Visual lung damage CT score at hospital admission of COVID-19 patients and 30-day mortality.

OBJECTIVES: Chest CT has been widely used to screen and to evaluate the severity of COVID-19 disease in the early stages of infection without severe acute respiratory syndrome, but no prospective data are available to study the relationship between extent of lung damage and short-term mortality. The objective was to evaluate association between standardized simple visual lung damage CT score (vldCTs) at admission, which does not require any software, and 30-day mortality. METHODS: In a single-center prospective cohort of COVID-19 patients included during 4 weeks, the presence and extent of ground glass opacities(GGO), consolidation opacities, or both of them were visually assessed in each of the 5 lung lobes (score from 0 to 4 per lobe depending on the percentage and out of 20 per patient = vldCTs) after the first chest CT performed to detect COVID-19 pneumonia. RESULTS: Among 210 confirmed COVID-19 patients, the number of survivors and non-survivors was 162 (77%) and 48 (23%), respectively at 30 days. vldCTs was significantly higher in non-survivors, and the AUC of vldCTs to distinguish survivors and non-survivors was 0.72 (95%CI 0.628-0.807, p < 0.001); the best cut-off vldCTs value was 7. During follow-up, significant differences in discharges and 30-day mortality were observed between patients with vldCTs ≥ 7 versus vldCTs < 7: (98 [85.2%] vs 49 [51.6%]; p < 0.001 and 36 [37.9%] vs 12 [12.4%]; p < 0.001, respectively. The 30-day mortality increased if vldCTs ≥ 7 (HR, 3.16 (1.50-6.43); p = 0.001), independent of age, respiratory rate and oxygen saturation levels, and comorbidities at admission. CONCLUSIONS: By using chest CT in COVID-19 patients, extensive lung damage can be visually assessed with a score related to 30-day mortality independent of conventional risk factors of the disease. KEY POINTS: • In non-selected COVID-19 patients included prospectively during 4 weeks, the extent of ground glass opacities(GGO) and consolidation opacities evaluated by a simple visual score was related to 30-day mortality independent of age, respiratory rate, oxygen saturation levels, comorbidities, and hs-troponin I level at admission. • This severity score should be incorporated into risk stratification algorithms and in structured chest CT reports requiring a standardized reading by radiologists in case of COVID-19.

Exceptional thyrolipoma and thymolipoma association: is there a syndrome?

Thyrolipoma and thymolipoma are rare neoplasms of the thyroid gland and thymus, respectively. Their simultaneous occurrence is exceptional. Up to now, only 2 cases have been reported in literature in 1966 and in 1997. For the first time, we report the occurrence of both neoplasms associated with myasthenia gravis in a 64-year-old woman. The value of this case report lies not only in the fact that it allows us to speculate on the presence of a syndrome but also because a complete radiological work-up (computed tomography scanner, magnetic resonance imaging, Positron emission tomography (PET) with fluorodeoxyglucose (FDG)) is reported.

Dynamic magnetic resonance imaging in unilateral diaphragm eventration: knowledge improvement before and after plication.

BACKGROUND: The assessment before surgical plication for unilateral hemidiaphragm (HD) eventration is not clearly defined and no precise criteria exist to really understand which patient is operated with which results depending on the technique used. The goal of this study was to evaluate the place of dynamic magnetic resonance imaging (dMRI) before and after plication by developing measurement criteria. METHODS: Between 2006 and 2017, 18 patients (group1: Gp1) were operated for eventrations, 15 left-sided (Gp1L) and 3 right-sided (Gp1R). All had preoperative and postoperative evaluations including dMRI and pulmonary function tests. Five healthy volunteer subjects (group2: Gp2) had the same imaging protocol. For each HD, we measured the respiratory excursion at three fixed points (S1, S2, S3) and the height of curvature on sagittal plane. We also searched for upward paradoxical diaphragm movements. RESULTS: Before surgery, no excursion (n=13) or extremely reduced excursion (n=5) was detected on the injured HD (IHD) in Gp1. Upward paradoxical movements were identified only in Gp1L (n=6). Compared with Gp2 subjects, the healthy HD for Gp1L patients had significantly reduced excursion values at three sites S1 (P=0.038), S2 (P=0.006), and S3 (P=0.004). After plication, the decreasing height of curvature confirmed a tightening of the IHD in all patients (median value from 100 to 39.5 mm in Gp1L and 92 to 74 mm in Gp1R, P=0.0001). All upward paradoxical movements disappeared. Healthy HD excursions in Gp1L normalised their values. All those imaging improvements were correlated with postoperative improvements of dyspnoea score (P<0.0001) and vital capacity (P=0.002). CONCLUSIONS: dMRI and the standardised grid we developed not only improve the knowledge of unilateral diaphragm eventration but also permit to evaluate the quality of its surgical repair. It also demonstrates that a dysfunction of the healthy HD contralateral to eventration is possible and reversible after plication of the IHD.

