Autopsy and Case Reports
Autopsy and Case Reports
Clinical Case Report

Lymphoplasmacyte-rich meningioma with hematologic signs and PD-L1 over-expression

Gabriele Gaggero; Michela Campora; Davide Taietti; Giannamaria Cerruti; Enrico Lo Bue; Monica Truffelli; Marco Ceraudo; Pietro Fiaschi

Downloads: 1
Views: 764


Lymphoplasmacyte-rich meningioma (LPRM) is one of the rarest variants of grade I meningiomas. It can be clinically associated with prominent peripheral blood abnormalities, anemia, and/or various gammopathy, which usually disappear after surgical removal of the tumor. We document a case of right frontal LPRM in a 72-year-old male who presented general cognitive decadence. The patient suffered from mild anemia. The LPRM is a rare variant of meningioma, with only a few cases globally reported in the literature. It has been categorized as a grade I tumor in the 2021 World Health Organization (WHO) classification central nervous system. Due to the rarity, this meningioma variant origin and biological behavior are still not clear. Immunohistochemistry profile showed prominent PD-L1 expression, leading to additional interrogation on LPRM immunomorphological characteristics, the significance of the inflammatory tumoral microenvironment and its correlation with the immune-checkpoints.


Meningioma, PD-L1, Microenviroment


1 Ostrom QT, Cioffi G, Waite K, Kruchko C, Barnholtz-Sloan JS. CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2014-2018. Neuro-oncol. 2021;23(12, Suppl 2):iii1-105. PMid:34608945.

2 IARC Publications. Central Nervous System Tumours: WHO Classification of Tumours. 5th. Lyon: IARC Publications; 2021 [cited 2022 June 25]. Available from:

3 Rogers L, Barani I, Chamberlain M, et al. Meningiomas: knowledge base, treatment outcomes, and uncertainties. A RANO review. J Neurosurg. 2015;122(1):4-23. PMid:25343186.

4 Liu JL, Zhou JL, Ma YH, Dong C. An analysis of the magnetic resonance imaging and pathology of intracal lymphoplasmacyte-rich meningioma. Eur J Radiol. 2012;81(5):968-73. PMid:21354736.

5 Firdaus, M., Gill AS, Andriani R et al. A rare cystic lymphoplasmacyte-rich meningioma: a case report and review of the literature. Surg Neurol Int 2020;11:391.

6 Zhu HD, Xie Q, Gong Y, et al. Lymphoplasmacyte-rich meningioma: our experience with 19 cases and a systematic literature review. Int J Clin Exp Med. 2013;6(7):504-15. PMid:23936588.

7 Yongjun L, Xin L, Qiu S, Jun-Lin Z. Imaging findings and clinical features of intracal lymphoplasmacyte-rich meningioma. J Craniofac Surg. 2015;26(2):e132-7. PMid:25643346.

8 Lal A, Dahiya S, Gonzales M, et al. IgG4 overexpression is rare in meningiomas with a prominent inflammatory component: a review of 16 cases. Brain Pathol. 2014;24(4):352-9. PMid:24467316.

9 Tao X, Wang K, Dong J, et al. Clinical, radiologic, and pathologic features of 56 cases of intracranial lymphoplasmacyte-rich meningioma. World Neurosurg. 2017;106:152-64. PMid:28673887.

10 de Almeida GB, Januário G. Lymphoplasmacyte-rich meningioma: a rare histologic variant of benign meningioma with atypical bone invasion. Radiol Case Rep. 2022;17(3):922-7. PMid:35079313.

11 Wang H, He B, Wang Y, Chen H, Huang S, Xu J. Lymphoplasmacyte-rich meningioma in the central nervous system: an unusual case report. Medicine (Baltimore). 2021;100(52):e27991. PMid:34967348.

12 Li J, Zan X, Feng M, Deng X, Zhang S, Liu W. Case report: creeping growth in lymphoplasmacyte-rich meningioma—a radiologic variant. Front Surg. 2021;8:775560. PMid:34970592.

