Pathology and Radiology: Oncology Flashcards
(188 cards)
1
Q
What is a traumatic neuroma?
What is often a cause?
What is a common example?
How do they feel?
A
- Tangle of axons, Schwann cells, and fibroblasts in a collagen matrix as a result of injury to a nerve
- Surgery is a common cause (e.g. oral surgery)
- Morton neuroma
- Painful and rubbery
2
Q
Schwann cells are derived from which cell type?
What disorder are schwannomas associated with?
A
Neural crest cells
NF2
3
Q
What are the common nerve sites for Schwannomas to form intracranially?
A
Superior vestibular nerve where entering IAM
Trigeminal nerve
4
Q
What types of nerves do spinal schwannomas form on?
In rare cases where there is an intra-axial schwannoma where did it likely form?
A
- Spinal sensory nerve roots
- Perivascular nerves
5
Q
What are the two histological patterns seen with Schwannomas?
A
Antoni A (Compact, dense reticular cells, fusiform cells, and collagen matrix)
Antoni B (Loose, stellate cells with stoma surrounding)
6
Q
What are Verocay bodies and in what peripheral nerve sheath tumor are they seen in?
A
Anuclear material with palisading cells in Antoni A areas, seen in Schwannomas
7
Q
Schwannomas stain positive for what on IHC?
A
S100
Name is from its solubility in 100% saturated ammonium sulfate at neutral pH
Family of multigenic group of nonubiquitous cytoplasmic homologous intracellular Ca2+ binding proteins
Marker of schwann cells and melanocytes; useful for evaluating nerve sheath tumors and melanoma
Differentiate benign nerve sheath tumors (S100 strong and diffuse) from malignant peripheral nerve sheath tumors (typically weak or negative S100)
Help differentiate between schwannoma (stains all cells) and neurofibroma (mixture of positive and negative cells)
Distinguish Langerhans cell histiocytosis from other histiocytosis syndromes
8
Q
How do Schwannomas appear on T1 and enhanced MRI?
A
T1: hypointense to isointense
They do enhance
Rare for them to have calcification
9
Q
Do neurofibromas occur intracranially?
Definition of NF
A
No
10
Q
What cell types make up neurofibromas?
A
Scwann cells and fibroblasts in conjunction with collagen and reticulin
11
Q
What do cutaneous neurofibromas stain positive for on IHC?
A
S100, Vimentin, Leu7
Occasionally GFAP
12
Q
Describe the characteristics of cutaneous neurofibromas. What disease are they associated with and do they often undergo malignant transformation?
A
Dermal or subcutaneous, painless, and unencapsulated lesions. Assd with NF1 and rarely malignantly transform
Path: Loose, wavy nuclei in an axonal matrix detected by silver stain
Aka vonRecklinghausen, Ch17
13
Q
Describe the characteristics of intraneural neurofibromas. What disease are they associated with and do they often undergo malignant transformation?
A
Involve large nerve trunks and occasionally plexi (i.e. plexiform neurofibromas). Assd with NF1 and may undergo malignant transformation
14
Q
What rare peripheral nerve sheath tumor may cause a motor mononeuropathy in adolescents? What types of cells make it up and what does it stain for on IHC?
A
Perineuroma
Perineural cells
EMA positive, S100 negative
15
Q
What peripheral nerve sheath tumor is painful, involves proximal nerves, and demonstrates a high recurrence rate with pathology noting high cellularity, mitotic figures, and necrosis?
A
Malignant peripheral nerve sheath tumor
16
Q
Where are colloid cysts normally located?
What is thought to be their cellular origin?
What are the cysts filled with?
Does the cyst enhance at all?
What is a good size threshold above which to consider surgery?
A
- Roof of third ventricle between columns of fornices
- Endodermal, from paraphysis
- Mucous (mucopolysaccharides)
- Yes, just the rim which is usually a single layer of Goblet cells
- > 7 mm
17
Q
What causes a neuroepithelial cyst?
Where are they commonly located?
A
Infolding of developing neuroectoderm
Located in ependyma, choroid plexus, and choroidal fissure
18
Q
What are neurenteric cysts filled with?
What is their thought developmental origin?
A
Filled with endoderm of GI/respiratory mucosa, have interspersed Goblet cells
Fusion of notochord and gut
19
Q
What is cavum septum pellucidum?
A
The cavum septum pellucidum (CSP) is a potential cavity between the membranous leaves of the septum pellucidum, separated by at least 1 mm, and is considered a normal anatomical variation.
