The 2021 World Health Organization Classification of Tumors of the Central Nervous System: What Neuroradiologists Need to Know.

Neuroradiologists play a key role in brain tumor diagnosis and management. Staying current with the latest classification systems and diagnostic markers is important to provide optimal patient care. Publication of the 2016 World Health Organization Classification of Tumors of the Central Nervous System introduced a paradigm shift in the diagnosis of CNS neoplasms. For the first time, both histologic features and genetic alterations were incorporated into the diagnostic framework, classifying and grading brain tumors. The newly published 2021 World Health Organization Classification of Tumors of the Central Nervous System, May 2021, 5th edition, has added even more molecular features and updated pathologic diagnoses. We present, summarize, and illustrate the most salient aspects of the new 5th edition. We have selected the key "must know" topics for practicing neuroradiologists.

The many faces of facial nerve schwannoma.

BACKGROUND AND PURPOSE: The imaging appearance of facial nerve schwannomas (FNSs) has been described as an enhancing tubular mass (using T1-enhanced MR) within an enlarged facial nerve canal (using CT). The purpose of this study is to identify how often the FNS imaging findings conform to this description and determine whether there are underlying anatomic explanations for the discrepant imaging appearances identified. MATERIALS AND METHODS: The clinical, pathologic, and radiologic records of 24 FNS in 23 patients were retrospectively reviewed. Each FNS was evaluated for location along the facial nerve. The lesions were cataloged by facial nerve segment with the imaging characteristics of each segment described. RESULTS: The average age at time of first imaging was 39 years (age range, 10-70 years). Eighteen (71%) of the 24 FNSs were pathologically confirmed, while the others were determined intraoperatively or diagnostically by the presence of both enlargement of the facial nerve canal and enhancement on contrast-enhanced T1 MR examination. The most common location was in the geniculate fossa (83%), followed by the labyrinthine and tympanic segments of the facial nerve (both 54%). The most common clinical presentation was facial neuropathy (42%). CONCLUSION: The classic description of FNS on enhanced T1 MR is that of a well-circumscribed fusiform enhancing mass along the course of the intratemporal facial nerve with bone algorithm CT showing sharply defined bony canal enlargement. Modern imaging techniques, however, demonstrate the importance of the surrounding anatomic landscape, leading to various imaging appearances. Lesions traversing the labyrinthine segment can demonstrate a dumbbell appearance. When FNSs track along the greater superficial petrosal nerve, they may present as a round mass projecting up into the middle cranial fossa. FNS of the tympanic segment of the facial nerve preferentially pedunculate into the middle ear cavity, clinically presenting as a middle ear mass. When the mastoid segment of the facial nerve is involved, irregular and "invasive" tumor margins seen on MR can be explained on CT as tumor breaking into surrounding mastoid air cells.

Imaging and clinical characteristics of temporal bone meningioma.

BACKGROUND AND PURPOSE: Imaging characteristics of temporal bone meningioma have not been previously reported in the literature. CT and MR imaging findings in 13 cases of temporal bone meningioma are reviewed to define specific imaging features. METHODS: A retrospective review of our institutional case archive revealed 13 cases of histologically confirmed temporal bone meningioma. CT and MR imaging studies were reviewed to characterize mass location, vector of spread, bone changes, enhancement characteristics, and intracranial patterns of involvement. Clinical presenting signs and symptoms were correlated with imaging findings. RESULTS: Thirteen temporal bone meningiomas were reviewed in 8 women and 5 men, aged 18-65 years. Meningiomas were stratified into 3 groups on the basis of location and tumor vector of spread. There were 6 tegmen tympani, 5 jugular foramen (JF), and 2 internal auditory canal (IAC) meningiomas. Tegmen tympani and JF meningiomas were characterized by spread to the middle ear cavity. IAC meningiomas, by contrast, spread to the cochlea and vestibule. Hearing loss was the most common clinical presenting feature in all cases of temporal bone meningioma (10/13). The presence of tumor adjacent to the ossicles strongly correlated with conductive hearing loss (7/9). CONCLUSION: Meningioma involving the temporal bone is rare. Three subgroups of meningioma exist in this location: tegmen tympani, JF, and IAC meningioma. Tegmen tympani and JF meningiomas spread to the middle ear cavity. IAC meningiomas spread to intralabyrinthine structures. Conductive hearing loss is commonly seen in these patients and can be surgically correctable.

