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Manifestations of Schwannoma in the Neuraxis and Peripheral Nerves: A Radiological Spectrum eEdE-243a Viet Nguyen, MD Maria Valencia, MD Achint Singh, MD Wilson Altmeyer, MD Carlos Bazan III, MD Bundhit Tantiwongkosi, MD Disclosure Statement The authors have no financial interest to disclose. Objectives • Review the characteristic imaging findings of schwannoma with respect to the neuraxis and peripheral nerves • Understand the radiologic spectrum seen with schwannomas • Become familiar with the appearances and locations of schwannoma outside the neuraxis Table of Contents Introduction Cranial Nerves Spine Skull base / Neck Chest Abdomen Extremities / MSK Click to proceed directly to section of choice. Introduction • Concept of schwannoma involving cranial nerves is well known in the radiology literature • Benign, slow growing tumors arising from differentiated neoplastic Schwann cells of any cranial, peripheral, or autonomic nerves • Classic imaging appearance of sharply marginated enhancing mass with smooth enlargement of the bony foremen involved Frequency of Schwannomas • Intracranium – 95% vestibulocochlear nerve (CN VIII) • vestibular >> cochlea division – 1-5% other CNs 99% arise from cranial • Trigeminal nerve (CN V), most common nerve • CN IX > X > VII > XI > XII • CN III, IV, & VI more associated with neurofibromatosis 2 (NF 2) • Spine – <70-75% intradural extramedullary – 15% completely extradural – 15% both intra- and extradural (transforaminal, dumbbellshaped) CN VIII • • • Enhancing mass with fusiform (IAC) or “ice scream (CPA) on cone (IAC)” appearance Bilateral CPA-IAC metastases may be confused with vestibular schwannomas in NF2 Cystic component is more common in schwannoma than meningioma MR images reveal the IAC & CPA components of a vestibular schwannoma, which has increased T2 signal & heterogeneous enhancement. Residual enhancing tumor is evident within the IAC after this patient underwent resection. CN VIII Axial MR IAC imaging reveals a CPA-IAC cystic mass with heterogenous intermediate T2 signal & enhancement. There is associated mass effect on the brainstem, right middle cerebellar peduncle & 4th ventricle. Subsequent axial MR imaging post resection via right occipital craniectomy & fat grafting demonstrates residual enhancing tumor. Note the right cerebellar edema with high T2 signal & CT hypodensity. Cochlear Division of CN VIII Coronal MR T1W image through the IAC demonstrates a round, enhancing lesion in the inferior compartment of the left IAC. CN V Multiplanar MR imaging shows a large dumbbell-shaped, extra-axial mass with heterogenous high T2 signal & enhancement in the left superior CPA extending anteriorly into the Meckel’s cave. Note the dumbbell waist located at trigeminal porus. There is associated mass effect on the brainstem, cerebellum & 4th ventricle. Foci of gradient susceptibility indicate hemorrhages within the mass. An enlarged foramen ovale with smooth margin is evident on CT. CN VII Coronal MR T2W image through the IAC demonstrates a T2 hypointense mass in the superior compartment of the right IAC. This lesion shows avid enhancement with extension to the geniculate ganglion, an important distinguishing feature from CN VIII lesion. CN V1 Coronal & axial MR images demonstrate an orbital mass with heterogeneous high T2 signal & intense enhancement in the right superomedial extraconal space that displaces the globe & superior rectus muscle inferolaterally. This was proven to be schwannoma in the expected course of CN V1. CN III Axial & coronal MR imaging shows a tubular, intraconal orbital mass with low T1/high T2 signal & intense enhancement in the superior orbital fissure extending posteriorly into the cavernous sinus. Note the medial displacement of the tortuous CN II. CN IX, X, or XI • • • Fusiform, enhancing mass in an enlarged jugular foramen Lack of flow voids help differentiate from glomus jugulare paraganglioma (most common jugular foramen tumor) 90% present with SNHL clinically similar to vestibular schwannoma Heterogeneously enhancing mass with increased T2 signal & calcifications (rarely seen) appears to arise from the left jugular foramen & produces mass effect on the brainstem & effacement of the fourth ventricle. CN IX, X, or XI Nonvestibular schwannoma in another patient with heterogeneous low T1/high T2 signal & enhancement. This mass arises from the left cerebellomedullary cistern, which is inferior to the cerebellopontine cistern. Similar mass effect on the brainstem & effacement of the 4th ventricle is identified. Cervical Spine (C3) Solitary extradural schwannoma within the right C2-C3 neural foramen demonstrates predominantly hyperintense T2 signal, homogenous enhancement & neural foraminal enlargement. Cervical Spine (C5) Well circumscribed, “dumbbell shaped” mass within the expanded left C4-C5 neural foramen demonstrates heterogeneous low T1/high T2 signal & homogenous enhancement. There is spinal cord compression. The lesion also exerts mild mass effect & cause luminal narrowing of the left vertebral artery. Lumbar Spine (L3) A small intradural, extramedullary mass at the L3 level reveals T2 hypointensity & homogenously intense enhancement favoring schwannoma. Other consideration includes drop metastasis, ependymoma, & granuloma. The resected mass was proven to be schwannoma on histology. Lumbar Spine (L5) Mildly enhancing mass with intermediate T2/STIR signal involves the left L5 nerve root & extends into the left neural foramen without bony foraminal expansion. Lumbar Spine A large, expansile cystic intradural mass spanning T12 – S2 reveals low