Abstract
This chapter presents a concise yet comprehensive overview of neuropathology covering a broad range of topics starting with general neurohistology and reaction to injury. A generous section is devoted for the discussion of various CNS neoplasms (glial, neuronal, glioneuronal, meningeal, and nonglial) with a summary of the clinical, radiographic, gross, and microscopic pathology and differential diagnosis of each entity. Other topics discussed include trauma, ischemic/anoxic injury, vascular disorders, neurodegenerative diseases, inflammatory and infectious diseases, pediatric and seizure-associated neuropathology, toxic and metabolic disorders, and sellar and suprasellar pathology including diseases of the pituitary gland. The text is oriented towards the general anatomic pathology resident for use during rotation in neuropathology, autopsy pathology, and preparation for anatomic pathology boards and in-service examinations. It may also be of use for practicing pathologists, neurosurgery and neurology residents, and faculty as a quick reference and preparation for various exams. The chapter has been updated to include changes present in the 2007 World Health Organization (WHO) classification system.
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Gliomas
Brat DJ, Scheithauer BW, Staugaitis SM, Cortez SC, Brecher K, Burger PC. Third ventricular chordoid glioma: a distinct clinicopathologic entity. J Neuropathol Exp Neurol 1998;57:283–290.
Burger PC. Pathology of brain stem astrocytomas. Pediatr Neurosurg 1996;24:35–40.
Burger PC, Pearl DK, Aldape K, et al. Small cell architecture – a histological equivalent of EGFR amplification in glioblastoma multiforme? J Neuropathol Exp Neurol 2001;60:1099–1104.
Choi YE, Chi JG, Suh YL. CD99 immunoreactivity in ependymoma. Appl Immunohistochem Mol Morphol 2001;9:125–129.
Emestus RI, Schroder R, Stutzer H, et al. The clinical and prognostic relevance of grading in intracranial ependymomas. Br J Neurosurg 1997;11:421–428.
Fernandez C, Figarella-Branger D, Girard N, et al. Pilocytic astrocytomas in children: prognostic factors – a retrospective study of 80 cases. Neurosurgery 2003;53:544–555.
Giannini C, Scheithauer BW, Burger PC, et al. Pleomorphic xanthoastrocytoma. What do we really know about it? Cancer 1999;85:2033–2045.
Hirose T, Scheithauer BW, Lopes MBS, et al. Tuber and sub-ependymal giant cell astrocytoma associated with tuberous sclerosis: an immunohistochemical, ultrastructural, and immunoelectron microscopic study. Acta Neuropathol 1995;90:387–399.
Kepes JJ. Pleomorphic xanthoastrocytoma: the birth of a diagnosis and a concept. Brain Pathol 1993;3:269–274.
Komotar RJ, Burger PC, Carson BS, et al. Pilocytic and pilomyxoid hypothalamic/chiasmatic astrocytomas. Neurosurgery 2004;54:72–80.
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, (eds.). WHO Classification of Tumours of the Central Nervous System. WHO Classification of Tumours (4th Edition). Lyon: IARC Press; 2007.
Min KW, Scheithauer BW. Clear cell ependymoma: a mimic of oligodendroglioma: clinicopathologic and ultrastructural considerations. Am J Surg Pathol 1997;21:820–826.
Morales H, Gaskill-Shipley M. Imaging of common adult and pediatric primary brain tumors. Semin Roentgenol 2010;45:92–106.
Perry A. Oligodendroglial neoplasms: current concepts, misconceptions, and folklore. Adv Anatomic Pathol 2001;8:183–199.
Perry A. Pathology of low-grade gliomas. An update of emerging concepts. Neuro-Oncology 2003;5: 168–178.
Pfister S, Hartmann C, Korshunov A. Histology and molecular pathology of pediatric brain tumors. J Child Neurol 2009;24:1375–1386.
Reifenberger G, Kaulich K, Wiestler OD, Blumcke I. Expression of the CD34 antigen in pleomorphic xanthoastrocytomas. Acta Neuropathol 2003;105:358–364.
Rosenblum MK. Ependymal tumors: a review of their diagnostic surgical pathology. Pediatr Neurosurg 1998;28:160–165.
Sato K, Kubota T, Ishida M, Yoshida K, Takeuchi H, Handa Y. Immunohistochemical and ultrastructural study of chordoid glioma of the third ventricle: its tanycytic differentiation. Acta Neuropathol 2003;106:176–180.
