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primary intracranial germinoma
Epidemiology:
-> most commonly present in 2nd decade of life
-> prevalent tumor type in suprasellar region, basal ganglia, thalamus
Clinical manifestations:
1) hypothalamic-pituitary dysfunction
-> diabetes insipidus
2) visual field defects
3) disturbances in memory or mood
4) hydrocephalus
Pathology:
1) may be benign, but more often aggressive & invasive
2) generally occur within or adjacent to the 3rd ventricle, including pineal region
Histopathology:
1) histologically identical to testicular seminoma
2) uniform cells resembling primitive germ cells
3) large vesicular nuclei with prominant nucleoli
4) relatively abundanty glycogen rich cytoplasm
5) pattern may be sheets, lobules, cords in desmoplastic stroma
6) lymphoid or lymphoplasmacytic infiltrates
7) possibly scattered syncytiotrophoblastic giant cells
8) mitoses present
9) necrosis uncommon
Immunopathology:
- mostly positive:
- placental alkaline phosphatase: surface membrane more common
- variable:
- beta-HCG
- human placental lactogen
- cytokeratins
Radiology:
-> uniformly-enhancing lesions with or without well-defined borders
Management:
1) surgical excision is treatment of choice
2) focal irradiation for non-resectable tumor
3) cisplatin-based chemotherapy if radiation therapy not feasible
4) tumors generally radiosensitive & chemosensitive
5) prognosis is good, 5 year survival is > 85%
General
germinoma
primary intracranial germ cell neoplasm
References
- WHO Classification Tumours of the Nervous System.
Kleihues & Cavenee eds. IARC Press 2000
- Harrison's Online, Chapter 370, McGraw-Hill, 2002