field-of-view (FOV): ZoomitPro (1.5 T Siemens); IRIS (3 T Philips). Head-and-neck and flexible coils., Assessment: SNR Efficiency, geometrical distortions, and susceptibility artifacts were quantified for different b-values in a phantom. ADC accuracy/agreement was quantified in phantom and for 51 patients. In vivo image quality was independently rated by four experts., Statistical Tests: QIBA methodology for accuracy: trueness, repeatability, reproducibility, Bland-Altman 95% Limits-of-Agreement (LOA) for ADC. Wilcoxon Signed-Rank and student tests on P < 0.05 level., Results: The ZoomitPro small FOV sequence improved b-image efficiency by 8%-14%, reduced artifacts and observer scoring for most raters at the cost of smaller FOV compared to EPI. The TSE-SPLICE technique reduced artifacts almost completely at a 24% efficiency cost compared to EPI for b-values ≤500 sec/mm 2 . Phantom ADC 95% LOA trueness were within ±0.03 × 10 -3  mm 2 /sec except for small FOV IRIS. The in vivo ADC agreement between techniques, however, resulted in 95% LOAs in the order of ±0.3 × 10 -3  mm 2 /sec with up to 0.2 × 10 -3  mm 2 /sec of bias., Data Conclusion: ZoomitPro for Siemens and TSE SPLICE for Philips resulted in a trade-off between efficiency and artifacts. Phantom ADC quality control largely underestimated in vivo accuracy: significant ADC bias and variability was found between techniques in vivo., Level of Evidence: 3 TECHNICAL EFFICACY STAGE: 2., (© 2023 The Authors. Journal of Magnetic Resonance Imaging published by Wiley Periodicals LLC on behalf of International Society for Magnetic Resonance in Medicine.)

After chemotherapy, patients with non-seminomatous germ cell tumors (NSGCTs) with residual masses >1 cm on computed tomography (CT) undergo surgery. However, in approximately 50% of cases, these masses only consist of necrosis/fibrosis. We aimed to develop a radiomics score to predict the malignant character of residual masses to avoid surgical overtreatment. Patients with NSGCTs who underwent surgery for residual masses between September 2007 and July 2020 were retrospectively identified from a unicenter database. Residual masses were delineated on post-chemotherapy contrast-enhanced CT scans. Tumor textures were obtained using the free software LifeX. We constructed a radiomics score using a penalized logistic regression model in a training dataset, and evaluated its performance on a test dataset. We included 76 patients, with 149 residual masses; 97 masses were malignant (65%). In the training dataset ( n = 99 residual masses), the best model (ELASTIC-NET) led to a radiomics score based on eight texture features. In the test dataset, the area under the curve (AUC), sensibility, and specificity of this model were respectively estimated at 0.82 (95%CI, 0.69-0.95), 90.6% (75.0-98.0), and 61.1% (35.7-82.7). Our radiomics score may help in the prediction of the malignant nature of residual post-chemotherapy masses in NSGCTs before surgery, and thus limit overtreatment. However, these results are insufficient to simply select patients for surgery.