T1, high T2/STIR signal intensity & peripheral/internal enhancement. Note the enlarged spinal canal & osseous remodeling/scalloping of the vertebral bodies. Ethmoid Sinus Coronal & axial CECT images demonstrate a well-defined, homogenously enhancing mass causing smooth bony remodeling of the right ethmoid. A mucocele can have similar appearance but does not enhance uniformly. Nasal Cavity Multiplanar CECT images of the neck reveal a well-defined, mildly enhancing solid mass in the anterior nasal cavity causing rightward deviation of the nasal septum. Base of Tongue Axial & sagittal CECT of the neck reveals a rounded, wellcircumscribed heterogeneously enhancing mass arising from the base of the tongue resulting in near complete obliteration of the oropharyngeal airway. The mass demonstrates heterogeneously high T2/low T1 weighted signal, & heterogeneous enhancement. Recurrent Laryngeal Nerve (CN X) Axial & coronal CECT images through the subglottic region reveal an isodense mass in the right tracheoesophageal groove causing narrowing of the trachea & exerting mass effect on adjacent thyroid lobe. CN XI Multiplanar CECT neck images demonstrates an elongated, heterogenously enhancing lesion at left level II, underneath the sternocleidomastoid muscle in the expected location of CN XI, which was histologically diagnosed as schwannoma. By location, differential consideration is an enlarged lymph node. Posterior Cervical Space Multiplanar CECT neck images demonstrates a lobulated fusiform, peripherally enhancing lesion in the right posterior cervical space/spinal accessory chain, which was initially described as reactive level VA lymphadenopathy. Carotid Space Two suprahyoid carotid space schwannomas with peripheral enhancement & central hypodensity on multiplanar CECT neck. Heterogeneously high T2 signal & heterogeneous enhancement seen on MR raised concern for necrotic level IIB lymphadenopathy. Chest PA/Lateral radiographs reveal a rounded, wellcircumscribed extrapleural posterior mediastinal mass arising from the right paraspinal soft tissues at the level of the T6 vertebral body without osseous remodeling. Follow up cross-sectional imaging reveals a paraspinous mass with mild enhancement on MR as well as heterogenous intermediate T2 signal. The mass is mildly enhancing on CECT. Thoracic Wall Multiplanar CECT through the abdomen reveals large, well-defined, multiseptated, lobulated masses within the subpulmonic extrapleural space & soft tissues of the chest wall. There is mass effect upon the underlying liver with fat planes respected. Visceral Organ Coronal & axial abdominal CECT shows a large, well-defined soft tissue mass projecting into the lumen of the gastric fundus, along the greater curvature of the stomach. This was found to be schwannoma on endoscopic biopsy. Illiopsoas Compartment Sagittal & coronal MR imaging through the lower abdomen reveals a well-circumscribed, multiseptated predominately low T1/high T2 signal intensity lesion within the left iliopsoas muscle. Mildly enhancing rim/septations are seen within the mass. It is hypodense on NECT with irregular septations. Initial differential considerations included lymphangioma, cystic neoplasm, or less likely atypical synovial cyst extension from the hip joint. Psoas Muscle Coronal & axial abdominal CECT shows a hypodense, well circumscribed, elongated solid mass within the psoas muscle without invasion of the adjacent soft tissues. This mass demonstrates T2 hyperintensity & heterogenous enhancement on MR. Superficial Soft Tissue of Thigh Axial NECT imaging through the proximal thigh reveals a round, hypodense mass in the subcutaneous soft tissues. Axial fused PET/CT shows intense FDG activity. No significant internal vascularity is shown on Doppler. Hands A round mass in the ulnar soft tissues just medial to the left small finger MTP joint demonstrates low T1/high T2 signal intensity & homogenous enhancement. This mass appears separate from the underlying joint capsule but contiguous with the adjacent musculature. Similar lesion is also seen in the radial soft tissues of the right ring finger at the level of the P1 segment. Ankle A well circumscribed mass at the level of the posteromedial ankle joint shows hyperintense T2 signal & diffuse heterogenous enhancement. This mass abuts & medially displaces the posterior tibial neurovascular bundle. Ankle An intermediate T1/high T2 signal intensity, mildly enhancing mass lies within the tarsal tunnel at the level of the talus in the expected course of the tibial nerve. An additional partially visualized T2 hyperintense mass on sagittal view is also seen. Foot A rounded low T1/intermediate to high T2 signal intensity superficial soft tissue mass in the dorsum of the foot demonstrates avid post contrastenhancement. Initial differential considerations included complex epidermoid cyst, neural-based tumor, or less likely atypical fibroma, hematoma or giant cell tumor of tendon sheath. Summary • The following imaging findings suggest the diagnosis of schwannoma: mass lesion within the nerve or nerve sheath, smooth widening of the neural foramen, hypodensity on CT, T2 hyperintensity and enhancement. • Cystic changes are a common finding. • The above findings should suggest the diagnosis schwannoma even if the location is atypical. References • • • • • • • • • • • • Adani, Roberto, et al. "Schwannomas of the upper extremity: diagnosis and treatment." La Chirurgia degli organi di movimento 92.2 (2008): 85-88. Beaman, Francesca D., Mark J. Kransdorf, and David M. Menke. "Schwannoma: RadiologicPathologic Correlation 1." 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