TY - JOUR
T1 - Intermediate pilomyxoid astrocytoma and diencephalic syndrome
T2 - imaging findings.
AU - Nakamura, Olavo Kyosen
AU - Pinho, Marco da Cunha
AU - Odone Filho, Vicente
AU - Rosemberg, Sergio
PY - 2012/1/1
Y1 - 2012/1/1
N2 - Pilomyxoid astrocytoma, an entity described as a histological variant of pilocytic astrocytoma, is a rare primary tumor of the central nervous system. It is usually located in the hypothalamic-chiasmatic area, affecting children with a mean age of 10 months. It has a high rate of recurrence and cerebrospinal fluid dissemination, which may be present throughout the neuroaxis. Due to its topography, it may present developmental delay in childhood and diencephalic syndrome, characterized by extreme weight loss, lack of fat accumulation, hyperactivity, euphoria and alertness. Magnetic resonance imaging has an important role in its diagnosis, staging and follow-up of pilomyxoid astrocytoma. However, for a definitive diagnosis, anatomopathology is particularly important to differentiate it from pilocytic astrocytoma. Some cases, as in this present one, have simultaneous histological features of pilocytic and pilomyxoid astrocytomas, constituting a group called intermediate pilomyxoid astrocytoma. Surgery is the best treatment option and it usually requires adjuvant therapy.
AB - Pilomyxoid astrocytoma, an entity described as a histological variant of pilocytic astrocytoma, is a rare primary tumor of the central nervous system. It is usually located in the hypothalamic-chiasmatic area, affecting children with a mean age of 10 months. It has a high rate of recurrence and cerebrospinal fluid dissemination, which may be present throughout the neuroaxis. Due to its topography, it may present developmental delay in childhood and diencephalic syndrome, characterized by extreme weight loss, lack of fat accumulation, hyperactivity, euphoria and alertness. Magnetic resonance imaging has an important role in its diagnosis, staging and follow-up of pilomyxoid astrocytoma. However, for a definitive diagnosis, anatomopathology is particularly important to differentiate it from pilocytic astrocytoma. Some cases, as in this present one, have simultaneous histological features of pilocytic and pilomyxoid astrocytomas, constituting a group called intermediate pilomyxoid astrocytoma. Surgery is the best treatment option and it usually requires adjuvant therapy.
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U2 - 10.1590/S1679-45082012000200020
DO - 10.1590/S1679-45082012000200020
M3 - Article
C2 - 23052462
AN - SCOPUS:84891421696
SN - 1679-4508
VL - 10
SP - 236
EP - 238
JO - Einstein (São Paulo, Brazil)
JF - Einstein (São Paulo, Brazil)
IS - 2
ER -