SFE/SFHTA/AFCE primary aldosteronism consensus: Introduction and handbook.

The French Endocrinology Society (SFE) French Hypertension Society (SFHTA) and Francophone Endocrine Surgery Association (AFCE) have drawn up recommendations for the management of primary aldosteronism (PA), based on an analysis of the literature by 27 experts in 7 work-groups. PA is suspected in case of hypertension associated with one of the following characteristics: severity, resistance, associated hypokalemia, disproportionate target organ lesions, or adrenal incidentaloma with hypertension or hypokalemia. Diagnosis is founded on aldosterone/renin ratio (ARR) measured under standardized conditions. Diagnostic thresholds are expressed according to the measurement units employed. Diagnosis is established for suprathreshold ARR associated with aldosterone concentrations >550pmol/L (200pg/mL) on 2 measurements, and rejected for aldosterone concentration<240pmol/L (90pg/mL) and/or subthreshold ARR. The diagnostic threshold applied is different if certain medication cannot be interrupted. In intermediate situations, dynamic testing is performed. Genetic forms of PA are screened for in young subjects and/or in case of familial history. The patient should be informed of the results expected from medical and surgical treatment of PA before exploration for lateralization is proposed. Lateralization is explored by adrenal vein sampling (AVS), except in patients under 35 years of age with unilateral adenoma on imaging. If PA proves to be lateralized, unilateral adrenalectomy may be performed, with adaptation of medical treatment pre- and postoperatively. If PA is non-lateralized or the patient refuses surgery, spironolactone is administered as first-line treatment, replaced by amiloride, eplerenone or calcium-channel blockers if insufficiently effective or poorly tolerated.

SFE/SFHTA/AFCE consensus on primary aldosteronism, part 4: Subtype diagnosis.

To establish the cause of primary aldosteronism (PA), it is essential to distinguish unilateral from bilateral adrenal aldosterone secretion, as adrenalectomy improves aldosterone secretion and controls hypertension and hypokalemia only in the former. Except in the rare cases of type 1 or 3 familial hyperaldosteronism, which can be diagnosed genetically and are not candidates for surgery, lateralized aldosterone secretion is diagnosed on adrenal CT or MRI and adrenal venous sampling. Postural stimulation tests and (131)I-norcholesterol scintigraphy have poor diagnostic value and (11)C-metomidate PET is not yet available. We recommend that adrenal CT or MRI be performed in all cases of PA. Imaging may exceptionally identify adrenocortical carcinoma, for which the surgical objectives are carcinologic, and otherwise shows either normal or hyperplastic adrenals or unilateral adenoma. Imaging alone carries a risk of false positives in patients over 35 years of age (non-aldosterone-secreting adenoma) and false negatives in all patients (unilateral hyperplasia). We suggest that all candidates for surgery over 35 years of age undergo adrenal venous sampling, simultaneously in both adrenal veins, without ACTH stimulation, to confirm the unilateral form of the hypersecretion. Sampling results should be confirmed on adrenal vein cortisol assay showing a concentration at least double that found in peripheral veins. Aldosterone secretion should be considered lateralized when aldosterone/cortisol ratio on the dominant side is at least 4-fold higher than contralaterally.

The value of a rapid contrast-enhanced angio-MRI protocol in the detection of head and neck paragangliomas in SDHx mutations carriers: a retrospective study on behalf of the PGL.EVA investigators.

