SEER is an authoritative source of information on cancer incidence and survival in the United States. SEER currently collects and publishes cancer incidence and survival data from population-based cancer registries covering approximately 28 percent of the U.S. population.
Aggressive B-cell lymphoma usually confined to pleural, pericardial, peritoneal body cavities, presenting as serous effusions without detectable tumor masses, occurring primarily but not exclusively in HIV-infected patients, caused by gamma 2 herpes virus (Kaposi sarcoma-associated herpes virus KSHV, aka human herpes virus type 8, HHV-8)
PEL is universally associated with human herpes virus 8 (HH8).
PEL is unusual in that the majority of cases arise in body cavities, such as the pleural, pericardial, and peritoneal cavities. Because there are no ICD-O-3 codes for the pleural space, pericardium, or peritoneal cavity, code the primary site to pleura C384 when the neoplasm arises in the pleural cavity, to pericardium C380 when it occurs in the pericardium, and to peritoneal cavity C482 when it occurs in the peritoneum. Typically only one body cavity is involved. Extracavitary tumors with morphologic and phenotypic characteristics similar to PEL occur in extranodal sites including the GI tract, skin, lung, and CNS, or may involve lymph nodes.
PEL is generally resistant to cancer chemotherapy drugs that are active against other lymphomas, and carries a poor prognosis.
Definitive Diagnostic Methods
Immunoglobulin genes clonally rearranged and hyperstimulated