Central Nervous System Tumors Pathology Study Guide

Central Nervous System Tumors Pathology Video

CNS Tumors

Central nervous system (CNS) tumors are abnormal growths that involve the central nervous system.

Types of central nervous system (CNS) tumors include:

Glioblastoma multiforme (GBM)
Meningioma
Schwannoma
Oligodendroglioma
Pilocytic astrocytoma
Medulloblastoma
Ependymoma
Craniopharyngioma

Approximately 50 percent of central nervous system (CNS) tumors are metastatic.

Approximately 50 percent of central nervous system (CNS) tumors are primary.

Metastatic tumors to the central nervous system (CNS) typically appear at the gray-white matter junction as a number of distinct lesions.

Common sources of metastasis to the include the kidney, breast, and lung.

Primary central nervous system (CNS) tumors are categorized based on the cell type that has given rise to them which includes:

Astrocytes
Meningothelial cells
Ependymal cells
Oligodendrocytes
Neuroectoderm

In adults, primary central nervous system (CNS) tumors are typically supratentorial.

In children, primary central nervous system (CNS) tumors are usually infratentorial.

The three most prevalent central nervous system (CNS) tumors in children are:

Pilocytic astrocytoma
Ependymoma
Medulloblastoma

Primary malignant central nervous system (CNS) tumors rarely metastasize.

Primary malignant central nervous system (CNS) tumors are locally devastating.

Expression of CD150 in human central nervous system (CNS) tissues.(A) Immunohistochemical analysis of CD150 expression in human primary CNS tumors. Tumor paraffin sections of glioblastoma, anaplastic astrocytoma, diffuse astrocytoma, anaplastic ependymoma and anaplastic oligodendroglioma were stained with antibodies to CD150 (upper panel), glial fibrillary acidic protein, GFAP, astrocyte marker (middle panel) and nestin, neural stem/progenitor cell marker (lower panel). CD150 expression was detected in all presented histological variants of CNS tumors (upper panel). (B) Expression of CD150 in different regions of human normal brain tissues: cerebellum, thalamus, subventricular zone ependyma. The expression of CD150 was not found in human normal brain tissues. DAB staining shows specific reactions in brown. Cell nuclei are lightly counterstained with haematoxylin. Microscopic magnification of 400× was used for all images.Expression of CD150 in tumors of the central nervous system: identification of a novel isoform. Romanets-Korbut O, Najakshin AM, Yurchenko M, Malysheva TA, Kovalevska L, Shlapatska LM, Zozulya YA, Taranin AV, Horvat B, Sidorenko SP - PloS one (2015). Not Altered. CC.
LIN28A immunohistochemistry in pediatric malignant CNS tumors. Microscopic appearance of ETMR composed of clusters of multilayered rosettes embedded in abundant neuropil (initially diagnosed as ETANTR) (a). Intense LIN28A expression in poorly differentiated areas containing rosettes and the absence of expression in neuropil of tumor (b). Microscopic appearance of ETMR composed of numerous rosettes with clusters of small poorly differentiated cells (initially diagnosed as ependymoblastoma) (c). Strong and diffuse LIN28 expression in all parts of the tumor (d). AT/RT. Focal LIN28A expression in single collections of tumor cells (e). Anaplastic ependymoma. Immunonegativity for LIN28A in tumor region with numerous ependymal rosettes (f). LIN28A immunoreactivity is a potent diagnostic marker of embryonal tumor with multilayered rosettes (ETMR). Korshunov A, Ryzhova M, Jones DT, Northcott PA, van Sluis P, Volckmann R, Koster J, Versteeg R, Cowdrey C, Perry A, Picard D, Rosenblum M, Giangaspero F, Aronica E, Schüller U, Hasselblatt M, Collins VP, von Deimling A, Lichter P, Huang A, Pfister SM, Kool M - Acta neuropathologica (2012). Not Altered. CC.

Glioblastoma Multiforme (GBM)

Glioblastoma multiforme (GBM) is a malignant, high-grade tumor of astrocytes.

Glioblastoma multiforme (GBM) is the most common primary malignant central nervous system (CNS) tumor in adults.

The cerebral hemisphere is where the “butterfly” lesion associated with glioblastoma multiforme (GBM), which often spans the corpus callosum, develops.

