Medulloblastoma: Malignant Brain Tumor in Children

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Questions and Answers

What is the primary focus of the research paper 'Genome sequencing of SHH medulloblastoma predicts genotype-related response to smoothened inhibition'?

  • Investigating the role of smoothened inhibition in medulloblastoma treatment
  • Identifying genomic characteristics of SHH medulloblastoma (correct)
  • Analyzing the prognosis of medulloblastoma patients
  • Examining the effect of chemotherapy on medulloblastoma treatment

In which part of the brain is medulloblastoma typically found?

  • Occipital lobe
  • Temporal lobe
  • Cerebellum or dorsal brainstem (correct)
  • Frontal lobe

What is the significance of the study by Rutkowski et al. (2010)?

  • It reported a prognostic factor for early childhood medulloblastoma (correct)
  • It identified a new genetic marker for medulloblastoma diagnosis
  • It found a correlation between medulloblastoma subtype and patient outcome
  • It compared the effectiveness of different chemotherapy regimens

What is medulloblastoma?

<p>A malignant small round cell tumor (B)</p> Signup and view all the answers

What is the title of the review article by Ramaswamy et al. (2016)?

<p>Risk stratification of childhood medulloblastoma in the molecular era (A)</p> Signup and view all the answers

What is the most common type of malignant CNS tumor in children?

<p>Medulloblastoma (C)</p> Signup and view all the answers

What is the theme of the study by Rutkowski et al. (2005)?

<p>Postoperative chemotherapy for early childhood medulloblastoma (A)</p> Signup and view all the answers

Which of the following genetic changes is associated with high-risk medulloblastoma?

<p>Chromosome 7 gain, chromosome 8 loss, and chromosome 11 loss (A)</p> Signup and view all the answers

What is a common theme among the referenced studies?

<p>Examining the molecular characteristics of medulloblastoma (A)</p> Signup and view all the answers

What is a common histopathological feature of medulloblastoma?

<p>Epitheloid ribboning and nuclear moulding (A)</p> Signup and view all the answers

What characteristic is shared by secretory, angiomatous, microcystic, and lymphoplasmacyte rich meningioma variants?

<p>Prominent peritumoral edema (A)</p> Signup and view all the answers

What is the diagnosis that would best fit the histology shown in the image?

<p>Meningioma (A)</p> Signup and view all the answers

Which of the following meningioma variants is characterized by prominent secretory activity?

<p>Secretory meningioma (B)</p> Signup and view all the answers

What is the appropriate set of immunostains to determine the diagnosis of the tumor?

<p>EMA, Vimentin, and SSTR2 (D)</p> Signup and view all the answers

What is the age of the patient with the intracranial tumor?

<p>57 years old (D)</p> Signup and view all the answers

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Study Notes

Medulloblastoma

  • A malignant small round cell tumor found in the cerebellum or dorsal brainstem
  • The most common malignant CNS tumor in children

Histopathological Features

  • Characterized by epitheloid ribboning and nuclear moulding of tumor cells
  • Reticulin stain highlights "pale islands" in desmoplastic medulloblastoma

Genetic Features

  • Group 3 and 4 can be separated into favorable and high-risk cases based on chromosome 7 gain, chromosome 8 loss, and chromosome 11 loss
  • i17q diploid cases are classified as high-risk

Subgroups and Classification

  • Histopathological and genetic classification is integrated to diagnose medulloblastoma
  • Subgroup-specific localization of human medulloblastoma can be based on pre-operative MRI

Genomic Analysis

  • Recurrent medulloblastomas may have secondary glioblastoma after radiotherapy
  • WNT-activated medulloblastomas may have secondary SHH pathway activation

Prognostic Factors and Treatment

  • Survival and prognostic factors of early childhood medulloblastoma include age, metastasis, and histology
  • Postoperative chemotherapy alone can be used to treat early childhood medulloblastoma
  • Risk stratification of childhood medulloblastoma can be done in the molecular era

Meningioma Diagnosis

  • A 57-year-old man presented with an intracranial convexity-based tumor requiring immunostains to determine the diagnosis.

Meningioma Variants

  • Low-grade meningioma variants that may have prominent peritumoral edema are: • Secretory • Angiomatous • Microcystic • Lymphoplasmacyte rich

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