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BMC Neurology
Published in: BMC Neurology 1/2023

Open Access 01-12-2023 | Case report

Lymphomatoid granulomatosis with the central nervous system involvement as the main manifestation: a case report

Authors: Dawei Chen, Jing Zhou, Weiwen Lu, Liuzhe Lu, Bo Chen, Wenzhong Li

Published in: BMC Neurology | Issue 1/2023

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Abstract

Background

Lymphomatoid granulomatosis (LyG) is a rare extralymphatic lymphoproliferative disease characterized by lymphocytic invasion into vascular walls and damage to blood vessels. The lungs are affected in 90% of LyG cases, followed by the skin, central nervous system (CNS), kidneys and liver.

Case presentation

Here we report a case of a young woman with LyG, with CNS involvement as the initial clinical manifestation. Computer tomography (CT) scans showed multiple nodular, patchy and flocculent high-density shadows in both lungs without mediastinal lymph node enlargement. Magnetic resonance imaging (MRI) scans showed multiple abnormal signal intensities in the right cerebellar hemisphere, frontal, parietal and temporal lobes, and dorsal brainstem, which became patchy and annular after enhancement. The post-operative pathological analysis of lesion samples confirmed the diagnosis of grade II LyG.

Conclusions

LyG should be concerned in young adults showing multiple radiological brain and lung lesions. Resection and postoperative medication of steroid hormones and IFN-α may be effective in the treatment of LyG.
Literature
1.
2.
Carbone A, Gloghini A, Dotti G. EBV-associated lymphoproliferative disorders: classification and treatment. Oncologist. 2008;13:577–85.CrossRefPubMed
3.
Sigamani E, Chandramohan J, Nair S, Chacko G, Thomas M, Mathew LG, et al. Lymphomatoid granulomatosis: a case series from South India. Indian J Pathol Microbiol. 2018;61:228–32.CrossRefPubMed
4.
5.
Moreno-Estébanez A, González-Pinto T, Agirre-Beitia G, González LM. Primary lymphomatoid granulomatosis of the central nervous system: a diagnostic challenge. Neurologia (Engl Ed). 2021;36:625–8.CrossRefPubMed
6.
Song JY, Pittaluga S, Dunleavy K, Grant N, White T, Jiang L, et al. Lymphomatoid granulomatosis–a single institute experience: pathologic findings and clinical correlations. Am J Surg Pathol. 2015;39:141–56.CrossRefPubMedPubMedCentral
7.
8.
Sabattini E, Bacci F, Sagramoso C, Pileri SA. WHO classification of tumours of haematopoietic and lymphoid tissues in 2008: an overview. Pathologica. 2010;102:83–7.PubMed
9.
Ishiura H, Morikawa M, Hamada M, Watanabe T, Kako S, Chiba S, et al. Lymphomatoid granulomatosis involving central nervous system successfully treated with rituximab alone. Arch Neurol. 2008;65:662–5.CrossRefPubMed
10.
Sheehy N, Bird B, O’Briain DS, Daly P, Wilson G. Synchronous regression and progression of pulmonary nodules on chest CT in untreated lymphomatoid granulomatosis. Clin Radiol. 2004;59:451–4.CrossRefPubMed
11.
Lee JS, Tuder R, Lynch DA. Lymphomatoid granulomatosis: radiologic features and pathologic correlations. AJR Am J Roentgenol. 2000;175:1335–9.CrossRefPubMed
12.
Pittaluga S, Wilson WH, Jaffe ES. WHO classification of tumors of Haematopietic and lymphoidtissues. 4th ed. Lyon Oxford: IARC; 2008.
13.
Patsalides AD, Atac G, Hedge U, Janik J, Grant N, Jaffe ES, et al. Lymphomatoid granulomatosis: abnormalities of the brain at MR imaging. Radiology. 2005;237:265–73.CrossRefPubMed
14.
Kiryu S, Okubo T, Takeuchi K, Inoue Y, Endo T, Odawara T, et al. Magnetic resonance imaging and diffusion tensor analysis of lymphomatoid granulomatosis of the brain. Acta Radiol. 2006;47:509–13.CrossRefPubMed
15.
Patil AK, Alexander M, Nair B, Chacko G, Mani S, Sudhakar S. Clinical, imaging and histopathological features of isolated CNS lymphomatoid granulomatosis. Indian J Radiol Imaging. 2015;25:56–9.CrossRefPubMedPubMedCentral
16.
He C, Wang Y, Zhang L, Lu C, Ge W, Zhang Q, et al. Isolated lymphomatoid granulomatosis of the central nervous system: a case report and literature review. Neuropathology. 2019;39:479–88.CrossRefPubMed
17.
Gaha M, Souillard-Scemama R, Miquel C, Godon-Hardy S, Naggara O, Meder JF. MR imaging of the brain and spinal cord in lymphomatoid granulomatosis: a case report and review of the literature. J Neuroradiol. 2013;40:364–7.CrossRefPubMed
18.
Lucantoni C, De Bonis P, Doglietto F, Esposito G, Larocca LM, Mangiola A, et al. Primary cerebral lymphomatoid granulomatosis: report of four cases and literature review. J Neurooncol. 2009;94:235–42.CrossRefPubMed
19.
Harting I, Hartmann M, Jost G, Sommer C, Ahmadi R, Heiland S, et al. Differentiating primary central nervous system lymphoma from glioma in humans using localised proton magnetic resonance spectroscopy. Neurosci Lett. 2003;342:163–6.CrossRefPubMed
20.
Schalk E, Krogel C, Scheinpflug K, Mohren M. Lymphomatoid granulomatosis in a patient with rheumatoid arthritis receiving methotrexate: successful treatment with the anti-CD20 antibody mabthera. Onkologie. 2009;32:440–1.CrossRefPubMed
21.
22.
Chavez JC, Sandoval-Sus J, Horna P, Dalia S, Bello C, Chevernick P, et al. Lymphomatoid granulomatosis: a single Institution experience and review of the literature. Clin Lymphoma Myeloma Leuk. 2016;16(Suppl):170–4.CrossRef
23.
Johnston A, Coyle L, Nevell D. Prolonged remission of refractory lymphomatoid granulomatosis after autologous hemopoietic stem cell transplantation with post-transplantation maintenance interferon. Leuk Lymphoma. 2006;47:323–8.CrossRefPubMed
24.
Siegloch K, Schmitz N, Wu HS, Friedrichs B, van Imhoff GW, Montoto S, et al. Hematopoietic stem cell transplantation in patients with lymphomatoid granulomatosis: a european group for blood and marrow transplantation report. Biol Blood Marrow Transplant. 2013;19:1522–5.CrossRefPubMed
25.
Katzenstein AL, Doxtader E, Narendra S. Lymphomatoid granulomatosis: insights gained over 4 decades. Am J Surg Pathol. 2010;34:e35–48.CrossRefPubMed
Metadata
Title
Lymphomatoid granulomatosis with the central nervous system involvement as the main manifestation: a case report
Authors
Dawei Chen
Jing Zhou
Weiwen Lu
Liuzhe Lu
Bo Chen
Wenzhong Li
Publication date
01-12-2023
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2023
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-023-03257-9

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