Scheithauer BW, Fuller GN, VandenBerg SR. The 2007 WHO classification of tumors of the nervous system: controversies in surgical neuropathology. Brain Pathol 2008;18:307–316.
Sonneland PR, Scheithauer BW, Onofrio BM. Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer 1985;56:883–893.
Trembath D, Miller CR, Perry A. Gray zones in brain tumor classification: evolving concepts. Adv Anat Pathol 2008;15:287–297.
Vege KDS, Giannini C, Scheithauer BW. The immunophenotype of ependymomas. Appl Immunohistochem Mol Morphol 2000;8:25–31.
Neuronal/Glioneuronal Neoplasms
Behdad A, Perry A. Central nervous system primitive neuroectodermal tumors: a clinicopathologic and genetic study of 33 cases. Brain Pathol2010;20:441–450.
Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, et al. Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. Neurosurgery 1988;23:545–556.
Eberhart CG, Burger PC. Anaplasia and grading in medulloblastomas. Brain Pathol 2003;13:376–385.
Edgar MA, Rosenblum MK. Mixed glioneuronal tumors: recently described entities. Arch Pathol Lab Med 2007;131:228–233.
Fuller CE, Pfeifer J, Humphrey P, Bruch LA, Dehner LP, Perry A. Chromosome 22q dosage in composite extrarenal rhabdoid tumors: clonal evolution or a phenotypic mimic? Hum Pathol 2001;32: 1102–1108.
Giangaspero F, Perilongo G, Fondelli MP, et al. Medulloblastoma with extensive nodularity: a variant with favorable prognosis. J Neurosurg 1999;91:971–977.
Hassoun J, Söylemezoglu F, Gambarelli D, et al. Central neurocytoma: a synopsis of clinical and histological features. Brain Pathol 1993; 3:297–306.
Hirose T, Scheithauer BW, Lopes MBS, et al. Olfactory neuroblastoma. As immunohistochemical, ultrastructural, and flow cytometric study. Cancer 1995;76:4–19.
Ho DMT, Hsu CY, Wong TT, Ting LT, Chiang H. Atypical teratoid/rhabdoid tumor of the central nervous system: a comparative study with primitive neuroectodermal tumor/medulloblastoma. Acta Neuropathol 2000;99:482–488.
Honavar M, Janota I, Polkey CE. Histological heterogeneity of dysembryoplastic neuroepithelial tumour: identification and differential diagnosis in a series of 74 cases. Histopathology 1999;34:342–356.
Luyken C, Blumcke I, Fimmers R, Urbach H, Wiestler OD, Schramm J. Supratentorial gangliogliomas: histopathologic grading and tumor recurrence in 184 patients with a median follow-up of 8 years. Cancer 2004;101:146–155.
McManamy CS, Lamont JM, Taylor RE, et al. Morphophenotypic variation predicts clinical behavior in childhood non-desmoplastic medulloblastomas. J Neuropathol Exp Neurol 2003;62:627–632.
Perry A. Medulloblastomas with favorable vs. unfavorable histology. How many small blue cell tumor types are there in the brain? Adv Anat Pathol 2002;9:345–350.
Rorke LB, Packer RJ, Biegel JA. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg 1996;85:56–65.
Schmidt MH, Gottfried ON, von Koch CS, Chang SM, McDermott MW. Central neurocytoma: a review. J Neurooncol 2004;66:377–383.
Tamburrini G, Colosimo C, Giangaspero F, Riccardi R, Di Rocco C. Desmoplastic infantile ganglioglioma. Childs Nerv Syst 2003;19:292–297.
VandenBerg SR. Desmoplastic infantile ganglioglioma and desmoplastic cerebral astrocytoma of infancy. Brain Pathol 1993;3:275–281.
Meningeal Neoplasms
Couce ME, Aker FV, Scheithauer BW. Chordoid meningioma. A clinicopathologic study of 42 cases. Am J Surg Pathol 2000;24:899–905.
Ecker RD, Marsh WR, Pollock BE, et al. Hemangiopericytoma in the central nervous system: treatment, pathological features, and long-term follow up in 38 patients. J Neurosurg 2003;98:1182–1187.
Ludwin SK, Rubinstein LJ, Russell DS. Papillary meningiomas: a malignant variant of meningioma. Cancer 1975;36:1363–1373.
