Tumors of the Sellar Region Flashcards
Craniopharyngiomas are thought to develop from residual cells of Rathke’s pouch (craniopharyngeal duct) in the sellar region. These constitute 5 to 11% of pediatric brain tumors, which makes them the most common non-neuroepithelial intracerebral tumor in children. These tumors are considered be-
nign in histology and malignant in behavior. Why these tumors are malignant in behavior?
● A. Because of distant metastasis of these tumors
● B. Because of extensive damage to the bone in sellar region
● C. Due to involvement of neural tissue which makes them difficult to cure
● D. Because it often involves the floor of the third ventricle with resultant hydrocephalus
● E. Because of damage to optic apparatus
C. Due to involvement of neural tissue which makes them difficult to cure
Craniopharyngiomas, with bimodal age distribution (childhood peak age 5–15 years, adult peak age 45–60 years), and solid and cystic squamous epithelial tumor with stellate reticulum, wet keratin, and basal palisades, tend to arise from the superior anterior margin of pituitary. Which statement is incorrect regarding location and imaging of adamantinomatous craniopharyngiomas?
● A. Most occur in the region of sella turcica and pituitary infundibulum
● B. Most are purely sellar lesions, with rare extension into the suprasellar region
● C. Pure involvement of the third ventricle is more common with the papillary craniopharyngiomas
● D. 90% of adamantinomas exhibit at least two of the following C features: cyst formation, prominent calcifications, contrast enhancement in the cyst wall
● E. Fluid in the cyst varies but usually contains cholesterol crystals
B. Most are purely sellar lesions, with rare extension into the suprasellar region
Papillary craniopharyngioma (CP) constitutes 10% of all CPs and occurs almost exclusively in adults with a mean age of 40 to 55 years. These are less frequently cystic than adamantinomas and are most commonly located at which of the following?
● A. Intrasellar region
● B. Infundibular recess and tuber cinereum of the floor of the third ventricle
● C. Invade extensively into sphenoid sinus
● D. Cause destruction of surrounding bones and grow into these
● E. These extend extensively into the parasellar region
B. Infundibular recess and tuber cinereum of the floor of the third ventricle
Arterial feeders that supply the craniopharyngiomas arise from which artery?
● A. Anterior cerebral artery
● B. Anterior communicating artery
● C. Internal carotid artery
● D. Posterior communicating artery
● E. All of the above
E. All of the above
Which of the following statements is incorrect regarding treatment of craniopharyngioma?
● A. With microscope possible approaches through subfrontal and frontotemporal regions include: subchiasmatic (between optic nerves and anterior to chiasma), opticocarotid region, lamina terminalis, transfrontal–transsphenoidal with drilling of tuberculum sellae
● B. Alternatives to frontotemporal approaches include: pure transsphenoidal, transcallosal, combined subfrontal pterional approach and extended transnasal transsphenoidal endoscopic resection
● C. Postoperative radiotherapy (XRT) has no role in preventing regrowth when residual is left behind and also it does not cause endocrine dysfunction, optic neuritis, and dementia
● D. Steroids are given in physiologic doses in all patients as all of these are considered hypoadrenal
● E. Diabetes insipidus often shows up early may be as a part of triphasic response and treated initially with fluid replacement
C. Postoperative radiotherapy (XRT) has no role in preventing regrowth when residual is left behind and also it does not cause endocrine dysfunction, optic neuritis, and dementia
In most series, 5-year survival after craniopharyngioma (CP) operation is 55 to 85% with 5 to 10% mortality. What is the most cause of mortality in these patients?
● A. From injury to brain stem which causes long tract signs and cranial nerve palsies
● B. Damage to hypothalamus bilaterally which may lead to hyperthermia, somnolence, and loss of thirst sensation
● C. Postoperative malignant hydrocephalus
● D. Postoperative sudden brain herniation
● E. All of the above
B. Damage to hypothalamus bilaterally which may lead to hyperthermia, somnolence, and loss of thirst sensation
A patient presents with bitemporal hemianopia, hypopituitarism, headache, and diabetes insipidus. Imaging characteristics of these tumors are similar to that of adenomas with cure requiring gross total resection with a higher recurrence rate. These tumors include pituicytoma, granular cell tumor of the sellar region, and spindle cell oncocytoma. Where do these low-grade tumors originate from?
