Primary central nervous system lymphoma (PCNSL) is a rare aggressive lymphoma1 belonging to the group of extranodal non-Hodgkin lymphomas2 that is confined to the central nervous system at the time of initial diagnosis. If it is merely a lymphoma diagnosed in the central nervous system, where a manifestation is already present at another site in the body, this is referred to as secondary CNS lymphoma. Neurosurgical treatment is generally limited to performing a biopsy to establish the diagnosis3, as, unlike other brain tumors, lymphomas respond well to radiotherapy and chemotherapy4. Compared with peripheral lymphomas, survival in primary CNS lymphoma is generally shorter5.
Epidemiology
Primary CNS lymphomas can develop in both immunosuppressed and immunocompetent patients. In immunocompetent patients, primary CNS lymphomas account for approximately 4 % of all intracranial space-occupying lesions and 4 % - 6 %
of all extranodal lymphomas6. In patients over 60 years of age, the incidence is 0.5 per 100,000 per year7. Approximately 1500 new diagnoses are recorded in the United States each year8.
Clinical Presentation
Patients with primary CNS lymphoma usually become symptomatic due to neurological deficits that progress over days or weeks9.
| Symptom complex | Frequency10 |
|---|---|
| Focal neurological deficit | 56 % - 70 % |
| Personality changes | 32 % - 43 % |
| Symptoms of increased intracranial pressure (headache, nausea, vomiting, papilledema) | 32 % - 33 % |
| Seizures | 11 % - 14 % |
In 66 % of cases, primary CNS lymphomas are solitary lesions, which are located supratentorially in 87 % of cases and frontoparietally in 39 % of cases11.
Imaging
The gold standard for imaging primary CNS lymphomas is MRI. A definitive diagnosis can only be made histopathologically, as imaging cannot reliably distinguish them from other brain tumors, such as glioblastoma.
Surgical Treatment
As mentioned above, the role of neurosurgery is mainly limited to establishing the diagnosis with a biopsy because of the highly diffuse and infiltrative tumor growth and the good treatment options available with chemotherapy and radiotherapy.
Retrospective studies have shown no survival benefit from subtotal or gross total resection12 13 14.
Prognosis
The outcome for patients with primary CNS lymphoma is unsatisfactory. The 5-year survival rate is 20-30 %, with a median survival time of 10 to 20 months15 16.
References
Korfel, Agnieszka, and Uwe Schlegel. "Diagnosis and treatment of primary CNS lymphoma." Nature Reviews Neurology 9.6 (2013): 317-327. ↩ ↩ ↩ ↩
Grommes, Christian, and Lisa M. DeAngelis. "Primary CNS lymphoma." Journal of Clinical Oncology 35.21 (2017): 2410. ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩
Villano JL, Koshy M, Shaikh H, et al. : Age, gender, and racial differences in incidence and survival in primary CNS lymphoma. Br J Cancer 105:1414-1418, 2011 ↩ ↩
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-Hodgkins lymphoma.
ONeill BP, Decker PA, Tieu C, Cerhan JR. Am J Hematol. 2013 Dec; 88(12):997-1000. ↩ ↩ ↩ ↩Report of an international workshop to standardize baseline evaluation and response criteria for primary CNS lymphoma.
Abrey LE, Batchelor TT, Ferreri AJ, Gospodarowicz M, Pulczynski EJ, Zucca E, Smith JR, Korfel A, Soussain C, DeAngelis LM, Neuwelt EA, ONeill BP, Thiel E, Shenkier T, Graus F, van den Bent M, Seymour JF, Poortmans P, Armitage JO, Cavalli F, International Primary CNS Lymphoma Collaborative Group. Clin Oncol. 2005 Aug 1; 23(22):5034-43. ↩ ↩Primary intracerebral malignant lymphoma: report of 248 cases. Bataille B, Delwail V, Menet E, Vandermarcq P, Ingrand P, Wager M, Guy G, Lapierre F. J Neurosurg. 2000 Feb; 92(2):261-6. ↩ ↩
Therapeutic management of primary central nervous system lymphoma in immunocompetent patients: results of a critical review of the literature. Reni M, Ferreri AJ, Garancini MP, Villa E. Ann Oncol. 1997 Mar; 8(3):227-34. ↩ ↩
Surgical removal of primary central nervous system lymphomas (PCNSL) presenting as space occupying lesions: a series of 33 cases. Bellinzona M, Roser F, Ostertag H, Gaab RM, Saini M Eur J Surg Oncol. 2005 Feb; 31(1):100-5. ↩ ↩
Rubenstein, James, Andrés JM Ferreri, and Stefania Pittaluga. "Primary lymphoma of the central nervous system: epidemiology, pathology and current approaches to diagnosis, prognosis and treatment." Leukemia & lymphoma 49.sup1 (2008): 43-51. ↩ ↩
Ferreri, Andrés JM, et al. "Summary statement on primary central nervous system lymphomas from the Eighth International Conference on Malignant Lymphoma, Lugano, Switzerland, June 12 to 15, 2002." Journal of clinical oncology 21.12 (2003): 2407-2414. ↩ ↩