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Table 1 Summary of clinical characteristics and clinical course of three cases

From: Pediatric-type high-grade gliomas with PDGFRA amplification in adult patients with Li-Fraumeni syndrome: clinical and molecular characterization of three cases

 

Case 1

Case 2

Case 3

Sex

Female

Male

Male

Age

43

45

32

Past medical history

Pituitary adenoma (36y)

Mandibular osteosarcoma (26y)

None

 

Left adrenal adenoma (36y)

Gastric cancer (42y)

 
 

Bilateral breast cancer (40y)

Colon cancer (44y)

 

Family history

Mother; breast cancer (38y)

Grand father; gastric cancer (70y)

Mother; bilateral breast cancer (32y)

Brother; brain tumor (17y)

 

Daughter; osteosarcoma (18y)

Grand mother; gastric cancer (50y)

 
 

Son; medulloblastoma (10y)

Aunt; pancreatic cancer (68y)

 

Symptom

Left hemiparesis

Gait disturbance

Left hemiparesis

 

Dysarthria

 

Left sensory disturbance

Tumor location

Left cerebellar peduncle

Right parietal lobe

Right pons

   

Right parietal lobe

Surgery

Biopsy

Subtotal resection

Partial resection

Adjuvant therapy

TMZ, BEV

TMZ

TMZ, TTF

 

Focal irradiation (60 Gy/30fr)

Focal irradiation (60 Gy/30fr)

Focal irradiation (60 Gy/30fr)

Salvage therapy

Best supportive care

Gross total removal

BEV

  

TMZ, BEV, TTF

Focal irradiation (20 Gy/5Fr)

PFS (months)

17.0

5.1

9.2

OS (months)

21.7

18.6

15.3