Craniospinal irradiation is employed in the treatment of:

A: OligodendrogliomaB: Pilocytic astrocytomaC: Mixed oligoastrocytomaD: Medulloblastoma

Correct Ans:DExplanation

Craniospinal irradiation is employed in the treatment of medulloblastoma.

Ref: Harrison’s Principles of Internal Medicine, 15th Edition, Page 2449

Which of the following tumor causes polycythemia due to increased erythropoietin production?

A: Cerebellar hemangioblastomaB: Medulloblastoma

C: EpendymomaD: OligodendrogliomaCorrect Ans:AExplanationSecondary erythrocytosis involves increased erythropoietin (EPO) signalling in the bone marrow. Several types of neoplasm are known to produce excess EPO, including renal cell carcinoma, uterine fibroids, hemangioblastoma, and hepatocellular carcinoma. EPO production can also be increased following renal transplant

 Ref: Verhovsek M., McFarlane A. (2012). Chapter 173. Abnormalities in Red Blood Cells. In G.V. Lawry, S.C. McKean, J. Matloff, J.J. Ross, D.D. Dressler, D.J. Brotman, J.S. Ginsberg (Eds), Principles and Practice of Hospital Medicine.  

A 50-year-old woman undergoes neurosurgery for resection of a well-circumscribed intracranial neoplasm attached to the dura. The tumor compressed the underlying brain parenchyma without infiltration. Which of the following is the most likely diagnosis?

A: Arteriovenous malformationB: Glioblastoma multiforme

C: MedulloblastomaD: Meningioma

Correct Ans:DExplanation

The gross features of this dural-based tumor are consistent with meningioma, the most frequent benign intracranial neoplasm. It arises from meningothelial cells and histologically consists of whorls of elongated cells with scattered psammoma bodies. The benign behavior of this tumor is apparent from its "pushing" pattern of growth. The tumor tends to expand downward, compressing the brain, but without invading it. For this reason, this tumor can be easily removed at surgery.

An arteriovenous malformation is a vascular aggregate of arteries, veins, and vessels with intermediate characteristics. These lesions are usually intracerebral and manifest with hemorrhage or seizures.

Glioblastoma multiforme is the most frequent malignant intracerebral tumor. It arises from neoplastic transformation of astrocytes within the white matter. Grossly, it is characterized by a variegated appearance, with areas of solid tumor alternating with necrosis and hemorrhage.

Medulloblastoma develops from the cerebellar vermis, usually in children. It is composed of small, primitive-appearing neoplastic cells.

A 10-year-old child with new onset of visual field abnormalities and diabetes insipidus undergoes a head CT. The CT scan demonstrates a 3cm mass lesion with focal calcification involving the area above the sella turcica. Needle biopsy of this mass shows that the tumor is composed of tissue resembling tooth enamel. Which of the following diagnoses is most likely?

A: CraniopharyngiomaB: Glioblastoma multiformeC: Large pituitary adenomaD: Medulloblastoma

Correct Ans:AExplanation

The tumor is a craniopharyngioma, alternatively known as an adamantinoma or ameloblastoma. Craniopharyngiomas may arise in, or more commonly above, the sella turcica. The histological pattern recapitulates the enamel organ of the tooth, with nests or cords of stratified squamous or columnar epithelium embedded in a loose fibrous stroma.

Calcification (and even metaplastic bone formation) is common in these benign tumors, which are thought to arise from vestigial remnants of Rathke's pouch.

Glioblastoma multiforme characteristically shows at least some enlarged cells with bizarre nuclei.

 Large pituitary adenomas contain nests of uniform glandular cells.

 Medulloblastoma is made of small basophilic cells with relatively large nuclei for their size.

 

Mutations in merlin protein is associated with:

A: AstrocytomaB: Meningioma

C: MedulloblastomaD: OligodendrogliomaCorrect Ans:BExplanationThe most common cytogenetic abnormality is loss of chromosome 22, especially the long arm (22q). The deletions include the region of 22q12 that harbors the NF2 gene, which encodes the protein merlin.

 Meningiomas are a common lesion in the setting of NF2.           Ref: Robbins 8th edition Chapter 28.

