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.
This is a rare type of extranodal large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of vessels, particularly capillaries, with exception of larger arteries and veins.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see code 9680/3.)
Lymph nodes are usually spared. Two major patterns of clinical presentation have been recognized:
1. Western form characterized by symptoms related to the main organ involved, predominantly neurological or cutaneous
2. Asian variant in which the patients present with multi-organ failure, hepatosplenomegaly, pancytopenia, and hemophagocytic syndrome. B symptoms very common in both types of presentations. This is an aggressive lymphoma which responds poorly to chemotherapy. The poor prognosis reflects in part frequent delays in diagnosis because of a lack of detectable tumor masses.
An isolated cutaneous variant has been identified invariably in Western females. It is characterized by limitation of tumor to the skin and has a better prognosis.