1.es kép
20
Q
What is cavum vergae?
A
A posterior communication of cavum septum pellucidum
2.es kép
21
Q
What is cavum velum interpositum?
A
A cavum in 3rd ventricle due to failed fusion of tela choroidea
3.as kép
22
Q
In what disease does a chloroma form? What part of the intracranium does it usually affect?
What is the appropriate treatment?
A
Leukemia, may affect the leptomeninges and is thus safe guarded from chemo by the BBB
If LP is positive then patients should received prophylactic radiation and IT chemo with MTX
23
Q
Where is Histicytosis X (Langerhans cell histiocytosis) often found? What is a classic symptom?
A
Bone
Diabetes Insipidus
24
Q
Multinucleated giant cells and Birbeck bodies are classically seen in what neoplastic process?
A
Langerhans cell histiocytosis
Birbeck granules looks like tennis racket
The function of Birbeck granules is debated, but one theory is that they migrate to the periphery of the Langerhans cells and release their contents into the extracellular matrix. Another theory is that the Birbeck granule functions in receptor-mediated endocytosis, similar to clathrin-coated pits.
25
What is Letterer-Siwe disease?
Acute fulminant histiocytosis occurring in children 2-4 years old, affecting multiple organs, and causing death often by 2 years of age
26
What is another name for unifocal Langerhans histiocytosis?
Describe what the lesion is like?
Where may these lesions be found?
Eosinophillic granuloma
**Painful, solitary**, punched-out, **lytic** bone lesions with clear margins (rim is NOT sclerotic)
Lesions are full thickness and may involve brain, spinal cord, dura, and even vertebrae (**vertebrae plana**)
27
Any patient with unifocal Langerhans histiocytosis should be worked up with what?
What is treatment?
Skeletal survey
| Excision and/or radiation
28
Respiratory infections, and infiltrates to lymph nodes, liver spleen, bones, orbit, pituitary, and hypothalamus may be indicative of what type of histiocytosis?
Multifocal histiocytosis
29
What triad of symptoms are seen in Hand-Schuller-Christian disease?
**Exophthalmos, lytic bone lesions, diabetes insipidus**
Hand-Schüller-Christian disease belongs to the spectrum of disorders, now known as "eosinophilic granulomatosis" or "Langerhans cell granulomatosis". It occurs in children and is characterized by the clinical triad of diabetes inspidus, exophthalmos, and lytic bone lesions
30
What is the difference between plasmacytoma and multiple myeloma?
Where do lesions typically occur?
What is classically seen on H+E?
Plasmacytoma is a single lesion, multiple myeloma is diffuse
Vertebral bodies skull, ribs
**Russell bodies**: Eosinophilic intracytoplasmic inclusions filled with Igs
31
Are there lytic lesions seen in Waldenstrom macroglobulinemia?
What are examples of neurologic complications seen?
Waldenstrom macroglobulinemia is considered a type of non-Hodgkin's lymphoma. It's sometimes called lymphoplasmacytic lymphoma.
No
Stroke, peripheral neuropathy, SAH
32
Primary CNS lymphoma may have a number of appearances on imaging. What are some coincident conditions which increase risk?
Wiskott-Aldricg syndrome, transplant patients, AIDS, collagen vascular disease, cancer, EBV infection
33
What does lymphoma look like on MRI imaging? What is the appropriate treatment?
Enahnces
| Chemotherapy, steroids, and radiation
34
Are most primary CNS lymphomas B or T cell derived?
B cell (98%)
35
What primary tumors commonly metastasize to skull?
Breast, prostate, multiple myeloma, lung
36
What primary tumors commonly metastasize to epidural space (usually thoracic)?
Breast, prostate, and lung
A number of additional ones can but not as commonly
37
What primary tumors metastasize to dura?
Breast, lung, lymohoma, leukemia, melanoma, GI tumors
38
What primary tumors have leptomeningeal spread?
Breast, lung, melanoma, and gastric carcinoma
39
What are symptoms of leptomeningeal carcinomatosis?
Cranial neuropathies and CSF obstruction due to outflow obstrcuction (remember: leptomeninges are subarachnoid and pia)
40
What primary tumors have parenchymal spread and list them in descending order of prevalence?
Lung > Breast > Kidney > Melanoma > GI
41
What mets are commonly hemorrhagic?