Imaging Psoas Sign in Lumbar Spinal Infections: Evaluation of Diagnostic Accuracy and Comparison with Established Imaging Characteristics.

BACKGROUND AND PURPOSE: Lumbar discitis-osteomyelitis has imaging characteristics than can overlap with noninfectious causes of back pain. Our aim was to determine the added accuracy of psoas musculature T2 hyperintensity (imaging psoas sign) in the MR imaging diagnosis of lumbar discitis-osteomyelitis. MATERIALS AND METHODS: This retrospective case-control study evaluated lumbar spine MR imaging examinations, during a 30-month period, that were requested for the evaluation of discitis-osteomyelitis. Of this pool, 50 age-matched control patients were compared with 51 biopsy-proved or clinically diagnosed patients with discitis-osteomyelitis. Two reviewers, blinded to the clinical information, assessed the randomly organized MR imaging examinations for abnormalities of the psoas musculature, vertebral bodies, discs, and epidural space. RESULTS: Psoas T2 hyperintensity demonstrated a high sensitivity (92.1%; 95% CI, 80%-97.4%) and specificity (92%; 95% CI, 80%-97.4%), high positive likelihood ratio (11.5; 95% CI, 4.5-29.6), low negative likelihood ratio (0.09; 95% CI, 0.03-0.20), and individual area under the receiver operating characteristic curve of 0.92; 95% CI, 0.87-0.97. Identification of psoas T2 abnormality significantly improved (P = .02) the diagnostic accuracy of discitis-osteomyelitis in noncontrast examinations from an area under the receiver operator characteristic curve of the established variables (vertebral body T2 and T1 signal, endplate integrity, disc T2 signal, and disc height) from 0.93 (95% CI, 0.88-0.98) to 0.98 (95% CI, 0.96-1.0). Psoas T2 abnormalities also had the highest interobserver reliability with a κ coefficient of 0.78 (substantial agreement). CONCLUSIONS: Psoas T2 hyperintensity, the imaging psoas sign, is highly correlated with discitis-osteomyelitis. T2 hyperintensity in the psoas musculature, particularly when there is clinical suspicion of spinal infection, improves the diagnostic accuracy of discitis-osteomyelitis compared with routine noncontrast variables alone.

Dumbbell schwannomas of the internal auditory canal.

BACKGROUND AND PURPOSE: Benign tumors of the internal auditory canal (IAC) may leave the confines of the IAC fundus and extend into inner ear structures, forming a dumbbell-shaped lesion. It is important to differentiate dumbbell lesions, which include facial and vestibulocochlear schwannomas, from simple intracanalicular schwannomas, as surgical techniques and prognostic implications are affected. In this article, the imaging and clinical features of these dumbbell schwannomas are described. METHODS: A dumbbell lesion of the IAC is defined as a mass with two bulbous segments, one in the IAC fundus and the other in the membranous labyrinth of the inner ear or the geniculate ganglion of the facial nerve canal, spanned by an isthmus. Twenty-four patients with dumbbell lesions of the IAC had their clinical and imaging data retrospectively reviewed. Images were evaluated for contour of the mass and extension into the membranous labyrinth or geniculate ganglion. RESULTS: Ten of 24 lesions were facial nerve dumbbell lesions. Characteristic features included an enhancing "tail" along the labyrinthine segment of the facial nerve and enlargement of the facial nerve canal. Dumbbell schwannomas of the vestibulocochlear nerve (14/24) included transmodiolar (8/14), which extended into the cochlea, transmacular (2/14), which extended into the vestibule, and combined transmodiolar/transmacular (4/14) types. CONCLUSION: Simple intracanalicular schwannomas can be differentiated from transmodiolar, transmacular, and facial nerve schwannomas with postcontrast and high-resolution fast spin-echo T2-weighted MR imaging. Temporal bone CT is reserved for presurgical planning in the dumbbell facial nerve schwannoma group.

