Extradural ependymal tumor
with myxopapillary and ependymoblastic differentiation in a case of
Schinzel-Giedion syndrome.Acta
Neuropathol. 2007 Mar;113(3):339-46.
Primary extradural
ependymomas are rare neoplasms usually of the myxopapillary type. Reports on
malignant primary extradural ependymal tumors are exceptionally rare. We
here report on a 3-year-old boy with Schinzel-Giedion syndrome (SGS), who
presented with lumbar spina bifida occulta and a progressive extraspinal
lesion in the subcutaneous sacrococcygeal region. Microscopic examinations
revealed an uncommon ependymal tumor with well-differentiated regions
reflecting myxopapillary ependymoma and highly anaplastic regions with
numerous mitoses, necroses, ependymal rosettes and ependymoblastic rosettes.
Final neuropathologic diagnosis was an extraspinal anaplastic ependymal
tumor with myxopapillary and ependymoblastic differentiation, corresponding
to WHO grade IV. SGS is a very rare disorder with a likely autosomal
recessive pattern of inheritance. So far, 42 cases have been reported, among
them 7 were diagnosed to have malignant neoplasms, including three malignant
sacrococcygeal teratomas, two sacrococcygeal primitive neuroectodermal
tumors (PNET), one hepatoblastoma and one malignant kidney tumor. The
present case is the first report on an ependymal tumor with a mixture of
myxopapillary, anaplastic and ependymoblastic features and the first report
on an ependymal tumor arising on the genetic background of SGS.
Sacrococcygeal extraspinal
myxopapillary ependymoma.Klin
Padiatr. 2006 Jul-Aug;218(4):243-5.
A 9-year-old boy was
admitted with a circumscript subcutaneous tumor of the sacral region. Serum
alpha-fetoprotein was not elevated. The lesion was excised en bloc including
the top of the coccygis. Histology revealed a myxopapillary ependymoma
without bony involvement. Metastases were excluded. Extraspinal
myxopapillary ependymoma is a rare tumor of the sacrococcygeal region with a
potential risk to develop metastases and for local relapse. Long-term
follow-up is recommended. |