Histologically, glioblastoma multiforme (GBM) tumor cells are glial fibrillary acidic protein (GFAP) positive and are characterized by areas of necrosis surrounded by proliferating endothelial cells.

Glioblastoma multiforme (GBM) has a poor prognosis.

Glioblastoma Multiforme. Lower magnification histopathology, showing necrosis surrounded by pseudopalisades of tumor cells, conferring a diagnosis of glioblastoma rather than anaplastic astrocytoma. No machine-readable author provided. KGH assumed (based on copyright claims). - Not altered. CC BY-SA 3.0
Glioblastoma Multiforme. Gliobastoma (astrocytoma) WHO grade IV - MRI coronal view, post contrast. 15 year old boNot altered. CC BY 2.5
Glioblastoma Multiforme. Axial post-contrast T1 (top) and T2 (bottom) weighted MRI showing an IDH1 mutant frontal lobe glioblastoma with sparse enhancement despite large size. J Ppoozler - Not altered. CC BY-SA 4.0
Glioblastoma Multiforme. GBM in the frontal right lobe as seen on CT scan James Heilman, MD - Not altered. CC BY-SA 4.0
Glioblastoma Multiforme. Histopathology of glioblastoma, showing high grade astrocytoma features of marked nuclear pleomorphism, multiple mitoses (one at white arrow) and multinucleated cells (one at black arrow), with cells having a patternless arrangement in a pink fibrillary background on H&E stain. Mikael Häggström, M.D. Not altered.CC0

Meningioma

Meningiomas are benign tumors of arachnoid cells.

Meningiomas are the most common benign central nervous system (CNS) tumor in adults.

Meningiomas are more common in females than males.

Meningiomas may cause seizures due to the tumor compressing the cortex without invading it.

Radiology of meningiomas typically show a spherical mass linked to the dura is seen through imaging.

The histology of meningiomas is characterized by a whorled pattern with psammoma bodies.

Meningioma. MRI image of a meningioma with contrast James Heilman, MD - Not altered. CC BY-SA 4.0
Meningioma. A meningioma that previously had been operated on, with surrounding edema James Heilman, MD - Not altered. CC BY-SA 3.0
Meningioma. A contrast enhanced CT scan of the brain, demonstrating the appearance of a Meningioma. Glitzy - Not altered. Public Domain
Meningioma. Micrograph of a meningioma with brain invasion (WHO Grade II). The tumor (bottom/right of image) has the typical "pushing border" invasion into the cerebral cortex (top/left of image), HPS stain. Nephron - Not altered. CC BY-SA 3.0
Meningioma. Meningioma seen at autopsy, as a tumor of the dura mater extending into the cranial cavity, and can be detached from the bone along with the rest of the dura mater Mikael Häggström, M.D. not altered. CC0
Meningioma. Micrograph of a meningioma showing the characteristic whorling, HPS stain Nephron. Not altered. CC BY-SA 3.0
a) Histopathologic feature with H and E, ×400 of fibroblastic meningioma; (b) Histopathologic feature with H and E, ×40 of transitional meningioma; (c) Histopathologic feature with H and E, ×400 of meningotheliomatous meningioma. Multiple meningiomas consisting of fibrous meningioma, transitional meningioma, and meningotheliomatous meningioma in one adult patient. Yudoyono F, Sidabutar R, Arifin MZ, Faried A - Asian journal of neurosurgery (2015 Oct-Dec). Not Altered. CC.

Schwannoma

Schwannomas are benign tumors of Schwann cells.

Schwannomas may involve spinal or cranial nerves.

In the head, the vestibular cochlear (VIII) nerve at the cerebellopontine angle is most usually involved by schwannomas.

The histology of schwannomas is characterized by spindle cells that are S-100 stain positive.

Bilateral schwannomas are often seen in patients with neurofibromatosis type II (NF2).