13 Kurmi DJ, Sharma A, Mittal RS, Singhvi S. Lymphoplasmacyte-rich meningioma with invasion of bone: a case report and review of literature. Asian J Neurosurg. 2016;11(4):448. PMid:27695559.

14 Nohara H, Furuya K, Kawahara N, et al. Lymphoplasmacyte-rich meningioma with atypical invasive nature. Neurol Med Chir (Tokyo). 2007;47(1):32-5. PMid:17245013.

15 Banerjee AK, Blackwood W. A subfrontal tumour with the features of plasmocytoma and meningioma. Acta Neuropathol. 1971;18(1):84-8. PMid:5579381.

16 Weidenheim KM, Campbell WG Jr, Goldman HW. Atypical monoclonal plasma cell hyperplasia of the central nervous system: precursor of plasmacytoma with evolutionary considerations. Neurosurgery. 1989;24(3):429-34. PMid:2927619.

17 Gregorios JB, Bay JW, Dudley AW Jr. Extramedullary hematopoiesis in a malignant meningioma. Neurosurgery. 1983;13(4):447-51. PMid:6633840.

18 Bruno MC, Ginguené C, Santangelo M, et al. Lymphoplasmacyte rich meningioma: a case report and review of the literature. J Neurosurg Sci. 2004;48(3):117-24, discussion 124. PMid:15557881.

19 Louis DN, Perry A, Wesseling P, et al. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro-oncol. 2021;23(8):1231-51. PMid:34185076.

20 Gi H, Nagao S, Yoshizumi H, et al. Meningioma with hypergammaglobulinemia. Case report. J Neurosurg. 1990;73(4):628-9. PMid:2398394.

21 Kepes JJ, Chen WY, Connors MH, Vogel FS. “Chordoid” meningeal tumors in young individuals with peritumoral lymphoplasmacellular infiltrates causing systemic manifestations of the castleman syndrome. A report of seven cases. Cancer. 1988;62(2):391-406.<391::AID-CNCR2820620226>3.0.CO;2-7. PMid:3383139.

22 Stam FC, van Alphen HAM, Boorsma DM. Meningioma with conspicuous plasma cell components. A histopathological and immunohistochemical study. Acta Neuropathol. 1980;49(3):241-3. PMid:6989148.

23 Johnson MD. PD-L1 expression in meningiomas. J Clin Neurosci. 2018;57:149-51. PMid:30153998.

24 Han SJ, Reis G, Kohanbash G, et al. Expression and prognostic impact of immune modulatory molecule PD-L1 in meningioma. J Neurooncol. 2016;130(3):543-52. PMid:27624915.

25 Mair MJ, Pajenda S, Ilhan-Mutlu A, et al. Soluble PD-L1 is associated with local and systemic inflammation markers in primary and secondary brain tumours. ESMO Open. 2020;5(6):e000863. PMid:33184096.

26 Du Z, Abedalthagafi M, Aizer AA, et al. Increased expression of the immune modulatory molecule PD-L1 (CD274) in anaplastic meningioma. Oncotarget. 2015;6(7):4704-16. PMid:25609200.

27 Karimi S, Mansouri S, Mamatjan Y, et al. Programmed death ligand-1 (PD-L1) expression in meningioma; prognostic significance and its association with hypoxia and NFKB2 expression. Sci Rep 2020;10:14115.

28 Li YD, Veliceasa D, Lamano JB, et al. Systemic and local immunosuppression in patients with high-grade meningiomas. Cancer Immunol Immunother. 2019;68(6):999-1009. PMid:31030234.

29 Karimi S, Mansouri S, Nassiri F, et al. Clinical significance of checkpoint regulator “Programmed death ligand-1 (PD-L1)” expression in meningioma: review of the current status. J Neurooncology. 2021;151:443-9.

30 Tsukamoto M, Imai K, Ishimoto T, et al. PD-L1 expression enhancement by infiltrating macrophage-derived tumor necrosis factor-α leads to poor pancreatic cancer prognosis. Cancer Sci. 2019;110(1):310-20. PMid:30426611.

