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Washington University Experience | NEOPLASMS (HEMATOLYMPHOID) | Lymphoma, Intravascular | 7A0 Case 7 History
Case 7 History ---- The patient was a 57 year old man admitted to BJH on 4/8 with a 6 month history of progressive dementia, low back pain and gait disturbance. In November he experienced sudden onset of left arm weakness and numbness with slurring speech which lasted 5 to 10 minutes. A right carotid arteriogram was also normal. His discharge diagnosis was TIA and treated with aspirin. Two days later however he began screaming with low back pain which spread into both legs. Because of persistence of this pain he was admitted to an OSC. CT scan of the head and lumbar myelogram were normal. CSF showed glucose 44, protein 480 and 5 monocytes. All other cerebrospinal tests including viral fungal and mycobacterial cultures were normal. ESR was 40. During the first 24 hours of his hospitalization at the OSC, his mental status deteriorated and then gradually returned to baseline over the next 10 days. A repeat LP revealed a CSF glucose and cell count to be normal and the protein was 121 mg%. Over the next 2 months the patient was unable to work or care for himself he was oriented to name only and used neologisms. He followed only occasional single step commands with a very short attention span. He had lower extremity weakness with atrophy and intermittent fasciculations. At this point knee and ankle jerks were absent. Fluctuating episodic clinical course and TIA suggested angiopathy or multiple emboli. It was felt that CJD was doubtful. A trial of high dose pulse steroids was given with no response. The CSF IGG albumin ratio was normal, and oligoclonal bands were negative. Arsenic in the hair and serum manganese provided no support for a toxic etiology. A sural nerve biopsy was performed. Sections of sural nerve and muscle did not exhibit intravascular tumor. There was no vasculitis or other inflammation seen. The patient remained semicomatose and unresponsive. He was found dead in bed in on May 4.---- At the time of autopsy a 5 cm region of cortical duskiness was identified in the right superior parietal region on the right with mild focal degree of atherosclerosis. The examination of the remainder of the cerebral hemispheres shows multifocal areas of dusky grey discoloration of the cortex. ---- Microscopic sections demonstrate diffuse involvement of the brain blood vessels by a neoplastic process consisting of small caliber vessels which contain predominantly detached neoplastic cells without apparent intercellular connections. Small, but grossly visible, cystic infarcts are present in the head of the left caudate, the left putamen, the right cerebellar hemisphere watershed region, and the splenium of the corpus callosum. The cerebral white matter shows multiple microscopic subacute infarcts, characterized by axonal spheroids, macrophages and variable astrocytosis. Small capillary vessels are associated with microthrombi. ---- The diagnosis made on this case in 1979 was neoplastic angioendotheliomatosis. At that time immunohistochemistry was only beginning to be applied and the intravascular nature of the malignant cells as lymphomatous in origin had not been established.