Melanoma, renal cell carcinoma, choriocarcinoma
42
What cell type do esthesioneuroblastomas (aka olfactory neuroblastoma) typically arise from?
Are they capable of spreading into the CNS?
How old are patients who typically get this?
Neuro**secretory cells**/Basal cells in the high nasal cavity
It can spread to CNS
> 50 yo
(New Latin, from Greek aisthēsis sensation, perception, feeling, from aisthanesthai to perceive, feel.)
43
What is another name for a chemodectoma?
Where does it form? Is it painful?
What substance may they produce?
What is treatment?
Chemodectoma is a rare tumor of the neuroendocrinne tissue of the carotid body. In the most of cases is benign (noncancerous), but can have a malignant behave
Carotid body tumor (**paraganglioma**)
Carotid bifurcation; painless
Catecholamines
Surgery and/or radiation
44
In what patient population do glomus jugulare tumors often arise in?
What tissue does this tumor arise from?
What is treatment and what are important treatment considerations?
Middle aged patients, more often female
Paraganglion tissue of adventitia of dome of jugular bulb, may produce catecholamines
Mastoidectomy followed by removal and radiation, since they're very vascular thought should be lent towards preoperative embolization
45
Where are chordomas often found?
| What is mean age of identification and sex predominance?
Clivus (40%) and Sacrum (60%)
| 20 to 60 yo, male
46
What developmental structure do chordomas arise from?
| Are they benign or malignant?
**Notochord**
Technically benign but given their location and ability to be locally aggressive they have malignant characteristics
47
Can chordomas metastasize?
| Can they transform to other tumor types?
Yes, they do in 25-40% of cases
| Yes, sarcoma (fibrosarcoma)
48
What do chordomas look like under a microscope?
| What do they stain positive for?
Lobulated with **physaliphorous**/bubble-bearing, large, vacuolated cellls which are surrounded by mucin.
(Cytoplasmic mucin=vacoulated)
Stains have characteristics of mesenchyme and epithelium. Cytokeratin, EMA (epithelial), and S100 (mesenchymal, neural crest)
## Footnote
Physaliphorous is a combination of two words – Greek word Physallis meaning “bubble” and Phoros meaning “bearing.” Thus, physaliphorous cells literally mean cells having bubbly or vacuolated appearance.
49
Chondrosarcoma is positive for what stain?
S100 (but not cytokeratin or EMA, like typical chordoma)
50
What is the name of the variant of chordoma which contains cartilage and has a better prognosis?
Chondroid chordoma
51
What is treatment for a chordoma?
Surgery with radiation
Wide En-block resection
| survival is 5-7 years
Sacrum: if below S2 , normal bladder bowl
If above: 😞
52
Where in the brain are lipomas commonly found?
In midline, usually above corpus callosum, at quadrigeminal plate, 3rd ventricle, CPA, or sylvian fissure
@ other midline abnormalities
53
Lipomas are thought to originate from maldifferentiation of _______, a mesenchym derivative of ectodermal and mesodermal origins which helps form dura and arachnoid
Meninx primitiva
54
There are two variants of lipomas, tubulonodular and curvilinear. Distinguish the two?
Tubulonodular are often anterior to corpus callosum and associated with corpus callosum dysgenesis, cephaloceles, and frontal lobe abnormalities
Curvilinear are often near splenium and the corpus callosum is normal
55
Where intracranially are dermoid cysts/tumors often located?
Midline (parasellar/4th ventricular/interhemispheric)
56
Describe the contents of dermoid cysts. What may occur if they rupture?
Oily, sebaceous material sometimes with sweat glands, hair follicles/shafts, and teeth.
If they rupture they may cause a chemical meningitis
57
What are two means of acquiring dermoid cysts?
Either congenitally or via trauma such as a lumbar puncture
58
Where are epidermoid cysts often located?
=**cholesteatoma**
CPA, Suprasellar, Intraventricular, Thalamic
59
How do epidermoid cysts appear on MRI?
Hypointense on T1 (almost completely dark like CSF) but intense on DWI and FLAIR.
Based on the latter it should be easily distinguished from an arachnoid cyst
60
A smooth, encapsulated mass with stratified cuboidal squamous epithelium and dry, flaky keratin describes either dermoid or epidermoid cyst?
Epidermoid cyst
61
What is Mollaret meningitis and with what intracranial pathology may it occur?
