Desmoplastic astrocytoma: new insights into its clinical profile, diagnosis, and treatment.
BACKGROUND
Desmoplastic astrocytoma (DA) is a rare intracranial tumor which usually affects pediatric patients. The aim of this study is to describe the clinical features and management of DA based on a joint analysis of the cases reported in the scientific literature.


METHODS
A thorough review was carried out, gathering those pathologically proven DAs reported since the first description of this entity. Two new own cases were included in order to illustrate this review. Epidemiological, clinical, radiological, therapeutic, and follow-up data were analyzed with the software SPSS version 20.


RESULTS
A total of 52 DAs were recorded. Most cases occurred in the first 2 years of life, although older patients were also reported. Patients mainly presented symptoms and signs of elevated intracranial pressure. According to their radiological features, we were able to classify DAs in four main groups, with distinct differential diagnosis and prognosis. After treatment, 14.2 % of patients presented persistent neurological impairment and the mortality rate was close to 10 %.


CONCLUSIONS
DAs can be diagnosed at any age from birth to adolescence. These neoplasms can show up a wider range of radiological morphologies than previously thought. Surgery represents the treatment of choice for DAs, although chemotherapy can also be useful in the setting of recurrence or progression of the disease. Those DAs lacking classic radiological features, especially type 4 tumors, were linked with a poorer clinical outcome.
Department of Neurosurgery, Ramón y Cajal University Hospital, Ctra. de Colmenar Viejo Km 9,100, 7th floor, Madrid, 28034, Spain. luismarianorojas@gmail.com.|Department of Pediatric Neurosurgery, Virgen del Rocío University Hospital, Sevilla, Spain. luismarianorojas@gmail.com.
L M
LM
Rojas-Medina
Department of Neurosurgery, Ramón y Cajal University Hospital, Ctra. de Colmenar Viejo Km 9,100, 7th floor, Madrid, 28034, Spain.
R
R
Carrasco-Moro
Department of Pediatric Neurosurgery, Virgen del Rocío University Hospital, Sevilla, Spain.
M
M
Rivero-Garvía
Department of Neurology, Nuestra Señora del Rosario Hospital, Madrid, Spain.
L
L
Esteban-Fernández
Department of Pathology, Virgen del Rocío University Hospital, Sevilla, Spain.
E
E
Rivas-Infante
Department of Pediatric Neurosurgery, Virgen del Rocío University Hospital, Sevilla, Spain.
J
J
Márquez-Rivas
D000293:Adolescent; D000970:Antineoplastic Agents / Q000008:administration & dosage; D001254:Astrocytoma / Q000000981:diagnostic imaging* / Q000628:therapy*; D001932:Brain Neoplasms / Q000000981:diagnostic imaging* / Q000628:therapy*; D018288:Carcinoma, Small Cell / Q000000981:diagnostic imaging* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007427:Intracranial Pressure; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D019635:Neurosurgical Procedures / Q000379:methods; D016896:Treatment Outcome
D000970:Antineoplastic Agents
Desmoplastic astrocytoma; Infancy; Infantile neuroepithelial tumors