Tumoral presentation of primary central nervous system lymphomatoid granulomatosis

Abstract

Making a preoperative diagnosis of CNS-LYG appearing initially as a tumoral mass is difficult because of the lack of pathognomonic clinical symptoms or imaging signs. Surgical management with radical resection of the mass is almost always followed by the long-term local control of the lesion, although the disease may have a disseminated, systemic or malignant evolution.

Primary lymphomatoid granulomatosis in the central nervous system: A report of three cases

“…23 LYG frequently occurs in immunosuppressive patients following organ transplantation or chemotherapy for hematolymphoid malignancies; however, it can also be seen in immunocompetent patients such as ours. 11 These EBV antigens result in an abnormal interaction of T-and B-cells and dysregulation of tumor suppressor genes and activation of oncogenes that ultimately lead to neoplastic transformation. 11 These EBV antigens result in an abnormal interaction of T-and B-cells and dysregulation of tumor suppressor genes and activation of oncogenes that ultimately lead to neoplastic transformation.…”

Fatal lymphomatoid granulomatosis with primary CNS-involvement in an immunocompetent 80-year-old woman

“…The pathogenesis of LYG remains elusive, but a defect in T-cell activity, allowing EBV-infected B cells to express EBV-encoded proteins, which results in disturbance of the interaction between B cells and T cells and consecutive lymphoproliferation, has been hypothesised 4. Thus, EBV plays a pivotal role in LYG pathogenesis, placing LYG in the group of EBV-related disorders along with Burkitt's and Hodgkin’s lymphoma, nasopharyngeal carcinoma, some diffuse large-cell B-cell lymphomas and others 15.…”

“…The lungs are usually affected at diagnosis of LYG, but involvement of the kidneys, the central and peripheral nervous system, and the skin, along with constitutional symptoms, are also reported 1. While LYG is mainly seen in immunocompromised patients suffering from AIDS, cases of solid-organ transplantation and in others,2 3 there are reports on LYG in immunocompetent persons as well 4 5. Presentation of LYG with initial CNS symptoms is rare.…”

Primary Cerebral Lymphomatoid Granulomatosis Progressing to Methotrexate-associated Lymphoproliferative Disease Under Immunosuppressive Therapy

“…In addition, a retrospective study of rituximab-containing chemotherapy for high-grade LYG documented a progressionfree survival of 40% at a median follow-up of 28 months (6). HIV-negative patients with primary cerebral LYG die approximately one year after receiving a diagnosis (9, 10), while patients with completely resectable tumoral cerebral lesions may survive for > three years (11). Recently, various reports have described patients with EBVnegative primary cerebral LYG exhibiting a favorable prognosis with corticosteroid therapy alone (19)(20)(21).…”

“…The presence of a uniform population of large atypical EBV-positive B-cells without a polymorphous background is beyond the spectrum of LYG and should be classified as diffuse large B-cell lymphoma (4). Although CNS lesions are found in 20-50% of LYG patients, usually proximal to sites of pulmonary LYG (5,8), there are fewer reports of primary cerebral LYG versus primary pulmonary LYG (9)(10)(11)(12)(13).…”