At the University Hospital, Hamburg, from 1980-85, 17 children had pineal region tumours among 102 children with CNS tumours. The authors conclude: (1) that the incidence in Germany is higher than assumed and at least equal to that in Japan, (2) radiation without histological confirmation is not justified, (3) surgical removal, successful in 10 cases, is reasonably safe, (4) cranio-spinal axis radiation, either post-operatively or alone, is indicated for germinomas that have a tendency for CSF ependymal proliferation and/or seeding, (5) all children treated successfully by both surgery and radiation have not relapsed. [1]
COMMENT: Pineal tumours are of 2 main types: (1) germinomas of the pineal or suprasellar region, and (2) pineal parenchymatous tumours (pineoblastomas in younger children and pineocytomas in older children and adults). Spinal seeding, more common with germinomas, occurs in 2-15% of pineal tumours.
Parinaud’s syndrome (impairments of upward gaze, convergence and accommodation) is the classical presentation, usually with signs of increased intracranial pressure. Visual loss, diabetes insipidus, precocious puberty, and emaciation point to anterior hypothalamic involvement. Precocious puberty only occurs in males. Suprasellar teratomas present with visual loss, hypopituitarism, and diabetes insipidus.
Calcification occurs in 25-75% of pineal area tumours, rarely in children under 10 years of age, and especially in germinomes of embryonal carcinomas. CT scan demonstrates location but may not differentiate type of tumour. Oncofetal antigens (human chorionic gonadotrophin and alpha-fetoprotein) in serum and spinal fluid was help in diagnosis and response to therapy.
Operative mortality and morbidity in the past have been high, Shunt surgery and/or biopsy followed by radiotherapy has a 50-80% survival rate. The present authors make a point for surgical removal and post-operative radiation. [2, 3, 4]