unusual location of primary central nervous system lymphoma · anamnesis in 2015, previously...

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Unusual location of primary central nervous system lymphoma Milda Sarkinaite 1 , Kristina Banioniene 1 , Rymante Gleizniene 2 1 Lithuanian University of Health Sciences 2 Hospital of Lithuanian University of Health Sciences Kaunas Clinics, Department of Radiology Key words Primary central nervous lymphoma, hypothalamus, high grade glioma, B-cell, diagnosis Introduction Primary central nervous system lymphoma (PCNSL) is a high grade extranodal Non-Hodging’s lymphoma beginning in the central nervous system (CNS) in the lack of systemic disease. PCNSL is mainly diffuse large B-cell lymphoma and represents 1% of all non-Hodgkin lymphomas and 4% of all malignant brain tumors [1,2]. The hypothalamus is one of the rare locations of lymphoma in adults. Other lesions that can mainly involve the hypothalamus are glioma, metastasis, neurosarcoidosis. PCNSL has higher incidence in patients with immunodeficient status and high prevalence is organ transplants [3]. Case Report Course of the disease. Part 1 Diagnostic tests No palpable lymphadenopathy or organomegaly during clinical examination. Neurological examination showed loss of muscle coordination. The complete blood count, renal and liver function tests were normal, serology for HIV and Ebstain Bar virus - negative. No pathological findings on abdominal and thoracic investigations. Patient was referred for magnetic resonance imaging (MRI); scans were performed at ,,Affidea’’ diagnostic centre and revealed contrast-enhanced hypothalamus tumor (Figure 1). Differential diagnosis of the lesion consisted of glioma and lymphoma and the patient was referred for biopsy. A stereotactic biopsy at LSMU Kaunas Clinics led to a diagnosis of diffuse large B-cell lymphoma, primarily located in the central nervous system. Anamnesis In 2015, previously healthy 69-year-old woman presented at Ukmerge Hospital with a 4-week history of: acute memory disturbancies – impaired short term memory; dizziness; weakness; rapidly worsening loss of orientation and walking impairment. There was no associated history of headache, vomiting, fever, seizures, urine incontinence or gait disturbance. She had no history of congenital immunodeficiency disease, previous organ transplantation or immunosuppressive therapy. Patient completed a few courses of chemotherapy treatment, but condition remained critical (acute confusional state with the need of 24-hour nursing care) although MRI scans showed no evidence of hypothalamus tumor (Figure 2). References 1. Gallop-Evans E. Primary central Nervous System Lymphoma. Clin Oncol. 2012;24(5):329-38. 2. Plotkin SR, Batchelor TT. Primary nervous-system lymphoma. Lancet Oncol. 2001;2(6):354-65. 3. Nisar Tanki HN, Malik KN, Makhdoomi R, Feroz S, Ramzan AU. Primary Hypothalamic Lymphoma in an Adult Male: A Case Report and Literature Review. Oman Med J. 2018 Jul; 33(4): 346–351. Figure 1. The first MRI: 1 – T2W/FLAIR axial plane; 2 -T1W with contrast axial and coronal planes; images show a well-defined hypothalamic lesion with marked homogeneous contrast enhancement. Course of the disease. Part 2 Figure 2. Post-treatment MRI: 1 – T2W/FLAIR, 2 - T1W with contrast images the same axial plane as Figure 1. Previously detected lesions are no longer recognizable. Figure 3. MRI repeated after 1 year from the beginning of the disease. T1W with contrast coronal images: emerging multifocal parenchymal nodulus. Patient's health status kept worsening. After 1 year MRI was repeated and revealed no hypothalamus tumor but emerging multifocal parenchymal changes (Figure 3). Discussion The most remarkable finding of our case is the unusual location of lymphoma involving hypothalamus and acute memory loss as clinical manifestation of the disease. Usually lymphoma is diagnosed by performing clinical examination or blood tests. Radiological diagnosis of PCNSL is often difficult because imaging findings may mimic other pathologies, although in this case MRI results led to the diagnosis of primary lymphoma. 1 2 1 2

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Page 1: Unusual location of primary central nervous system lymphoma · Anamnesis In 2015, previously healthy 69-year-old woman presented at UkmergeHospital with a 4-week history of: • acute