OBJECTIVES: To assess the performance of a simplified MRI protocol consisting of a contrast-enhanced three-dimensional MR angiography (CE-MRA) in association with a post-contrast T1-weighted sequence (T1WIV) for the detection of HNPGLs in SDHx mutation carriers. METHODS: This retrospective sub-study is based on the multicenter PGL.EVA cohort, which prospectively enrolled SDHx mutation carriers from 2005 to 2009; 157 index cases or relatives were included. CE-MRA and the T1WIV images were read solely with knowledge of the clinical data but blind to the diagnosis. Sensitivity, specificity and likelihood ratios for the simplified MRI protocol were compared to the full MRI protocol reading results and to the gold standard status obtained through the consensus of an expert committee. RESULTS: The sensitivity and specificity of the readings of the simplified MRI protocol were, respectively, 88.7 % (95 % CI = 78.1-95.3) and 93.7 % (95 % CI = 86.8-97.7) versus 80.7 % (95 % CI = 68.6-89.6) and 94.7 % (95 % CI = 88.1-98.3) for the readings of the full MRI protocol. CONCLUSIONS: The simplified post-contrast MRI with shorter duration (5 to 10 minutes) showed no performance difference compared to the lengthy standard full MRI and can be proposed for the detection of head and neck paragangliomas (HNPGLs) in SDHx mutation carriers. KEY POINTS: • Rapid angio-MRI protocol and the usual lengthy protocol show equal diagnostic performance. • The CE-MRA is the key sequence for the detection of HNPGLs. • The T1WIV sequence assists in localizing HNPGLs.

Peritoneal implantation of pheochromocytoma following tumor capsule rupture during surgery.

CONTEXT: Patients with pheochromocytoma (PH) or paraganglioma (PGL) may suffer from tumor persistence or recurrence after resection of the primary tumor. Malignancy and genetic determinants account for the vast majority of these cases, but tumor recurrence or persistence may also arise from tumor spillage during primary resection, followed by peritoneal implantation. We report here five such cases. MATERIALS AND METHODS: Thirty-nine of the 181 patients referred to our unit for a PH experienced tumor persistence or recurrence as a result of malignant disease (n = 12), new PGL in a context of genetic determinants (n = 18) or incomplete primary surgical resection (n = 4). Another five patients presenting with adrenal PH could not be categorized into these three groups. RESULTS AND DISCUSSION: All five patients (age range 45-63 years) presented evidence of tumor capsule rupture documented upon macroscopic examination or in the surgical report. Initial diagnostic examinations provided no evidence of malignancy. All had a period of apparent remission, lasting from 24 to 106 months. The principal site of recurrence was invariably the peritoneum or the retroperitoneum. Two patients suffered solid organ metastasis, involving the liver (n = 2), bones (n = 1), and lung (n = 1). Therapeutic management involved a combination of (131)I-metaiodobenzylguanidine therapy and surgery. Two patients died due to tumor progression. One patient experienced tumor progression despite surgery. Two patients are currently in a satisfactory condition. CONCLUSION: Tumor rupture during surgical resection, with subsequent peritoneal and retroperitoneal dissemination, is a potentially lethal complication of primary pheochromocytoma resection. Even in cases of apparently benign disease, it may lead to peritoneal carcinomatosis and metastatic disease. Complete primary surgery is, therefore, crucial for a good prognosis in PH patients. Furthermore, in cases of tumor rupture, careful follow-up is mandatory, because recurrences may occur after long periods of apparent remission.

Sarcoidosis presenting as severe renin-dependent hypertension due to kidney vascular injury.

Renal sarcoidosis embraces a wide variety of clinical patterns. Renal vascular involvement has seldom been reported and usually in the setting of systemic vasculitis. We report the case of a 22-year-old patient in whom inaugural manifestation of renal sarcoidosis consisted of severe hypertension associated with bilateral perfusion defects and tumour-like nodules. In the setting of renal sarcoidosis, our case suggests that renin-dependant hypertension may arise from renal ischaemia as a result of extrinsic compression of kidney blood vessels due to severe granulomatous inflammation.

Diagnostic accuracy of unenhanced, contrast-enhanced perfusion and angiographic MRI sequences for pulmonary embolism diagnosis: results of independent sequence readings.