Which of the following tumors is associated with drop metastasis?

A: AstrocytomaB: EpendymomaC: MedulloblastomaD: OligodendrogliomaCorrect Ans:CExplanationAt the edges of the main tumor mass, medulloblastoma cells have a propensity to form linear chains of cells infiltrating through cerebellar cortex to aggregate beneath the pia, penetrate the pia, and seed into the subarachnoid space. Dissemination through the CSF is a common complication, presenting as nodular masses elsewhere in the CNS, including metastases to the cauda equina that are sometimes termed drop metastases.

 Ref: Robbins 8th edition Chapter 28.

Which among the following is the commonest type of intracranial tumour?

A: AstrocytomaB: MedulloblastomaC: MeningiomaD: Secondaries

Correct Ans:DExplanationSecondary metastatic deposits are more common than primary brain tumors in adults and the opposite is true in children. Brain metastases are three times more common than all primary brain tumors combined. 

 Must know:

Brain metastases arise from hematogenous spread and frequently arise from either a lung primary or are associated with pulmonary metastases. 

Most metastases develop at the gray matter–white matter junction in the watershed distribution of the brain where intravascular tumor cells lodge in terminal arterioles. 

The distribution of metastases in the brain approximates the proportion of blood flow such that about 85% of all metastases are supratentorial and 15% occur in the posterior fossa. 

The most common sources of brain metastases are lung and breast carcinomas; melanoma has the greatest propensity to metastasize to the brain, being found in 80% of patients at autopsy. 

Ovarian and esophageal carcinoma rarely metastasize to the brain.  Prostate and breast cancer also have a propensity to metastasize to the

dura and can mimic meningioma.  Leptomeningeal metastases are common from hematologic malignancies

and also breast and lung cancers.  Spinal cord compression primarily arises in patients with prostate and

breast cancer, tumors with a strong propensity to metastasize to the axial skeleton.

Ref: DeAngelis L.M., Wen P.Y. (2012). Chapter 379. Primary and Metastatic Tumors of the Nervous System. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo (Eds), Harrison's Principles of Internal Medicine, 18e.

A 3 year old boy is brought to the emergency department after the acute onset of headache, vomiting, nuchal rigidity, and impaired mental status. MRI reveals a posterior fossa tumor that fills the 4th ventricle. Surgery is immediately started, and intraoperative consultation leads to a "frozen section" diagnosis of medulloblastoma. Which of the following pathologic mechanisms most likely accounts for this child's clinical presentation?

A: Acute hemorrhage into the 4th ventricleB: Alteration of medullary function

C: Increased intracranial pressure

D: Infiltration of the cerebellar vermis by the neoplasmCorrect Ans:CExplanationAny tumor "filling the 4th ventricle" blocks the circulation of cerebrospinal fluid (CSF). This blockage leads to increased intracranial pressure, which manifests with nausea, vomiting, headache,nuchal rigidity, and mental status changes. If surgery is not performed promptly, cerebellar tonsillar herniation and rapid death will ensue. In children, medulloblastoma and ependymoma are the most frequent neoplasms presenting in this manner.

 There is no evidence in this case suggesting that acute hemorrhage into the 4th ventricular cavity has occurred, nor is medulloblastoma typically associated with this complication. CNS tumors that frequently bleed are metastases from melanoma, renal cell carcinoma, and choriocarcinoma.

 Alterations in medullary function lead to cardiorespiratory instability and may be caused by direct tumor compression or infiltration of the medulla, neither of which is supported by MRI findings in this case.

 Infiltration of the cerebellar vermis is certainly seen in many cases of medulloblastoma, a tumor that arises from this midline cerebellar structure. However, this would lead to truncal ataxia and gait instability, not symptoms of increased intracranial pressure.

 Ref: Ropper A.H., Samuels M.A. (2009). Chapter 30. Disturbances of Cerebrospinal Fluid and Its Circulation, Including Hydrocephalus, Pseudotumor Cerebri, and Low-Pressure Syndromes. In A.H. Ropper, M.A. Samuels (Eds), Adams and Victor's Principles of Neurology, 9e.

