Letters to the Editor

Emergent management with favorable outcome of an unusual presentation of a primary CNS lymphoma in an immunocompetent patient

demonstrating a angio‑centric growth pattern without follicles.[2] In immunocompetent patients most of the PCNSL (90‑95%) are B cell lymphomas.[2]

Sir, Primary CNS lymphomas (PCNSL) are extra‑nodal, malignant tumors arising within the brain, eyes, leptomeninges and spinal cord in the absence of systemic involvement at the time of diagnosis.[1,2] PCNSL is seen in both immunocompromised and immunocompetent patients. [1,2] PCNSL differs from its systemic counterpart by having a poorer prognosis and

A 48‑year‑old male patient presented to the emergency room in an altered sensorium of 1 h duration with preceding progressive hemiparesis of six months duration. The neurological examination revealed a deeply comatose patient with a Glasgow Coma score of 7/15 [E1V1M5]. The patient had a dilated right pupil with left hemiparesis of grade 2. MRI of the brain [Figure 1] showed a well‑defined lobulated parasagittal lesion.

PCNSL occur in decreasing order of frequency in the cerebral hemisphere (38%), basal ganglia and thalamus (16%) corpus callosum (14%), ventricular region (12%), and the cerebellum (9%), demonstrating a proclivity toward the midline peri‑ventricular structures.[1,3] The commonest presenting features are neuropsychiatric.[1,3] Although features of raised intracranial pressure may be present in as many as 33% (mainly due to hydrocephalus), impending herniation during the presentation is extremely rare and only three such cases have been reported in the literature [Table 1].[1‑5]

a

b

g

e

c

f

d

h

i

Figure 1: MRI of the brain revealing a right parasagittal lesion measuring 7.4 × 3.8 × 6.0 cm. It was hypointense on TIW images (a) Hyperintense on T2W images (b) With mass effect and midline shift. Figure 1c and d depict the contrast enhancement on T1W coronal and sagittal images. There was restriction on DWI (e) And hypointensity on ADC. (f) The MR spectroscopy revealed an elevated choline to creatinine ratio and loss of NAA on MRS (g) With surrounding peri‑lesional edema with subfalcine/uncal herniation. H and i are post‑operative TIW gadolinium images depicting complete excision of lesion 88

Journal of Neurosciences in Rural Practice | January - March 2014 | Vol 5 | Issue 1

Letters to the Editor

Table I: Review of literature of PCNSL operated as an emergency Year Author

Age/sex Presentation

Acute Location herniation signs Present Left frontal lobe

2008 Kim IY et al.[5]

49/F

2012 Ilker A, et al.[4]

33/F

Deterioration in sensorium, hemiparesis, raised ICP Headache, drowsiness N.A

2013 Cheatle JT et al.[3]

31/F

Headache/ataxia

N.A

2013 Matsuyama J et al.[6] 2013 Present case

64/M

Comatose

Present

48/M

Headache

Present

raised ICP

Cause of acute Procedure Follow up presentation Hemorrhage Decompression 10 months

Frontal – septum Increase in pellucidum size and mass effect during pregnancy Septum Increase in pellucidum size and mass effect during pregnancy^ Right frontal Hemorrhage R posterior frontal

Acute herniation

Tumor Death decompression delayed 2 weeks* Decompression 3 years

Endoscopic Death evacuation Emergency 3 months craniotomy and decompression

*Tumor decompression delayed until fetal lung maturity was attained, ^ Biopsied earlier‑ inconclusive, R: Right, ICP: Intracranial pressure, N.A: Not available, PCNSL: Primary CNS lymphomas

The patient underwent emergency craniotomy and decompression of the lesion. Intra‑operatively the tumor had a poor plane with the brain in some areas. Histopathology clinched the diagnosis of a B cell non‑Hodgkin’s lymphoma [Figure 2]. Post‑operatively the patient improved in sensorium as did his motor power to grade 4. Evaluation for systemic lymphoma was negative. The patient underwent whole brain radiotherapy of 60 Gy and received high dose of methotrexate chemotherapy. Despite advancement in the treatment modalities the prognosis of PCNSL remains poor.[3] Symptomatic therapy results in a median survival of 2‑3 months.[1,2] Steroids decrease the tumor volume by inducing apoptosis in the cells and prolongs the median survival to 4‑5 months.[2] The standard of care has been systemic chemotherapy with or without radiotherapy.[1‑5] Surgery has been traditionally contraindicated in PCNSL due to its diffusely infiltrative nature[1,2] and has mainly been limited to tissue diagnosis especially in the cases of atypical radiology and partial decompression has no proven benefit over a stereotactic biopsy and has sometimes been associated with a poorer prognosis.[1,2] There has been one recent review by Weller et al. which challenges this traditional view favoring cytoreductive surgery at least for a subset of patients with PCNSL[7] We have done a review of the literature highlighting the very few instances in which a PCNSL should be operated [Table 1]. In this group of patients, the presentation was due to increased size during the pregnancy in two cases and hemorrhage in two cases.[3‑6] There have been no cases reported in which the patient had impending herniation due to the size of the tumor, as in the present case.