OBJECTIVES: To independently evaluate unenhanced, contrast-enhanced perfusion and angiographic MR sequences for pulmonary embolism (PE) diagnosis. METHODS: Prospective investigation, including 274 patients who underwent perfusion, unenhanced 2D steady-state-free-precession (SSFP) and contrast-enhanced 3D angiographic MR sequences on a 1.5-T unit, in addition to CTA (CT angiography). Two independent readers evaluated each sequence independently in random order. Sensitivity, specificity, predictive values and inter-reader agreement were calculated for each sequence, excluding sequences judged inconclusive. Sensitivity was also calculated according to PE location. RESULTS: Contrast-enhanced angiographic sequences showed the highest sensitivity (82.9 and 89.7 %, reader 1 and reader 2, respectively), specificity (98.5 and 100 %) and agreement (kappa value 0.77). Unenhanced angiographic sequences, although less sensitive overall (68.7 and 76.4 %), were sensitive for the detection of proximal PE (92.7 and 100 %) and showed high specificity (96.1 and 99.1 %) and good agreement (kappa value 0.62). Perfusion sequences showed lower sensitivity (75.0 and 79.3 %), specificity (84.8 and 89.7 %) and agreement (kappa value 0.51), and a negative predictive value of 84.8 % at best. CONCLUSIONS: Compared with contrast-enhanced angiographic sequences, unenhanced sequences demonstrate lower sensitivity, except for proximal PE, but high specificity and agreement. The negative predictive value of perfusion sequences was insufficient to safely rule out PE. KEY POINTS: • Unenhanced angiographic MR sequences are very specific and can identify proximal PE. • Contrast-enhanced MR angiographic sequences show high sensitivity for PE diagnosis. • A normal MR perfusion result does not exclude PE. • Inter-reader agreement is better for angiographic than perfusion MR sequences.

Imaging work-up for screening of paraganglioma and pheochromocytoma in SDHx mutation carriers: a multicenter prospective study from the PGL.EVA Investigators.

CONTEXT: Recommendations have not been established concerning imaging to screen SDHx mutation carriers for paraganglioma and pheochromocytoma. OBJECTIVE: Our objective was to compare the performance of gadolinium-enhanced magnetic resonance angiography, contrast-enhanced computed tomography, and [(123)I]metaiodo-benzylguanidine and somatostatin receptor scintigraphies for detecting head and neck and thoracic-abdominal-pelvic paragangliomas in SDHx mutation carriers. DESIGN AND SETTING: We conducted a prospective, multicenter study from June 2005 to December 2009 at 23 French medical centers. PATIENTS: A total of 238 index cases or relatives carrying mutations in SDHD, SDHB, or SDHC genes were included. INTERVENTION: Images obtained by each technique were analyzed blind, without knowledge of results from other tests, first in each local center and then centrally. MAIN OUTCOME MEASURES: We evaluated sensitivity, specificity, and likelihood ratios for individual and combinations of tests, the gold standard being the consensus of an expert committee. RESULTS: Two hundred two tumors were diagnosed in 96 subjects. At local assessment, the sensitivity of anatomical imaging for detecting all tumors was higher (85.7%) than that of both scintigraphic techniques (42.7% for [(123)I]metaiodo-benzylguanidine and 69.5% for somatostatin receptor scintigraphy), except for thoracic localizations where somatostatin receptor scintigraphy was more sensitive (61.5 vs. 46.2% for anatomical imaging and 30.8% for [(123)I]metaiodo-benzylguanidine scintigraphy). The best diagnostic performance during local assessment was obtained by combining anatomical imaging tests and somatostatin receptor scintigraphy (sensitivity 91.7%). Central assessment significantly increased the sensitivity (98.6%) of tests in combination. CONCLUSIONS: In routine practice, the imaging work-up for screening SDHx mutation carriers should include thoraco-abdomino-pelvic computed tomography, head and neck magnetic angiography, and somatostatin receptor scintigraphy. Expert centralized image assessment is recommended.

A novel TMEM127 mutation in a patient with familial bilateral pheochromocytoma.

