Primary central nervous system lymphoma epidemiology

Primary central nervous system lymphoma (PCNSL) is a relatively common central nervous system tumor.

It is rare in children with immunocompromise as an important risk factor.

The majority of Primary central nervous system lymphomas (PCNSL) (95%) are considered diffuse large B cell lymphomas (DLBCLs).

Because of the low incidence of primary central nervous system lymphoma (PCNSL) in non-HIV individuals and because of the lack of specific clinical manifestations and auxiliary examinations, the disease is easily missed or misdiagnosed.

Primary CNS lymphomas are estimated to account for up to 1% of all lymphomas, 4–6% of all extranodal lymphomas, and about 3% of all CNS tumors ¹⁾ ²⁾ ³⁾ ⁴⁾ ⁵⁾. ⁶⁾

The incidence of primary central nervous system lymphoma (PCNSL) has increased in the last two decades and the clinical research regarding the treatment for PCNSL patients has also increased. After a continuous rise in the incidence of primary CNS lymphoma during the 1980s and 1990s, epidemiological data in high-income countries show a decrease in incidence, particularly among young patients with AIDS ⁷⁾.

By contrast, the incidence of primary CNS lymphoma continues to rise in elderly patients, who represent most patients in immunocompetent populations ⁸⁾ ⁹⁾ ¹⁰⁾.

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Kluin PM, Deckert M, Ferry JA. Primary diffuse large B-cell lymphoma of the Central Nervous System. In: Swerdlow SH, Campo E, Harris NL, eds. World Health Organization classification of tumours pathology and genetics of tumours of the haematopoietic and lymphoid tissues. Lyon: IARC Press, 2008: 240–41.

²⁾

Abbi KK, Muzaffar M, Gaudin D, Booth RL Jr, Feldmeier JJ, Skeel RT. Primary CNS lymphoplasmacytic lymphoma: a case report and review of literature. Hematol Oncol Stem Cell Ther. 2013;6(2):76–78. doi: 10.1016/j.hemonc.2013.06.002.

³⁾

Ueba T, Okawa M, Abe H, Inoue T, Takano K, Hayashi H, Nabeshima K. et al. Central nervous system marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type involving the brain and spinal cord parenchyma. Neuropathology. 2013;33(3):306–311. doi: 10.1111/j.1440-1789.2012.01350.x.

⁴⁾

Ney DE, Deangelis LM. Non- Hodgkin Lymphomas. Philadelphia: Lippincott Willilams & Wilkins; 2010. Management of central nervous system lymphoma; pp. 527–539.

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Report of Brain Tumor Registry of Japan (1984-2000) Neurol Med Chir (Tokyo) 2009;49(Suppl):PS1–96.

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Gestner ER, Batchelor TT. Primary Central Nervous System Lymphoma. Arch Neurol 2010;67:291–7.

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Kadan-Lottick NS, Skluzacek MC, Gurney JG. Decreasing incidence rates of primary central nervous system lymphoma. Cancer 2002; 95: 193–202.

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Bessell EM, Dickinson P, Dickinson S, et al. Increasing age at diagnosis and worsening renal function in patients with primary central nervous system lymphoma. J Neurooncol 2011; 104: 191–93.

⁹⁾

Villano JL, Koshy M, Shaikh H, et al. Age, gender, and racial differences in incidence and survival in primary CNS lymphoma. Br J Cancer 2011; 105: 1414–18.

¹⁰⁾

O’Neill BP, Decker PA, Tieu C, et al. The changing incidence of primary central nervous system lymphoma is driven primarily by the changing incidence in young and middle-aged men and differs from time trends in systemic diffuse large B-cell non-Hodgkin’s lymphoma. Am J Hematol 2013; 88: 997–1000.