9678/3 Primary effusion lymphoma


Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C83.8

/span>rimary effusion lymphoma (PEL) is a large B-cell neoplasm usually presenting as serous effusions without detectable tumour masses. It is universally associated with human herpesvirus 8 (HHV8), also named Kaposi sarcoma herpesvirus (KSHV). It most often occurs in the setting of immunodeficiency. Some patients with PEL secondarily develop solid tumours in adjacent structures such as the pleura. Rare HHV8-positive lymphomas indistinguishable from PEL present as solid tumour masses, and have been termed extracavitary PEL.