A type of recurrent aseptic meningitis with large cells due to epidermoid cysts
Like endothel/macrophage cells
62
Do epidermoid cysts recur?`
Yes they can, they can also be associated with trauma from lumbar puncture and failing to re-insert stylet before exiting
63
Remnant of the craniopharyngeal duct may lead to ...
Rathke cleft cyst
64
What symptoms may present due to Rathke cleft cysts?
Visual changes and/or increased PRL
65
How do Rathke cleft cysts appear on MRI?
Either T1 hypo- or hyperintense and homogenous, 50% have some rim enhancement
66
What is the primary cause for empty sella syndrome?
A defect in **diaphragm sellae** rather allows arachnoid to push in and compress the pituitary. Can be seen after radiation, surgery, stroke, Sheehan syndrome (intrapartum shock with ischemic necrosis of pituitary), or pseudotumor cerebri
67
What is lymphocytic hypophysitis? What is treatment?
Pituitary insufficiency from **autoimmune reaction in peripartum woman** (B and T cell involvement). If necessary, treatment is surgical decompression with hormonal replacement
68
What is a null cell tumor (Oncocytoma)?
Type of pituitary tumor which is 2nd most common after PRL
Null cell adenoma is a benign tumor of the **anterior pitutiary gland that is made up of adenohypophyseal cells** without cell-type-specific differentiation. This is proven via immunohistochemistry for pituitary hormones and transcription factors
69
Cushings disease represents what pathology ...
ACTH secreting tumor in pituitary gland
70
How does Nelson syndrome develop?
After **total adrenalectomy** patients may develop pituitary enlargement and ACTH hypersecretion even years after. These patients will also be hypermelanotic as well
71
What is a medical treatment for GH secreting pituitary adenomas?
Octreotide (somatostatin analog)
72
What PRL level is consistent with prolactinoma vs stalk effect?
PRL > 150 is consistent with a prolactinoma, mild increases may be due to stalk effect
73
Pituitary microadenomas may be part of what endocrine syndrome?
Multiple Endocrine Neoplasias 1 (MEN1)
74
What hormones are secreted from acidophils and what are from basophils?
Acidophils: PRL, GH, FSH, LH
Basophils: ACTH, Thyroid hormone
75
Where do germ cell tumors develop from embryonically?
Yolk sac endoderm
76
Where are the most frequent tumor locations for germ cell tumors?
Pineal, suprasellar, third ventricular, posterior fossa,
= **midline**
midline mediastinum, and retroperitoneum
77
How do germinomas appear on:
T1
T2
and CT?
T1: Isointense
T2: Hypointense
CT: Hyperdense
78
Large polygonal clear cells with prominent nucleoli and T cell infiltrates is characteristic of what tumor?
Germinoma
79
What are germinomas positive for on staining?
Placental alkaline phosphatase (pALP) = placental alkalin phosphates
| 15% have elevated beta hCG
80
How are germinomas treated?
Radiation to entire neuraxis (very radiation sensitive)
81
What germ cell tumor has elevated AFP and B-hCG with necrosis and hemorrhage?
Embryonal carcinoma
## Footnote
Embryonal carcinomas are typically positive for placental alkaline phosphatase (PLAP), c-kit (CD117), keratins (8, 18, 19), and CD30. In addition, embryonal carcinomas test positive for other markers now being used in the diagnosis, including NANOG, SOX2 (sex-determining region Y [SRY] – box 2), and OCT3/4.
The usual IHC markers used in GCTs are PLAP, SALL4, OCT 3/4, CD117, Glypican 3 (GPC3), CD30, and β-HCG, applied in combination as and when the morphology deems it necessary.
82
What rare germ cell tumor has elevated AFP and contains Schiller-Duval bodies?
Yolk sac tumor (Endodermal sinus tumor)
* Schiller - Duval bodies look like glomeruli and are also seen in the same type of cancer when located in the testes
83
What germ cell tumor has elevated B-hCG with a very poor prognosis?
How may they appear on CT scan?
Choriocarcinoma - these tumors likely to hemorrhage bc of thin walled vessels
84
What germ cell tumor contains tissue from all 3 embryonic layers?
What serum marker may be elevated?
Teratomas, often in the pineal region
CEA
85
Where is AFP normally secreted from?
Mucous glands of GI tract; is seen elevated in yolk sac tumors and hepatic carcinomas
86
Where is B-hCG normally secreted from?
Placenta; elevated in choriocarcinoma
87
What hormones are secreted by the pineal gland?
| At what age does it usually calcify?
