Intravascular large B-cell lymphoma affecting multiple cranial nerves: A histopathological study.

ABSTRACT

Intravascular large B-cell lymphoma (IVLBCL) is a rare form of lymphomas with poor prognosis, characterized by atypical lymphocytes selectively growing within the lumen of small or medium-sized vessels. Here, we report a case of intracerebral IVLBCL in a 54-year-old man who died three months after symptom onset. The diagnosis was made by postmortem pathological examination, based on the identification of multiple ischemic lesions, with small or medium-sized vessels filled with malignant B-cells, in the cerebral hemispheres, cerebellum, midbrain, and medulla oblongata, including the external cuneate nucleus and trigeminal spinal tract nucleus. Apart from necrotic lesions, specific histopathological search for occluded vessels in the other brain stem structures permitted identification of significant involvement of the cuneate nucleus, solitary tract nucleus, hypoglossal nucleus, and inferior olivary complex. Small vessels affected by IVLBCL were also found in the trunks of the oculomotor, trigeminal, glossopharyngeal, vagal, and hypoglossal nerves. These histopathological findings were consistent with some cranial nerve symptoms/signs ascertained during hospitalization, such as diplopia, dysphonia, and asymmetry/hypomotility of the palatal veil. The case study presented here reports novel insights on radiological, anatomical, and clinical correlations of the IVLBCL, including the possible involvement of nuclei and trunks of multiple cranial nerves. The reported findings may help clinicians in the early identification of this rapidly progressive disease that can be easily misdiagnosed, through integrated neuroradiological, neurological and neuropathological approaches.