Intravascular lymphoma forming massive aortic tumors complicated with sarcoidosis and focal segmental glomerulosclerosis: a case report and literature review

Abstract

BackgroundIntravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal diffuse large B-cell lymphoma characterized by proliferation of B cells within small vessels. Herein, we report a case of a 77-year-old man who presented with IVLBCL and massive tumor formation on the aortic wall who was previously diagnosed with sarcoidosis and focal segmental glomerulosclerosis (FSGS). To our knowledge, this is the first reported case of an IVLBCL with aortic tumor formation.Case presentationA 77-year-old ambulatory man with sarcoidosis and FSGS had neurological symptoms for nine months. The patient presented to the emergency department with sudden left leg pain, and was diagnosed with acute femoral artery occlusion. Emergency thrombectomy was performed subsequently. Pathological evaluation of the thrombi revealed that its surface was filled with large atypical B cells. Bone marrow biopsy showed infiltration of large atypical B cells within the small vessels. IVLBCL was suspected and further examination was planned, but the patient died due to sudden respiratory and cardiac arrest on hospital day twelve. Autopsy revealed intravascular tumors adherent to the aortic arch, left ventricle, and the abdominal aorta. All enlarged lymph nodes and the ventricular septum of the heart showed hyalinized lesions with granular formation consistent with sarcoidosis. The patient was diagnosed with IVLBCL with aortic tumor formation complicated with sarcoidosis and FSGS.ConclusionsIVLBCL may present with tumor formation on the aortic wall. Although the cause of its affinity to the aortic wall is yet unknown, autopsy findings imply that arteriosclerosis may have contributed to the tumor formation. The literature suggests that T-cell abnormalities could possibly be the common etiology of intravascular lymphoma, sarcoidosis, and FSGS.

Comprehensive treatment for primary right renal diffuse large B-cell lymphoma with a renal vein tumor thrombus: A case report

“…The current study highlights the importance of blood urea nitrogen and creatinine levels. The results of one study indicated that swollen retroperitoneal lymph node enlargement causes ureteral obstruction or adverse reactions to nephrotoxic chemotherapy drugs as their level increases[ 3 ]. An intermediate relationship between LDH and lymphoma has been reported in the literature, and high LDH indicates a high turnover rate of lymphoma cells and the release of degradation products into the blood (Figure 3 ).…”

A case of malignant lymphoma invading the aorta, which mimicked aortic dissection

“…In some reported cases involving adhesion of malignant lymphoma to the aorta, surgical treatment was required. These reports note that invasion of the aorta by lymphoma may cause aortic disease 2,[5][6][7][8] . Alexander et al and Matthew et al reported that ectopic lymphoma cells in the aortic wall may cause degeneration of the vessel wall and promote the development of aortic aneurysm 6,9) .…”

“…Alexander et al and Matthew et al reported that ectopic lymphoma cells in the aortic wall may cause degeneration of the vessel wall and promote the development of aortic aneurysm 6,9) . In addition, Oda et al reported that tumor cells may have affinity to arteriosclerotic lesions because they appeared not only in the aortic aneurysm but also in the arteriosclerotic parts without a visible mass 7) . Some other studies have reported that the malignant lymphoma sometimes causes aortic disorder.…”

“…Table 1 shows the aortic disorder, type of lymphoma, and treatment in each case. Six of these patients were treated by open surgery and seven were treated by endovascular surgery; none of the patients died due to the invesigations [2][3][4][5][6][7][8][9] . This may mean that both open surgery and endovascular procedures are both sufficiently effective and indicate that it is appropriate for surgeons to choose the approach on an individual basis.…”