Introduction: Autologous fat grafting (AFG) is a promising breast reconstruction technique, following surgery. However, fat necrosis after AFG can cause palpable nodules, inducing anxiety and prompting additional investigations. We aimed to determine the occurrence rate and to identify the risk factors of palpable nodules in breast cancer patients prompting complementary explorations., Materials and Methods: We retrospectively reviewed the computerized files of consecutive breast cancer patients who underwent AFG after mastectomy or breast conservative treatment (BCT) at our center from January 2013 to December 2016. We collected data regarding palpable nodule incidences and suspicious breast imaging findings that led to additional explorations., Results: Of the 252 patients reviewed, 222 (88%) underwent AFG for breast reconstruction, while 30 (12%) underwent AFG for correction of defects after BCT. The follow-up period ranged from 0 to 74 months (median: 27 months), and 201 patients had follow-ups beyond 1 year. Of the 252 patients, 66 (26.2%) underwent at least one control imaging and 24 (10%) underwent a biopsy, including 6 (2.4%) locoregional recurrence. Sixty patients (24%) were diagnosed with nodules during follow-ups. Among them, 52 (87%) patients had additional imaging, and 17 patients (28%) had a biopsy, including 82% benign histology. Patients with BCT had significantly more palpable nodules at 1 year than patients who underwent total mastectomy (odds ratio = 2.50, 95% confidence interval: 1.04. 6.03, p = 0.04)., Conclusion: This study underlines the importance of preoperative patient information. This intervention and its follow-up should be reserved for experienced teams to limit unnecessary additional explorations., Level of Evidence Iv: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 ., (© 2021. Springer Science+Business Media, LLC, part of Springer Nature and International Society of Aesthetic Plastic Surgery.)

Purpose: The purpose of this study was to create a deep learning algorithm to infer the benign or malignant nature of breast nodules using two-dimensional B-mode ultrasound data initially marked as BI-RADS 3 and 4., Materials and Methods: An ensemble of mask region-based convolutional neural networks (Mask-RCNN) combining nodule segmentation and classification were trained to explicitly localize the nodule and generate a probability of the nodule to be malignant on two-dimensional B-mode ultrasound. These probabilities were aggregated at test time to produce final results. Resulting inferences were assessed using area under the curve (AUC)., Results: A total of 460 ultrasound images of breast nodules classified as BI-RADS 3 or 4 were included. There were 295 benign and 165 malignant breast nodules used for training and validation, and another 137 breast nodules images used for testing. As a part of the challenge, the distribution of benign and malignant breast nodules in the test database remained unknown. The obtained AUC was 0.69 (95% CI: 0.57-0.82) on the training set and 0.67 on the test set., Conclusion: The proposed deep learning solution helps classify benign and malignant breast nodules based solely on two-dimensional ultrasound images initially marked as BIRADS 3 and 4., (Copyright © 2021 Société française de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

Purpose: The 2020 edition of these Data Challenges was organized by the French Society of Radiology (SFR), from September 28 to September 30, 2020. The goals were to propose innovative artificial intelligence solutions for the current relevant problems in radiology and to build a large database of multimodal medical images of ultrasound and computed tomography (CT) on these subjects from several French radiology centers., Materials and Methods: This year the attempt was to create data challenge objectives in line with the clinical routine of radiologists, with less preprocessing of data and annotation, leaving a large part of the preprocessing task to the participating teams. The objectives were proposed by the different organizations depending on their core areas of expertise. A dedicated platform was used to upload the medical image data, to automatically anonymize the uploaded data., Results: Three challenges were proposed including classification of benign or malignant breast nodules on ultrasound examinations, detection and contouring of pathological neck lymph nodes from cervical CT examinations and classification of calcium score on coronary calcifications from thoracic CT examinations. A total of 2076 medical examinations were included in the database for the three challenges, in three months, by 18 different centers, of which 12% were excluded. The 39 participants were divided into six multidisciplinary teams among which the coronary calcification score challenge was solved with a concordance index > 95%, and the other two with scores of 67% (breast nodule classification) and 63% (neck lymph node calcifications)., (Published by Elsevier Masson SAS.)