31 Ding Y, Qiu L, Xu Q, Song L, Yang S, Yang T. Relationships between tumor microenvironment and clinicopathological parameters in meningioma. Int J Clin Exp Pathol. 2014;7(10):6973-9. PMID: 25400783.

32 van Kempen LCLT, Ruiter DJ, van Muijen GNP, Coussens LM. The tumor microenvironment: a critical determinant of neoplastic evolution. Eur J Cell Biol. 2003;82(11):539-48. PMid:14703010.

33 Yu P, Rowley DA, Fu YX, Schreiber H. The role of stroma in immune recognition and destruction of well-established solid tumors. Curr Opin Immunol. 2006;18(2):226-31. PMid:16459066.

34 Ladányi A. Prognostic and predictive significance of immune cells infiltrating cutaneous melanoma. Pigment Cell Melanoma Res. 2015;28(5):490-500. PMid:25818762.

35 Radvanyi LG, Bernatchez C, Zhang M, et al. Specific lymphocyte subsets predict response to adoptive cell therapy using expanded autologous tumor-infiltrating lymphocytes in metastatic melanoma patients. Clin Cancer Res. 2012;18(24):6758-70. PMid:23032743.

36 Liao Y, Ou J, Deng J, et al. Clinical implications of the tumor-infiltrating lymphocyte subsets in colorectal cancer. Med Oncol. 2013;30(4):727. PMid:24026663.

37 Gooden MJM, de Bock GH, Leffers N, Daemen T, Nijman HW. The prognostic influence of tumour-infiltrating lymphocytes in cancer: a systematic review with meta-analysis. Br J Cancer. 2011;105(1):93-103. PMid:21629244.

38 Kryczek I, Wei S, Zhu G, et al. Relationship between B7-H4, regulatory T cells, and patient outcome in human ovarian carcinoma. Cancer Res. 2007;67(18):8900-5. PMid:17875732.

39 Woo EY, Yeh H, Chu CS, et al. Cutting edge: regulatory T cells from lung cancer patients directly inhibit autologous T cell proliferation. J Immunol. 2002;168(9):4272-6. PMid:11970966.

40 Zou W. Regulatory T cells, tumour immunity and immunotherapy. Nat Rev Immunol. 2006;6(4):295-307. PMid:16557261.

41 Nakano O, Sato M, Naito Y, et al. Proliferative activity of Intratumoral CD8 T-Lymphocytes as a prognostic factor in human renal cell carcinoma: clinicopathologic demonstration of Antitumor Immunity 1. Cancer Res. 2001;61(13):5132-6. PMid:11431351.

42 Nelson BH. CD20+ B cells: the other tumor-infiltrating lymphocytes. J Immunol. 2010;185(9):4977-82. PMid:20962266.

43 Nielsen JS, Nelson BH. Tumor-infiltrating B cells and T cells: working together to promote patient survival. OncoImmunology. 2012;1(9):1623-5. PMid:23264915.

44 Sharma P, Shen Y, Wen S, et al. CD8 tumor-infiltrating lymphocytes are predictive of survival in muscle-invasive urothelial carcinoma. Proc Natl Acad Sci USA. 2007;104(10):3967-72. PMid:17360461.

45 Sato E, Olson SH, Ahn J, et al. Intraepithelial CD8+ tumor-infiltrating lymphocytes and a high CD8+/regulatory T cell ratio are associated with favorable prognosis in ovarian cancer. Proc Natl Acad Sci USA. 2005;102(51):18538-43. PMid:16344461.

46 Liao Y, Ou J, Deng J, et al. Clinical implications of the tumor-infiltrating lymphocyte subsets in colorectal cancer. Med Oncol. 2013;30(4):727. PMid:24026663.

47 Hanahan D, Weinberg RA. Hallmarks of cancer: the next generation. Cell. 2011;144(5):646-74. PMid:21376230.

Submitted date:

Accepted date:

Publication date:

62f161f7a95395188a4e1f53 autopsy Articles
Links & Downloads

Autops Case Rep

Share this page
Page Sections