Intratemporal facial nerve neuroma: anatomical location and radiological features.

OBJECTIVES: To present the imaging findings and anatomical locations of a series of 88 facial nerve neuromas from two centers over a 30-year period. We describe the salient radiological features of neuromas in each anatomical location and outline the ways in which modern imaging techniques have altered our perception of this entity. STUDY DESIGN: A retrospective review of tumors presenting to two tertiary care referral institutions since 1970. METHODS: The charts and available imaging of patients with the diagnosis of facial neuroma were reviewed. These patients presented to the House Ear Clinic between 1970 and 1994 and to the University of Utah Medical Center (Salt Lake City, UT) between 1986 and August 2000. We examined anatomical location to determine patterns of tumor presentation and compared the findings before and after the era of magnetic resonance imaging (MRI). RESULTS: All segments of the facial nerve were represented. Overall, multiple-segment tumors were almost twice as common (63.6%) as single-segment tumors (36.4%). Before the advent of MRI, all segments of the nerve from the cerebellopontine angle to the tympanic portion were almost equally represented (29.5%-36.3%). After MRI, the geniculate ganglion (68.2%) and labyrinthine portion (52.3%) were by far the most commonly affected areas. Before MRI, there were, on average, 1.89 segments involved per tumor. After MRI, this average number increased to 2.57 segments per tumor. Radiologically, the high-resolution computed tomography and MRI features cannot be generalized. Rather, the imaging features depend on which segments are involved. This is because of the variation in the surrounding anatomical landscape of the facial nerve in its course through the temporal bone. CONCLUSION: The more sensitive imaging provided by newer radiological techniques has altered our perception of facial neuroma. It has provided us with an increased ability to diagnose and fully evaluate this neoplasm preoperatively, allowing improved patient counseling and surgical planning.

Craniopharyngeal canal and its spectrum of pathology.

BACKGROUND AND PURPOSE: The craniopharyngeal canal is a rare, well-corticated defect through the midline of the sphenoid bone from the sellar floor to the anterosuperior nasopharyngeal roof. We reviewed a series of craniopharyngeal canals to determine a system of classification that might better our understanding of this entity, highlight the range of associated pathologic conditions, and optimize patient treatment. MATERIALS AND METHODS: Available MR imaging, CT, and clinical data (from 1989-2013) of 29 patients (10 female, 15 male, 4 unknown; median age, 4 years; age range, 1 day-65 years) with craniopharyngeal canals were retrospectively examined. Qualitative assessment included orthotopic or ectopic adenohypophysis and the presence of a tumor and/or cephalocele. The midpoint anteroposterior diameter was measured. Clinical and imaging data were evaluated for pituitary dysfunction and accompanying anomalies. RESULTS: Craniopharyngeal canals were qualitatively separated into 3 types: incidental canals (type 1); canals with ectopic adenohypophysis (type 2); and canals containing cephaloceles (type 3A), tumors (type 3B), or both (type 3C), including pituitary adenoma, craniopharyngioma, dermoid, teratoma, and glioma. Quantitative evaluation showed a significant difference (P < .0001) in the anteroposterior diameters of type 1 canals (median, 0.8; range, 0.7-1.1 mm), type 2 canals (median, 3.9, range, 3.5-4.4 mm), and type 3 canals (median, 9.0; range, 5.9-31.0 mm) imparting small, medium, and large descriptors. Canals with cephaloceles all contained an ectopic adenohypophysis. The craniopharyngeal canals were associated with pituitary dysfunction (6/29) and congenital anomalies (8/29). CONCLUSIONS: Accurate diagnosis and classification of craniopharyngeal canals are valuable to characterize lesions requiring surgery, identify patients with potential pituitary dysfunction, and avoid iatrogenic hypopituitarism or CSF leak during surgical resection of nasopharyngeal masses.

