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Case Study 50

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Case Study 50 Edward D. Plowey Discussion Angiocentric glioma occurs mostly in pediatric patients and young adults, but has been reported in elderly adults, and is ... – PowerPoint PPT presentation

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Title: Case Study 50


1
Case Study 50
  • Edward D. Plowey

2
Case History
  • The patient is a 2 year old girl with normal
    birth and developmental histories who presented
    with new onset seizures. A video EEG study
    confirmed complex partial seizures arising from
    the left frontal lobe.
  • The patient underwent an MRI of the brain.

3
Question 1
  • Describe the radiologic abnormality on the
    following MRI.

4
(No Transcript)
5
Answer
  • There is a 4 cm left cingulate gyrus lesion with
    heterogenous T2 FLAIR and T1 signal abnormalities
    involving the cortex and subcortical white
    matter. The FLAIR abnormality focally involves
    the corpus callosum and crosses the midline. The
    lesion is surrounded by subtle edema and exerts
    mild mass effect that expands the left cingulate
    gyrus and displaces the falx to the right but
    shows no significant effect on the left frontal
    horn. No clear contrast enhancement is seen.
  • With involvement of the motor strip and the
    corpus callosum, the lesion was deemed
    unresectable. A stereotactic needle biopsy was
    performed.

6
Question 2
  • What is the differential diagnosis of this
    non-enhancing flair lesion in a 2 year old child?

7
Answer
  • The differential diagnosis includes
  • dysembryoplastic neuroepithelial tumour
  • ganglioglioma
  • diffuse glioma, low grade
  • Glioneuronal hamartoma
  • The radiologic features are less favorable for
    pleomorphic xanthoastrocytoma and
    meningioangiomatosis.

8
Question 3
  • Describe the findings and render a diagnosis for
    the following intraoperative consultation smear
    preparation.

9
Answer
  • The intraoperative smear preparation shows a
    glial neoplasm comprised of spindled cells with
    delicate bipolar cytoplasmic processes. The
    nuclear chromatin is coarsely granular. There is
    prominent gliovascular structuring. There are
    several entrapped normal-appearing neurons.
    However, high grade features (mitotic activity,
    endothelial hyperplasia, necrosis) are not seen.
  • Intraoperative Consultation Diagnosis
  • A. Neoplastic.
  • B. Low grade glioma.

10
Question 4
  • Describe the findings on the HE stained
    permanent sections of the stereotactic biopsy
    material.
  • Click here to view virtual slide.

11
Answer
  • Histologic sections demonstrate a glial neoplasm
    with prominent gliovascular structuring around
    vessels of variable caliber. A couple of
    perivascular pseudorosettes are seen in the
    largest fragment. The glioma cells show spindle
    nuclei with granular chromatin. No mitotic
    figures are seen. Endothelial hyperplasia and
    necrosis are not seen. There are numerous
    entrapped mature neurons in the tumor. No
    binucleate neurons or phenotypically
    indeterminate cells are seen.

12
Question 5
  • What immunostains will you order to further
    characterize this neoplasm?

13
Answer
  • GFAP (click hyperlink to view the virtual slide)
  • EMA (click hyperlink to view the virtual slide)
  • Ki67 (click hyperlink to view the virtual slide)

14
Question 6
  • What further information to these immunostains
    convey?

15
Answer
  • A GFAP immunostain shows reactivity in tumor cell
    processes of perivascular pseudorosettes and in
    reactive glial processes. An EMA immunostain
    shows perivascular dot-like, microlumen-type
    immunoreactivity in some of the tumor cells. A
    Ki67 immunostain confirms a low proliferative
    index of less that 1.

16
Question 7
  • What is the final diagnosis?

17
Answer
  • Diagnosis ANGIOCENTRIC GLIOMA, WHO GRADE 1.

18
Discussion
  • Angiocentric glioma is new and rare entity in the
    WHO Classification of Tumors of the Central
    Nervous System (2007). Angiocentric glioma
    usually follows a benign clinical course. Most
    reported cases have been treated with gross total
    or subtotal resection a couple of cases have
    been treated with radiation. In some reported
    cases, excision of the lesion eliminated
    seizures. Only 1 reported case has shown
    recurrence and progression to a high grade glioma
    following resection.
  • Wang et al. J Neuropathol Exp Neurol. 64875-81,
    2005.
  • Preusser M et al. Am J Surg Pathol. 311709-18,
    2007.
  • The histogenesis of angiocentric glioma, which
    shows ependymal features, is unknown. One group
    of authors has suggested a radial glia origin
    (Preusser et al, 2007).

19
Discussion
  • Angiocentric glioma occurs mostly in pediatric
    patients and young adults, but has been reported
    in elderly adults, and is usually associated with
    medically intractable epilepsy.
  • A similar case of a frontoparietal angiocentric
    glioma in a 2 year old child with seizures has
    been previously reported (J Child Neurol.
    24852-6, 2009.
  • A recent retrospective study by Prayson
    (Pathology. 42426-31, 2010) estimates that
    angiocentric glioma underlies approximately 2 of
    tumor-associated medically intractable seizures
    in pediatric patients.
  • Given the non-resectable nature of the tumor in
    this case, the patient has undergone radiation
    therapy. A 3 month interval MRI evaluation
    demonstrated stable to a slight decrease in tumor
    size.
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