A primary CNS lymphoma in spontaneous remission for 3.5 years after initial detection of the lesions by MRI

Case Details

Disease Location

Cns (corpus callosum, tectum)

Personal Characteristics

61 -year-old male, pediatrician september 1996

Clinical Characteristics

Patient noticed disorientation and difficulties with writing and walking when he was 61 years old MRI findings diagnosed him with having hydrocephalus due to aqueductal stenosis, there were no abnormal intensity lesions or masses detected a ventriculoperitoneal shunt operation was performed via a right lateral ventricular approach 3 months after the operation, symptoms subsided significantly and patient returned to work. 3 months later a follow-up MRI revealed high-intensity regions in the corpus callosum and tectum on the flair andt2 images, the lesions were not enhanced after admin of gd-dtpa, and the patient was symptom free except for slightly unsteady walking, and no radiotherapy, chemo, or steroid therapy was administered for the next 3.5 years. Periodic mris were performed and the lesions were disclosed without significant alterations in april 2000, the patient noticed upper gaze palsy and then rigidity extending to the extremities, he became mentally confused and was admitted in june 2000, he suffered respiratory disturbance follwed by pneumonia and was intubated MRI didnt reveal marked dilatation of the ventricular system, and functionally insufficient csf circulation was suspected due to aqueductal stenosis, so pressure was reduced of the memos shunt system, but the patient didn't improve endoscopic third ventriculostomy was performed follow-up MRI in august revealed the high-intensity lesion in the cc had disappeared but the lesion in the tectum was unchanged, consciousness levels recovered transiently and he responded to verbal commands but then reverighted to a mute state, he was eventually transferred in september an MRI revealed the lesion in the tectum had developed into a linear lesion that was enhanced by the contrast medium and showed a tendency to spread on the dorsal side of the midbrain and pons. The cc continued to show no enhanced or high-intensity lesions the beta2-microglobulin content of his csf raised (8305 ng/ml), lesions were radiological diagnosed as malignant lymphomas and his poor condition didnt allow for chemo or radiotherapy, or to take biopsies... After given prednisolone, he didn't improve and eventually suffered respiratory arrest and need artificial respiratory supporight. MRI revealed a high-intensity mass in the right caudate nucleus and diffuse enhanced lesions around the third and fourighth ventricles and in the brain stem. He died in december at age 65 and an autopsy was performed. The brain weight 1100 grams and a vivid tumor was found in the right caudate nucleus where diffuse proliferation of large lumphoid cells with occasional mitotic figures and coagulation necrosis were evident. Infiltration around the blood vessels by small lymphocytes and macrophages was seen. Silver staining found an intricate reticulin network surrounding the tumor cells immunohistochemical staining found a large proportion of the large lymphoid cells were labeled with the anti-l-26 antibody, whereas the small lymphocytes were mainly labeled with the anti-uchl1 antibody. Malignant, diffuse large b-cell lymphoma was diagnosed, the lymphoma cells infiltrated the adjacent white matter and reactive astrocytes with atypical, elongated processes wre found at the infiltrating edges of the tumor exam of a midsagittal section of the cerebrum revealed a portion of the body of the cc had become attenuated histological findings included showing a partial loss of callosal fibers, reactive astrocytosis, and remnants of lymphoma cells around the vessels. Further examination found diffuse infiltration of the basal side of the cerebrum, midbrain, pontine, and medullary tegmentum and cerebellar peduncles by lymphoma cells; numerous lymphoma cells were scattered diffusely in the tectum. Visceral organs were found to contain no lymphoma cells

Remission Characteristics

In august 2000, the lesion in the corpus callosum disappeared, but the tectum lesion was unchanged

Treatment & Mechanisms

Proposed Remission Mechanisms

No specific mechanism proposed for this case, but it was alluded that immunological competence of the host has influence it may be immunophenotypic or henotypic characteristic, although is unknown

Clinical Treatment

Ventriculoperitoneal shunt for hydrocephalus intubated endotracheally endoscopic third ventriculostomy prednisolone artificial respiratory supporight

Non-Clinical Treatment

None reported