Mesenchymal, Nonmeningothelial Tumors Flashcards
A patient presented in neurosurgery OPD with brain imaging showing meningioma-like features. Surgery was done and histopathology showed variably cellular tumor composed of spindled to ovoid cells arranged around a branching and hyalinized vasculature and variable stromal collagen deposition and STAT6 nuclear expression. This tumor has a high rate of recurrence as late as decades. What is the most likely diagnosis?
● A. Atypical meningioma
● B. Pleomorphic astrocytoma
● C. Solitary fibrous tumor
● D. Carcinomatous fibrous tumor
● E. None of the above
C. Solitary fibrous tumor
A patient is found to have tumor with tightly packed capillary-sized and cavernous vessels with a single layer of benign epithelial cells. This tumor is found most commonly in the spine followed by skull and uncommonly in the brain parenchyma, nerve roots, and cauda equina with a predilection for thoracic and lumbar region (often multiple). Following are the imaging findings in a patient with hemangioma except?
● A. Polka-dot sign is found on axial images
● B. Corduroy pattern is found on longitudinal images
● C. Calcifications are very rare in these tumors
● D. T2 MRI shows mixed signal internally
● E. The surrounding hemosiderin ring is low density on T2 with blooming artifact
C. Calcifications are very rare in these tumors
A patient presents with nausea, vomiting, headache, cerebellar signs, and obstructive hydrocephalus. CT of brain showed low density cystic lesion with contrast enhancing nodule in the posterior fossa, MRI showed serpentine vascular signal voids with cystic lesion, and blood workup showed polycythemia. These tumors are most common primary intra-axial tumors in the adult posterior fossa and 30% of these occur as part of Von
Hippel-Lindau (VHL) disease. What is the most likely diagnosis in this patient?
● A. Hemangioma
● B. Pilocytic astrocytoma
● C. Epidermoid tumor
● D. Hemangioblastoma
● E. Dermoid tumor
D. Hemangioblastoma
Which of the following statements is incorrect regarding treatment of hemangioblastoma?
● A. Gross total resection with removal of mural nodule and leaving cyst wall is optimal treatment (unless there is evidence of thickened wall cysts with tumor on MRI or direct visualization during surgery)
● B. Preoperative embolization may help reduce the vascularity
● C. Prognosis is excellent following GTR and is better for cases with VHL than for sporadic cases
● D. In case of cystic brainstem hemangioblastoma, solid tumor of the nodule is removed under microscope
● E. Radiotherapy is helpful for multiple small deep lesions, inoperable brainstem hemangioblastomas, for patients who are not surgical candidate, although it does not prevent regrowth following subtotal excision
C. Prognosis is excellent following GTR and is better for cases with VHL than for sporadic cases
A 50-year-old male patient presents with backache, pain in legs, sphincter disturbance, and nerve root symptoms. CT and MRI show centrally located bony destruction with an anterior soft tissue mass (with small calcifications) which is confined anteriorly by soft presacral fascia. This lesion is hypointense on
T1, hyperintense on T2, and contrast enhancing. A firm fixed mass is palpable between rectum and sacrum on rectal examination. What is the most likely diagnosis in this patient?
● A. Spinal chordoma
● B. Chondrosarcoma
● C. Osteosarcoma
● D. Osteochondroma
● E. Osteoblastoma
A. Spinal chordoma
Chordomas are rare tumors which arise from remnant of the primitive notochord (which normally differentiates into the nucleus pulposus of the intervertebral disks). Histologically these tumors are considered low grade but still why are these primary malignant tumors?
● A. Difficulty of total removal
● B. A high recurrence rate of 85% following surgery
● C. They metastasize in 5 to 20% of cases
● D. Locally aggressive and osteo-destructive
● E. All of the above
E. All of the above
Four types of chordomas have been identified, namely, conventional, chondroid, dedifferentiated, and poorly differentiated. Which statement is incorrect regarding location of chordoma?
● A. Chordoma can arise anywhere along entire the neuraxis where there is remnant of notochord
● B. 35% occur cranially in the spheno-occipital region (clivus)
● C. 53% occur in the spine at the sacrococcygeal region
● D. 3% are intracranial tumors while 1% are primary spine tumors
● E. Less commonly, these may occur in the spine above sacrum
D. 3% are intracranial tumors while 1% are primary spine tumors
What is the most appropriate management options regarding chordomas?
● A. For chordomas caudal to the third sacral segment, a posterior approach with wide en block excision and postoperative radiation is usually the best option
● B. Decompression is best avoided because entering the mass serves to spread the tumor (surgically induced metastasis) which will then regrow
● C. Chordomas located in the C2 are usually not amenable to en bloc resection
● D. If s2 nerve roots are the most caudal nerve roots spared, there is 50% chance of normal bladder and bowel function while above this, most will have impaired bladder control and bowel problems
● E. All of the above
E. All of the above
Which statement is correct regarding radiotherapy in chordoma patients?
● A. Radiotherapy does prevent recurrence when done in conjunction with palliative or debulking surgery
● B. It is indicated only for residual, recurrent, or inoperable cases
● C. Best results are obtained only with en bloc excision combined with high dose radiotherapy
● D. Proton beam therapy alone or combined with high-energy X-ray photon therapy is more effective than conventional radiotherapy and is readily available
● E. Early radiation has no effect on longer survival
C. Best results are obtained only with en bloc excision combined with high dose radiotherapy
Chemotherapeutic agent imatinib (a tyrosine kinase inhibitor) has some antitumor effect in chordoma. Which subtype of chordoma has worse prognosis?
● A. Dedifferentiated
● B. Poorly differentiated
● C. Conventional
● D. Chondroid
● E. Both A and B
E. Both A and B
Radiologically, a solitary fibrous tumor may be mistaken for which tumor?
● A. Astrocytoma
● B. Meningioma
● C. Osteosarcoma
● D. Fibrous dysplasia
● E. Oligodendroglioma
B. Meningioma
What is the characteristic of WHO grade 3 solitary fibrous tumor?
● A. Hypocellular tumor, < 2.5 mitosis/10 HPF
● B. Dense tumor, > 2.5 mitosis/mm2 (> 5 mitosis/10 HPF)
● C. Dense tumor, > 5 mitosis/mm2 (> 10 mitosis/10 HPF)
● D. > 2.5 mitosis/mm2 (> 5 mitosis/10 HPF) with tumoral necrosis
● E. Dense tumor, > 5 mitosis/mm2 (> 10 mitosis/10 HPF) with tumoral necrosis
D. > 2.5 mitosis/mm2 (> 5 mitosis/10 HPF) with tumoral necrosis
Which of the following is the most common site of hemangiomas?
● A. Skull
● B. Spine
● C. Brain parenchyma
● D. Nerve roots
● E. Cauda equina
B. Spine
Which of the following is the most common primary intraaxial tumor in the adult posterior fossa?
● A. Pilocytic astrocytoma
● B. Ependymoma
● C. Choroid plexus papilloma
● D. Hemangioblastoma
● E. Medulloblastoma
D. Hemangioblastoma
If the cases of intracerebral hemorrhage (ICH) are carefully examined, which tumor is likely to be found as an underlying cause?
● A. AVM
● B. Hemangiomas
● C. Hemangioblastoma
● D. Cavernoma
● E. Hemangioblastoma
C. Hemangioblastoma
