Pathology and Radiology: Oncology Flashcards
What is a traumatic neuroma?
What is often a cause?
What is a common example?
How do they feel?
- 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
Schwann cells are derived from which cell type?
What disorder are schwannomas associated with?
Neural crest cells
NF2
What are the common nerve sites for Schwannomas to form intracranially?
Superior vestibular nerve where entering IAM
Trigeminal nerve
What types of nerves do spinal schwannomas form on?
In rare cases where there is an intra-axial schwannoma where did it likely form?
- Spinal sensory nerve roots
- Perivascular nerves
What are the two histological patterns seen with Schwannomas?
Antoni A (Compact, dense reticular cells, fusiform cells, and collagen matrix)
Antoni B (Loose, stellate cells with stoma surrounding)
What are Verocay bodies and in what peripheral nerve sheath tumor are they seen in?
Anuclear material with palisading cells in Antoni A areas, seen in Schwannomas
Schwannomas stain positive for what on IHC?
S100
How do Schwannomas appear on T1 and enhanced MRI?
T1: hypointense to isointense
They do enhance
Rare for them to have calcification
Do neurofibromas occur intracranially?
No
What cell types make up neurofibromas?
Scwannn cells and fibroblasts in conjunction with collagen and reticulin
What do cutaneous neurofibromas stain positive for on IHC?
S100, Vimentin, Leu7
Occasionally GFAP
Describe the characteristics of cutaneous neurofibromas. What disease are they associated with and do they often undergo malignant transformation?
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
Describe the characteristics of intraneural neurofibromas. What disease are they associated with and do they often undergo malignant transformation?
Involve large nerve trunks and occasionally plexi (i.e. plexiform neurofibromas). Assd with NF1 and may undergo malignant transformation
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?
Perineuroma
Perineural cells
EMA positive, S100 negative
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?
Malignant peripheral nerve sheath tumor
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?
- 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
What causes a neuroepithelial cyst?
Where are they commonly located?
Infolding of developing neuroectoderm
Located in ependyma, choroid plexus, and choroidal fissure
What are neurenteric cysts filled with?
What is their thought developmental origin?
Filled with endoderm of GI/respiratory mucosa, have interspersed Goblet cells
Fusion of notochord and gut
What is cavum septum pellucidum?
CSF between the sheets of the septum
What is cavum vergae?
A posterior communication of cavum septum pellucidum
What is cavum velum interpositum?
A cavum in 3rd ventricle due to failed fusion of tela choroidea
In what disease does a chloroma form? What part of the intracranium does it usually affect?
What is the appropriate treatment?
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
Where is Histicytosis X (Langerhans cell histiocytosis) often found? What is a classic symptom?
Bone
Diabetes Insipidus
Multinucleated giant cells and Birbeck bodies are classically seen in what neoplastic process?
Langerhans cell histiocytosis
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
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)
Any patient with unifocal Langerhans histiocytosis should be worked up with what?
What is treatment?
Skeletal survey
Excision and/or radiation
Respiratory infections, and infiltrates to lymph nodes, liver spleen, bones, orbit, pituitary, and hypothalamus may be indicative of what type of histiocytosis?
Multifocal histiocytosis
What triad of symptoms are seen in Hand-Schuller-Christian disease?
Exophthalmos, lytic bone lesions, diabetes insipidus
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
Are there lytic lesions seen in Waldenstrom macroglobulinemia?
What are examples of neurologic complications seen?
No
Stroke, peripheral neuropathy, SAH
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
What does lymphoma look like on MRI imaging? What is the appropriate treatment?
Enahnces
Chemotherapy, steroids, and radiation
Are most primary CNS lymphomas B or T cell derived?
B cell (98%)
What primary tumors commonly metastasize to skull?
Breast, prostate, multiple myeloma, lung
What primary tumors commonly metastasize to epidural space (usually thoracic)?
Breast, prostate, and lung
A number of additional ones can but not as commonly
What primary tumors metastasize to dura?
Breast, lung, lymohoma, leukemia, melanoma, GI tumors
What primary tumors have leptomeningeal spread?
Breast, lung, melanoma, and gastric carcinoma
What are symptoms of leptomeningeal carcinomatosis?
Cranial neuropathies and CSF obstruction due to outflow obstrcuction (remember: leptomeninges are subarachnoid and pia)
What primary tumors have parenchymal spread and list them in descending order of prevalence?
Lung > Breast > Kidney > Melanoma > GI
What mets are commonly hemorrhagic?
Melanoma, renal cell carcinoma, choriocarcinoma
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?
Neurosecretory cells/Basal cells in the high nasal cavity
It can spread to CNS
> 50 yo
What is another name for a chemodectoma?
Where does it form? Is it painful?
What substance may they produce?
What is treatment?
Carotid body tumor
Carotid bifurcation; painless
Catecholamines
Surgery and/or radiation
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
Where are chordomas often found?
What is mean age of identification and sex predominance?
Clivus (40%) and Sacrum (60%)
20 to 60 yo, male
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
Can chordomas metastasize?
Can they transform to other tumor types?
Yes, they do in 25-40% of cases
Yes, sarcoma
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.
Stains have characteristics of mesenchyme and epithelium. Cytokeratin, EMA (epithelial), and S100 (mesenchymal, neural crest)
Chondrosarcoma is positive for what stain?
S100 (but not cytokeratin or EMA, like typical chordoma)
What is the name of the variant of chordoma which contains cartilage and has a better prognosis?
Chondroid chordoma
What is treatment for a chordoma?
Surgery with radiation
survival is 5-7 years
Where in the brain are lipomas commonly found?
