Alpha-fetoprotein secretion in a craniopharyngioma. Are craniopharyngiomas part of the germ cell tumor family?
A 14-months-old girl was admitted to our hospital because of excessive irritability and abnormal eye movements over the last two months. Brain CT and MRI revealed a suprasellar cystic and partially solid mass with calcifications. The laboratory investigation revealed increased serum levels of AFP. These findings were suggestive for a brain germ cell tumor. Therefore, systemic chemotherapy was started. After two courses there was a reduction in the levels of AFP but the tumor size remained unchanged. Subtotal tumor excision was performed that revealed the presence of a craniopharyngioma. One month later there was enlargement of the cystic part of the tumor, while serum AFP was elevated. The child received again systemic chemotherapy and placement of a reservoir into the cystic part of the tumor. Analysis of the intracystic flouid revealed the presence of beta-HCG and AFP. Following that the patient received brachytherapy with intracavity yttrium placement. Three months later repeated MRI showed a decrease in the size of the cystic part, while the solid part remained unchanged. Thus, the solid part was treated by radiosurgery. One year later the patient was stable but with complete loss of vision. These observations support the theory of a germ cell tumor family, in which craniopharyngioma and germ cell tumor present the two sides of the same entity, while between them a wide variety of tumors, with variable type of secretion of AFP and/or beta-HCG, may exist.
First Department of Pediatrics, Athens University Medical School, Greece.
Maria
M
Moschovi
George A
GA
Alexiou
Anastasia
A
Dastamani
Kalliopi
K
Stefanaki
Neofytos
N
Prodromou
Hristiana
H
Hatzigiorgi
Kalliopi
K
Karamolegou
Fotini
F
Tzortzatou-Stathopoulou
D003397:Craniopharyngioma / Q000145:classification / Q000000981:diagnostic imaging / Q000378:metabolism; D005260:Female; D006801:Humans; D007223:Infant; D009373:Neoplasms, Germ Cell and Embryonal / Q000145:classification*; D010911:Pituitary Neoplasms / Q000145:classification / Q000000981:diagnostic imaging / Q000378:metabolism; D014057:Tomography, X-Ray Computed; D000509:alpha-Fetoproteins / Q000378:metabolism*
C097353:AFP protein, human; D000509:alpha-Fetoproteins