Intracranial undifferentiated malign neuroglial tumor in Smith-Lemli-Opitz syndrome: A theory of a possible predisposing factor for primary brain tumors via a case report

Aslan, Ayfer; BÖRCEK, ALP; Pamukcuoglu, Selma; Baykaner, M.

doi:10.1007/s00381-016-3214-z

Intracranial undifferentiated malign neuroglial tumor in Smith-Lemli-Opitz syndrome: A theory of a possible predisposing factor for primary brain tumors via a case report

Aslan A., BÖRCEK A. Ö., Pamukcuoglu S., Baykaner M. K.

CHILDS NERVOUS SYSTEM, cilt.33, sa.1, ss.171-177, 2017 (SCI-Expanded, Scopus)

Yayın Türü: Makale / Tam Makale
Cilt numarası: 33 Sayı: 1
Basım Tarihi: 2017
Doi Numarası: 10.1007/s00381-016-3214-z
Dergi Adı: CHILDS NERVOUS SYSTEM
Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
Sayfa Sayıları: ss.171-177
Anahtar Kelimeler: Brain surgery, Intracranial undifferentiated malignant tumor, Smith-Lemli-Opitz syndrome, Sonic Hedgehog signaling pathway, SONIC HEDGEHOG, ANIMAL DEVELOPMENT, CELLS, MEDULLOBLASTOMA, MORPHOGENESIS, ACTIVATION, SUPPRESSOR, G-ALPHA(S), DISORDERS, PHENOTYPE
Gazi Üniversitesi Adresli: Evet

Özet

Smith-Lemli-Opitz Syndrome (SLOS) is a rare hereditary autosomal recessive disorder with broken cholesterol synthesis causing by 7-dehydrocholesterol reductase deficiency. Although the clinical features and pathogenesis is well-defined, it is unknown whether there is a relationship between SLOS and neoplastic processes, especially brain neoplasms.