title: Primary Pericardial Sarcoma with Right Atrial Invasion and Multiple Bilateral Pulmonary Metastases in a Patient with Hereditary Nonpolyposis Colorectal Cancer.
abstract: Primary tumours originating from the pericardium are extremely rare. Previous studies have reported that these tumours account for only 6.7-12.8% of all mediastinal tumours with an overall prevalence of 0.001% to 0.007%. The majority of these tumours are benign lipomas or pericardial cysts. The most common pericardial malignancy is mesothelioma. Sarcomas are soft-tissue mesenchymal malignancies originating from various parts of the body but are extremely rare in this area. We report a case of a 52-year-old female who was diagnosed with a primary sarcoma with rhabdoid differentiation originating from the pericardium. The patient presented to her GP with a four-week history of progressive dyspnea and chest pain on exertion. Chest X-Ray demonstrated a prominent pericardial effusion and suspicious chest and pericardial lesions. Biopsies of the effusion and primary tumour identified on FDG/PET scans revealed the diagnosis of primary undifferentiated sarcoma. On thoracotomy, it was noted that the tumour had invaded the right atrium; therefore, pericardial window was aborted and a drain inserted instead. The patient was then started on chemotherapy; however, progression soon occurred and the patient died within 4 months, suggesting there is urgent need for efficacious treatments for sarcomatous lesions.
authors.affiliation: Royal North Shore Hospital, Reserve Road, St Leonards, NSW 2065, Australia.
authors.forename: Eugene
authors.identifier: 0000-0002-5799-5083
authors.initials: E
authors.lastname: Wong
authors.affiliation: Royal North Shore Hospital, Reserve Road, St Leonards, NSW 2065, Australia.
authors.forename: Lawrence J
authors.identifier: 0000-0002-3548-2148
authors.initials: LJ
authors.lastname: Oh
authors.affiliation: Royal North Shore Hospital, Reserve Road, St Leonards, NSW 2065, Australia.
authors.forename: Kazi
authors.initials: K
authors.lastname: Nahar
authors.affiliation: Royal North Shore Hospital, Reserve Road, St Leonards, NSW 2065, Australia.
authors.forename: Adrian
authors.initials: A
authors.lastname: Lee
authors.affiliation: Royal North Shore Hospital, Reserve Road, St Leonards, NSW 2065, Australia.
authors.forename: Stephen
authors.initials: S
authors.lastname: Clarke