Abstract
Primary central nervous system lymphomas (PCNLs) and histiocytic tumors are neoplasms that correspond to their nodal or systemic counterparts but lack an apparent systemic manifestation at presentation. The most common “typical” types include diffuse large B cell lymphoma and Langerhans cell histiocytosis, respectively, being the many other subtypes considerably rarer in the CNS. Given the usually deep-seated nature of these tumors and the fact that PCNSL is not considered a surgical disease, surgery is often restricted to stereotactic biopsy. Because misdiagnosis of lymphoma is one of the most common errors in frozen section evaluation, smears are recommended for the intraoperative procedure. In this chapter, the cytomorphologic features and differential diagnosis of lymphomas and histiocytic tumors arising in the CNS are discussed.
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Batchelor T, Loeffler JS. Primary CNS lymphoma. J Clin Oncol. 2006;24:1281–8.
Bubolz AM, Weissinger SE, Stenzinger A, Arndt A, et al. Potential clinical implications of BRAF mutations in histiocytic proliferations. Oncotarget. 2014;5:4060–70.
Chen KTK. Crush cytology of Rosai-Dorfman disease of the central nervous system. A report of 2 cases. Acta Cytol. 2003;47:1111–5.
Demellawy DE, Young JL, de Nanassy J, Chernetsova E, Nasr A. Langerhans cell histiocytosis: a comprehensive review. Pathology. 2015;47:294–301.
Dubuisson A, Kaschten B, Lénelle J, Martin D, et al. Primary central nervous system lymphoma: report of 32 cases and review of the literature. Clin Neurol Neurosurg. 2004;107:55–6.
Estupiñán-Díaz B, Salazar-Rodríguez S, Jiménez-Galainena J, García-Maeso N, et al. Primary histiocytic sarcoma of the central nervous system. A case report and review of the literature. Rev Esp Patol. 2015;48:222–30.
Giannini C, Dogan A, Salomão DR. CNS lymphoma: a practical diagnostic approach. J Neuropathol Exp Neurol. 2014;73:478–94.
Haroche J, Charlotte F, Arnaud L, von Deimling A, et al. High prevalence of BRAF V600E mutations in Erdheim-Chester disease but not in other non-Langerhans cell histiocytoses. Blood. 2012;120:2700–3.
Hung YP, Lovitch SB, Qian X. Histiocytic sarcoma: new insights into FNA cytomorphology and molecular characteristics. Cancer Cytopathol. 2017;125(8):604–14.
Idbaih A, Mokhtari K, Emile JF, et al. Dramatic response of a BRAF V600E-mutated primary CNS histiocytic sarcoma to vemurafenib. Neurology. 2014;83:1478–80.
Nahm JH, Yoon G, Do SI, Kim HS. Squash smear cytology of Langerhans cell histiocytosis. Int J Clin Exp Pathol. 2015;8:7998–8007.
Namiki TS, Nichols P, Young T, Martin SE, Chandrasoma P. Stereotaxic biopsy diagnosis of central nervous system lymphoma. Am J Clin Path. 1988;90:40–5.
Plasswilm L, Herrlinger U, Korfel A, Weller M, et al. Primary central nervous system (CNS) lymphoma in immunocompetent patients. Ann Hematol. 2002;81:415–23.
Purav P, Ganapathy K, Mallikarjuna VS, Balamuguram M. Rosai-Dorfman disease of the central nervous system. J Clin Neurosci. 2005;12:656–9.
Rodríguez-Pereira C, Borrás-Moreno JM, Pesudo-Martínez JV, Vera-Román JM. Cerebral solitary Langerhans cell histiocytosis: report of two cases and review of the literature. Br J Neurosurg. 2005;19:192–7.
Rushing EJ, Kaplan KJ, Mena H, Sandberg GD, Koeller K, Bouffard JP. Erdheim-Chester disease of the brain: cytological features and differential diagnosis of a challenging case. Diagn Cytopathol. 2004;31:420–2.
Scott BJ, Douglas VC, Tihan T, Rubenstein JL, Josephson SA. A systematic approach to the diagnosis of suspected central nervous system lymphoma. JAMA Neurol. 2013;70:311–9.
Sherman ME, Erozan YS, Mann RB, Kumar AJ, et al. Stereotactic brain biopsy in the diagnosis of malignant lymphoma. Am J Clin Pathol. 1991;95:878–83.
Sun W, Nordberg ML, Fowler MR. Histiocytic sarcoma involving the central nervous system: clinical, immunohistochemical, and molecular genetic studies of a case with review of the literature. Am J Surg Pathol. 2003;27:258–65.
Tian Y, Wang J, Li M, Lin S, et al. Rosai-Dorfman disease involving the central nervous system: seven cases from one institute. Acta Neurochir. 2015;157:1565–71.
Tu PH, Giannini C, Judkins AR, Schwalb JM, et al. Clinicopathologic and genetic profile of intracranial marginal zone lymphoma: a primary low-grade CNS lymphoma that mimics meningioma. J Clin Oncol. 2005;23:5718–27.
Weidauer S, von Stuckrad-Barre S, Dettmann E, Zanella FE, Lanfermann H. Cerebral Erdheim-Chester disease: case report and review of the literature. Neuroradiology. 2003;45:241–5.
Yu GH, Montone KT, Frias-Hidvegi D, Cajulis RS, Brody BA, Levy RM. Cytomorphology of primary CNS lymphoma: review of 23 cases and evidence for the role of EBV. Diagn Cytopathol. 1996;14:114–20.
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Lacruz, C.R., Saénz de Santamaría, J., Bardales, R.H. (2018). Lymphomas and Histiocytic Tumors. In: Central Nervous System Intraoperative Cytopathology. Essentials in Cytopathology, vol 13. Springer, Cham. https://doi.org/10.1007/978-3-319-98491-9_16
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DOI: https://doi.org/10.1007/978-3-319-98491-9_16
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