Unusual location of primary central nervous system lymphomaMilda Sarkinaite1, Kristina Banioniene1, Rymante Gleizniene2

1 Lithuanian University of Health Sciences2 Hospital of Lithuanian University of Health Sciences Kaunas Clinics, Department of Radiology

Key wordsPrimary central nervous lymphoma, hypothalamus, high grade glioma, B-cell, diagnosis

IntroductionPrimary central nervous system lymphoma (PCNSL) is

a high grade extranodal Non-Hodging’s lymphoma

beginning in the central nervous system (CNS) in the lack

of systemic disease. PCNSL is mainly diffuse large B-cell

lymphoma and represents 1% of all non-Hodgkin

lymphomas and 4% of all malignant brain tumors [1,2]. The

hypothalamus is one of the rare locations of lymphoma in

adults. Other lesions that can mainly involve the

hypothalamus are glioma, metastasis, neurosarcoidosis.

PCNSL has higher incidence in patients with

immunodeficient status and high prevalence is organ

transplants [3].

Case Report

Course of the disease. Part 1

Diagnostic tests

• No palpable lymphadenopathy or organomegaly during clinical

examination.

• Neurological examination showed loss of muscle coordination.

• The complete blood count, renal and liver function tests were

normal, serology for HIV and Ebstain Bar virus - negative.

• No pathological findings on abdominal and thoracic

investigations.

• Patient was referred for magnetic resonance imaging (MRI);

scans were performed at ,,Affidea’’ diagnostic centre and

revealed contrast-enhanced hypothalamus tumor (Figure 1).

Differential diagnosis of the lesion consisted of glioma and

lymphoma and the patient was referred for biopsy.

• A stereotactic biopsy at LSMU Kaunas Clinics led to a diagnosis

of diffuse large B-cell lymphoma, primarily located in the

central nervous system.

Anamnesis

In 2015, previously healthy 69-year-old woman

presented at Ukmerge Hospital with a 4-week history of:

• acute memory disturbancies – impaired short term

memory;

• dizziness;

• weakness;

• rapidly worsening loss of orientation and walking

impairment.

There was no associated history of headache, vomiting,

fever, seizures, urine incontinence or gait disturbance.

She had no history of congenital immunodeficiency

disease, previous organ transplantation or

immunosuppressive therapy.

Patient completed a few courses of chemotherapy treatment, but condition

remained critical (acute confusional state with the need of 24-hour nursing care)

although MRI scans showed no evidence of hypothalamus tumor (Figure 2).

References1. Gallop-Evans E. Primary central Nervous System Lymphoma. Clin Oncol. 2012;24(5):329-38.

2. Plotkin SR, Batchelor TT. Primary nervous-system lymphoma. Lancet Oncol. 2001;2(6):354-65.

3. Nisar Tanki HN, Malik KN, Makhdoomi R, Feroz S, Ramzan AU. Primary Hypothalamic Lymphoma in an Adult Male: A Case Report and Literature Review. Oman Med J. 2018 Jul; 33(4): 346–351.

Figure 1. The first MRI: 1 – T2W/FLAIR axial plane; 2 -T1W with contrast axial and coronal planes; images show a well-defined hypothalamic lesion with marked homogeneous contrast enhancement.

Course of the disease. Part 2

Figure 2. Post-treatment MRI: 1 – T2W/FLAIR, 2 - T1W with contrast images the same axial plane as Figure 1. Previously detected lesions are no longer recognizable.

Figure 3. MRI repeated after 1 year from the beginning of the disease. T1W with contrast coronal images: emerging multifocal parenchymal nodulus.

Patient's health status kept worsening. After 1 year MRI was

repeated and revealed no hypothalamus tumor but emerging

multifocal parenchymal changes (Figure 3).

DiscussionThe most remarkable finding of our case is the unusual location of lymphoma involving hypothalamus and

acute memory loss as clinical manifestation of the disease. Usually lymphoma is diagnosed by performing

clinical examination or blood tests. Radiological diagnosis of PCNSL is often difficult because imaging findings

may mimic other pathologies, although in this case MRI results led to the diagnosis of primary lymphoma.

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