Norepinephrine, Serotonin, Melatonin
| 16 yo
88
What is the most frequent tumor the pineal gland?
Germinoma
| Pineocytoma and pineoblastoma are second
89
At what age is pineocytoma most common?
| What is seen on pathology?
30 years old
| Homer-wright Rosettes with central fibrillary material
90
What age is pineoblastoma usually seen?
Does this have a propensity to metastasize?
What is mean survival?
< 20 yo
Yes, mets to bone, lungs, and lymph nodes. Seeds CSF
< 2 years survival (median)
PNET tumor: Primitive neuroectodermal tumors (PNET) and pineoblastoma are a group of tumors defined by their appearance and are thought to develop from primitive (undeveloped) nerve cells in the brain. They appear similar to medulloblastoma and were once considered a single tumor.
91
What is the peak age of craniopharyngioma?
| Where are they often located?
0-20 years with a second peak > 50 yo
| Often intrasellar and suprasellar
92
Are craniopharyngiomas benign or malignant?
What cells are they derived from?
What may they contain?
Benign but may invade vital structures
Squamous cells from Rathke cleft
Oily fluid with cholesterol crystals and often calcifications
93
How do craniopharyngiomas appear on pathology?
Adamantinomatous pattern with epithelial cell rests surrounded by columnar basal cells and separated by myxoid, stellate cells and wet keratin
94
Papillary craniopharyngiomas often extend to the third ventricular and have what prognosis compared to typical craniopharyngiomas?
Better
95
What is the mean age and sex predominance of hemangioblastoma?
Where is it located most often?
Males, 20-40 yo
Cerebellar hemisphere/vermis (posterior fossa), cervical spinal cord, brainstem
96
How do hemangioblastomas appear on imaging?
| Pathology?
Cystic mass in posterior fossa with enhancing mural nodule; if in spinal cord often with associated syrinx.
Circular and yellow due to lipid content.
Path: capillaries with hyperplastic endothelial cells and pericytes surrounded by stromal cells with vacuoles and lipids
97
Where is it thought hemangiopericytomas develop from? Where do they develop in the brain?
Pericytes surrounding capillaries
Dural based and well demarcated with high vascularity, usually supratentorial (originally thought to be a type of meningioma but now characterized as distinct)
98
What is the recurrence rate of hemangiopericyomoma?
Do they metastasize?
How is their response to radiation and chemo?
70% recur
Yes, to lungs and bone (30% metastasize)
No, they have poor response
99
What are risk factors for meningioma development?
Radiation
NF2
Mild increase among females
100
Why may meningiomas increase in size during pregnancy and in patients with breast cancer?
They have receptors for estrogen, progesterone, peptides, amines, androgens, glucocorticoids, somatostatin, and cholecystokinin
101
Where do meningiomas receive their blood supply?
Commonly ECA branches such as middle meningeal artery or anterior falcine artery, sometimes pial vessels are involved giving dual ECA-ICA supply
102
Bony changes from meningiomas are hyperostotic or lytic?
Hyperostotic
103
Meningiomas develop from what layer of the dura?
| What classic finding is seen on pathology?
Arachnoi cap cells, often by arachnoid granulations and tela choroidea
Psammoma bodies
104
What is a medulloblast, by definition?
Cell with bipotential capabilities to develop into neurons or glia (coined by Bailey and Cushing in 1925)
105
What are the age peaks for the development of medulloblastoma?
< 15 yo and at 28 years
106
What is the most frequent genetic abnormality seen in PNET?
| What syndrome may they be associated with?
Isochromosome 17q
Gorlin (basal cell nevus syndrome) due to mutation of PTCH gene on chromosome 9q
107
Retinoblastoma is a type of PNET derived from what precursor cell?
What are two common eponyms seen on pathology?
How is it treated?
Neural crest precursor of sympathetic ganglion
Homer-Wright and Flexner-Wintersteiner rosettes
Surgery and radiations
108
What is trilateral retinoblastoma?
Bilateral retinoblastoma with pineoblastoma (Latter is also PNET form)
109
Loss of what type of gene causes retinoblastoma?
A tumor suppressor gene, often in kids < 5 yo
110
What chromosome's gene deletion is associated with ATRT? (=atypical teratoid/rhabdoid tumor)
| At what age is this usually seen?