A newborn with congenital heart failure, which is not responding to treatment, has bulging anterior fontanelles with a bruit on auscultation. On trans fontanelle USG a hypoechoic midline mass is seen with dilated lateral ventricles. Most probable diagnosis is:

A: Vein of Galen malformationB: Arachnoid cystC: MedulloblastomaD: Encephalocele

Correct Ans:AExplanation

A neonate with fulminant CHF, bulging anterior fontanelles with USG showing hypoechoeic midline mass is with dilated lateral ventricles suggests a diagnosis of Vein of Galen Malformation.

Ref: Pediatric Brain And Spine: An Atlas Of MRI And Spectroscopy, Springer, 2005 – Pg 332, 333, 334; Neurosurgery Case Review: Questions and Answers, Thieme, 02-Dec-2009 – Pg 66, 67; Pediatric Neurovascular Disease: Surgical, Endovascular, and Medical Management, Thieme, 17-Oct-2005 – Pg 116, 117; Volpe’s neurology of newborn 3rd/802.

An 8 year old child presented with signs of increased intracranial tension. Imaging studies showed a growth in the brain. Which is the commonest tumor in a child in the posterior fossa of head?

A: AstrocytomaB: MedulloblastomaC: CraniopharyngiomaD: Meningioma

Correct Ans:BExplanationMedulloblastoma arises in the posterior part of the cerebellar vermis and neuroepithelial roof of the fourth ventricle in children. It accounts for 20 percent of childhood brain tumors.

 

 

 

Primitive neuroectodermal tumor is the most common type of medulloblastoma. Most occur in the first decade of life, but there is a second peak around age 30. Medulloblastoma is the most common malignant pediatric brain tumor. They are usually midline. Most occur in the cerebellum and present with symptoms of increased ICP.

 

 

 

Ref: Ropper A.H., Samuels M.A. (2009). Chapter 31. Intracranial Neoplasms and Paraneoplastic Disorders. In A.H. Ropper, M.A. Samuels (Eds), Adams and Victor's Principles of Neurology, 9e.

 

A newborn presents with congestive heart failure. On examination there is bulging anterior fontanelle with a bruit on auscultation. Transfontanellar USG shows a hypoechoic midline mass with dilated lateral ventricles. What is the MOST likely diagnosis?

A: Medulloblastoma

B: EncephaloceleC: Vein of Galen malformationD: Arachnoid cyst

Correct Ans:CExplanationThis newborn is suffering from vein of Galen malformation which often presents with features of hydrocephalus and congestive heart failure. Prenatal ultrasonography shows characteristic midline tubular anechoic structure superior to the thalamus which is contiguous with the dilated sagittal sinus (comet tail sign). Final diagnosis of this malformation is provided by cerebral angiography. 

 

 

 

The vein of Galen is a centrally located short venous structure formed by the confluence of the internal cerebral veins and the basal vein of Rosenthal. 

 

It empties into the straight sinus at its junction with the inferior sagittal sinus.

Structures drained by vein of Galen are thalamus, medial temporal lobe, occipital lobe and superior cerebellar vermis.

 

Ref: Nursing Care of the Pediatric Neurosurgery Patient page 47, Textbook of Neurosurgery edited by Prakash Narain Tandon, page 1105.

 

A 4 year old boy diagnosed of having a malignancy. On physical examination, he found to have aniridia. Which of the following tumor is associated with aniridia?

A: HepatoblastomaB: MedulloblastomaC: NephroblastomaD: Retinoblastoma

Correct Ans:CExplanationWilms Tumor (Nephroblastoma):

 

 

 

After neuroblastoma, this is the second most common abdominal tumor in children. Symptoms consist of abdominal enlargement, pain, hematuria, malaise, weakness, anorexia, weight loss and fever.

