Corpus Callosum Primary Central Nervous System Lymphoma

J.Sales-Llopis

Neurosurgery Service, Alicante University General Hospital, Alicante, Spain.

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Twenty-seven patients were included (median age: 67 years, median Karnofsky Performance Status: 70); cognitive impairment and balance disorders were present in 74% and 59%, respectively. At diagnosis, neuropsychological test results were abnormal for global cognitive efficiency (63% of patients), memory (33-80% depending on the test) and executive functions (44-100%). Results for visuospatial and language tests were normal. All patients received high-dose methotrexate-based polychemotherapy, followed in one patient by whole-brain radiotherapy; 67% of patients achieved complete response (CR). With a median follow-up of 48 months (range 6-156), patients in CR had persistent abnormal test results for global cognitive efficiency in 17%, executive function in 18-60%, depending on the test, and memory in 40-60%. Splenium location and age ≥ 60 years were significantly associated with worse episodic memory scores throughout the follow-up.

PCNSL-CC is associated with frequent cognitive dysfunctions, especially memory impairment, which may recover only partially despite CR and warrant specific rehabilitation. Older age (≥ 60) and splenium location are associated with worse neurocognitive outcomes ¹⁾.

A 52-year-old man attended the emergency department with a 2-week history of progressively worsening abnormal behavior and gait disturbance. Magnetic resonance imaging on admission revealed an oval lesion in the splenium of the corpus callosum. The follow-up magnetic resonance imaging performed 2 months after disease onset revealed multiple high-signal areas in the bilateral cerebral white matter on T2-weighted images and diffusion-weighted images. The blood test results showed an elevated level of lactate dehydrogenase and serum-soluble interleukin-2 receptor. These findings were compatible with the diagnosis of IVL. IVL is often difficult to diagnose due to a wide variety of clinical presentations and imaging findings ²⁾

A 49-year-old female is taken to the emergency department due to disorientation while at work. The woman refers to asthenia, dyspneic sensation, and unquantified weight loss.

According to companions, the patient presented mood swings in the last month with forgetfulness of basic activities of her work with the impossibility of doing it.

In addition, she presented an episode that, due to the clinical characteristics that they explain to us, impresses with global amnesia without completing an imaging study.

Intraventricular tumor with aggressive characteristics with signs of subependymal spread/drop metastasis. To assess, among others, Choroid plexus tumor, ependymoma, metastasis…

The intraventricular lesion up to 54x34x30mm that extends through both lateral ventricles crossing the midline; more on the left where it makes extensive contact with the choroid plexus. It appears minimally hyperintense on T2 and minimally hypointense on T1 with diffusion restriction and very low signal on ADC associated with striking and homogeneous enhancement without intralesional bleeding or calcification. Shows signs of the infiltration of the adjacent corpus callosum with edema/increased signal in both corona radiates A 4mm enhancement focus was associated in the most medial area of the left caudate and periventricular adjacent to the posterior horn of the ipsilateral ventricle as well as a 9mm enhancement in the IV ventricle and on the left edge of the mime; impressing this affectation of subependymal dissemination, drop metastasis….. Secondary hydrocephalus with an Evans index of 0.31, but with increased diameter in the temporal and occipital horns.

Primary central nervous system lymphoma CD45 + , S100 protein - , AE1/AE3 - .

In the next days rapid neurological deterioration

CT

Hyperdense solid mass in the midline, supratentorial, located in both lateral ventricles.

Bilateral frontal skin incision over Kocher's point. Trephine at that level. Dural opening with the help of monopolar and forceps. Introduction of a Bactiseal external ventricular catheter with clear CSF output at very high pressure.

¹⁾

Nilles C, Delgadillo D, Sarazin M, Nichelli L, Mokhtari K, Mathon B, Choquet S, Feuvret L, Alentorn A, Ribeiro M, Hoang-Xuan K, Houillier C. Primary CNS lymphoma of the corpus callosum: presentation and neurocognitive outcomes. J Neurooncol. 2022 May;158(1):99-109. doi: 10.1007/s11060-022-04014-7. Epub 2022 Apr 21. PMID: 35445956.

²⁾

Masuzawa H, Suzuki F, Amemiya S, Orimo K, Ishiura H, Hara R, Toda T, Nakazawa T, Honda A, Tanaka M, Hinata M, Abe O. A case of intravascular lymphoma presenting with a lesion in the splenium of the corpus callosum. Radiol Case Rep. 2023 Mar 16;18(5):1929-1932. doi: 10.1016/j.radcr.2023.02.025. PMID: 36970234; PMCID: PMC10030823.