|
|
Intracranial Germ Cell Tumors: A Single
Institution Experience and Review of the Literature
A.A. Smith, E. Weng, M. Handler, N.K. Foreman
Department of Pediatric Oncology, The
Children's Hospital (A.A.S.); Department of Radiation Therapy (E.W.), Department
of Neurosurgery (M.H.), Department of Pediatrics (N.K.F.), Division of Pediatric
Oncology, University of Colorado Health Sciences Center; Denver, CO, USA
There is little literature to guide therapy in children and young adults
with intracranial germ cell tumors.
We present 17 consecutively diagnosed intracranial germ cell tumors at The
Children's Hospital, Denver, from 1995 to 2001.
Of 17 patients, 3 had considerable delay in diagnosis.
Two with suprasellar tumors presented with dementia, blindness and
pan-hypopituitarism and another with recurrent subarachnoid hemorrhage.
Seven had germinoma, three were metastatic at diagnosis.
Ten had non-germinomatous germ cell tumors (NGGCT), 5/10 were alpha feto-protein
(AFP) positive only, one β-human
chorionic growth (βHCG) factor positive only, 3 positive for AFP and βHCG,
and 1 malignant teratoma.
Therapy for metastatic patients consisted of chemotherapy followed by
craniospinal radiation (CSI).
Patients with localized disease received chemotherapy followed by focal
radiation.
Two patients received chemotherapy only, one because she died of sepsis while
receiving chemotherapy and one because of neurologic injury incurred during
surgery parents elected for no therapy.
Three patients have died, one of tumor recurrence, one from a remote
complication of surgery and one of sepsis.
Twelve patients are alive without evidence of disease from 10 to 68 months
(median 31.5 months).
All five children with only AFP positivity, treated with chemotherapy and focal
radiation are alive without evidence of disease at 10, 16, 22, 41 and 41
months.
Thus, there is little evidence that CSI is necessary in non-metastatic
germinomas and AFP positive NGGCTs when combined chemotherapy and radiation
therapy is used.
However, complications of delayed diagnosis, surgery and chemotherapy are
important causes of mortality, with only one patient dying of tumor.
Keywords: chemotherapy, germinoma, intracranial, non-germinomatous germ cell tumor,
radiation
Copyright
©
2004 Kluwer Academic Publishers.
All rights reserved
Source: http://journals.kluweronline.com/article.asp?PIPS=5138410
|
