primary intracranial germinoma
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Epidemiology
- most commonly present in 2nd decade of life
- prevalent tumor type in suprasellar region, basal ganglia, thalamus
Pathology
- may be benign, but more often aggressive & invasive
- generally occur within or adjacent to the 3rd ventricle, including pineal region
Microscopic pathology
- histologically identical to testicular seminoma
- uniform cells resembling primitive germ cells
- large vesicular nuclei with prominant nucleoli
- relatively abundanty glycogen rich cytoplasm
- pattern may be sheets, lobules, cords in desmoplastic stroma
- lymphoid or lymphoplasmacytic infiltrates
- possibly scattered syncytiotrophoblastic giant cells
- mitoses present
- necrosis uncommon
Immunophenotype
- mostly positive:
- placental alkaline phosphatase: surface membrane more common
- variable:
Clinical manifestations
- hypothalamic-pituitary dysfunction
- visual field defects
- disturbances in memory or mood
- hydrocephalus
Radiology
- uniformly-enhancing lesions with or without well-defined borders
Management
- surgical excision is treatment of choice
- focal irradiation for non-resectable tumor
- cisplatin-based chemotherapy if radiation therapy not feasible
- tumors generally radiosensitive & chemosensitive
- prognosis is good, 5 year survival is > 85%