Chromosome 22 and the INI1/hSNF5 gene
Infants and very young children
## Footnote
Genetic Testing
* Deletions, mutations in INI1/hSNF5/SMARCB1/BAF47 gene
in almost all cases
* Mutation in SMARCA4/BRG1 gene (rare)
○ Retained nuclear INI1 immunostaining in rare AT/RTs
with SMARCA4/BRG1 mutation
* Loss of all (monosomy) or part (deletion) of chromosome
22 by FISH in almost all cases
111
What is the third most common tumor in children besides leukemia and brain tumors?
Neuroblastoma (although only 2% affect brain)
112
Neuroblastoma may have mets where?
Spinal epidural mets
113
What does medulloepithlioma derive from?
Ventricular matrix cells
Is the most primitive of PNETS, WHO IV, and affectes very young children
114
What are the PNET tumors?
Medulloblastoma, Retinoblastoma, ATRT, Central Neuroblastoma, Medulloepithelioma, Pineoblastoma, Ependymoblastoma
115
Where are PNET tumors often located? (Descending order)
Vermis > Cerebellar hemispheres > Pineal > Cerebrum > Spinal Cord > Brainstem
116
How do PNETs appear on CT?
T1?
T1 enhanced?
Slightly hyperdense on CT
Hypointense on T1, enhancing
Frequently cystic
117
Linear array of small, blue cells may be seen with what tumors?
PNET ('Indian file arrangement')
118
Do PNETs metastasize often?
What is appropriate treatment?
What is survival?
Yes, 50% (either intracranially and/or to bone and lung)
Surgery, chemo, and rad
>50% survival 5 yrs with above treatment, if complete surgical resection achieved then > 75% survival at 5 yrs
119
Name the tumor.
Usually cystic and with a calcified nodule and occurring in temporal lobes with seizure as presentation, often before 30 yrs of age?
Ganglioglioma
A ganglioglioma is low-grade tumor of mixed cell type. It is very rare and contains properties of both glial cells — responsible for providing the structural support of the central nervous system, and neuronal cells — the functioning component of the central nervous system.
120
What are gangliogliomas positive for on IHC?
Neurofilament, synaptophysin, neurosecretory granules, and GFAP
## Footnote
Ganglion-like cells: MAP2, neurofilament, synaptophysin (neural markers)
Neoplastic glial cells: GFAP, Olig2,CD34
BRAF V600E mutation specific antibody
121
If ganglioglioma is a combo of neoplastic neurons and glia what is gangliocytoma?
Mostly neoplastic neurons without glia
Gangliocytomas are rare indolent CNS tumours (WHO grade 1), primarily encountered in children, and frequently discovered as the cause of epilepsy. They are considered one of the **long-term epilepsy-associated tumours (LEATs)**.
They differ from gangliogliomas by the absence of neoplastic glial cells, although both tumours are defined by the presence of **displaced ganglion cells** (large mature neurones that show cytological or architectural abnormalities).
On imaging, these tumours are usually characterised by cortical solid lesions with little associated mass effect and minimal or no surrounding vasogenic oedema. Calcification and cyst formation can occur, and contrast enhancement is generally present.
Terminology
Gangliocytomas should not be confused with dysplastic cerebellar gangliocytoma of the cerebellum, better known as Lhermitte-Duclos disease.
122
At what age do desmoplastic infantile gangliomas occur?
How do they appear on imaging and usually in what location?
Often before 18 months
Massive frontal lesions adherent to dura with a desmoplastic reaction, has enhancement, often cystic
## Footnote
**Desmoplastic component**: vimentin+, GFAP+, type IV collagen+, reticulin (pericellular pattern), trichrome (stroma)
**Astrocytic cells**: GFAP+, S100+
**Neuronal cells**: synaptophysin+, NeuN+, neuron specific enolase+
**Poorly differentiated** small cells: vimentin+, GFAP+, synaptophysin+
Proliferation index: < 2% (higher in the poorly differentiated small cell component)
123
Dysembryonic neuroepithelial tumor presents how? Where are they usually located?
What cell types make it up?
What are the treatment and prognosis?
Seizures
Temporal region as cystic, multinodular, and superficial masses
**Normal neurons with abnormal oligodendrocytes and astrocytes**
Surgery, often curative
124
Where do central neurocytomas often form in the brain?
| Do they enhance?
Septum pellucidum into lateral or third ventricle
| Yes they enhance
125
Arachnoid cyst locations
60% middle fossa
10% suprasellar
10% post.fossa
10% cisterna quadrigemina
126
What is a neuroepithelial cyst?