The new World Health Organization Classification of Central Nervous System Tumors: what can the neuroradiologist really say?

The WHO Classification of Tumors of the Central Nervous System has become the worldwide standard for classifying and grading brain neoplasms. The most recent edition (WHO 2007) introduced a number of significant changes that include both additions and redefinitions or clarifications of existing entities. Eight new neoplasms and 4 new variants were introduced. This article reviews these entities, summarizing both their histology and imaging appearance. Now with more than 3 years of clinical experience following publication of the newest revision, we also ask, "What can the neuroradiologist really say?" Are there imaging findings that could suggest the preoperative diagnosis of a new tumor entity or variant?

Intralabyrinthine schwannomas: imaging diagnosis and classification.

BACKGROUND AND PURPOSE: ILS is a rare lesion that has a different management from the more common "acoustic" schwannoma. To date, only 137 cases have been reported. We present a classification scheme based on labyrinthine anatomy to describe and localize these lesions. Treatment and prognosis hinge on the appropriate localization of these tumors; thus, a concise terminology that can be used by both the otolaryngologist and radiology communities is desirable. MATERIALS AND METHODS: After approval of the institutional review board, a retrospective study of all patients with the diagnosis of ILS imaged between 1996 and 2010 was performed. Clinical and imaging data were collected. Patients were imaged with thin-section high-resolution T2 and contrast-enhanced MR imaging. RESULTS: There were 45 patients with a diagnosis of ILS. Forty-three had complete histories. There were 18 male and 25 female patients with an age range of 21-78 years with a mean age of 53 years. The most common presenting symptom was progressive sensorineural hearing loss. Lesions were characterized on the basis of their location. Intracochlear was most common (14/45) followed by transmodiolar (13/45), intravestibular (7/45), vestibulocochlear (5/45), transmacular (4/45), and transotic (2/45). Sixteen patients underwent surgical resection. The remaining patients were followed clinically and by serial MR imaging. CONCLUSIONS: ILS is an uncommon but under-reported tumor. We characterized the MR imaging appearance of these tumors by using high-resolution techniques. In addition, an anatomically based classification system is presented that will help the radiologist accurately describe ILS within the inner ear and help the surgeon determine which tumors are potential surgical candidates.

An unusual sinonasal tumor: soft tissue perineurioma.

Soft tissue perineuriomas are an unusual type of peripheral nerve sheath tumors distinct from schwannomas and neurofibromas, with interesting histologic findings. They are not well characterized on radiographic examination. We report this case of a patient with sinonasal perineurioma to help define the imaging and pathologic features of this rare head and neck tumor.

Extraosseous chordoma of the nasopharynx.

BACKGROUND AND PURPOSE: Chordoma is a relatively rare tumor of the skull base and sacrum thought to originate from embryonic remnants of the notochord. Chordomas arising from the skull base/clivus are typically locally aggressive with lytic bone destruction. When chordomas occur in an extraosseous location, they may mimic other lesions of the nasopharynx. We present 5 cases of primarily extraosseous chordoma involving the nasopharynx in an effort to improve the preoperative diagnosis of this rare tumor. In addition, we review regional notochordal embryology to explain this variant tumor location. MATERIALS AND METHODS: We reviewed the clinical and imaging data of 5 pathologically proved cases of extraosseous chordoma of the nasopharynx seen or reviewed at our institution during the last decade. All cases had both CT and MR imaging. The study had institutional review board approval. RESULTS: The primary clinical complaint in the 5 patients with extraosseous nasopharyngeal chordoma was nasal obstruction. The extraosseous chordomas were centered in the nasopharynx. Bony lytic changes along the anterior surface of the clivus were seen on 5 of 5 CT studies. A midline sinus tract was seen in 3 of 5 patients. MR imaging showed heterogeneous hyperintense T2 signal intensity (5/5). CONCLUSIONS: Extraosseous nasopharyngeal chordoma is a rare but important lesion to be considered in the differential diagnosis of nasopharyngeal masses. When a midline nasopharyngeal mass is found with an associated clival sinus tract, extraosseous chordoma moves to the top of the differential diagnosis list. Complete removal of the soft-tissue tumor and the clival sinus tract is the treatment of choice in such cases.