Mena H, Ribas JL, Pezeshkpour GH, et al. Hemangiopericytoma of the central nervous system: a review of 94 cases. Hum Pathol 1991;22:84–91.
Perry A, Gutmann DH, Reifenberger G. Molecular pathogenesis of meningiomas. J Neurooncol 2004;70: 183–202.
Perry A, Scheithauer BW, Stafford SE, et al. “Rhabdoid” meningioma: an aggressive variant. Am J Surg Pathol 1998;22:1482–1490.
Perry A, Stafford SE, Scheithauer BW, et al. “Malignancy” in meningiomas: a clinicopathologic study of 116 patients. Cancer 1999;85:2046–2056.
Rajaram V, Brat DJ, Perry A. Anaplastic meningioma vs. meningeal hemangiopericytoma: immunohistochemical and genetic markers. Hum Pathol 2004;135:1413–1418.
Riemenschneider MJ, Perry A, Reifenberger G. Histological classification and molecular genetics of meningiomas. Lancet Neurol 2006;5:1045–1054.
Tihan T, Viglione M, Rosenblum MC, Olivi A, Burger PC. Solitary fibrous tumors in the central nervous system. A clinicopathologic review of 18 cases and comparison to meningeal hemangiopericytomas. Arch Pathol Lab Med 2003;127:432–439.
Zorludemir S, Scheithauer BW, Hirose T, et al. Clear cell meningioma. A clinicopathologic study of a potentially aggressive variant of meningioma. Am J Surg Pathol 1995;19:493–505.
Other Nonglial Neoplasms
Bataille B, Delwail V, Menet E, et al. Primary intracerebral malignant lymphoma: report of 248 cases. J Neurosurg 2000;92:261–266.
Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D. Hemangioblastomas of the central nervous system in von Hippel–Lindau syndrome and sporadic disease. Neurosurgery 2001;48:55–63.
Crotty TB, Scheithauer BW, Youn WF Jr., et al. Papillary craniopharyngioma: a clinicopathological study of 48 canes. J Neurosurg 1995;83: 206–214.
Hoang MP, Amirkhan RH. Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma. Am J Surg Pathol 2003;27:1152–1156.
Matsutani M, Sano K, Takakura K, et al. Primary intracranial germ cell tumors: a clinical analysis of 153 histologically verified cases. J Neurosurg 1997;86:446–455.
Paulus W. Classification, pathogenesis and molecular pathology of primary CNS lymphomas. J Neurooncol 1999;43:203–208.
Perry A, Parisi JE, Kurtin PJ. Metastatic adenocarcinoma to the brain. An immunohistochemical approach. Hum Pathol 1997;28:938–943.
Schild SE, Scheithauer BW, Haddock MG, et al. Histologically confirmed pineal tumors and other germ cell tumors of the brain. Cancer 1996;78:2564–2571.
Srodon M, Westra WH. Immunohistochemical staining for thyroid transcription factor-1: a helpful aid in discerning primary site of tumor origin in patients with brain metastases. Hum Pathol 2002;33:642–645.
Wolff JEA, Sajedi M, Brant R, Coppes MJ, Egeler RM. Choroid plexus tumours. Br J Cancer 2002;87:1086–1091.
Trauma
Crout SE, Flanders AE. Neuropathology of human spinal cord injury. Adv Neurol 1997;72:317–323.
Geddes JF, Whitwell HE, Graham DI. Traumatic axonal injury: practical issues for diagnosis in medicolegal cases. Neuropathol Appl Neurobiol 2000;26:105–116.
Graham DI, Adams JH, Nicoll JAR, et al. The nature, distribution and causes of traumatic brain injury. Brain Pathol 1995;5:397–406.
Hardman JM, Manoukian A. Pathology of head trauma. Neuroimaging Clin North Am 2002;12:175–187.
Pearl GS. Traumatic neuropathology. Clin Lab Med 1998;18:39–64.
Ischemic/Anoxic/Vascular Disorders
Black M, Graham DI. Sudden unexplained death in adults caused by intracranial pathology. J Clin Pathol 2002;55:44–50.
Kalimo H, Ruchoux MM, Vitanen M, Kalaria RN. CADASIL: a common form of hereditary arteriopathy causing brain infarcts and dementia. Brain Pathol 2002;12:371–384.