Schwannoma. Schwannoma with Antoni A and Antoni B areas. H&E stain. Original article authors: Hodges, Tiffany R.; Karikari, Isaac O.; Nimjee, Shahid M.; Tibaleka, June; Cummings, Thomas J.; Radhakrishnan, Senthil; Friedman, Allan H. Annotated by: Mikael Häggström, M.D. Not altered, CC BY 3.0
(A) Preoperative pure tone audiogram revealed moderate sensorineural hearing loss on the right and severe mixed hearing loss on the left. (B) Postoperative aided hearing threshold. Right ear was aided by hearing aid, and left ear was aided by CI. (C) The right side was aided by hearing aid and the left side by cochlear implant. Low signal on T2 and high signal enhanced T1 (D) indicate that the tumor (arrow) is a schwannoma. (E) The tumor (arrow) was totally removed via a translabyrinthine approach. The cochlear nerve was anatomically preserved during the operation. (F) Cochlear implantation was performed simultaneously. The internal device electrode was inserted through a posterior tympanotomy site (arrow). To prevent cerebrospinal fluid leakage, the tympanotomy site was plugged with a small piece of temporalis muscle after implantation. Simultaneous Translabyrinthine Tumor Removal and Cochlear Implantation in Vestibular Schwannoma Patients. Yonsei Medical Journal. Not Altered. CC.
Schwannoma. Histopathologic image of schwannoma of the subcutis, representing an Antoni B type. The same case as shown in a file "Subcutaneous_schwannoma_(1)_Antoni_B.jpg". S-100 immunostain. No machine-readable author provided. KGH assumed (based on copyright claims). - No machine-readable source provided. Not altered. CC BY-SA 3.0
Schwannoma. Histopathologic image of schwannoma of the subcutis, representing an Antoni B type. KGH assumed (based on copyright claims). Not altered. CC BY-SA 3.0
Schwannoma. Histopathologic image of schwannoma of the subcutis, representing an Antoni B type. KGH assumed (based on copyright claims). - Not altered. CC BY-SA 3.0
Schwannoma. Subcutaneous schwannoma No machine-readable author provided. KGH assumed (based on copyright claims). Not altered. CC BY-SA 3.0
Schwannoma. Intermediate magnification micrograph of a schwannoma. HPS stain. A cellular Antoni A area is seen on the right of the image. A paucicellular Antoni B area is seen on the left of the image. Related images Intermed. mag. Very high mag. Nephron - Not altered. CC BY-SA 3.0
Schwannoma. Antoni A area of schwannoma with Verocay bodies (one annotated by circle) Pezzali, Luíza Guazzelli; Rostirolla, Gabriela Francoes; Luiz, Carina Bauer; Gomes, Lúcio Brandão; Rivero, Raquel Camara; Valério, Edimárlei Gonsales; Vettorazzi, Janete - (2019). Not altered.CC BY 4.0
Schwannomas. (a) Microphotograph of conventional schwannoma showing hypercellular, Antoni A areas (black arrowhead), and hypocellular Antoni B areas (red arrowhead). (b) Schwannoma with extensive hyalinized areas. (c-f) Microphotograph of “ancient” schwannoma showing degenerative nuclear atypia (black arrowhead), hyalinized areas (red arrowhead), cystic change (blue arrowhead), areas of hemorrhage (*) and myxoid change (+) (H and E, ×40). (g) whole-mount section of plexiform schwannoma notes the subcutaneous multiple nodules (H and E, ×4), (h and i) higher magnification of the same showing hypocellular and hypercellular areas with verocay body (black arrow).
Schwannoma. Pathologic findings of brain schwannoma. (A) The tumor shows typical microscopic appearance of a schwannoma. It is composed of spindle cells arranged in interlacing fascicles (Antoni A: cellular area, H&E: ×200) showing transition with loose meshwork of delicate collagen fibers (Antoni B: acellular area). (B) The tumor cells strongly and diffusely express the S-100 protein (×200). Metachronous schwannoma in the colon with vestibular schwannoma. Jung EJ, Han HS, Koh YC, Cho J, Ryu CG, Paik JH, Hwang DY - Annals of surgical treatment and research (2014). Not Altered. CC.

Oligodendroglioma

Oligodendrogliomas are malignant tumors of oligodendrocytes.

Radiology of oligodendrogliomas typically show a calcified tumor involving the central nervous system (CNS).

Oligodendrogliomas typically affects the frontal lobe.

Oligodendrogliomas may cause seizures due to mass effect.

Histology of oligodendrogliomas typically show “fried egg” appearing cells that are pathognomonic for this tumor.

