Clinical features of IP-LBCL
| . | PCNSL . | PVRL . | PTL . | IVLBCL∗ . |
|---|---|---|---|---|
| Incidence | ∼4% of malignant brain tumors; ∼1700 cases annually in the United States | A rare subset of PCNSL; ∼50 cases annually in United States | <5% of testicular cancers; commonest testicular malignancy in men aged >60 y | Rare |
| Age | Median age 55 to 60 y; incidence rising in those aged >70 y | Median age 50 to 60 y | Median age 65 to 68 y | Median age 60 to 70 y |
| Presentation | Focal neurologic deficits, cognitive/behavioral changes, or increased intracranial pressure | Blurry vision and floaters; impaired visual acuity; symptoms precede diagnosis for years | Firm, painless testicular mass; 40% associated with hydrocele | Fever of unknown origin common, rapid weight loss; progressive neurologic signs; hemophagocytosis-associated variant |
| Behavior | Aggressive | Indolent | Aggressive | Aggressive |
| CNS involvement | 100% | 100% involve the eye; 60%-80% progress to CNS | ∼25% relapse in CNS (can be late); most CNS progressions involve brain parenchyma | Common |
| Anatomic sites | >90% involve brain parenchyma; 30% to 40% are multifocal; 15%-25% with concurrent brain/CSF; may relapse outside of CNS | Infiltration of the vitreous; may also affect the retina | 5%-10% involve contralateral testis; 20% to 30% have concurrent disease at extranodal sites | Largely restricted to lumina of small blood vessels; the bone marrow, spleen, liver, skin, and CNS |
| . | PCNSL . | PVRL . | PTL . | IVLBCL∗ . |
|---|---|---|---|---|
| Incidence | ∼4% of malignant brain tumors; ∼1700 cases annually in the United States | A rare subset of PCNSL; ∼50 cases annually in United States | <5% of testicular cancers; commonest testicular malignancy in men aged >60 y | Rare |
| Age | Median age 55 to 60 y; incidence rising in those aged >70 y | Median age 50 to 60 y | Median age 65 to 68 y | Median age 60 to 70 y |
| Presentation | Focal neurologic deficits, cognitive/behavioral changes, or increased intracranial pressure | Blurry vision and floaters; impaired visual acuity; symptoms precede diagnosis for years | Firm, painless testicular mass; 40% associated with hydrocele | Fever of unknown origin common, rapid weight loss; progressive neurologic signs; hemophagocytosis-associated variant |
| Behavior | Aggressive | Indolent | Aggressive | Aggressive |
| CNS involvement | 100% | 100% involve the eye; 60%-80% progress to CNS | ∼25% relapse in CNS (can be late); most CNS progressions involve brain parenchyma | Common |
| Anatomic sites | >90% involve brain parenchyma; 30% to 40% are multifocal; 15%-25% with concurrent brain/CSF; may relapse outside of CNS | Infiltration of the vitreous; may also affect the retina | 5%-10% involve contralateral testis; 20% to 30% have concurrent disease at extranodal sites | Largely restricted to lumina of small blood vessels; the bone marrow, spleen, liver, skin, and CNS |
IVLBCL is currently categorized as a discrete biologic entity in both the World Health Organization and International Consensus Classification systems.