13 yo girl with LGG

Hospital: Dharmais NCC MSBTG Case #: -

Age: 13 yo
Presenter: Fatimah Saidah
Gender: F
Attending Physician: Mururul Aisyi
Length to Diagnosis from initial Sx: 3 months
Presentation Date: March 8 th 2024

Previously Presented Date (if applicable): N/A

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 Initial Presentation
History of Illness / Symptoms:
• January 2023: Recurrent headache  CT scan: SOL with hydrocephalus  referred to PON hospital

PON hospital
• VP shunt placement 1st March 2023
• Removal tumor 13th April 2023
• Histopathology: pilocytic astrocytoma WHO grade I
• MRI post surgery July 2023: residual mass 3.4 x 3.2 x 3.7 cm (previously 5.4 x 5.5 x 5. cm)
• Referred to Dharmais NCC

Physical Findings:
No history of seizure, no motoric deficit, no cranial nerve palsy, good vision
No clinical sign of NF

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CSF Cytology
• N/A

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Tumour Characteristics
Pathology:
• pilocytic astrocytoma WHO grade I

Molecular Biology:
N/A
(e.g. IDH mutation, RELA fusion, BRAF alterations,
H3K27M mutation, MGMT methylation)

Risk Group Assignment:

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Surgery
Surgical procedure (CSF diversion/ tumour biopsy/ tumour resection):
• VP shunt placement 1st March 2023
• Removal tumor 13th April 2023

Extent of Resection (biopsy only / Complications / Morbidities:

subtotal / near total / gross total):
Susbtotal resection

Postop MRI (not done / done on which

postoperative day): 3 months post op
MRI post surgery July 2023: residual mass
3.4 x 3.2 x 3.7 cm (previously 5.4 x 5.5 x 5.
cm)

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Chemotherapy
Overall Course: Complications / Morbidities:
Not done

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Radiation Therapy
Overall Course: Complications / Morbidities:
Not done

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Pathology review
Pathology:
• The preparation shows that the tissue in the form of brain parenchymal tissue contains spots of
calcification.
• At the edges there is a tumor that almost entirely shows a 'clear cell' appearance with nuclei that tend
to have mild anisocytosis (round-ovoid cell nuclei).
• Between the tumors there are many blood vessels, some of which suggest an increase in collagen
components in the perivasculature.
• Tumor cell mitosis is difficult to find.

Conclusion:
• Review preparation (location information: midcerebellum)
• Histology is more suggestive of a 'Clear cell meningioma' (CNS WHO grade 2).
• Immunohistochemical examination is required to confirm the diagnosis/ exclude the possibility of an
Astrocytoma: EMA, Vimentin, GFAP, Ki67.

• Note: Is there a history of Meningioma in the family? Because 'Clear cell meningioma' in children is one
of the 'Genetic tumor syndromes' ('Clear cell meningioma predisposition syndrome'/CCMPS)
• Immunohistochemical examination to confirm CCMPS (SMARCE-1) is not yet available.
Summary/Plan
pLGG post surgery (STR) April 2023 with residual mass, good clinical
presentation

MRI review:
• Not progressive, can be extra-axial

Pathology review:
• Histology is more suggestive of a 'Clear cell meningioma' (CNS WHO
grade 2).
• IHC in process

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Questions to be Discussed
Diagnostic
Treatment plan

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