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Suprasellar
germ cell tumors (GCT): experience with this less common intracranial GCT at St.
Bartholomew's Hospital
Piers N
Plowman, David Tsang, Alexandra Taylor, Jerome St. George
St
Bartholomews Hospital, London, UK
Over
24 years, 11 patients with suprasellar GCT presented to SBH, 8 with
germinomatous and 3 with non-germinomatous GCT, M:F=6:5, a less male dominant
ratio than for pineal GCT, median age 20 years (6-49 years).
The median duration of symptoms had been 17 months (1-35 months).
Polyuria (10/11) and visual failure (6/11) were the commonest symptoms.
Diabetes insipidus occurred in all patients with partial or complete anterior
pituitary failure; hyperprolactinaemia was present in 9/11 patients.
Where biopsy was risky (optic chiasm) and markers negative, PET scanning helped
to narrow the diagnosis.
Since our introduction of CNS-friendly chemo-radiotherapy (Clin. Oncol 1997; 9:
48-53) our five year survival in 8 patients has been 100% and late morbidity
negligible.
The problem of simultaneous diabetes insipidus and thirst derangement makes
fluid balance management problematic during platinum based chemotherapy.
No patient recovered from previously abnormal pituitary function but 3/6
recovered some visual failure.
Our CNS-friendly chemo-radiotherapy (Clin Oncol 1992; 4: 345-50 and 351-4) will
cure the vast majority of cases of this rare disease and is relevant in that the
recent International Trial endorses the continuing need for radiotherapy (Proc
Am Soc Clin Oncol 2001; Abstr 204).
We stress the advantages of differential daily dosage radiotherapy (Brit J
Radiol 1991; 64: 603-7), the selected use of less than neuraxis radiotherapy,
the graded reduction in total radiation dosage, carboplatin for cis-platin and,
possibly, our recently described post-hoc radiation response modifier: gamma
linolenic acid (Proc Am Soc Clin Onocol 2001; Abstr. 260).
The diagnosis of this rare disease is frequently delayed and at least three
other childhood cases are currently the subject of litigation in the UK for this
reason (visual).
© Copyright 2002
American
Society of Clinical Oncology
Source:
http://www.asco.org/ac/1,1003,_12-002324-00_18-002002-00_19-002103-00_29-00A-00_42-00ONeill-00_43-00-00_44-00-00_45-00
Author-00_46-00Title-00_47-00Title-00_48-00and-00_49-00and,00.asp?cat=CNS+Tumors&parent=CENTRAL+NERVOUS+SYSTEM+TUMORS
&returnpid=2323
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