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Rosette-Forming Glioneural tumor of the fourth ventricle

A case report and literature review

Sarah A. Bin Abdulqader, Salwa Al-Shibani, Wafa Alshawakeer and Gmaan Alzhrani
Neurosciences Journal July 2021, 26 (3) 284-288; DOI: https://doi.org/10.17712/nsj.2021.3.20200163

Article Figures & Data

Figures

  • Figure 1
    Figure 1

    - Timeline showing the clinical course of the patient and outcomes.

  • Figure 2
    Figure 2

    - Pre-operative images. A and B) posterior fossa mass with coarse calcifications and a cystic component as well as hydrocephalus demonstrated on CT scan. C and H) T1-weighted post contrast MRI showing a hypointense lesion with heterogenous enhancement with gadolinium. D and I) Axial and sagittal T2-weighted MRI demonstrating heterogenous, hyperintense lesion in the fourth ventricle. E) Diffusion weighted images showing a large heterogeneous cystic lesion centered within the fourth ventricle compressing the adjacent structures with no significant restricted diffusion. F) Susceptibility-weighted images showing multiple areas of susceptibility effects in the lesion corresponding to calcification with some areas representing hemorrhage. G) The lesion again seen demonstrating heterogeneous signal intensity with adjacent small edema in the cerebellum on Fluid attenuated inversion recovery images (FLAIR). J-L) Postoperative MRI obtained at 6-month postoperatively showing no evidence of recurrence.

  • Figure 3
    Figure 3

    - Histopathological and immunohistochemical findings of rosette-forming glioneuronal tumors, A) A smear shows true rosettes embedded in a loose fibrillary background, B) Hematoxylin and eosin (H-E) staining shows a ring like neurocytic rosette arranged around a neuropil core, C) Pilocytic astrocytoma like component exhibiting rosenthal fibers, D) Oligodendrocyte-like cells with perinuclear halo. (E) Neurocytic rosettes are positive for synaptophysin, f, g) GFAP and S100 showing positive staining in the pilocytic astrocytoma area, H) Prominent hyalinization in the glial area.

Tables

  • Table 1

    - Summary of CNS tumors with Rosette formation.

    Tumor typeAgeLocationMorphologyPositive antibodies
    Ependymoma GII, GIIIChildren/ young adultsWall of the ventricles Spinal canal-Uniform round to oval cells with salt and pepper chromatin.- Perivascular pseudorosettes >trueGFAP S100 EMA dot and ring
    AstroblastomaChildren/ young adultsCerebrum- Astroblastic pseudorosettes- “stout” - (not fibrillar) processes - Prominent vascular hyalinization.GFAP S100EMA focal cytoplasmic or dotlike
    Rosettes forming glioneuronal tumor G1Young adults4th ventricle CerebellumBiphasic:-- Neuronal component: small uniform cells forming neurocytic true or pseudorossettes.– Glial component: pilocytic astrocytoma or oligodendroglioma likeNeurocytic rosettes: SynaptophysinNeuNGlial component:GFAP S100
    Medulloblastoma GIVChildrenCerebellum- Small round blue cell tumor- Brisk mitotic activity- Prominent karyorrhexis- Homer Wright rosettesSynaptophysin B-catenin CMYC P53 YAPGAB
    Embryonal tumor with multilayered rosettes / NOS GIVChildrenCerebrum Brain stem Cerebellum3 histological patterns in ETMR:- Embryonal tumor with abundant neuropil and true rosettes (ETANTR)– Medulloepithelioma– EpendymoblastomaSynaptophysin C19MC altered
    Pineoblastoma GIVChildrenPineal gland- Small round blue cell tumor-Homer Wright rosettes- Flexner–Wintersteiner rosettesSynaptophysin
    Pituitary adenomaAdultsPituitary gland- Uniform nuclear morphology- Abundant cytoplasm- Perivascular rosettes - PapillaeSynaptophysin Pit. Hormones Transcription factor
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