Primary Central Nervous System Lymphoma in the Fourth Ventricle

Abstract

Primary central nervous system lymphoma (PCNSL), a highly malignant and infrequent tumor, is rarely found within the fourth ventricle. Here we report a case of isolated lymphoma within the fourth ventricle: a 61-year-old male presented with headache, mental disorder, abnormal gait and urinary incontinence. Based on the clinical symptoms and neuroimage, He was initially diagnosed as choroid plexus papilloma. However, post-surgery pathology corrected the diagnosis as a diffuse large B-cell lymphoma. To the best of our knowledge, only nine cases are documented in the literature and all of them were initially diagnosed as other tumours or not determined. Therefore, Thesis unconventional location and atypical clinical manifestation of PCNSL in the fourth ventricle should be included on the list of differential diagnosis of the fourth ventricle tumors. Case ReportA 61-year-old man presented to our hospital complaining of headache for one month and somnolence, abnormal gait and urinary incontinence for one week. During admission, he was in a state of somnolence and physical examination only revealed the mild cervical resistance. His past medical history and laboratory tests including routine blood parameters, blood biochemistry (including lactate dehydrogenase (LDH) HIV testing), routine coagulation parameters and erythrocyte sedimentation rate were normal as well.Brain magnetic resonance imaging (MRI) revealed one irregular nodule occupying the fourth ventricle with a hypo intensity on T1 weighted image (T1WI) and is intensity on T2 weighted image (T2WI) (Figure 1). Post-contrast T1WI showed well-defined and marked homogenous enhancement without necrosis. Furthermore, contrast enhancement image demonstrated that the primary nidus of the mass originated from the roof of the forth ventricle, which was surrounded with choroid plexus (Figure 2). The intraventricular location of the mass and MRI manifestation led us to clinical diagnosis as choroid plexus papilloma (CPP). PET-CT of the whole body showed high metabolism in the fourth ventricle. The special location of the mass prompted our surgical team to choose surgical excision over stereotactic biopsy.Interestingly, post-surgery pathology diagnosed as a diffuse large Bcell lymphoma (Figure 3). The patient refused to undergo radiotherapy or chemotherapy immediately after the surgery. At 6-week follow-up after the resection, brain MRI demonstrated enhanced nodules in the third ventricle and midbrain aqueduct area, which were considered as implantation metastases. Consequently, the patient was administrated with chemotherapy combined with radiotherapy. Another month later, these lesions amazingly disappeared on brain MRI. Currently, 20 months post-surgery, the patient is still under active follow-up.

Primary CNS Lymphoma Arising from the 4th Ventricle: A Case Report and Review of the Literature

“…Imaging typically reveals a single brain lesion (66%), commonly in the supratentorial region (87%) [23]. Literature review of patients with 4 th ventricular PCNSL indicates all patients were immunocompetent, had a median age of onset at 61-years-old, and had a male-to-female predominance (3.25:1) and intracranial metastatic disease in 35.3% of cases, with 50% of these documenting metastases to other ventricles (Table 1, Figure 4) [7–22].…”

“…However, this advantage was lost when controlling for the total number of lesions [35]. Of patients with 4 th ventricular PCNSL, 35.3% of patients received ITC [8–11, 14, 16], and 52.9% of patients with 4 th ventricular PCNSL had gross subtotal or total resection [8, 11–17, 22]. These patients also received various combinations of chemotherapy and chemoradiation so whether either of these treatment modalities conferred a survival benefit in this select patient population is not known.…”

“…The most common subtype is DLBCL, accounting for approximately 90% of PCNSLs [6]. While PCNSL is an infrequent diagnosis, rarer still is the diagnosis of intraventricular lymphoma, with a limited number of cases to contribute to the knowledge of clinical manifestations, diagnostic modalities, and optimal treatment [7–22]. The following case adds to the limited clinical knowledge of 4 th ventricular PCNSL.…”