Oligodendroglioma. An oligodendroglioma as seen on MRI James Heilman, MD. Not altered. CC BY-SA 3.0
Oligodendroglioma. High magnification micrograph of an oligodendroglioma. H&E stain. Description: Highly cellular lesion composed of: cells resembling fried eggs with: distinct cell borders moderate-to-marked nuclear atypia, and a clear cytoplasm. acutely branched capillary sized vessels - "chicken-wire" like appearance. Related images Low mag. High mag. Nephron Not altered. CC BY-SA 3.0
Oligodendroglioma. Low magnification micrograph of an oligodendroglioma showing the characteristic, small, branching, chicken wire-like blood vessels. H&E stain. Nephron - Not altered. CC BY-SA 3.0
Photomicrographs of anaplastic oligodendroglioma and arteriovenous malformation (AVM) histology (a) anaplastic oligodendroglioma showing uniform cellularity, uniformly round nuclei, and perinuclear halos. This section shows classic delicate microvasculature, although microvascular proliferation was seen in other areas. Inset highlights an atypical mitotic figure of which many were identified (H and E; original magnification, ×200). (b) Secondary features of oligodendroglioma include scattered microcalcifications (center of the image) and clonal nodules of heightened cellularity (right side of image) (H and E original magnification, ×100). (c) Irregular large vessels of the AVM with intervening cellular oligodendroglioma (*) (H and E original magnification, ×20). (d) Irregularly contoured vessels of the AVM filled with black granular embolization material. An intimal cushion is identified in one of the fibrotic vessel walls (*) (H and E original magnification, ×50)Arteriovenous malformation within an isocitrate dehydrogenase 1 mutated anaplastic oligodendroglioma. Lai G, Muller KA, Carter BS, Chen CC - Surgical neurology international (2015). Not Altered. CC.

Pilocytic Astrocytoma

A pilocytic astrocytoma is a benign tumor of astrocytes.

Pilocytic astrocytomas are the most typical central nervous system tumor in children.

Pilocytic astrocytomas tends to develop in the cerebellum.

Radiology of pilocytic astrocytomas typically show a mural nodule and a cystic lesion are seen on imaging.

Histology of pilocytic astrocytomas shows eosinophilic granular aggregates and Rosenthal fibers (thick eosinophilic processes of the astrocytes).

The tumor cells of pilocytic astrocytomas are glial fibrillary acidic protein (GFAP) positive.

Pilocytic Astrocytoma. Stereotactic MRI brain scan showing a recurrent postoperative brain stem cystic pilocytic astrocytoma. Raef FA Hafez - Not altered CC BY 2.0
Pilocytic Astrocytoma. Histopathology of Rosenthal-fibres. H&E staining showing these elongated eosinophilic structures in the case of pilocytic astrocytoma. Magnification 400x Marvin 101 - Not altered. CC BY-SA 3.0
Pilocytic Astrocytoma. Histopathology of pilocytic astrocytoma (grade I WHO). H&E stain. Original magnification 200x. Marvin 101 - Not altered CC BY-SA 3.0
Pilocytic Astrocytoma. Axial non-contrast CT in a nine-year-old girl showing a slightly hypodense mass in the tectum of the brainstem, compressing the aqueduct of Sylvius and causing obstructive hydrocephalus. - Not altered. CC BY-SA 3.0
Pilocytic Astrocytoma. Very high magnification micrograph of a pilocytic astrocytoma, showing the characteristic bipolar cells with long hair-like processes. Smear. H&E stain. Related images Very high mag. High mag. Nephron. Not altered. CC BY-SA 3.0

Medulloblastoma

Medulloblastomas are a malignant tumor originating from the cerebellum’s granular cells (neuroectoderm).

Medulloblastomas typically occurs in children.

Medulloblastomas typically grow very rapidly.

The histology of medulloblastomas shows small, rounded blue cells are visible on histology, and Homer-Wright rosettes might be present.

Medulloblastomas are characterized by a poor prognosis.

