Sinonasal adamantinoma-like Ewing sarcoma: A case report.

We describe the case of a sinonasal adamantinoma-like Ewing sarcoma in a 41-year-old male. Histologically, the tumor exhibited distinctive areas of nested growth pattern with prominent stromal fibrosis and metaplastic bone formation. The tumor cells were small and uniform with minimal amount of pale eosinophilic to clear cytoplasm and round or oval nuclei with finely dispersed chromatin and small nucleoli. Approximately 20% of the tumor parenchyma comprised of small clusters of basaloid cells within an osteofibrous background resembling adamantinoma. The tumor showed strong expression of keratins, p63, CD99 and Fli-1, and EWSR1 rearrangement. The diagnosis of sinonasal Ewing family tumors is particularly problematic owing to the large number of potential mimics. For any poorly differentiated or undifferentiated head and neck tumor, cellular monotony and CD99 immunoreactivity should prompt consideration for molecular studies that include analysis of both EWSR1 and FLI1, even in the presence of strong cytokeratin expression or focal keratinization.

Malignant tenosynovial giant cell tumor with CDKN2A/B genomic alteration: a histological, immunohistochemical, and molecular study.

Diffuse-type tenosynovial giant cell tumor (D-T TSGCT) is regarded as a benign but locally aggressive neoplasm with significant recurrent potential. We report a case of malignant D-T TSGCT with pleural metastases arising in the left knee in a 57-year-old man. The tumor demonstrated atypical features, including a solid infiltrative pattern with spindling of the tumor cells, nuclear pleomorphism with prominent nucleoli, and markedly increased mitotic activity (>20 mitoses/10 high-power fields). The immunoprofile demonstrated clusterin+, D2-40+, CD68+, p63+, MDM2+, and p16+ tumor. The next-generation sequencing-based assay demonstrated loss of the CDKN2A/B gene. Pleural metastases with identical histologic and immunohistochemical features were identified 2 years later after primary tumor resection. To the best of our knowledge, this is the first reported case of D-T TSGCT with CDKN2A/B genomic alteration, MDM2 expression, and p16 loss. Clinicians and pathologists should be aware of the morphologic variability and the metastatic propensity of this entity.

A Papillary Fibroelastoma Involving Aortic and Pulmonary Valves: Findings on Multimodality Imaging.

Cardiac papillary fibroelastoma, a rare entity, is the second most common benign primary cardiac tumor. Commonly involving the cardiac valves, this entity is increasingly diagnosed using different imaging modalities. We present a rare case of simultaneous involvement of both the aortic and pulmonary valves in an asymptomatic patient who underwent different imaging modalities, including transthoracic and transesophageal echocardiography, nongated and gated computed tomography, and magnetic resonance imaging. We will discuss the imaging findings and differential diagnosis.

Endovascular diagnosis and management of chronic cerebrospinal venous insufficiency: retrospective analysis of 30-day morbidity and mortality in 95 consecutive patients.

OBJECTIVE: The purpose of this study is to report the 30-day morbidity and mortality associated with the endovascular diagnosis and management of chronic cerebrospinal venous insufficiency. MATERIALS AND METHODS: The medical records of 95 consecutive patients (60 women and 35 men) with a mean age of 48 years (age range, 25-66 years) who underwent diagnostic endovascular evaluation and intervention for chronic cerebrospinal venous insufficiency between June 2010 and September 2011 were reviewed retrospectively. All patients had a diagnosis of multiple sclerosis by McDonald criteria. Endovascular evaluation of the internal jugular and azygos veins was performed with digital subtraction venography and intravascular ultrasound. Indications for percutaneous transluminal angioplasty (PTA) were venographic findings of a greater than 50% diameter stenosis, the presence of reflux on digital subtraction venography, greater than 50% cross-sectional area stenosis by intravascular ultra-sound, or a finding of abnormal thick valves or webs by either method. The primary endpoint of this study was the 30-day mortality, and the secondary endpoint was the presence of major complications. Results are presented as means and percentages. RESULTS: A total of 107 procedures were performed in 95 patients. Endovascular evaluation showed venous lesions requiring intervention in 90 of 95 patients (94.7%) and was negative in five of 95 patients (5.3%). A total of 193 venous lesions were treated; angioplasty was technically successful in 188 of 193 (97.4%) lesions. Internal jugular vein thrombosis after PTA was identified in three of 95 (3.2%) of the treated patients. Bleeding at the puncture site not requiring transfusion occurred in four of 95 patients (4.2%). There were no reported procedure-related deaths. CONCLUSION: The results of the current study suggest that endovascular evaluation and management of chronic cerebrospinal venous insufficiency is safe, with low morbidity and no procedure-related mortality.

Balloon-assisted over-the-wire technique for placement of the venous outflow component of the Hemodialysis Reliable Outflow (HeRO) device.

A modified technique for placement of the venous outflow component (VOC) of the Hemodialysis Reliable Outflow (HeRO) device (Hemosphere Inc, Minneapolis, Minn) is described. The purpose of the technique is to improve the system's trackability and facilitate device insertion in patients with central venous occlusion. Device preparation requires placement of a 6-mm × 4-cm angioplasty balloon within the leading end of the VOC. The leading 2 cm of the balloon are placed just distal to the radiopaque marker of the VOC. The balloon is inflated to profile and locked in this position within the leading end of the VOC. The VOC and balloon combination is advanced over the wire through the 20F peel-away sheath provided by the manufacturer. The described technique was used to successfully implant the HeRO device in 12 patients with central venous occlusion. This technique is recommended for placement of the VOC of the HeRO device in patients with central venous occlusions.