Person:

Shaheen, Shagufta

Primary effusion lymphoma (PEL) is a subset of large B cell lymphomas and has been mostly associated with human immunodeficiency virus infection. Rare cases have been reported in organ transplant recipients and chronic hepatitis C patients. It typically presents as an effusion in the pleural and pericardial spaces but rarely disseminates. However, involvement of the gastrointestinal tract, lymph nodes and bone marrow has been reported. Diagnosis is based on characteristic clinical, histopathological and immunohistochemical features. We present a case with a right atrial mass which tested positive for human herpes virus 8 (HHV-8), CD20, CD30 and lambda light chains and negative for CD138, kappa light chain, PAX5, Epstein-Barr virus, latent membrane protein 1, CD2, CD3, CD8 and CD56. Bilateral pleural effusions and pericardial effusions were noted which tested positive for HHV-8, CD30 and CD45. The patient responded well to the R-EPOCH regimen with complete resolution of the effusions and a significant decrease in the size of the right atrial mass. This case report illustrates the atypical manifestation of PEL as a right atrial mass.

Uterine leiomyosarcoma is a rare malignancy and carries a poorer prognosis when compared to endometrial carcinoma. It has been observed to metastasize to all the major organs. It presents with symptoms of abdominal distension, vaginal bleeding and may pass unnoticed until an advanced stage in patients with leiomyomas. Surgery is a viable option in patients with disease limited to the uterus, but metastasis to the heart may require surgery to prevent acute and catastrophic complications. The case described here involves metastasis to the tricuspid valve, which caused severe tricuspid regurgitation in the setting of acute pulmonary embolism. Surgical resection restored cardiac function and stabilized the patient. This case illustrates a rare site of metastasis of leiomyosarcoma which required immediate intervention and resulted in a favorable outcome.