OBJECTIVE: In this report, we describe a new patient with unexplained familial bilateral pheochromocytoma. Following the recent description of TMEM127 as a new pheochromocytoma susceptibility gene, the aim of this study was to test the hypothesis of a causative TMEM127 gene mutation in this patient. DESIGN: Pheochromocytoma susceptibility genes were analyzed in germline DNA and losses of heterozygosity (LOH) assessed by BAC array comparative genomic hybridization in tumor DNA. SDHB expression and S6 kinase (S6K) phosphorylation were analyzed by immunohistochemistry. Genome-wide expression microarray studies were performed, and vascular density was quantified after CD34 immunohistochemistry. RESULTS: A first germline variant was identified in the SDHB gene (c.158G>A; p.Gly53Glu). However, a positive SDHB immunostaining in the tumor indicated that this SDHB variant was a non-functional polymorphism. A novel TMEM127 germline mutation (c.140C>A, p.Ala47Asp) associated with a 2q11 LOH was found. Transcriptome and immunohistochemical analyses showed that TMEM127-related pheochromocytoma clusterized with NF1-related and RET-related tumors in a large series of pheochromocytomas and paragangliomas, exhibited a reduced TMEM127 mRNA expression and displayed a low vascularization. The phosphorylation of S6K observed in this tumor was suggestive of an activation of the MTOR pathway. CONCLUSIONS: Pathological and genomic data demonstrated that a TMEM127 gene mutation not previously described was causative of a new case of familial bilateral pheochromocytoma. This report highlights the importance of supplementary analyses on tumor tissue to provide an accurate pheochromocytoma/paraganglioma genetic testing result to affected patients.

Unusual cardiovascular events in Behçet's disease.

OBJECTIVES: To report on cardiovascular involvement in Behçet's disease (BD). METHODS: A retrospective analysis of clinical, EKG, echodoppler, CT-scan, MRI, conventional angiography, treatment and follow-up data was undertaken in 4 patients suffering BD. RESULTS: Cardiac specific complications included coronary artery involvement (n=3), endomyocardial fibrosis (n=1), left ventricle spontaneous rupture with giant wall pseudo-aneurysm (n=1), and massive left ventricle thrombosis (n=1). Follow-up ranged from 1 month to 17 years. Surgery was complicated with vascular patch leakage, recurrent pseudo-aneurysm or upper-limb venous thrombosis in 2 patients who did not receive pre-operative specific treatment because of delayed BD diagnosis. High-dose steroids (n=4), colchicine (n=4), immunosuppressants (n=3) and anticoagulants (n=4) were eventually prescribed and stabilised cardiac disease in all cases. CONCLUSIONS: At time of life-threatening cardiac complications, BD was often overlooked. Prompt initiation of steroids and immunosuppressive treatment may prevent post-operative complications, recurrences and death.

Single-lung transplantation in patients with previous contralateral pneumonectomy: technical aspects and results.

OBJECTIVE: Single-lung transplantation (SLTX) in patients with previous contralateral pneumonectomy (PN) is a rarely observed situation. Intrathoracic anatomical changes caused by mediastinal shift may complicate the surgical procedure. We collected observations from different transplantation centres to analyse the technical aspects and results. PATIENTS AND METHODS: Between July 1990 and September 2008, 14 patients (seven women and seven men) with previous PN underwent SLTX for end-stage pulmonary failure. Patients were categorised in three groups according to lung disease: cystic fibrosis bronchiectasis (group 1; n=4), non-cystic fibrosis bronchiectasis (group 2; n=6) and bronchioloalveolar carcinoma (group 3; n=4). We reviewed patients' characteristics according to mediastinal shift, thoracic approach, bypass cannula procedure, perioperative difficulties and immediate and long-term results. RESULTS: Median age was 19.5, 33.5 and 52.5 years in groups 1, 2 and 3, respectively; there were nine left and five right cases of SLTX. Surgery was performed by sternotomy (n=4), anterolateral thoracotomy (n=4), clamshell (n=4) or posterolateral thoracotomy (n=2). Cannulas for bypass were inserted into femoral (n=7) or central vessels (n=5) or both (n=2). Mediastinal shift did not complicate surgical procedure but rendered cannulation more difficult with ensuing cardiopulmonary bypass dysfunction (n=3) and early bronchial complications (n=2). In-hospital mortality rate was 29% (4 out of 14 patients), including two deaths due to perioperative difficulties linked to mediastinal shift. Median global survival was 108 months. Median survival was higher in group 2 (108 months vs 1 month in the other groups) and in case of PN during childhood (n=6, median survival 108 months corresponding to one death). CONCLUSIONS: SLTX after PN is associated with high perioperative morbidity and mortality due to mediastinal shift. Best results are observed in patients undergoing PN for non-cystic fibrosis bronchiectasis and during childhood. Anatomical changes induced by PN must be anticipated to adapt the thoracic approach and cardiopulmonary bypass access.

Management of lobar torsion following lung transplantation.

