Case Report Primary CNS Lymphoma & Role Of Surgery – A Narrative Review With Illustrative Cases: Surgery for Primary CNS Lymphoma

Amol Mittal; Chandrashekhar Deopujari; Chandan Mohanty; Vikram Karmarkar

doi:10.15713/ins.bhj.153

Authors

Amol Mittal Bombay Hospital https://orcid.org/0000-0001-7468-9611
Chandrashekhar Deopujari Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, New Marine Lines, Mumbai, Maharashtra, India – 400020
Chandan Mohanty Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, New Marine Lines, Mumbai, Maharashtra, India – 400020
Vikram Karmarkar Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, New Marine Lines, Mumbai, Maharashtra, India – 400020

DOI:

https://doi.org/10.15713/ins.bhj.153

Keywords:

Chemotherapy, Diffuse large B-cell Lymphoma, Primary CNS Lymphoma, Stereotactic Biopsy, Surgical Excision

Abstract

Aim: Primary CNS Lymphoma (PCNSL) accounts for 6.7% of malignant primary CNS tumours and is the second most common malignant tumour of the brain after gliomas. The most common type of CNS lymphoma is the primary diffuse large B cell lymphoma (DLBCL) of the CNS which occurs in the immunocompetent individual. However, it is also prevalent in immunocompromised patients and recently has been classified as a separate subgroup. The prognosis has shown a substantial improvement in these patients with the advent of high-dose methotrexate (HDMTX) and rituximab-based regimens with or without radiotherapy. Traditionally, surgery in the form of only a biopsy was considered for the definitive diagnosis of PCNSL though craniotomy for surgical excision is being recommended by some recent studies. This article focuses on the role of surgery in PCNSL in immunocompetent patients.

Background: The role of surgical intervention has been limited to stereotactic biopsy. Older research articles demonstrated a high rate of complications and no additional benefit with craniotomy/surgical excision. However, more recent studies have reported a similar or a lower complication rate with excision and are associated with better overall survival (OS) in selected patient subsets.

Conclusion: HDMTX with rituximab-based regimens with or without radiotherapy remains the cornerstone for the management of PCNSL. The diagnosis is established via stereotactic biopsy in cases with deep-seated, multiple lesions, older age, and poor KPS score. Cytoreductive surgery has shown improved survival in the selected subset of patients with solitary lesions in superficial locations, especially in the younger age group and good KPS score. However, the strength of the present evidence is low and future prospective trials may better elucidate the role of surgery in the management of PCNSL.

Author Biographies

Amol Mittal, Bombay Hospital

Chandrashekhar Deopujari, Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, New Marine Lines, Mumbai, Maharashtra, India – 400020

Professor & Head

Chandan Mohanty, Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, New Marine Lines, Mumbai, Maharashtra, India – 400020

Assistant Professor

Vikram Karmarkar, Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, New Marine Lines, Mumbai, Maharashtra, India – 400020

Associate Professor

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