● A. Pituicytes of the neurohypophysis (posterior pituitary)
● B. Infundibulum/pituitary stalk
● C. Pituicytes of anterior pituitary
● D. Remnants of Rathke’s pouch
● E. Both A and B
E. Both A and B
Pituitary adenoma is a clonal neoplasm of anterior pituitary hormone producing cells which are also called as pituitary neuroendocrine tumors (PitNET). Following are included in the essential diagnostic criteria of pituitary adenoma/PitNET tumors except?
● A. Reticular fiber disruption
● B. Sellar suprasellar location
● C. Histologic features of low-grade neuroendocrine tumors that display destruction of the normal anterior gland acinar structure
● D. Subclassified based on immunoreactivity for pituitary hormones
● E. Subclassified on the basis of lineage-specific transcription factors
A. Reticular fiber disruption
A patient presents with progressive blindness, ophthalmoplegia, CSF rhinorrhea, and hyperprolactinemia in blood. On MRI pituitary tumor with invasion of the surrounding structures was seen with encasement of internal carotid artery and noncontiguous metastasis was seen categorizing this tumor as pituitary carcinoma. What are the suggested management options for invasive pituitary tumors?
● A. Initial surgery by a surgeon with extensive experience in pituitary surgery
● B. If Ki67 index comes out to be more than 3 then p53 immunodetection and mitotic count should be evaluated as potential predictors of tumor’s aggressiveness
● C. Repeat surgery should be considered for residual or recurrent tumors while XRT can also be given for clinically relevant tumor remnant with markers that suggest aggressive behavior
● D. Chemotherapy for tumors showing continued growth on Stupp regimen (temozolomide 150–200 mg/m2 for 5 consecutive days for 28 days with XRT and continued for 6 months if clinical response is evident)
● E. All of the above
E. All of the above
Which of the following is not included in the Hardy’s classification (anatomic classification) of invasive pituitary tumors?
● A. Extension includes suprasellar extension (0, A, B, and C) and parasellar extension (D and E)
● B. 0, A, B, and C denote no tumor extension, extension into suprasellar cistern, extension into anterior recess of the third ventricle, and extension into the floor of the third ventricle, respectively
● C. Invasion/spread includes spread into the floor, into the sphenoid sinus, and distant metastasis
● D. Spread/invasion is classified as 1, 2, 3, 4, and 5 which indicate no sellar expansion with tumor < 10 mm, sellar expansion with tumor > 10 mm, localized perforation of sellar floor, diffuse destruction of sellar floor, and spread via CSF or blood, respectively
● E. All of the above statements are correct
E. All of the above statements are correct
Which craniopharyngioma with epithelium that forms stellate reticulum, wet keratin, and basal palisades and frequently shows CTNNB1 mutations and aberrant nuclear expression of beta-catenin is now considered a distinct tumor differentiated
by gene mutation?
● A. Adamantinomatous
● B. Papillary
● C. Pseudopapillary
● D. Xanthochromic
● E. Malignant
A. Adamantinomatous
In contrast to adamantinomatous craniopharyngioma which of the following is a part of the diagnostic criteria of papillary craniopharyngioma?
● A. Absence of BRAF p. V600E mutation
● B. Immunoreactivity for BRAF p. V600E
● C. Presence of nuclear beta-catenin immunoreactivity
● D. Presence of CTNNB1 mutation
● E. Keratinizing mature squamous epithelium covering fibrovascular cores or a cyst wall
B. Immunoreactivity for BRAF p. V600E
All of the following vessels supply blood to craniopharyngioma except?
● A. ACA
● B. A-comm
● C. ICA
● D. P-comm
● E. Basilar artery (BA) bifurcation
E. Basilar artery (BA) bifurcation
Which of the following is the most common presenting symptom of infundibular tumors?
● A. Hypopituitarism
● B. Headache
● C. Visual field defect
● D. Diabetes insipidus
● E. Secondary amenorrhea
C. Visual field defect
According to Wilson’s anatomic classification for invasive pituitary tumors, what is the grade for localized perforation of the sellar floor?
● A. 1
● B. A
● C. B
● D. 3
● E. C
D. 3