Lammie GA. Hypertensive cerebral small vessel disease and stroke. Brain Pathol 2002;12:358–370.
Pantoni L, Garcia JH. Cognitive impairment and cellular/vascular changes in the cerebral white matter. Ann N Y Acad Sci 1997;826:92–102.
Parisi JE, Moore PM. The role of biopsy in vasculitis of the central nervous system. Semin Neurol 1994;14:341–348.
Reevesz T, Holton JL, Lashley T, et al. Sporadic and familial cerebral amyloid angiopathies. Brain Pathol 2002;12:343–357.
Ruchoux MM, Maurage CA. CADASIL: cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. J Neuropathol Exp Neurol 1997;56:947–964.
Neurodegenerative Disorders
Budka H, Aguzzi A, Brown P, et al. Neuropathological diagnostic criteria for Creutzfeldt–Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases). Brain Pathol 1995;5:459–466.
Chen-Plotkin AS, Lee VM, Trojanowski JQ. TAR DNA-binding protein 43 in neurodegenerative disease. Nat Rev Neurol 2010;6:211–220.
Dickson DW. Neuropathology of Alzheimer’s disease and other dementias. Clin Geriatric Med 2001;17:209–228.
Forno LS. Neuropathology of Parkinson’s disease. J Neuropathol Exp Neurol 1996;55:259–272.
Hansen LA, Cram BJ. Making the diagnosis of mixed and non-Alzheimer’s dementias. Arch Pathol Lab Med 1995;119:1023–1031.
Ironside JW. Prion diseases in man. J Pathol 1998;186:227–234.
Jellinger KA. Neuropathology of movement disorders. Neurosurg Clin N Am 1998;9:237–262.
Li SH, Li XJ. Huntingtin-protein interactions and the pathogenesis of Huntington’s disease. Trends Genet 2004;20:146–154.
Liscic RM, Grinberg LT, Zidar J, Gitcho MA, Cairns NJ. ALS and FTLD: two faces of TDP-43 proteinopathy. Eur J Neurol 2008;15:772–780.
Mirra SS, Hart MN, Terry RD. Making the diagnosis of Alzheimer’s disease. A primer for practicing pathologists. Arch Pathol Lab Med 1993;117:132–144.
Nance MA. Clinical aspects of CAG repeat diseases. Brain Pathol 1997;7:881–900.
Trojanowski JQ, Dickson D. Update on the neuropathological diagnosis of frontotemporal dementias. J Neuropathol Exp Neurol 2001;60:l 123–1 126.
Vinters HV, Ellis WG, Zarow C, et al. Neuropathologic substrates of ischemic vascular dementia. J Neuropathol Exp Neurol 2000;59:931–945.
Yoshida M. Multiple system atrophy α-synuclein and neuronal degeneration. Neuropathol 2007;27:484–493.
Inflammatory/Infectious/Demyelinating Diseases
Botero D, Tanowitz HB, Weiss LM, Wittner M. Taeniasis and cysticercosis. Infect Dis Clin North Am 1993;7:683–697.
Burns DK, Risser RC, White CL. The neuropathology of human immunodeficiency virus infection. The Dallas, Texas experience. Arch Pathol Lab Med 1991;115:1112–1124.
Chimelli L, Mahler-Araujo MB. Fungal infections. Brain Pathol 1997;7:613–627.
Gray F. Bacterial Infections. Brain Pathol 1997:7:629–647.
Kepes JJ. Large focal tumor-like demyelinating lesions of the brain: intermediate entity between multiple sclerosis and acute disseminated encephalomyelitis? A study of 31 patients. Ann Neurol 1993;33:18–27.
Kleinschmidt-DeMasters BK. Central nervous system aspergillosis: a 20- year retrospective series. Hum Pathol 2002;33:116–124.
Kleinschmidt-DeMasters BK, Gilden DH. The expanding spectrum of herpesvirus infections of the nervous system. Brain Pathol 2001;11:440–451.
Kuhlmann T, Lassmann H, Brück W. Diagnosis of inflammatory demyelination in biopsy specimens: a practical approach. Acta Neuropathol 2008;115:275–287.
Nowak DA, Widenka DC. Neurosarcoidosis: a review of its intracranial manifestation. J Neurol 2001;248:363–372.
Omalu BI, Shakir AA, Wang G, Lipkin WI, Wiley CA. Fatal fulminant pan-meningopolioencephalitis due to West Nile virus. Brain Pathol 2003;13:465–472.