[Anatomic and pathologic spectrum of pituitary infundibulum lesions]. / Anatomía y espectro patológico de las lesiones del infundíbulo hipofisario.

OBJECTIVE: The pathologic spectrum of pituitary infundibulum disease is diverse. We reviewed 65 infundibular lesions in 44 adult and 21 pediatric patients, and summarized their imaging features and clinical presentation. CONCLUSION: The spectrum of pathology involving the pituitary infundibulum is broad yet distinct from other pathology in the sella and parasellar region. Pituitary stalk lesions can be grouped into three categories: congenital and developmental, inflammatory and infectious, and neoplastic. Knowledge of the imaging appearance of diseases specific to adults and to children is important for accurate diagnosis and treatment.

Early pathologic findings and long-term improvement in anti-Ma2-associated encephalitis.

A 67-year-old man sequentially developed anti-Ma2-associated paraneoplastic encephalitis (PNE) and contralateral herpes simplex encephalitis (HSE). Brain biopsy 1 month before HSE revealed extensive infiltrates of T cells, B cells, and plasma cells. Most T cells expressed the cytotoxic granule-associated protein TIA-1 and the membranolytic protein granzyme-B. Although recovery was thought to be unlikely, treatment of the PNE with corticosteroids and resection of the associated lung cancer resulted in dramatic improvement for 21 months.

Upper extremity bursitis.

Upper extremity bursae are injured through a number of processes, including overuse, hemorrhage, crystal deposition, autoimmune diseases and infection. These injuries may be disabling and can pose significant diagnostic and therapeutic challenges for the clinician. Treatment of the most common forms is directed at pain management and functional rehabilitation through a structured exercise program. Early recognition of infectious bursitis, followed by appropriate surgical and antibiotic treatment, is critical to prevent severe sequelae in these cases. This article reviews the pathophysiology, evaluation and treatment of the three most commonly involved upper extremity bursae: the subacromial, the olecranon and the subscapular bursae.

Lower extremity bursitis.

Bursitis is a common cause of lower extremity pain in patients presenting to primary care physicians. Several bursae in the lower extremity account for most of these injuries, including the ischiogluteal, greater trochanteric, pes anserine, medial collateral, prepatellar, popliteal and retrocalcaneal. Often the symptoms are mild, with the patient successfully self-treating through activity modification and other conservative measures. A systematic approach to the evaluation and treatment of patients with bursitis, including prevention, relative rest, ice, compression, elevation, anti-inflammatory medication and treatment modalities such as ultrasound and electrical stimulation, combined with a structured rehabilitation program, will greatly facilitate the healing process.

Primary intracranial germ cell tumors: clinicopathologic review of 32 cases.

Primary intracranial germ cell neoplasms are rare tumors and constitute a heterogeneous group. We have reviewed 32 cases, over a 21-year period, from the University of Florida. The cases include 22 germinomas, 6 mixed germ cell tumors, and 4 teratomas. The clinical presentations in these cases were more closely related to the location of the tumor, that is, pineal or suprasellar, rather than the histologic subtype. Neuroimaging evaluation was useful in distinguishing between germinomas, teratomas, and other mixed germ cell tumors (MGCTs), primarily by evaluation of cystic versus solid lesions (teratoma versus germinoma), contents of cysts (teratoma versus MGCT), and infiltrative nature of the tumors (MGCT), although cytologic-histopathologic confirmation remains necessary. Germinomas responded favorably to radiation therapy with survival periods of over 16 years; MGCTs were treated with combination chemotherapy and radiation, with a markedly poorer prognosis. This study underlines the critical significance of histopathologic evaluation of the tumor in determining therapeutic interventions as well as prognosis.