Neuroepithelial cysts are cavities with an ependymal or epithelial lining. They contain fluids of unidentified origin and develop in the central nervous system. Regarding ventricular or subarachnoid spaces, there is no clear communication between them.
127
What is a neurenteric cyst?
The neurenteric cyst is a rare lesion of the **spinal axis composed of heterotopic endodermal tissue**. During the third week of human embryogenesis, the neurenteric canal unites the yolk sac and the amniotic cavity as it traverses the primitive notochordal plate.
128
Tela choroidea definition
The tela choroidea is the thin, highly vascularized, loose connective tissue portion of pia mater that gives rise to the choroid plexus. Thus, it is basically the lamina propria of the ependyma and lies directly adherent to it, without any tissue in between the two
129
What is plasmocytoma?
Plasmacytoma is a tumor of plasma cells of bony or soft tissue and can occur anywhere in the body without evidence of systemic disease. It may present as a solitary or multiple mass anywhere in the body. It can progress to multiple myeloma if not evaluated and appropriately managed
130
What is myeloma multiplex?
Myeloma is a type of blood cancer that develops from plasma cells in the bone marrow. Myeloma is often called multiple myeloma because most people (90%) have multiple bone lesions at the time it is diagnosed. Plasma cells are a type of white blood cell found in the bone marrow.
131
What is glomus tumor?
132
Features of glomus jugulare tumor
133
10%rule of paragangliomas
134
Pathology of paragangliomas
135
Histology of glomus jugulare tumor
136
Spread of glomus jugulare tumor
137
Glomus tumor radiology
138
Glomis jugulare tumor feeding artery
139
Curvilinear lipoma finding
Around selenium
CC usually normal
140
Germ cell tumor deviation
141
Pineal region tumors
142
Pineal region masses
143
Pineal gland operative anatomy
Dorsally: Splenium, Tela choroidea
Ventrally: Quadrigeminal plate, tectum
Rostrally: 3rd ventricle
Caudally: vermis
144
Parinaud syndrome
Dorsal midbrain lesion
Nystagmus retractorius, pupils react to near bit not light
145
Psammoma bodies
Psammoma bodies (PBs) are concentric lamellated calcified structures, observed most commonly in papillary thyroid carcinoma (PTC), meningioma, and papillary serous cystadenocarcinoma of ovary but have rarely been reported in other neoplasms and nonneoplastic lesions.
146
Meningeoma locations
147
Olfactory groove meningeoma blood supply
Ophthalmic a. --> anterior ethmoid a.
148
Meningioma histology
syncytial pattern, psammoma bodies, whorls
149
Meningothelial/syncytial type meningioma
**Meningothelial meningiomas** (also known as syncytial or endothelial meningiomas) are the most common histological subtype of meningioma, found in ~60% of all meningiomas, most frequently combined with fibrous meningioma (40%) or in isolation (17%) 1
150
Fibroblastic meningioma
151
WHO grade I meningiomas (92%)
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WHO grade II meningiomas (6%)
hypercellularity, frequent mitosis, necrosis, brain invasion, 30% 5y survival, recurrence, 5%metastasize
153
WHO grade III meningiomas (2%)
invades, 30%metastasize, necrosis, higher rate of mitosis, 70%recurrance
154
Medulloblastoma subtypes
Medulloblastoma is the most common pediatric malignant brain tumor. Advances in molecular profiling have uncovered significant heterogeneity among medulloblastomas and led to the identification of four distinct subgroups (wingless [WNT], sonic hedgehog [SHH], group 3, and group 4) that represent distinct disease entities in both underlying biology and clinical characteristics.
155
Medulloblastoma prognosis
156
How does medulloblastoma metastasize?
157
Medulloblastoma MRI
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Retinoblastoma in general
Most common extraaxial solid tumor in children!
Retinoblastoma results from a biallelic mutation of the retinoblastoma gene (RB-1) in developing retinal cells, following the 2-hit model of tumor suppressor gene inactivation.2 In the heritable form, seen in up to 50% of patients, a germline mutation is followed by a second acquired somatic mutation. In the nonheritable form, 2 somatic mutations must occur in the same gene in a single cell.
Retinoblastoma originates in the retina and can display endophytic growth into the vitreous chamber; exophytic growth into the subretinal space; or diffuse, infiltrative growth along the retina. Most tumors demonstrate both endophytic and exophytic growth and can cause retinal detachment as well as vitreous and subretinal tumor seeding.
