Primary central nervous system lymphoma initially mimicking lymphomatosis cerebri: An autopsy case report

Abstract

A 59-year-old immunocompetent man was admitted to our hospital because of progressive dementia with concomitant bilateral uveitis. The first brain MRI revealed diffuse hyperintense lesions in the cerebral white matter of both hemispheres on a T2-weighted image and fluid-attenuated inversion recovery image. However, another MRI taken more than 1 month later revealed enhanced cohesive mass lesions in the bilateral thalami, in addition to the white matter lesions. The white matter lesions were slightly hyperintense on a diffusion-weighted image and apparent diffusion coefficient map image, suggesting vasogenic edema. One year after the onset of uveitis, he died of respiratory failure. Pathological diagnosis was diffuse large B-cell lymphoma with perivascular proliferation and diffuse scattered infiltration in the cerebrum and brainstem. Microscopically, cohesive mass lesions in the bilateral thalami were a massive cluster of lymphoma cells. This is a case of primary CNS lymphoma (PCNSL) mimicking 'lymphomatosis cerebri (LC)' at first but later exhibiting typical mass lesions, giving rise to the possibility that cases of LC might unmask features of regular lymphomas in their later course more often than believed thus far.

Utility of Proton MR Spectroscopy for Differentiating Typical and Atypical Primary Central Nervous System Lymphomas from Tumefactive Demyelinating Lesions

“…We also observed that the Cho/Cr ratio of typical, nodular PCNSLs was higher than that of atypical PCNSLs. This may be because homogeneously enhancing PCNSLs had more densely packed lymphoma cells than heterogeneously enhancing PCNSLs, 29 such that they had more active cell division, rapid cell turnover with synthesis of membrane phospholipids, and higher Cho/Cr ratios. The lower Cho/Cr ratios of atypical PCNSLs may result in the decreased diagnostic performance of the Cho/Cr ratio for discriminating between atypical PCNSLs and TDLs.…”

“…8,30 An autopsy report of a patient with PCNSL suggested that only scattered infiltration of malignant lymphomatous cells was observed in the nonenhancing tumor portion. 29 Therefore, we speculate that the low cellularity and low invasiveness contributed to the nonspecific MR spectrum in those cases.…”

Neurocognitive Features Distinguishing Primary Central Nervous System Lymphoma from Other Possible Causes of Rapidly Progressive Dementia

“…From 3238 results, with the earliest paper published in 1950, we winnowed down to 16 articles that met our inclusion criteria of English-language case reports describing dementia as the presenting syndrome in adults with PCNSL. These 16 articles, published between 1990 and 2013, contained 20 original case reports of patients with pathologically confirmed PCNSL: Bakshi et al, 1999; Carlson, 1996; Kanai et al, 2008; Kim et al, 2006; Leschziner et al, 2011; Lewerenz et al, 2007; Matosevic et al, 2010; Nakasu et al, 1990; Pandit et al, 2010; Rollins et al, 2005; Sugie et al, 2009; Sugino et al, 2013; Vital and Sibon, 2007; Weaver et al, 2007; Widdess-Walsh et al, 2005; and Zarabi et al, 1992.…”

An autopsy case of lymphomatosis cerebri showing pathological changes of intravascular large B-cell lymphoma in visceral organs

“…To our knowledge, there have only been 13 pathologically confirmed cases of this type of PCNSL demonstrated by MRI (Table 1). [6][7][8][17][18][19][20][21][22][23] These lesions showed either absent or mild contrast enhancement on MRI. The clinical features were progressive dementia, gait disturbance, and akinetic mutism.…”

“…Malignant cells were rarely found in the CSF or peripheral blood smears. Eight cases could be correctly diagnosed by brain biopsy, [6][7][8][17][18][19]22 three by autopsy, 6,21,23 and the other cases by CSF cytology and/or lymph node biopsy. 8,20 In the present case, brain biopsy was not helpful in making a diagnosis.…”