Clinical diagnosis
Case 11
【Follow-up】
The patient received biopsy under open craniotomy. Microscopic examination during operation revealed malignant lymphoma. Several days later, blood laboratory tests revealed diabetes insipidus and he lost consciousness probably because of the tumor progressively growing to hypothalamus and passed away.
【Discussion】
On diffusion-weighted magnetic resonance imaging (DWI), the lesion with the more restriction of water diffusion appears to be higher signal intensity (1, 2). Meanwhile, the apparent diffusion coefficient (ADC) value was lower in the lesion with the more restriction of water diffusion, inducing the relatively low signal intensity in ADC map (1, 2). Because malignant lymphoma lesions microscopically own high cellularity and high nuclear-to-cytoplasm ratio, water diffusion disorder is considered to occur (2). Then, a malignant lymphoma lesion such as our case has been shown as high signal intensity in DWI and low signal intensity in ADC map, compared to the surrounding tissue.
Lactate dehydrogenase (LDH) is known to be contained in malignant lymphoma (ML), leukemia and other active growing tumors. While the tumors rapidly increase, those are simultaneously destroyed. The increase speed surpasses the destruction speed, inducing the tumor growth. Then, LDH level often elevates in the growing ML. The ML patients with LDH levels of 500 U/ml or less is reported to significantly survive longer than those with LDH levels greater than 500 U (Two-year survival rates were 67% and 13%, respectively, documented in 1980) (3).
Interleukin-2 receptor (IL-2R) is known to be produced by activated lymphocytes or ML cells (4, 5). Harrington DS reported in 1988 that five-year actuarial survival was 20% for patients with IL-2R levels greater than 1000 U/mL and 86% for patients with those 1000 or less (4).
In our case, although levels of LDH and IL-2R were lower less than 500 U/ml and 1000 ml/U, respectively, the prognosis was poor after biopsy because the tumor invades to hypothalamus rapidly. A short survival case with malignant lymphoma originated from hypothalamus causing diabetes insipidus and consciousness disorder, was previously documented (6). A biopsy of the cerebral mass lesion might permit diagnosis, but appropriate and speedy therapy such as steroid and methotrexate should be administered.
【Summary】
A seventy one-year-old male came to our hospital for speaking disturbance and behaving slowly. Laboratory test revealed the high values of Interleukin 2 receptor 533 U/ml and LDH 460 U/ml. Non-enhanced and contrast-enhanced CT depicted homogeneous iso-density mass and homogeneously enhanced mass, respectively. DWI and ADC map depicted high signal intensity and low signal intensity respectively, indicating the typical images of ML. Although the prognosis was poor in our case because of the tumor invasion to hypothalamus, the speedy treatment was considered to be crucial.
【References】
1.Lin C, et al. Whole-body diffusion-weighted imaging in lymphoma - NCBI www.ncbi.nlm.nih.gov › NCBI › Literature › PubMed Central (PMC)
2.Lin C, et al. Whole-body diffusion-weighted magnetic resonance imaging with apparent diffusion coefficient mapping for staging patients with diffuse large B-cell lymphoma. Eur Radiol. 2010;20:2027–2038.
3.Schneider RJ, et al. Prognostic significance of serum lactate dehydrogenase in malignant lymphoma. Cancer. 1980;46:139-43.
4.Harrington DS, et al. Soluble interleukin 2 receptors in patients with malignant lymphoma. Arch Pathol Lab Med. 1988 ;112:597-601.
5.Tsujioka T, et al. The impact of serum soluble interleukin-2 receptor levels on the diagnosis of malignant lymphoma. Kawasaki Medical Journal 2011; 3：19－27.
6.Biasiotta A, et al. Primary hypothalamic lymphoma in a patient with systemic lupus erythematosus: case report and review of the literature. Neurol Sci. 2010;31:647-652. doi: 10.1007/s10072-010-0338-x. Epub 2010 Jun 29.
2016.06.22
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