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WHO embrional tumors (old PNET)
vermis, cerebellar hemispheres, cerebrum, spinal cord,
hyperdense ion CT, hypoontense, KA enhancing; pinkish-brown, sof-solid, maybe hemorrhagic. 80%cystic, no capsule
hist.: densely cellular, small, round cells, large nuclei, mitosis, necrosis. Homer Wright rosettes, pseudorisettes, Flexner-Wintersteiner rosetes,
50%mestastatize; complete resection provides 75% 5 y survival
160
WHO germ cell tumors
161
Immunohistochemistry in brain tumors
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DNET attributes
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s100
S100 proteins are normally present in cells derived from the **neural crest** (Schwann cells, and melanocytes), chondrocytes, adipocytes, myoepithelial cells, macrophages, Langerhans cells,[3][4] dendritic cells,[5] and keratinocytes. They may be present in some breast epithelial cells.
S100 proteins have been implicated in a variety of intracellular and extracellular functions,[6] such as regulation of protein phosphorylation, transcription factors, Ca2+ homeostasis, the dynamics of cytoskeleton constituents, enzyme activities, cell growth and differentiation, and the inflammatory response. S100A7 (psoriasin) and S100A15 have been found to act as cytokines in inflammation, particularly in autoimmune skin conditions such as psoriasis.[7]
Several members of the S100 protein family are useful as markers for certain tumors and epidermal differentiation. They can be found in **melanomas,[8] 100% of schwannomas, 100% of neurofibromas (weaker than schwannomas), 50% of malignant peripheral nerve sheath tumors (may be weak and/or focal), paraganglioma stromal cells, histiocytoma, and clear-cell sarcomas**. Further, S100 proteins are markers for inflammatory diseases and can mediate inflammation and act as antimicrobials.[9] S100 proteins have been used in the lab as cell markers for anatomic pathology.
164
What is a neurofibroma?
165
Calretinin
Calretinin, a Vitamin D-dependent calcium-binding protein, is a sensitive marker for **ganglion cells and nerve fibers** [1–3, 5]. It is a protein involved in calcium transport and modulates neuronal excitability. Absence of this protein leads to accumulation of calcium in cytoplasm.
166
EMA antigen
167
Alfa-synuclein
168
Lewy-body
alfa-synuclein
169
IDH
170
IDH staining
171
NFP / neurofilament
172
Epithel markers
For example, epithelial cells can be identified by the expression of markers such as cell adhesion proteins including **E-Cadherin** and Claudin family members and specific **Cytokeratin** intermediate filament proteins.
173
What is Flexner-Wintersteiner rosettes? Which tumor?
RB
174
What is Homer Wright rosette? Which tumor?
NB
175
Genetic patterns of medulloblastoma (IHC)
176
Germinoma radiology
177
Germinoma histology
178
Sindou grading system of menigiomas
179
Simpson grade menigioma
180
Genetic alterations menigioma
181
IHC 1
182
IHC 2
183
growing skull fracture
Leptomeningeal cysts, also known as growing skull fractures, are an enlarging skull fracture that occurs near post-traumatic encephalomalacia.
The exact pathogenesis remains unclear, but it is thought they occur secondary to skull fractures causing **dural tears, allowing the leptomeninges and/or cerebral parenchyma to herniate into it.** Pulsations from CSF **erode the fracture margin,** resulting in eventual expansion and non-union.
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L1CAM syndrome
L1 syndrome is a group of mild to severe X-linked recessive disorders that share a common genetic basis. The spectrum of L1 syndrome disorders includes X-linked complicated corpus callosum dysgenesis, spastic paraplegia 1, MASA syndrome, and X-linked hydrocephalus with stenosis of the aqueduct of Sylvius (HSAS).[1][2] It is also called L1CAM syndrome (for the disorder's causative gene) and **CRASH syndrome**, an acronym for its primary clinical features: **corpus callosum hypoplasia, retardation (intellectual disability), adducted thumbs, spasticity, and hydrocephalus**.[2]
185
DISH score
Diffuse idiopathic skeletal hyperostosis (DISH) is a systemic condition characterized by characteristic ossification patterns that can occur in the spine and peripheral entheses.[1] DISH most commonly affects the spine and often presents as back pain and stiffness
186
Cowden syndrome
187
Turcot syndrome
188