 

 

 

Malformations or syndromes associated with Wilms tumor:

 

Aniridia Hemihypertrophy Genitourinary malformations

o Cryptorchidismo Hypospadiaso Gonadal dysgenesiso Pseudohermaphroditismo Horseshoe kidney

Beckwith-Wiedemann syndrome Denys-Drash syndrome WAGR syndrome (Wilms tumor, aniridia, ambiguous genitalia, mental

retardation)

 

 

 

Ref: Graham D.K., Quinones R.R., Keating A.K., Maloney K., Foreman N.K., Giller R.H., Greffe B.S. (2012). Chapter 31. Neoplastic Disease. In W.W. Hay, Jr., M.J. Levin, R.R. Deterding, J.J. Ross, J.M. Sondheimer (Eds), CURRENT Diagnosis & Treatment: Pediatrics, 21e.

 

Which of the following malignancy is classified by Chang staging system?

A: RetinoblastomaB: RhabdomyosarcomaC: Ewings sarcomaD: MedulloblastomaCorrect Ans:DExplanationMedulloblastoma is the most common malignant pediatric brain tumor. They are usually midline. Most occur in the cerebellum and present with symptoms of increased ICP. Histologic characteristics include densely packed small round cells with large nuclei and scant cytoplasm. The Chang staging system is currently used to stage medulloblastomas; it categorizes the tumor by size and metastatic spread. They should undergo surgical resection followed by radiation therapy and chemotherapy.

 

 

 

Ref: Principles and Practice of Pediatric Neurosurgery edited by A. Leland Albright, P. David Adelson, Ian F. Pollack, 2008, Page 607.

 

Which of the following brain tumors does not metastasize via the Cerebrospinal fluid (CSF)?

A: Germ cell tumorsB: Medulloblastoma

C: CNS LymphomaD: CraniopharyngiomaCorrect Ans:DExplanation

Craniopharyngiomas are rare, usually suprasellar, partially calcified, solid or mixed solid-cystic benign tumors that arise from remnants of Rathke's pouch. They do not metastasise.

They have a bimodal distribution, occurring predominantly in children but also between the ages of 55 and 65 years. They present with headaches, visual impairment, and impaired growth in children and hypopituitarism in adults. Treatment involves surgery, radiotherapy, or the combination of the two.

Ref: Harrison’s Internal Medicine, 18th Edition, Page 2271 ; Scwartz’s Principles of Surgery, 9th Edition, Chapter 42 ; Williams Hematology, 8th Edition, Chapter 97

Which of the following is the most common type of Glial tumors?

A: AstrocytomasB: MedulloblastomasC: NeurofibromasD: Ependymomas

Correct Ans:AExplanation

Astrocytomas are the most common type of glial tumor. So astrocytomas is the single best answer of choice.

Ref: Harrison's Principles of Internal Medicine, 16th Edition, Page 2453;  Microneurosurgery By Mahmut Gazi Yasargil, Chad D, Page 95.

Which of the following brain tumors does not metastasize via the Cerebrospinal fluid (CSF)?

A: Germ cell tumorsB: Medulloblastoma

C: CNS LymphomaD: CraniopharyngiomaCorrect Ans:DExplanation

Craniopharyngiomas are rare, usually suprasellar, partially calcified, solid or mixed solid-cystic benign tumors that arise from remnants of Rathke's pouch. They do not metastasise.

They have a bimodal distribution, occurring predominantly in children but also between the ages of 55 and 65 years. They present with headaches, visual impairment, and impaired growth in children and hypopituitarism in adults. Treatment involves surgery, radiotherapy, or the combination of the two.

Ref: Harrison’s Internal Medicine, 18th Edition, Page 2271 ; Scwartz’s Principles of Surgery, 9th Edition, Chapter 42 ; Williams Hematology, 8th Edition, Chapter 97

Which of the following tumors has got the best prognosis?

A: Cerebellar pilocytic astrocytomaB: Grade IV astrocytomaC: MedulloblastomaD: Ependymoma

Correct Ans:AExplanation

Cerebellar pilocytic astrocytoma is Grade - I astrocytoma  and is associated with best prognosis. Grade - IV astrocytoma is Glioblastoma multiforme and has worst prognosis.

Grade - II astrocytoma is low-grade (fibrillary) astrocytoma, mixed oligoastrocytoma. They are relatively slow growing astrocytomas. These patients have 5 yr survival rate of 34% without treatment and 70% with radiation therapy.

