J Pathol Transl Med.  2017 May;51(3):284-291. 10.4132/jptm.2017.02.16.

A Small Case Series of Intravascular Large B-Cell Lymphoma with Unexpected Findings: Subset of Cases with Concomitant Extravascular Central Nervous System (CNS) Involvement Mimicking Primary CNS Lymphoma

• Poropatich K
• Dittmann D
• Chen YH
• Raparia K
• Wolniak K
• Gao J

• KMID: 2392595
• DOI: http://doi.org/10.4132/jptm.2017.02.16

Abstract

BACKGROUND
Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal lymphoma with growth mainly in the lumina of vessels. We studied a small series of IVLBCL and focused on its central nervous system (CNS) involvement.
METHODS
Searching the medical records of Northwestern Memorial Hospital, we identified five cases of IVLBCL from January 2007 to January 2015. Clinical information, hematoxylin and eosin stained histologic slides and immunohistochemistry studies were reviewed for all cases. Polymerase chain reaction (PCR) analysis for the immunoglobulin (Ig) heavy and light chain gene rearrangement was performed on all five cases.
RESULTS
Three of the five cases of IVLBCL were autopsies. Patients' age ranged from 56 to 84. CNS involvement was present in two cases"”in both patients, the CNS involvement showed an extravascular pattern with confluent sheet-like formation. PCR analysis confirmed that in one case the systemic intravascular and CNS extravascular components were clonally identical.
CONCLUSIONS
In a small case series of IVLBCL, we observed that CNS involvement by IVLBCL often has an extravascular morphology, but is clonally identical to the intravascular counterpart by PCR analysis. As IVLBCL can have a rapidly progressing poor outcome, it should be kept in the differential diagnoses for patients presenting with lymphoma of the CNS. The presence of extravascular growth patterns in the CNS should not exclude IVLBCL as a diagnosis.

Keyword

Intravascular large B-cell lymphoma

MeSH Terms

Autopsy
B-Lymphocytes*
Central Nervous System*
Diagnosis
Diagnosis, Differential
Eosine Yellowish-(YS)
Gene Rearrangement
Hematoxylin
Humans
Immunoglobulins
Immunohistochemistry
Lymphoma*
Lymphoma, B-Cell*
Medical Records
Polymerase Chain Reaction
Eosine Yellowish-(YS)
Hematoxylin
Immunoglobulins

Figure

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  Fig. 1. An 81-year-old male presented with rapidly progressive respiratory failure (case 1). (A) Lung parenchyma showed extensive vascular distension and intravascular collection of large lymphoid cells. (B) A CD20 immunohistochemical stain confirmed many large B cells filling the lumina of the vessels.

• 

  Fig. 2. A 76-year-old female presented with altered mental status and rapid neurologic decompensation (case 2). (A) Histology of the pituitary glands shows perivascular lymphoid infiltrates and areas of sheet-like architecture. (B) Perivascular lymphoid infiltrate cuffing the capillary vessels in the pons. (C) Higher magnification of the pituitary glands reveals sheet-like architecture and parenchymal effacement by abnormal large lymphoid cells with vesicular chromatin and prominent nucleoli. (D) The large lymphoma cells in the intravascular spaces of the lung interstitium from the same patient.

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  Fig. 3. A 61-year-old female with progressive skin rash and a diagnosis of intravascular large B-cell lymphoma who subsequently developed neurologic symptoms (case 4). (A) Brain biopsy (left frontal cortex) reveals lymphoid infiltrates in predominantly extravascular pattern. (B) CD20 highlights large B cells in the perivascular infiltrates. (C) Brain biopsy reveals sheets of large cells with vesicular chromatin and prominent nucleoli. (D) Diagnostic skin biopsy reveals large lymphoma cells filling the lumina of capillary vessels in the dermis in the same patient.

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  Fig. 4. Immunoglobulin heavy and light chain gene rearrangement performed on tumor cells with two distinct morphologic patterns (case 2). (A, B) Both the lung with intravascular pattern and central nervous system (CNS) pituitary with extravascular pattern show clonal heavy chain rearrangement with the same 254-bp clonal FR2 peak. (C, D) Both the lung with intravascular pattern and CNS meninges with extravascular pattern show clonal kappa light chain rearrangement with the same 149-bp and 201-bp clonal peaks.

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