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Meningeal tumor pathology

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Presentation on theme: "Meningeal tumor pathology"— Presentation transcript:

1 Meningeal tumor pathology
Define the role of mitotic activity in meningioma grading Discuss the effect of preoperative embolization on meningioma grading Name and grade the aggressive meningioma variants Why are CNS melanocytic tumors and supratentorial hemangioblastomas no longer classified as meningiomas? Compare the behavior of anaplastic meningiomas, hemangiopericytomas, and solitary fibrous tumors

2 “Meningioma cells recapitulate their lepidic function”

3

4 Epithelial Membrane Antigen (EMA)

5 Meningioma: Behavior ~90% are WHO Grade I
Excellent 5-year survival 20% recur within 10 years (GTR) ~10% are “Atypical” (WHO Grade II) 5-year recurrence = 40%, mortality = 20% ~2% are “Anaplastic” (WHO Grade III ) median survival = 1.5 years

6 Meningioma: WHO I Variants
Traditional Meningothelial, fibrous, transitional, psammomatous, angiomatous Deceptive Microcystic, metaplastic, secretory Questionable Lymphoplasmacyte-rich

7 Microcystic meningioma (FS: “Glioma”)

8 Metaplastic meningiomas

9 Secretory meningioma – may cause marked cerebral edema

10 Meningioma: Grading Atypical (WHO Grade II) Anaplastic (WHO Grade III)
High mitotic index (>4/10hpfs) or 3 of 5: Architectural loss, hypercellularity, macronucleoli, small cell differentiation, necrosis or Brain invasion Anaplastic (WHO Grade III) Excessive mitotic activity (>20/10hpfs) or Loss of meningothelial differentiation (“sarcomatous, carcinomatous, or melanomatous differentiation”)

11 Atypical meningioma (WHO II)
Hypercellularity Architectural loss (“sheeting”) N E C R O S I Small cell differentiation

12 Caveat emptor

13 WHO II – Brain Invasion

14 Not Brain Invasion

15 Meningioma: Grading Atypical (WHO Grade II) Anaplastic (WHO Grade III)
High mitotic index (>4/10hpfs) or 3 of 5: Architectural loss, hypercellularity, macronucleoli, small cell differentiation, necrosis or Brain invasion Anaplastic (WHO Grade III) Excessive mitotic activity (>20/10hpfs) or Loss of meningothelial differentiation (“sarcomatous, carcinomatous, or melanomatous differentiation”)

16 Anaplastic menigioma

17 Aggressive meningioma variants
WHO Grade II Chordoid meningioma Clear cell meningioma WHO Grade III Papillary meningioma Rhabdoid meningioma * The aggressive pattern should comprise >50% of the tumor volume

18 Chordoid meningioma

19 Clear cell menigioma

20 Clear cell v microcystic menigioma

21 Mengiomas with clear cells
meningioma Microcystic Age Predilection Young adults Older Adults Typical location CP angle Spinal Convexity Histo- chemistry Intracellular glycogen Extracellular glycoprotein Behavior Aggressive Benign

22 Papillary meningioma

23 Rhabdoid meningioma

24 Ex-meningiomas Melanocytic meningioma Angioblastic meningioma
Now Meningeal Melanocytoma Angioblastic meningioma Now Hemangioblastoma Hemangiopericytic meningioma Now Hemangiopericytoma, and maybe Solitary Fibrous Tumor

25 Meningeal melanocytoma

26 Immunocytochemistry Meningiomas EMA - positive S100 – positive in 10%
HMB-45 - negative Melanocytomas EMA – negative S100 – positive HMB-45 – positive

27 Case History 25 year-old man with two months of headache following automobile accident, now with double vision Family and personal history of “moles and skin lesions” Left frontal lobe mass

28

29

30 Brat, DJ et al. Am J Surg Path 23: 745, 1999
CNS Melanocytic Tumors Melanocytoma Melanocytic tumor, ID Primary melanoma Metatstatic melanoma Tight nests >50% No Invasion Yes Epithelioid cells Rare Most Nucleoli Indistinct Prominent Mitoses 0-1/10hpf 1-3/10hpf 2-15/ 10hpf 7-35/ 10hpf Necrosis ~20% ~90% Brat, DJ et al. Am J Surg Path 23: 745, 1999

31 17-73 Mean = 51 53,67,68 15-71 Mean=43 2/3 spinal 1/3 ST, 1PF
CNS Melanocytic Tumors Melanocytoma Melanotic tumor, ID Primary Malignant melanoma Age 17-73 Mean = 51 53,67,68 15-71 Mean=43 Location 2/3 spinal 1/3 ST, 1PF PF,conus, pineal ~equal dist: Spinal,PF,ST Recurrence None Poor f/u All STR 1/5 GTR* * 1 to 3 year follow-up

32 33 years later…

33

34 Cerebellar hemangioblastoma

35 Cerebellar & ST hemangioblastoma FS (“Glioma”)

36 Mengiomas with clear cells
meningioma Microcystic Hemangio- blastoma Age Predilection Young adults Older Adults Depends on VHL status Typical location CP angle Spinal Convexity Cerebellum Histo- chemistry Intracellular glycogen Extracellular glycoprotein lipid Immunohisto- EMA + Inhibin -- EMA – Inhibin + Behavior Aggressive Benign

37 Hemangioblastoma: Anti-inhibin IHC

38 Meningeal hemangiopericytoma

39 Solitary fibrous tumor
Anti-CD34 Anti-bcl2

40 HPC SFT Hyalinization Rare, Focal Diffuse Mitoses Common Rare EMA Negative CD34 Weak Strong Bcl-2 Recurrence (GTR) 80% None Metastasis 30% 5yr survival 70% 100%

41 Meningeal tumor pathology
Define the role of mitotic activity in meningioma grading Discuss the effect of preoperative embolization on meningioma grading Name and grade the aggressive meningioma variants Why are CNS melanocytic tumors and supratentorial hemangioblastomas no longer classified as meningiomas? Compare the behavior of anaplastic meningiomas, hemangiopericytomas, and solitary fibrous tumors

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