The patient is a 21 year old who received a kidney transplant more than 10 years ago.
The patient is a 20 year old with an intraventricular tumor
This patient presented in his 20s with 2 months of progressive headaches and was found to have a right cerebellar cystic mass.
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Hemangioblastoma (WHO grade I):
"Uncertain" Histogenesis
• Various cell lineages such as vascular, glial, neural, fibrohistiocytic, and smooth muscle/myofibroblastic have been proposed for the so-called stromal cells, which are thought to represent the neoplastic component of these lesions
- Neg for endothelial markers
- CD133, VEGF positive, Epo/EpoR coexpression'
- GFAP positive cells may result from phagocytosis
• The VHL gene is expressed particularly in Purkinje cells of the cerebellum (mutations may be inherited or somatic).
• VHL mutation may lead to developmental arrest of angioblasts. Pluripotent neoplastic angioblasts may be the cell of origin.
• Possible origin is 'angiomesenchyme' which may give rise to three cell types (stromal cells, pericytes, and endothelial cells).
Clinical Features
- 25 % occur in VHL Patients, 75 % occur sporadicly
- Locations : Cerebellum , brain stem,Spinal cord, Supretentorial sites more frequently in VHL patients
-Symptoms are associated with increased ICP, Secondary erythrocytosis secondary to EPO production
-MRI shows cystic lesion with enhancement
Histopathology
- Tumors consisting of numerous vessels intermixed with stromal cells
- Vessels have hyperplastic endothelial cells and pericytes
- Stromal cells have abundant lipid-rich clear cytoplasm with slight pleomorphic nuclei
-Rare mitosis
-Cyst wall may show reacive astrocytosis with Rosenthal Fibers
Immunohistochemistry
-Stromal cells are positive for: Vimentin,S100,Oil Red O
-Stromal cells are negative for :CD34,FVIII,CK,EMA,GFAP
DDX
-Metastatic Renal Cell CA: Non-cystic,Abundant mitosis,EMA,Vimentin and CK positive
-Pilocytic Astrocytoma: Fibrillary astrocytes, GFAP diffusely positive
The patient is a 17 month old female with a cystic insular and temporal lobe lesion. There is some enhancement.
Fibrohistiocytic neoplasms
Intracranial fibromatosis
Fibroblastic meningioma
Fibrosarcoma
Desmoplastic infantile ganglioglioma (DIG)
- WHO grade I, glial or glioneuronal superficial tumors with prominent desmoplasia.
- Present in the first 2 years of life
- Supratentorium: Frontoparietal
- Symtoms of increased ICP
Histopathology
- Distinctive features include collagenized regions dominated by spindly astrocytes in fascicular or storiform array and admixed small cells of primitive appearance in nodular aggregates.
Immunohistochemistry
- GFAP-positive astroglial cells within the desmoplastic regions
- Synaptophysin and Neurofilament positive neuronal cells in non-collagenized areas
- Small cell populations can be positive for GFAP, Neuronal markers ( Synaptophysin, Beta-tubulin, NF, MAP-2
DDX
This patient had a history of worsening bilateral leg numbness and was found with neuroimaging to have an extra-medullary, intradural thoracic mass.
The patient was a 35 year old woman who apparently suffered a cardiac event at home. She arrived at the hospital obtunded with minimal brainstem reflexes. Two days after admission she developed V-fib and became completely unresponsive. An MRI at this time showed severe anoxic changes. She was pronounced dead 12 days after the initial event.
This 2 year old was noted to have a head tilt for 1 week. A well-circumscribed, enhancing lesion near the foramina of Luschka displaced the pons.
Continue reading Ependymoblastoma.
The patient is a 5 year old who had a meningioma involving the cavernous sinus at age 2 1/2. The tumor recurred within 2 years and has not responded well to radiation therapy.
Clear cell meningioma:
Histology: Solid, clear glycogen rich cells, round and innocuous nuclei, inconspicuous nucleoli, lack mitotic activity, distinctive hyalinised stroma.
Immunohistochemistry: EMA +ve,Vimentin +ve, Inhibin -ve,GFAP -ve , PAS +ve diastase sensitive material.High MIB-1 LI accounts for the high-recurrence rate.
DDX:Differential diagnoses include microcystic meningioma, hemangioblastoma, clear cell ependymoma and metastatic carcinoma.
References: Greenfield's Neuropathology, Sixth Edition
1: Clear cell meningioma, an uncommon variant of meningioma: a clinicopathologic study of nine cases. J. Neurooncol. 2007 Feb;81(3):315-21.
- Incidence: 0.2 % of all meningiomas, rarest variant
- WHO Grade II
- Age: Younger patients
- Sex:Female predilection in Ped CCMs
- Locations: Cerebellopontine angle and Intraspinal
- Recurrence: High rate, up to 61%
Histology: Solid, clear glycogen rich cells, round and innocuous nuclei, inconspicuous nucleoli, lack mitotic activity, distinctive hyalinised stroma.
Immunohistochemistry: EMA +ve,Vimentin +ve, Inhibin -ve,GFAP -ve , PAS +ve diastase sensitive material.High MIB-1 LI accounts for the high-recurrence rate.
DDX:Differential diagnoses include microcystic meningioma, hemangioblastoma, clear cell ependymoma and metastatic carcinoma.
References: Greenfield's Neuropathology, Sixth Edition
1: Clear cell meningioma, an uncommon variant of meningioma: a clinicopathologic study of nine cases. J. Neurooncol. 2007 Feb;81(3):315-21.