Medulloblastoma. Cerebellar medulloblastoma in an adult No machine-readable author provided. KGH assumed (based on copyright claims). - No machine-readable source provided. Not altered. CC BY-SA 3.0
Medulloblastoma. Cerebellar medulloblastoma in an adult No machine-readable author provided. Not altered. CC BY-SA 3.0
Medulloblastoma. CT brain scan of child with medulloblastoma and resulting hydrocephalus Not altered. Public Domain
Histology showing (A) Classic medulloblastoma with nuclear β-catenin immunostaining; (B) Nodular medulloblastoma with cytoplasmic β-catenin immunostaining; (C) Anaplastic medulloblastoma with cytoplasmic β-catenin immunostaining.Pediatric medulloblastoma - update on molecular classification driving targeted therapies. DeSouza RM, Jones BR, Lowis SP, Kurian KM - Frontiers in oncology (2014). Not Altered. CC.

Ependymoma

Ependymomas are central nervous system tumors composed of malignant ependymal cells.

Ependymomas typically effect children.

Ependymomas most frequently occurs in the fourth ventricle and may be accompanied with hydrocephalus.

Histology of ependymomas classically show perivascular pseudorosettes are seen.

Ependymoma. Ependymoma of ventricle in MRI. Hellerhoff - Not altered. CC BY-SA 3.0
Ependymoma. Micrograph of a myxopapillary ependymoma. HPS stain. Nephron - Not altered. CC BY-SA 3.0
Ependymoma. Micrograph of an ependymoma. H&E stain. Related images Intermed. mag. Nephron. not altered. CC BY-SA 3.0
Inıtial microscopic evaluation of the case after first operation. (A) Moderately cellular tumor composed of monomorphic cells with round to oval nuclei containing “salt and pepper” pattern of chromatin. Radially arranged ependymal cell processes become thinner toward the vascular wall leaving an acellular zone around the blood vessel, called “perivascular pseudorosette,” a key histologic feature for ependymoma (hematoxylin and eosin [H&E] ×100). After the second operation (B) abundant endothelial proliferation, microvascular proliferation, hypercellularity with nuclear hyperchromasia, pleomorphism, numerous mitoses, and pseudopalisading necrosis warrants a diagnosis of anaplastic ependymoma when seen throughout the lesion (H&E ×200). Moreover (C), focus of coagulative necrosis, nuclear pseudopalisading was prominent around the necrotic areas, tumor cells oriented closer to viable areas emphasizing anaplastic changes (H&E ×200). Ki-67 immunolabeling index is an independent prognostic factor and accurate predictor of outcome in patients with intracranial ependymoma (D). The index was 10% in the first operation that increased to 35% for our case in this image (Ki-67 ×200).Malignant Transformation of Grade II Ependymoma in a 2-Year-Old Child: Case Report. Sarıkafa Ş, Çelik SE, Yarikkaya E, Sayılgan A - Journal of neurological surgery reports (2015). Not Altered. CC.

Craniopharyngioma

Craniopharyngiomas are central nervous system tumors that develop from Rathke’s pouch epithelial remnants.

In children or young adults, craniopharyngiomas manifests as a supratentorial mass.

Craniopharyngiomas have the potential to compress the optic chiasm and cause bitemporal hemianopsia.

Radiology of craniopharyngiomas typically shows calcifications.

Craniopharyngioma. CT scan showing a craniopharyngioma see above - Matthew R Garnett, Stéphanie Puget, Jacques Grill, Christian Sainte-Rose. Not altered. CC BY 2.0
Craniopharyngioma. Micrograph showing a papillary craniopharyngioma, HPS stain Nephron - Not altered. CC BY-SA 3.0
Craniopharyngioma. Micrograph showing the characteristic features of an adamantinomatous craniopharyngioma - cystic spaces, calcifications, and "wet" keratin, HPS stain Nephron - Not altered. CC BY-SA 3.0
Pathological findings. (a and b) Histopathology at 31 years of age. The tumor is covered with prickle cells, and a single layer of nonatypical basal cells and cholesterin crystals is present in the interstitial tissue. The diagnosis was adamantinomatous craniopharyngioma. (c and d) Histopathology at 36 years and 3 months of age. Densely packed squamous cells and stratification of basal cells with an atypical appearance are seen. The features of adamantinomatous type craniopharyngioma are no longer apparent. (c–d) Hematoxylin and eosin staining at the original magnification.Sequential pathological changes during malignant transformation of a craniopharyngioma: A case report and review of the literature. Negoto T, Sakata K, Aoki T, Orito K, Nakashima S, Hirohata M, Sugita Y, Morioka M - Surgical neurology international (2015). Not Altered. CC.