Grade - III astrocytoma is anaplastic astrocytoma. These patients often related to seizures, neurologic deficits, headaches, or changes in mental status. Individuals with grade 3 astrocytoma have a median survival time of 18 months with treatment (radiation and chemotherapy).

Which of the following intracranial tumors has the best prognosis?

A: GlioblastomaB: Cerebellar astrocytomaC: MedulloblastomaD: Ependymoma

Correct Ans:BExplanation

Cerebellar astrocytoma is the most common type of posterior fossa tumor in childhood and has an excellent prognosis following surgical removal. Next to cerebellar

astrocytoma, medulloblastoma is the most frequent tumor involving posterior cranial fossa structures and has a poor prognosis for recovery.

Which of the following is the most frequent primary malignant tumor of the CNS?

A: Glioblastoma multiformeB: Medulloblastoma

C: MeningiomaD: OligodendrogliomaCorrect Ans:AExplanationGlioblastoma multiforme (GBM) is the most frequent primary malignant tumor of the CNS. It usually affects middle-aged or elderly patients and most frequently arises in the cerebral white matter. GBM is a malignant astrocytoma; it is referred to as grade IV astrocytoma in the WHO classification and grade 4 astrocytoma in the St. Anne-Mayo grading system. This tumor is very aggressive; median survival is approximately 12-14 months following optimal treatment, ie, a combination of surgery and radiation therapy.

 Medulloblastoma, a much less frequent malignant tumor, affects children; it arises in the posterior fossa in this population. The histogenetic origin of this tumor is uncertain, although it is thought to develop from immature cell precursors that give rise to glial and neuronal cells.

 Meningioma and pituitary adenoma are frequent but benign brain tumors that arise from meningothelial cells and endocrine cells of the adenohypophysis, respectively.Oligodendrogliomas constitute 5% of all primary brain tumors. Although most oligodendrogliomas have benign histologic features (e.g., low mitotic rate and mild nuclear atypia), their complete surgical excision is virtually impossible owing to a diffuse, poorly circumscribed pattern of growth. This tumor allows a much longer survival (up to 7-10 years) than GBM, but eventually transforms into a malignant glioma indistinguishable from GBM.

 Ref: Ropper A.H., Samuels M.A. (2009). Chapter 31. Intracranial Neoplasms and Paraneoplastic Disorders. In A.H. Ropper, M.A. Samuels (Eds), Adams and Victor's Principles of Neurology, 9e.

 

Aniridia is associated with:

A: Retinoblastoma

B: Hepatoblastoma

C: Nephroblastoma

D: MedulloblastomaCorrect Ans:CExplanationAniridia and Wilm's tumor (nephroblastoma) are components of a rare genetic syndrome - WAGR syndrome W - Wilm's tumor A - Aniridia G - Genitourinary problems R - Mental Retardation Aniridia is a condition of complete or partial iris hypoplasia and foveal hypoplasia, resulting in reduced visual acuity and nystagmus. It may be found in isolation or in association with other syndromes. PAX6 is present on band p13 of chromosome 11, and its mutation results in aniridia. Syndromes associated with Aniridia: Miller syndrome Aniridia associated wilm's tumor WAGR syndrome Wilm's tumor, aniridia, genital abnormalities and mental retardation Gillespie’s syndrome Aniridia, mental retardation and cerebellar ataxia Axenfeld–Rieger syndrome Iris hypoplasia, corectopia and polycoria. Ref: Yanoff & Duker’s Opthalmology, 3rd Edition, Page 1208. 

Which is the second common malignancy in patients with retinoblastoma?

A: OsteosarcomaB: Ewing's sarcomaC: MedulloblastomaD: Osteoblastoma

Correct Ans:AExplanation

Heritable germ line cases of retinoblastoma develop bilateral tumours and multifocal tumours, and carry a predisposition to develop tumours most notably pinealoblastomas and osteosarcomas.

Retinoblastoma is most common primary intraocular malignanacy of childhood.

It is characterized by Flexner winterstiener rosette, homer - wright rosette

Leucocoria or cat's reflex is most common manifestation. Strabismus is second most common manifestation. Predisposing gene is 13ql4