Background Ewing’s sarcoma is a malignant neoplasm that mainly occurs in skeletal tissue but can rarely arise in soft tissues. Recently, small round cell tumors (including Ewing’s sarcoma) caused by chromosomal translocations have been collectively termed Ewing’s sarcoma family of tumors. We report a rare case of primary adrenal Ewing’s sarcoma family of tumors with tumor thrombus. Case presentation A 22-year-old Asian woman was referred to our hospital with a left retroperitoneal tumor 19 cm in diameter. Tumor thrombus was identified from the left adrenal vein to the inferior vena cava, infiltrating the right atrium. Total tumor excision with left adrenalectomy, nephrectomy, and thrombectomy was performed under hypothermic circulatory arrest, followed by seven courses of adjuvant chemotherapy. The patient has shown no signs of recurrence as of 26 months postoperatively. Conclusion Radical surgery combined with systemic chemotherapy may contribute to good prognosis in patients with primary adrenal Ewing’s sarcoma family of tumors.

Background The elephant trunk (ET) technique is a very useful surgical procedure; however, haemolysis in the aorta associated with ET has been previously reported. It normally occurs within several years after the surgery, and it is a rare case of rapidly progressing haemolysis 10 or more years after aortic arch replacement with ET. Case presentation A 53-year-old man with a history of Stanford type A aortic dissection (DeBakey type Ⅰ), who was treated with total arch aortic replacement and aorto-femoral bypass using a prosthetic graft 17 years ago, developed severe progressive haemolytic anaemia. The ET used for the initial surgery was narrowed, and mechanical haemolysis was suspected. We assumed that progressive mechanical haemolysis occurred because of degeneration of the prosthetic graft. Thoracic endovascular aortic repair was performed, and haemolysis and anaemia were mitigated postoperatively. Conclusions Haemolysis occurred 17 years after the initial surgery with ET. When haemolysis is suspected in a patient with ET, it must be identified as a cause of haemolysis even if 10 years or more have passed since the ET was inserted. To prevent this complication, attention should be paid to an appropriate ET length and diameter to avoid folding of the ET, particularly when the true cavity diameter is small.

Background: It is often difficult to distinguish a noninfectiousinflammatory response from infection in peri-graft fluid collection. The diagnosis of graft infection is usually based on clinical findings supported by the clinical course and radiological and microbiological investigations. Case presentation: We describe a 68-year-old man who underwent ascending aortic replacement and thoracic endovascular aortic repair. Four years later, the patient noticed right neck pain, and CT showed that the fluid in the mediastinum had expanded and extended to the neck. Echocardiography revealed progressive severity of aortic regurgitation and decreased ejection fraction. Given the progression of aortic regurgitation, decreased cardiac function, and rapidly expanding fluid collection causing neck pain, surgery was indicated. All microbiological tests, including polymerase chain reaction, were negative. The patient was being followed without antibiotics, and CT did not show peri-graft fluid 2 years postoperatively. Conclusions: We cannot exclude the infection completely, but we report the importance of medium selection, additional medium orders, extension of culture, genetic testing, and communication with microbiology laboratories when normal culture tests for general bacteria and fungi were all negative, which can successfully avoid increased drug-resistant bacteria count, elevated medical costs, and drug side effects due to the improper use of antibiotics through proper diagnosis.

Background: Cold agglutinin disease can lead to significant complications, specifically for patients undergoing arch repair requiring hypothermic circulatory arrest. Rituximab and plasmapheresis are treatments for cold agglutinin disease; therefore, we used them under consultation of hematologists before mild hypothermic aortic arch surgery maintaining the body temperature above thermal altitude. Case presentation: We describe an 86-year-old male with acute Type A aortic dissection who had been treated for rheumatoid arthritis and a 55-mm thoracic aortic aneurysm as an outpatient. The patient was scheduled to undergo urgent surgery for a Type A intramural hematoma and progressive aortic aneurysm; however, blood clotting was observed on a laboratory test and cold agglutinin was detected. Consequently, we rescheduled the urgent surgery. Under consultation of hematologists, rituximab was initiated 3 months before surgery, and plasmapheresis was performed 2 days before surgery for cold agglutinin disease. Then, total arch replacement under mild hypothermia with the frozen elephant trunk technique was performed by maintaining cerebral and lower body perfusion. The postoperative course was uneventful. On the 42nd postoperative day, the patient was discharged without any neurological deficits. Conclusions: We report a case of total arch replacement with mild hypothermia for aortic arch aneurysm with cold agglutinin disease after rituximab treatment and plasmapheresis.

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