1. Intracranial Germ Cell Tumor
Presented by Int 李振豪

2. Case Presentation

3. Identification Name：穆XX Chart No.：11787255 Age：19 y/o Gender：Male
Admission date：2007/05/08

4. Past History Birth History: Hospitalization history
G1P1, GA: FT weeks, DOIC(-), PROM(-)
Feeding: On full diet
Vaccination: On schedule
Growth and Development:
BW: 69 Kg, BL: 178 cm
Developmental milestones: WNL
Hospitalization history
95/11/16~96/1/4 Small bowel ileus with peritonitis Central Diabeteius Insipidus
Allergy to Vancomycin
Family History: Non-contributory

5. Chief Complaints
Persisted headache, vomiting and drowsy consciousness for 3 days

6. Discharge with endocrinologist's OPD F/U
Present Illness [1/2]
1. Peritonitis with small bowel ileus s/p exp laparotomy + serosa repair of patchy necrosis + appendectomy
2. Hypernatremia with serum hyperosmolality and low urine osmolality(around 200)  susp. central diabetes insipidus
Elevation of prolactin (81.46) without prominent decreased level of thyroid hormone, GH,
and ACTH
Discharge with endocrinologist's OPD F/U
Brain MRI
2006/11/16
11/30
12/08
2007/01/04

7. Present Illness [2/2] Headache, fever, cough with N/V
 improved after symptomlytic agent under the impression of URI
Fever, headache with vomiting, drowsy consciousness  ER
 discharge after septic workup
Back to ER due to persisted fever, headache with vomiting, and drowsy consciousness
 Brain CT  Brain MRI(5/8)
Prolactin 62.5
Admission
2007/03/06
04/15
05/04
05/07
05/08

8. Physical Examination
Drowsy consciousness
Others unremarkable

9. Laboratory Data (5/10) 1. Biochemistry 2. Hemogram
CREA mg/dL | BUN H 23 mg/dL
OSMO H 351 mOsm/Kg | NA H 164 mmol/L
K mmol/L | CRP <7.0 mg/L
2. Hemogram
WBC H 14.6 K/cmm | Hb L 12.9 g/dl
Hct L 37.7 % | Pl K/cmm
Blast % | Pro %
Myelo % | Meta %
Band % | Seg H 86.2 %
Eos % | Baso %
Mono % | Lymph L 9.6 %

10. Operation (05/12)

11. Pathological Diagnosis
Brain, suprasellar, tumor excision: Yolk sac tumor
CSF (5/14)
βHCG 8.4 mIU/mL
AFP ng/ml

12. Discussion — Germ cell tumor

13. Classification

14. Germ Cell Tumors [1/4]
Involvement of midline ( 80% in pineal and 20% in suprasellar region) in the CNS, rarely in the roof of the 3rd ventricle
Childhood and young adult
All malignancy except mature teratoma
Metastasis via CSF and systemically

15. Germ Cell Tumors [2/4]
Parinaud’s syndrome = syndrome of the Sylvian aqueduct  convergence, accommodation and supranuclear upward gaze palsy with lid retraction
Precocious puberty only in boys with choriocarcinomas or germinoma with LH-like effects of βHCG
Drop seeding via CSF  radiculopathy or myelopathy
Headache, vomiting, lethargy, memory disturbance, seizure, precocious puberty, radiculopathy, myelopathy, abnormally increasing head circumference in infants

16. Germ Cell Tumors [3/4] CSF tumor marker βHCG AFP
Choriocarcinoma, germinoma(10%)
Cerebral mets of uterine/testicular choriocarcinoma
0.5~2% of serum HCG in non-CNS tumor
AFP
Yolk sac tumor, embryonal carcinoma, Teratoma (occasionally)
Cerebral mets of testicular tumor(16~25%)

17. Germ Cell Tumors [4/4] Treatment EVD for hydrocephalus
Stereotactic procedure
Diagnosis, treatment
Chemotherapy
Cisplatin, Etoposide
Radiation treatment
Germinoma, Post-op for other malignancy tumors
Craniospinal XRT
Surgical treatment
Radioresistant (30~35% of pineal region tumor)
Benign (e.g., meningioma, teratoma)
Well encapsulated
Malignant germ cell tumors without evidence of mets

21. Reference Harrison's Internal Medicine, 16th ed.
Adams and Victor's Principles of Neurology, 8th ed.
Handbook of Neurosurgery, 6th ed.
CT of Pineal Tumors and Intracranial Germ-Cell Tumors. Tsuen Chang et al. AJR 153: , Dec. 1989
MR Imaging of Pineal Tumors, Robert D. Tien et al., AJR 155: , July 1990
Pineal Region Masses: Differential Diagnosis. James G. Smirniotopoulos et al. Radiographics 1992; 12:
The Treatment of Cranial Germ Cell Tumours. A.A. Brandes et al., Cancer Treatment Reviews 2000; 26: 233–242

22. ~ The End ~

24. 2007/05/07 Brain CT
Susp. Germ cell tumor with suprasellar and pineal region involvement with acute bleeding and hydrocephalus

25. 2007/05/08 Brain MRI
2.9x2.9x3.1cm suprasellar tumor and 1.1x0.7x1.6cm pineal tumor with hydrocephalus, favor germinoma

26. Laboratory data (5/8) CORTI AM H 73.18 ug/dL 6.2-19.4
CORTI PM H ug/dL
TT L 4.41 ug/dL
LH L mU/mL
FSH. L mIU/mL 1-14
HGH ng/ml
PRL H ng/ml
ACTH <10.0 pg/ml
HCG mIU/mL
AFP. H ng/mL