Adult-onset primary Ewing’s sarcoma of the right atrium: a case report

Abstract

BackgroundPrimary cardiac tumors, which are only detected in 0.001–0.03% of autopsies, are rare. Only 25% of primary cardiac tumors are malignant, of which 95% are sarcomas. Ewing’s sarcoma, one of the Ewing’s sarcoma of family tumors, is thought to be derived from neural crest cells. While Ewing’s sarcoma usually presents in the bone of children, Ewing’s sarcoma of cardiac origin is rare, with only a few reports described in the literature. The prognosis is unpredictable because of the scarcity and unestablished treatment. We herein report an extremely rare case of primary cardiac Ewing’s sarcoma in the right atrium of a 64-year-old man.Case presentationThe patient is a 64-year-old Japanese male who was referred to our hospital to treat a floating mass of the right atrium (RA). Although the patient was asymptomatic, we performed an operation to urgently resect the floating mass on the next day of admission due to the risk of pulmonary embolism. The operation was performed under cardiopulmonary bypass and cardiac arrest. We resected the tumor with at least 1.5 cm of the RA wall as a margin. The postoperative pathological diagnosis of the mass was compatible with a primitive neuroectodermal tumor (PNET, a form of Ewing’s sarcoma). The cells were positive for CD56, CD99, and Vimentin and negative for S-100 and Desmin. Although no malignant cells were observed in the margin of the resected RA wall and the sarcoma was completely resected, he was transferred to another hospital to receive adjuvant postoperative chemotherapy to improve the prognosis by preventing subclinical micrometastasis.ConclusionsWe experienced an extremely rare case of primary cardiac Ewing’s sarcoma in the right atrium of a 64-year-old man, which was successfully resected under cardiac arrest. Although the sarcoma was completely resected, postoperative chemotherapy and long-term follow-up are recommended for patients with primary cardiac sarcoma because of the high rates of metastasis and recurrence.

Autonomic dysfunction in postoperative primitive neuroectodermal tumor of heart

“…This was a rare case of PNET arising from the right atrium, for which there are few published reports. 2,5,6 This is also the only report mentioning syncope after surgery and its management by visual biofeedback. ECG showed normal rate and rhythm with normal morphology before surgery.…”

“…The standard regimen of chemotherapy was used, for which there are no reports on the occurrence of arrhythmias or autonomic dysfunction in the literature. 2,7,8 Among the sympathetic reactivity tests, dysfunction was noted in the HUT test whereas the CPT findings were normal. Orthostatic hypotension and loss of baroreflex-dependent sympathetic reactivity were noted on all three recordings.…”

Challenges of Diagnosing Primary Ewing’s Sarcoma in the Small Intestine of the Elderly: A Case Report

“…The most common primary sites of the disease are in the lower extremities (41%), pelvis (26%), chest wall (16%), upper extremities (9%), spine (6%), hands and feet (3%) and skull (2%) (12). ESS present in rare sites has been reported to occur in the atrium, esophagus, maxillary sinus, and iris (13)(14)(15)(16)(17)(18). In this case, the patient is a 69-year-old male presenting with an abdominal mass, making this case double rare (19,20).…”

Establishment and Characterisation of Metastatic Extraskeletal Ewing Sarcoma Mouse Models

“…Compared to skeletal Ewing sarcomas, which metastasise preferentially to the lung, bone and bone marrow, extraskeletal Ewing sarcomas feature more extensive metastatic tropism (12,13). It was therefore surprising that previous reports of mouse models of this form of the disease, which typically involved introducing unlabelled A673 cells into the tail vein and/or gastrocnemius muscle, only mentioned lung metastases (33-35, 39, 48).…”

“…Metastatic spread of skeletal Ewing sarcoma occurs most commonly to the lungs, other bones, and bone marrow (11). Extraskeletal Ewing sarcoma spreads most frequently to the bones or lungs (12) but approximately 14% are metastases within other organs, such as abdominal organs, the peritoneum, brain, or heart (12,13). Current treatments for Ewing sarcoma involve neoadjuvant chemotherapy, typically with vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide, followed by surgery and/or radiotherapy (5).…”