We report 4 cases of acute lobar torsion in patients who had undergone bilateral lung transplantation. Bronchoscopy showed complete obstruction in only 2 of them. Torsion was confirmed by computed tomography in coronal minimal and maximal intensity projections with reconstruction. At operation, 1 detorsion and 3 lobectomies were carried out. Early diagnosis and rapid surgical intervention can save the affected lobe.

Is It possible to recognize pulmonary infarction on multisection CT images?

PURPOSE: To retrospectively determine sensitivity and specificity of four findings for distinguishing pulmonary infarction from other causes of peripheral pulmonary consolidations on multidetector computed tomographic (CT) images, with other CT and clinical findings as reference. MATERIALS AND METHODS: Institutional review board approved the study and waived informed consent. Three independent radiologists blindly analyzed selected multisection CT images of 50 pulmonary infarctions-not showing direct arterial signs of pulmonary embolism-and 100 peripheral consolidations of other origins. Readers analyzed four findings: triangular shape, vessel sign (defined as presence of an enlarged vessel at the apex of consolidation), central lucencies, and air bronchograms. Interobserver agreement; frequency on CT images with and without infarct; and sensitivity, specificity, and positive likelihood ratio (LR) for diagnosis of pulmonary infarction were assessed for each finding. RESULTS: One hundred fifty peripheral consolidations were analyzed in 134 (75 men, 59 women) patients (mean age, 55.9 years+/-17.4 [standard deviation] vs 54.7+/-19.9; P=.71). Interobserver agreement was good for central lucencies and air bronchograms and poor to moderate for the other two findings (kappa<0.61). Compared with CT images without infarct, CT images with infarct had a higher frequency of vessel sign (32% [16 of 50] vs 11% [11 of 100], P=.029) and central lucencies (46% [23 of 50] vs 2% [two of 100], P<.001) and a lower frequency of air bronchograms (8% [four of 50] vs 40% [40 of 100], P=.003). Frequency of triangular shape was similar in both groups (52% [26 of 50] vs 40% [40 of 100], P=.17). Positive LR was 23.0 for central lucencies, 2.9 for vessel sign, 1.3 for triangular shape, and 0.2 for air bronchograms. Presence of central lucencies had 98% specificity and 46% sensitivity for pulmonary infarction. When the vessel sign and negative air bronchogram were combined with central lucencies, specificity increased to 99% but sensitivity decreased to 14%. CONCLUSION: Central lucencies in peripheral consolidations are highly suggestive of pulmonary infarction.

Ventricular volumes in Ebstein's anomaly: x-ray multislice computed tomography before and after repair.

BACKGROUND: This study was performed to measure right and left ventricular volumes in Ebstein's anomaly before and after operation. METHODS: Twenty-six consecutive patients were operated on with tricuspid valve repair and plication of the atrialized right ventricle, associated with a bidirectional cavopulmonary shunt in 15 patients. Right ventricular (RV) and left ventricular end-diastolic and end-systolic volume indexes were measured by x-ray computerized tomography before and after surgical repair. Left ventricular stroke index and ejection fraction were calculated. RESULTS: Before surgery, the mean stroke index of the atrialized RV was 36 +/- 33 mL/m2, with severe reduction in 9 patients and aneurysmal aspect in 2 patients. After surgery, the atrialized RV was no longer identifiable. Both RV end-diastolic volume index and stroke index of the remaining effective RV were reduced. Bidirectional cavopulmonary shunt was a determinant factor of decrease in the effective RV end-diastolic volume index after repair. Conversely, left ventricular ejection fraction and stroke index increased significantly after surgery. CONCLUSIONS: The atrialized RV with dyskinesia seems a good indication for a plication. RV end-diastolic volume index of the effective RV decreased after surgery. In severe cases, bidirectional cavopulmonary shunt was useful by decreasing RV end-diastolic volume, thus preventing further RV dilation. In all cases left ventricular ejection fraction and stroke volume index increased after surgery.

A rare inferior middle mediastinal tumor resection under extra-corporeal circulation.

An inferior middle mediastinal mass was observed in a 51-year-old woman initially presenting as pericardial effusion and mimicking lymph node disease. Thoracotomy demonstrated a tumor invading the posterior atrial wall. Complete resection was possible under the extracorporeal circulation. The tumor proved to be a soft tissue myoepithelioma in an exceptional location. Despite the difficult surgical resection due to location, prognosis of this absolutely rare tumor without cytologic features of malignancy should be good.