Purpose: MRI for radiotherapy planning requires spatial referencing using immobilization devices and markers. Clinical images of a difficult-to-interpret artifact are presented, resembling a metastasis, which occurs when combining CAIPIRINHA k-space-based parallel imaging (PI), 3D distortion correction, and external markers., Methods: A 3D variable flip angle Turbo Spin Echo sequence was used on a 1.5 T and 3 T MRI using flexible and head and neck coils. Two types of markers were tested: Liquimark LM1 and Spee-D-Mark. A silicone oil phantom was used that represents low signal intensity, such as gray matter. 3D Fourier transforms were also used to show the issue's origin., Results: The markers can appear in an unexpected region of a patient, not in the same original or reconstructed slice nor in a rectilinear direction in a slice, especially when using CAIPIRINHA acceleration with 3D distortion correction. The probability of occurrence was respectively 13% and 80% for distances of <=2 mm and >2 mm between marker and patient, for example when using thermoplastic masks. Clinical cases are shown where this semi-randomly occurring artifact appears post contrast only, and thus can be interpreted as metastases. The artifact did not appear when using compressed sensing acceleration., Conclusion: Markers used for radiotherapy MRI application can introduce additional artifacts that can be interpreted as metastases. However, other high signal intensity structures on the surface of a patient, such as the ear, can lead to an equivalent error., (Copyright © 2021 Associazione Italiana di Fisica Medica. Published by Elsevier Ltd. All rights reserved.)

Purpose: We evaluated the addition of breast magnetic resonance imaging (MRI) to standard radiologic evaluation on the re-intervention rate in women with ductal carcinoma in situ (DCIS) undergoing breast-conserving surgery., Patients and Methods: Women with biopsy-proven DCIS corresponding to a unifocal microcalcification cluster or a mass less than 30 mm were randomly assigned to undergo MRI or standard evaluation. The primary end point was the re-intervention rate for positive or close margins (< 2 mm) in the 6 months after randomization ( ClinicalTrials.gov identifier: NCT01112254)., Results: A total of 360 patients from 10 hospitals in France were included in the study. Of the 352 analyzable patients, 178 were randomly assigned to the MRI arm, and 174 were assigned to the control arm. In the intent-to-treat analysis, 82 of 345 patients with the assessable end point were reoperated for positive or close margins within 6 months, resulting in a re-intervention rate of 20% (35 of 173) in the MRI arm and 27% (47 of 172) in the control arm. The absolute difference of 7% (95% CI, -2% to 16%) corresponded to a relative reduction of 26% (stratified odds ratio, 0.68; 95% CI, 0.41 to 1.1; P = .13). When considering only the per-protocol population with an assessable end point, the difference was 9% (stratified odds ratio, 0.59; 95% CI, 0.35 to 1.0; P = .05). Total mastectomy rates were 18% (31 of 176) in the MRI arm and 17% (30 of 173) in the control arm (stratified P = .93). For 100 lesions seen on MRI, nonmass-like enhancement was more predominant (82%) than mass enhancement (20%). Nevertheless, no specific morphologic and kinetic parameters for DCIS were identified., Conclusion: The study did not show sufficient surgical improvement with the use of preoperative MRI to be clinically relevant in DCIS staging. However, this could be reconsidered with the improvement of new MRI sequences and new modalities in magnetic resonance techniques.