In midline, usually above corpus callosum, at quadrigeminal plate, 3rd ventricle, CPA, or sylvian fissure
Lipomas are thought to originate from maldifferentiation of _______, a mesenchym derivative of ectodermal and mesodermal origins which helps form dura and arachnoid
Meninx primitiva
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
Where intracranially are dermoid cysts/tumors often located?
Midline (parasellar/4th ventricular/interhemispheric)
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
What are two means of acquiring dermoid cysts?
Either congenitally or via trauma such as a lumbar puncture
Where are epidermoid cysts often located?
CPA, Suprasellar, Intraventricular, Thalamic
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
A smooth, encapsulated mass with stratified cuboidal squamous epithelium and dry, flaky keratin describes either dermoid or epidermoid cyst?
Epidermoid cyst
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
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
Remnant of the craniopharyngeal duct may lead to …
Rathke cleft cyst
What symptoms may present due to Rathke cleft cysts?
Visual changes and/or increased PRL
How do Rathke cleft cysts appear on MRI?
Either T1 hypo- or hyperintense and homogenous, 50% have some rim enhancement
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
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
What is a null cell tumor (Oncocytoma)?
Type of pituitary tumor which is 2nd most common after PRL
Cushings disease represents what pathology …
ACTH secreting tumor in pituitary gland
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
What is a medical treatment for GH secreting pituitary adenomas?
Octreotide (somatostatin analog)
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
Pituitary microadenomas may be part of what endocrine syndrome?
Multiple Endocrine Neoplasias 1 (MEN1)
What hormones are secreted from acidophils and what are from basophils?
Acidophils: PRL, GH, FSH, LH
Basophils: ACTH, Thyroid hormone
Where do germ cell tumors develop from embryonically?
Yolk sac endoderm
Where are the most frequent tumor locations for germ cell tumors?
Pineal, suprasellar, third ventricular, posterior fossa, midline mediastinum, and retroperitoneum
How do germinomas appear on:
T1
T2
and CT?
T1: Isointense
T2: Hypointense
CT: Hyperdense
Large polygonal clear cells with prominent nucleoli and T cell infiltrates is characteristic of what tumor?
Germinoma
What are germinomas positive for on staining?
Placental alkaline phosphatase (pALP)
15% have elevated beta hCG
How are germinomas treated?
Radiation to entire neuraxis (very radiation sensitive)
What germ cell tumor has elevated AFP and B-hCG with necrosis and hemorrhage?
Embryonal carcinoma
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
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
What germ cell tumor contains tissue from all 3 embryonic layers?
What serum marker may be elevated?
Teratomas, often in the pineal region
CEA
Where is AFP normally secreted from?
Mucous glands of GI tract; is seen elevated in yolk sac tumors and hepatic carcinomas
Where is B-hCG normally secreted from?
Placenta; elevated in choriocarcinoma
What hormones are secreted by the pineal gland?
At what age does it usually calcify?
Norepinephrine, Serotonin, Melatonin
16 yo
What is the most frequent tumor the pineal gland?
Germinoma
Pineocytoma and pineoblastoma are second
At what age is pineocytoma most common?
What is seen on pathology?
30 years old
Homer-wright Rosettes with central fibrillary material
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)
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
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
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
Papillary craniopharyngiomas often extend to the third ventricular and have what prognosis compared to typical craniopharyngiomas?
Better
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
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
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)
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
What are risk factors for meningioma development?
Radiation
NF2
Mild increase among females
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
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
Bony changes from meningiomas are hyperostotic or lytic?
Hyperostotic
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
What is a medulloblast, by definition?
Cell with bipotential capabilities to develop into neurons or glia (coined by Bailey and Cushing in 1925)
What are the age peaks for development of medulloblastoma?
< 15 yo and at 28 years
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
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
What is trilateral retinoblastoma?
Bilateral retinoblastoma with pineoblastoma (Latter is also PNET form)
Loss of what type of gene causes retinoblastoma?
A tumor suppressor gene, often in kids < 5 yo
What chromosome’s gene deletion is associated with ATRT?
At what age is this usually seen?
Chromosome 22 and the INI1/hSNF5 gene
Infants and very young children
What is the third most common tumor in children besides leukemia and brain tumors?
Neuroblastoma (although only 2% affect brain)
Neuroblastoma may have mets where?
Spinal epidural mets
What does medulloepithlioma derive from?
Ventricular matrix cells
Is the most primitive of PNETS, WHO IV, and affectes very young children
What are the PNET tumors?
Medulloblastoma, Retinoblastoma, ATRT, Central Neuroblastoma, Medulloepithelioma, Pineoblastoma, Ependymoblastoma
Where are PNET tumors often located? (Descending order)
Vermis > Cerebellar hemispheres > Pineal > Cerebrum > Spinal Cord > Brainstem
How do PNETs appear on CT?
T1?
T1 enhanced?
Slightly hyperdense on CT
Hypointense on T1, enhancing
Frequently cystic
Linear array of small, blue cells may be seen with what tumors?
PNET (‘Indian file arrangement’)
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
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
What are gangliogliomas positive for on IHC?
Neurofilament, synaptophysin, neurosecretory granules, and GFAP
If ganglioglioma is a combo of neoplastic neurons and glia what is gangliocytoma?
Mostly neoplastic neurons without glia
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
Dysembryonic neuroepithelial tumor presents how? Where are they usually located?
What cell types make it up?
What is treatment and prognosis?
Seizures
Temporal region as cystic, multinodular, and superficial masses
Normal neurons with abnormal oligodendrocytes and astrocytes
Surgery, often curative
Where do central neurocytomas often form in the brain?
Do they enhance?
Septum pellucidum into lateral or third ventricle
Yes they enhance