Thomas G, Murphy S, Staunton H, O’Neill S, Farrell MA, Brett FM. Pathogen-free granulomatous diseases of the central nervous system. Hum Pathol 1998;29:1110–1115.
Zagzag D, Miller DC, Kleinman GM, et al. Demyelinating disease versus tumor in surgical neuropathology. Am J Surg Pathol 1993;17:537–545.
Pediatric and Seizure Neuropathology
Arid Y, Edwards V, Becker LE. A comparison of cell phenotypes in hemimegalencephaly and tuberous sclerosis. Acta Neuropathol 1999;98:407–413.
Black M, Graham DI. Sudden unexplained death in adults caused by intracranial pathology. J Clin Pathol 2002;55:44–50.
Frater JL, Prayson RA, Morris HH, III, Bingaman WE. Surgical pathologic findings of extratemporal-based intractable epilepsy. A study of 133 consecutive resections. Arch Pathol Lab Med 2000;124:545–549.
Golden JA. Holoprosencephaly: a defect in brain patterning. J Neuropathol Exp Neurol 1998;57:991–999.
Luyken C, Blumcke I, Fimmers R, et al. The spectrum of long-term epilepsy-associated tumors: long-term seizure and tumor outcome and neurosurgical aspects. Epilepsia 2003;44:822–830.
Marcorelles P, Laquerriere A. Neuropathology of Holoprosencephaly. Am J Med Genet C Semin Med Genet 2010;154C:109–119.
Mizuguchi M, Takashima S. Neuropathology of tuberous sclerosis. Brain Dev 2001;23:508–515.
Norman MG. Malformations of the brain. J Neuropathol Exp Neurol 1996;55:133–143.
Pilz D, Stoodley N, Golden JA. Neuronal migration, cerebral cortical development, and cerebral cortical anomalies. J Neuropathol Exp Neurol 2002;61:1–11.
Prayson RA. Diagnostic challenges in the evaluation of chronic epilepsy-related surgical neuropathology. Am J Surg Pathol 2010;34:e1–e13.
Volpe JJ. Neurologic outcome of prematurity. Arch Neurol 1998;55:297–300.
Toxic/Metabolic Disorders
Harper C. The neuropathology of alcohol-related brain damage. Alcohol Alcohol 2009;44:136–140.
Kolodny EH, Cable WJ. Inborn errors of metabolism. Ann Neurol 1982;11:221–232.
Krill JJ. Neuropathology of thiamine deficiency disorders. Metab Brain Dis 1996;11:9–17.
Moser HW, Powers JM, Smith KD. Adrenoleukodystrophy: molecular genetics, pathology, and Lorenzo’s oil. Brain Pathol 1995;5:259–266.
Sellar/Suprasellar/Pituitary Pathology
Gaffey TA, Scheithauer BW, Lloyd RV, et al. Corticotroph carcinoma of the pituitary: a clinicopathological study. Report of four cases. J Neurosurg 2002;96:352–360.
Kovacs K, Scheithauer BW, Horvath E, et al. The World Health Organization classification of adenohypophyseal neoplasms. A proposed five-tier scheme. Cancer 1996;78:502–510.
Lloyd RV, Scheithauer BW, Kovacs K, Roche PC. The immunophenotype of pituitary adenomas. Endocr Pathol 1996;7:145–150.
Pernicone PJ, Scheithauer BW, Horvath E, Kovacs K. Pituitary and sellar region. In: Sternberg SS (ed) Diagnostic Surgical Pathology, 2nd Ed, Philadelphia, PA, Lippincott Williams & Wilkins; 1997:1053–1107.
Perry A, Scheithauer BW. Commentary: classification and grading of pituitary tumors. Observations of two working neuropathologists. Acta Neuropathol 2006;111:68–70.
Saeger W, Lüdecke DK, Buchfelder M, Fahlbusch R, Quabbe HJ, Petersenn S. Pathohistological classification of pituitary tumors: 10 years of experience with the German Pituitary Tumor Registry. Eur J Endocrinol 2007;156:203–216.
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Hattab, E.M., Hagen, M.C., Scheithauer, B.W., Perry, A. (2011). Neuropathology. In: Cheng, L., Bostwick, D. (eds) Essentials of Anatomic Pathology. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-6043-6_14
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