Objective: To investigate whether there were suspicious criteria on the initial MRI in BIRADS 3 lesions. To analyze the value of "blooming sign" as an additional criterion for malignancy., Materials and Methods: In this retrospective study the lesion morphological and enhancement characteristics were analyzed. The "blooming sign" (BS), defined as the lesion size increase between the early and the late phase after gadolinium was assessed. We determined the optimal cut-off value for the BS to distinguish benign and malignant breast lesions., Results: 100 lesions were classified BIRADS 3 in 75 patients (12%). Four of the five malignant lesions had suspicious BIRADS criteria on the index MRI. 45 lesions were stable and 30 lesions resolved spontaneously during the follow-up MRI. The optimal cut-off value for the BS was 8.54% with 100% sensitivity, 94% specificity, 44% positive and 100% negative predictive values. Using reclassification rule to upgrade benign BIRADS lesions with suspicious BS feature and downgrade suspicious BIRADS lesions with benign BS feature increased MRI specificity (89%), sensitivity (100%) while preserving NPV (100%)., Conclusions: This study showed the suggestive part of classified BIRADS 3 lesions. The blooming sign seems to be a good additional parameter to increase MRI specificity when associated to BIRADS criteria., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objective: The Response Evaluation Criteria in Solid Tumors (RECIST) can have limitations when used to evaluate local treatments for cancer, especially for liver malignancies treated by stereotactic body radiation therapy (SBRT). The aim of this study was to validate the relationship between the occurrence of lobulated enhancement (LE) and local relapse and to evaluate the utility of this relationship for predicting local progression., Patients and Methods: Imaging data of 59 lesions in 46 patients, including 281 computed tomographic (CT) scans, were retrospectively and blindly reviewed by 3 radiologists. One radiologist measured the lesion size, for each CT and overall, to classify responses using RECIST threshold criteria. The second studied LE occurrence. A third radiologist was later included and studied LE occurrence to evaluate the interobserver consistency for LE evaluation., Results: The mean duration of follow-up was 13.6 months. LE was observed in 16 of 18 progressive lesions, occurring before size-based progression in 50% of cases, and the median delay of LE detection was 3.2 months. The sensitivity of LE to predict progression was 89%, and its specificity was 100%. The positive predictive value was 100%, the negative predictive value was 95.3%, and the overall accuracy was 97%. The probability of local progression-free survival at 12 months was significantly higher for lesions without LE compared with all lesions: 0.80 (CI 95%: 0.65-0.89) versus 0.69 (CI 95%: 0.54-0.80), respectively. The overall concordance rate between the 2 readers of LE was 97.9%., Conclusion: Response assessment of liver metastases treated by SBRT can be improved by including LE. This study demonstrates the diagnostic and predictive utility of LE for assessing local progression at a size still eligible for local salvage treatment., (Copyright © 2015 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: To report experience with fiducial marker insertion and describe an advantageous, novel technique for fiducial placement in the liver for stereotactic body radiation therapy with respiratory tracking., Methods and Materials: We implanted 1444 fiducials (single: 834; linked: 610) in 328 patients with 424 hepatic lesions. Two methods of implantation were compared: the standard method (631 single fiducials) performed on 153 patients from May 2007 to May 2010, and the cube method (813 fiducials: 610 linked/203 single) applied to 175 patients from April 2010 to March 2013. The standard method involved implanting a single marker at a time. The novel technique entailed implanting 2 pairs of linked markers when possible in a way to occupy the perpendicular edges of a cube containing the tumor inside., Results: Mean duration of the cube method was shorter than the standard method (46 vs 61 minutes; P<.0001). Median numbers of skin and subcapsular entries were significantly smaller with the cube method (2 vs 4, P<.0001, and 2 vs 4, P<.0001, respectively). The rate of overall complications (total, major, and minor) was significantly lower in the cube method group compared with the standard method group (5.7% vs 13.7%; P=.013). Major complications occurred while using single markers only. The success rate was 98.9% for the cube method and 99.3% for the standard method., Conclusions: We propose a new technique of hepatic fiducial implantation that makes use of linked fiducials and involves fewer skin entries and shorter time of implantation. The technique is less complication-prone and is migration-resistant., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Primary vertebral osteosarcoma is a rare type of osteosarcoma, differing from the appendicular forms by an incidence peak occurring at a higher age and a poorer prognosis, due to the difficulties of the surgical treatment. We present five cases of histologically proven primary vertebral osteosarcomas followed in our institution between 2004 and 2012. They allow to illustrate some essential radiologic features, useful to evoke this rare entity., (Copyright © 2013 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.)

