Cranial and Paraspinal Nerve Tumors Flashcards
Schwannoma (WHO grade 1) is a benign nerve sheath tumor exclusively or primarily comprised of differentiated neoplastic Schwann cells with peak incidence in the fourth to sixth decades of life. Which statement is correct regarding location of
these tumors?
● A. They commonly arise from peripheral nerves in the skin and subcutaneous tissue of the head and neck, or on the flexor surfaces of the limbs
● B. Intracranially they frequently involve the vestibular division of cranial nerve eight
● C. Intracranially these can also arise from cranial nerve five or other cranial nerves
● D. In the spine, these tumors form dumbbell shaped tumors as they extend out through the neural foramen which constricts them
● E. All of the above
E. All of the above
A 10-year-old patient presents with multiple cutaneous lesions that are typically soft, mobile, and asymptomatic. On histopathology, these tumors are found to be composed of neoplastic Schwann cells interspersed with normal cells and infiltrative low cellularity spindle cell neoplasm that is associated
with a variably myxoid to collagenous stroma with mixed cell population. What is the most likely diagnosis in this patient?
● A. Schwannomatosis
● B. Neurofibroma
● C. Perineuroma
● D. Neurofibromatosis type 1
● E. Hybrid nerve sheath tumors
B. Neurofibroma
A perineuroma is a benign WHO grade 1 tumor which is comprised exclusively of neoplastic perineural cells. Soft tissue perineuroma arises in deep soft tissues and presents as a soft tissue mass with nonspecific symptoms. Which of the following is not included in the essential diagnostic criteria of perineuroma?
● A. Slender spindle cells with bipolar cytoplasmic processes
● B. Storiform and/or whorled architecture
● C. Positive for at least one perineural antigen (EMA, claudin-1, GLUT1)
● D. Always associated with nerve fiber
● E. Negative for S100
D. Always associated with nerve fiber
Hybrid nerve sheath tumors are also known as benign peripheral nerve sheath tumor not otherwise specified (NOS). On histopathology, there are intermingled features of two types of benign nerve sheath tumors and immunohistochemical staining for each component. What is the most common type of hybrid nerve sheath tumor?
● A. Neurofibroma/schwannoma
● B. Schwannoma/perineuroma
● C. Neurofibroma/perineuroma
● D. Schwannoma/meningioma
● E. None of the above
B. Schwannoma/perineuroma
Malignant melanocytic nerve sheath tumors are aggressive tumors that usually arise from a spinal or autonomic nerve. What neoplastic cells are found in this tumor?
● A. Cells with melanocytic features
● B. A + Schwann cells
● C. A + Neurofibroma cells
● D. A + Astrocytes
● E. A + Meningeal cells
B. A + Schwann cells
Malignant peripheral nerve sheath tumors originate from peripheral nerve or a pre-existing benign nerve sheath tumor or in patient with NF-1 (approximately 50% are associated with NF-1). Following are features of this tumor which are associated with a favorable prognosis except?
● A. Smaller tumor size
● B. Female gender and Hispanic white race
● C. No metastasis at presentation
● D. GTR
● E. No association with prior XRT
B. Female gender and Hispanic white race
A patient presents with low-back pain and radiculopathy. A surgery is performed, and the tumor attached to filum terminale is excised. On preoperative imaging, it showed serpiginous dilated and ectatic blood vessels with a cap sign. On histology, it showed uniform, round or polygonal chief cells congregate in lobules (Zellballen) encompassed by a barely discernible single layer of susentacular (type 2) cells. What is the most probable diagnosis in this patient?
● A. Schwannoma
● B. Myxopapillary ependymoma
● C. Cauda equina neuroendocrine tumor
● D. Neurofibroma
● E. Malignant filum terminale neuroma
C. Cauda equina neuroendocrine tumor
A 35-year-old patient presents with decreasing hearing in right ear, tinnitus in the same ear, difficulty in maintaining balance, abnormal corneal reflex, and nystagmus. Weber test was found lateralizing to the left ear. Further testing showed
pure tone audiogram threshold to be less than or equal to 50%, while speech discrimination score was more than or equal to 50% (serviceable hearing). MRI of brain showed oval enhancing tumor centered on IAC and trumpeting sign which was 2.2 cm in size and extended to the brainstem surface without displacing it. What is the Koos grading of this tumor?
● A. Grade 1
● B. Grade 2
● C. Grade 3
● D. Grade 4
● E. Grade 5
C. Grade 3
A patient presents with facial nerve palsy. On examination, this patient has obvious but not disfiguring asymmetry of face, slight to moderate motion of forehead, complete closure of eye with effort, and slightly weak mouth with maximal effort.
What is the House and Brackmann facial nerve grading in this patient?
● A. Grade 1
● B. Grade 2
● C. Grade 3
● D. Grade 4
● E. Grade 5
C. Grade 3
A patient presents with vestibular schwannoma in your clinic. What are the treatment options in this patient?
● A. Surgery through translabyrinthine approach
● B. Surgery through retrosigmoid approach
● C. Surgery through subtemporal approach
● D. Stereotactic radiosurgery
● E. All of the above
E. All of the above
All of the following are desirable for diagnosis of schwannoma except?
● A. Verocay bodies
● B. Subcapsular lymphocytes
● C. Hyalinized blood vessels
● D. Lattice-like pattern of CD34 staining
● E. Loss of SMARCB1 expression
D. Lattice-like pattern of CD34 staining
What is the most common subtype of hybrid nerve sheath tumors?
● A. Schwannoma/perineurioma
● B. Neurofibroma/schwannoma
● C. Neurofibroma/perineurioma
A. Schwannoma/perineurioma
Which of the following features is not associated with a favorable prognosis for MPNST?
● A. Smaller tumor size
● B. Female gender
● C. GTR
● D. No mets at presentation
● E. No association with prior XRT
B. Female gender
Which of the following tumors of cauda equina demonstrate dilated ectatic blood vessels with a cap sign on T2MRI or GRASS MRI?
● A. Myxopapillary ependymoma
● B. Astrocytoma
● C. Cavernoma
● D. Cauda equina NET
● E. AVM
D. Cauda equina NET
A 45-year-old female presented in OPD department with sudden hearing loss (SHL). Treatment options of SHL include all of the following except?
● A. 60-mg prednisone QID × 10 days
● B. Famciclovir 500 mg TID × 10 days
● C. Intravenous heparin
● D. Conservative
● E. Thrombolytic therapy
B. Famciclovir 500 mg TID × 10 days