Figure 2: Paraffin section of non‑Hodgkin lymphoma showing monomorphic round to oval cells with hyperchromatic nuclei, mitotic activity and (Inset) immunopositivity for CD20. (H and E ×400) [Inset: Avidin Biotin Complex Immunoperoxidase ×400]

Despite the criticism associated with surgery in PCNSL, it has a significant and special role in the case of rare emergency presentation and may have a favorable outcome such as in the present case. Arun S. Rao, Ravi Dadlani, Nandita Ghosal1, Alangar S. Hegde Departments of Neurosurgery, and 1Pathology, Sri Sathya Sai Institute of Higher Medical Sciences, Bangalore, Karnataka, India Address for correspondence:  Dr. Ravi Dadlani, Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP Area, Whitefield, Bangalore ‑ 560 066, Karnataka, India. E‑mail: [email protected]

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References 1. Gerstner E, Batcheolar T. Primary CNS lymphoma. Expert Rev Anticancer Ther 2007;7:689‑700. 2. Schlegel U, Schmidt‑Wolf  IG, Deckert M. Primary CNS lymphoma: Clinical presentation, pathological classification, molecular pathogenesis and treatment. J Neurol Sci 2000;181:1‑12. 3. Cheatle  JT, Aizenberg  MR, Weinberg  JS, Surdell  DL. Atypical presentation of primary central nervous system non‑Hodgkin lymphoma in immunocompetent young adults. World Neurosurg 2013;79:593.e9‑13. 4. Ilker  A, Aykut  B, Muge  H, Ibrahim  HM, Ulkua  OB, Tugba  D, et al. Primary brain T‑cell lymphoma during pregnancy. J  Pak Med Assoc 2012;62:394‑6. 5. Kim IY, Jung S, Jung TY, Kang SS, Choi C. Primary central nervous system lymphoma presenting as an acute massive intracerebral hemorrhage: Case report with immunohistochemical study. Surg Neurol 2008;70:308‑11. 6. Matsuyama  J, Ichikawa  M, Oikawa  T, Sato  T, Kishida  Y, Oda  K, et al. Primary CNS lymphoma arising in the region of the optic nerve presenting as loss of vision: 2  case reports, including a patient with a massive intracerebral hemorrhage. Brain Tumor Pathol 2013. 7. Weller M, Martus P, Roth P, Thiel E, Korfel A; German PCNSL Study Group. Surgery for primary CNS lymphoma? Challenging a paradigm. Neuro Oncol 2012;14:1481‑4.

with or without radiotherapy, and surgery is mainly limited to tissue diagnosis, due to its diffusely infiltrative nature. However, in some cases, such as a huge tumor with impending herniation or CNS lymphoma cases with intratumoral hemorrhage, emergency surgery for mass reduction is required. Intracerebral hemorrhage in primary CNS lymphoma is rare, but higher vascular endothelial growth factor (VEGF) immune reactivity in hemorrhagic cases is observed compared to non‑hemorrhagic cases.[2‑5] Also, serum elevation of VEGF levels are predictors of poor prognosis. In the previously reported cases of intratumoral hemorrhage in primary CNS lymphomas, emergent craniotomy and removal of mass reduction was performed. Although impending herniation of primary CNS lymphomas is rare, a mass reduction surgery should sometimes be considered in such unique cases.

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Junko Matsuyama

Website: www.ruralneuropractice.com

Department of Neurosurgery, Fukushima Medical University,1 Hikarigaoka, Fukushima, 960‑1295, Japan Address for correspondence: Dr. Junko Matsuyama, Department of Neurosurgery, Fukushima Medical University, 1 Hikarigaoka, Fukushima, 960‑1295, Japan. E‑mail: [email protected]

DOI: 10.4103/0976-3147.127925

Emergent management with favorable outcome of an unusual presentation of a primary central nervous system lymphoma in an immunocompetent patient Sir, This article is a report of a case with primary central nervous system (CNS) lymphoma, with impending brain herniation due to a huge mass, and surgical reduction was effective in obtaining neurological improvement.[1] Usually the standard therapy for primary CNS malignant lymphoma, at present, is high‑dose methotrexate therapy 90

References 1.

2. 3. 4. 5.

Rao  AS, Dadlani  R, Ghosal  N, Hegde  AS. Emergent management with favorable outcome of an unusual presentation of a primary CNS lymphoma in an immunocompetent patient. J  Neurosci Rural Pract 2014;5:88-90. Fukui  MB, Livstone  BJ, Meltzer  CC, Hamilton  RL. Emorrhage He Hemorrhagic presentation of untreated primary CNS lymphoma in a patient with AIDS. AJR Am J Roentgenol 1998;170:1114‑5. Rubenstein J, Fischbein N, Aldape K, Burton E, Shuman M. Hemorrhage and VEGF expression in a case of primary CNS lymphoma. J Neurooncol 2002;58:53‑6. Kim IY, Jung S, Jung TY, Kang SS, Choi C. Primary central nervous system lymphoma presenting as an acute massive intracerebral hemorrhage: Case report with immunohistochemical study. Surg Neurol 2008;70:308‑11. Matsuyama J, Ichikawa M, Oikawa T, Sato T, Kishida Y, Oda K, et al. Primary CNS lymphoma arising in the region of the optic nerve presenting as loss of vision: 2 case reports, including a patient with a massive intracerebral hemorrhage. Brain Tumor Pathol 2013 [Epub ahead of print].

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DOI: 10.4103/0976-3147.127926

Journal of Neurosciences in Rural Practice | January - March 2014 | Vol 5 | Issue 1

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Emergent management with favorable outcome of an unusual presentation of a primary CNS lymphoma in an immunocompetent patient.

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