Objective: To describe post-CyberKnife® imaging characteristics of liver metastases as an aid in assessing response to treatment, and a novel set of combined criteria (CC) as an alternative to response according to change in size (RECIST)., Subjects and Methods: Imaging data and medical records of 28 patients with 40 liver metastases treated with stereotactic body radiotherapy (SBRT) were reviewed. Tumor size, CT attenuation coefficient, and contrast enhancement of lesions were evaluated up to 2 years post SBRT. Rates of local control, progression-free survival, time to progression, and overall survival according to RECIST and CC were estimated., Results: Complete response (CR) was 3.6% (95% CI: 0.1-18%) and 18% (95% CI: 6-37%) according to RECIST and combined criteria, respectively. Two progressive diseases and two partial responses according to RECIST were classified as CR by the combined criteria and one stable response according to RECIST was classified as progressive by CC (Stuart-Maxwell test, p = 0.012). The disease control rate was 60.7% (95% CI: 41-78%) by RECIST and 64% (95% CI: 44%-81%) by CC., Conclusion: Use of response criteria based on change in size alone in the interpretation of liver response to SBRT may be inadequate. We propose a simple algorithm with a combination of criteria to better assess tumor response. Further studies are needed to confirm their validity.

Superficial soft-tissue sarcomas are malignant mesenchymal tumors located within the cutaneous and/or subcutaneous layers. Most superficial soft-tissue sarcomas are low-grade tumors; yet, the risk of local recurrence is high, and initial wide surgery is the main prognostic factor. Some of these superficial sarcomas may grow, following an infiltrative pattern, and their real extent may be underestimated clinically. Imaging techniques are useful to determine precisely the real margins of the tumor, especially in cases of clinically doubtful or recurrent or large superficial lesions. Imaging tools enable one to determine the relationship with the superficial fascia separating the subcutaneous layer from the underlying muscle. In our institution ultrasonographic examination is followed by magnetic resonance (MR) imaging when the size of the lesion exceeds 3-5 cm. Imaging assessment is performed prior to biopsy, enabling optimal surgical management. Imaging features of the main superficial sarcomas are detailed in the following article, according to their major locations: those arising in the epidermis and/or dermis, which are most often diagnosed by dermatologists, and the subcutaneous sarcomas.

The aim of this study was to compare the complete pathologic response (CPR) rate in 56 nonmetastatic inflammatory breast cancer patients according to the classification used and to look for a correlation between the CPR and overall survival. Initial biopsies and mastectomy specimens were reviewed by the same pathologist. The clinical response rate was 75%. A CPR was observed in 11 cases according to Sataloff, three according to Chevallier and five according to the NSABP. There was no correlation between the clinical and pathologic responses and none of them was predictive of relapse free survival or overall survival. We propose a standardization of the pathologic process of the mastectomy specimens so that a CPR has a clear definition across the institutions, with a good reproducibility whatever the classification used., (© 2010 Wiley Periodicals, Inc.)

Haematomas are common and sarcomas are rare. However the absence of trauma or a light trauma should alert the clinician to the possibility that the abnormality may represent haemorrhage into a tumor and not just haematoma, even in a haemophilic patient. Clinical findings, sonography with Doppler assessment and magnetic resonance images with contrast administration will help in the differential diagnosis. The diagnosis of a high grade sarcoma must be considered in these patients and any doubt should be resolved with a biopsy to avoid tragic consequences of missed sarcoma.

Background: Case reports of primary pulmonary artery sarcomas are very rare., Methods: We described herein eight new cases diagnosed between December 2000 and December 2004., Results: There were four men and four women, with median age of 52 years. Presenting symptoms mimicked pulmonary emboli in all cases. There were six "intimal sarcomas" and two leiomyosarcomas. In six cases, we observed initial metastasis in lung (six cases), in bone (two cases), and in brain (two cases), and adrenal gland (one case). The palliative treatments included surgical desobstruction (six cases), conformational radiotherapy (four cases), and chemotherapy (seven cases). Doxorubicin-based regimen failed in seven cases. All patients died (median survival: 8 months, extremes 5-20 months)., Conclusion: Those eight cases illustrate the high incidence of initial metastasis and the very poor outcome of primary pulmonary artery sarcomas despite classic doxorubicin-based chemotherapy.