Tumors Flashcards
Glioblastoma multiforme
= malignant astrocytic glioma, grade IV; in older ppl.
- primary: malign. mutations start w/ 1st cell
- secondary: mutations accumulate w/ time –> become malignant
Path Dx: “ring enhancement” w/ central necrosis & peripheral vascular proliferation.
most common & most deadly type of glioma
fibrillary astrocytoma
= glioma in younger ppl, solid or cystic, grade II or III;
path: poorly demarcated, diffuse infiltration throughout brain, w/ fibrillary astrocytes & GFAP
pilocytic astrocytoma
~benign astrocytoma that often involves optic n.
* most common CNS tumor in kids, cure w/ surgery.
Path: cystic tumor in cerebellum; alternating dense & loose astrocytes, w/ Rosenthal fibers
Oligodendroglioma
grade II glioma affecting 40s-50s+, in cerebral hemispheres.
Sx: seizures, headaches,
Path: ~well-circumscribed, uniform “fried egg” cells (small round blue cells), grow slow but infiltrate
** 1p19q LOH deletion = responder phenotype (easy to treat)**
Ependymoma
glioma in 4th ventricle/wall, or spinal canal (esp. cauda equina);
Path: perivascular pseudorosettes & true rosettes, well-diff & well-circumscribed.
Tx: resect – good prognosis, + radiation.
primary tumors in cerebral hemispheres (6)
- Glioblastoma 4. Astrocytoma
- Oligodendroglioma 5. Ependymoma
- CNS lymphoma 6. meningioma - benign
primary tumors in cerebellum, brainstem, etc. (posterior fossa) (7)
- Medulloblastoma 5. hemangiomablastoma
- pilocytic astrocytoma 6. schwannoma
- ependymoma 7. meningioma
- (diffuse) astrocytoma
primary tumors in the sellar region (4)
- craniolpharyngioma
- pituitary adenoma
- Germ cell neoplasms (esp. pineal region)
- pilocytic astrocytoma (esp. optic n, hypothalamus)
primary tumors in the spinal canal (5)
- astrocytoma (diffuse or pilocytic)
- ependymoma
- myxopapillary
- schwannoma
- meningioma
- neurofibrola
Meduloblastoma
primitive neuronal cell malignant tumor (grade IV) in cerebellum;
* mostly in children.
Path: homogenous, small round blue cells, invasive - spreads via CSF
Tx: radiation (#1), surgery & chemo
meningioma
benign (grade 1) tumor of meninges, esp. in falx cerebri.
* M>F, very young or older pts, NF2!
Path: “dural tail” w/ contrast enhanced imaging; encapsulated, whorls of cells & calcifications.
Tx: resect, but often recur.
hemangiopericytoma
a sarcoma, vascular tumor in meninges or elsewhere;
Path: “stag horn” blood vessels
Tx: resect, frequently recurs, aggressive
schwannoma
benign, slow growing Schwann cell tumor in PNS,
- esp. CN VIII (“acoustic schwannoma”) or spinal cord sens. n. roots
** NF2 ** M>F, middle age pts
Path: encapsulated, @ nerve margin, biphasic w/ spindle cells.
Tx: resect - easy to preserve nerve!
Neurofibroma
Schwann & fibroblastic cell infiltrative tumor.
** NF1 (w/ cafe au lait spots)**
Path: poorly defined, disordered spindle cells, axons run through (n. f(x) spared).
* 5-10% risk malignant degeneration.
von Rcklinghausen’s disease (NF-1)
type 1 neurofibromatosis,
= peripheral, w/ cafe au lait spots & cutaneous neurofibromas, optic gliomas, etc.
Mut: neurofibromin on chrom. 17
NF-2 neurofibromatosis
central form w/ >1 CN & spinal root schwannomas (ie: bilateral acoustic schwannomas) & gliomas, but NOT usually neurofibromas.
Mut: merlin (chrom 22)
Metastatic brain tumor characteristics
primary sites: lung (#1), breast, melanoma, kidney, colon
Path: multiple, necrosis/hemorrhage, ring enhancement of contrast scan; well-circumscribed w/ surrounding gliosis & edema.
* poor prognosis
Primary CNS Lymphoma
very rare, in older ppl or immunocompromised (esp. transplant or AIDS + EBV), @ deep in hemispheres, not CSF.
path: B-cell tumors w/ angiocentric growth & brain infiltration, w/ reactive astrocytosis.
Tx: radiationm, but often recur.
Secondary lymphomas
= metastatic from systemic lymphomas, not 1st presentation;
mostly in leptomeninges & dura, multiple.
Craniopharyngioma
benign tumor from Rathke’s pouch remains (pituitary stalk),
in children OR adults;
* may invade hypothalamus & compress optic chiasm
Path: lobular encapsulated stratified squam. epithelial, calcified w/ degen. keratin
germinoma
undifferentiated, malignant & invasive brain tumor, mostly pineal;
* young ppl, 95% M
path: large nucleoli, w/ T cell infiltrate +/- other malignant cell types
Tx: curable w/ radiation if pure germinoma
Teratoma
differentiated germ cell tumor, in pineal region.
- ecto, meso, & endodermal parts
- in children, 90+% M, benign OR w/ malignant parts
pituitary adenoma
hormone-producing tumors in sella (expand to brain parenchyma),
Sxs: visual field defects, + sudden severe headache if infarction or hemorrhage of tumor
Metastatic bone tumors
*MOST common bone tumor.
= Mets from breast (#1), prostrate, lung, kidney, thyroid, etc.
Tx: radiation, *reinforce bone 1st if high risk of fracture (functional pain) bc radiation slows healing!
Primary osteosarcoma
Age? Path? Bad?
MALIGNANT bone tumor in knee or humerus metaphysis;
“Primary” = young age, peak @ puberty (rapid growth).
“Secondary” = 50+, radiation, Paget’s disease or fibrous dysplasia.
Sx: pain, tender mass, fracture –> periosteal elevation
Tx: wide resection + chemo,
*check for mets in bone marrow!
Ewing’s Sarcoma
Where? What gene mut? How bad?
Malignant! primary bone (& soft tissue) tumor in pelvis/leg diaphyses;
5-30 yrs old, CD99+ & EWS/FLI-1 translocation = t(11:22)
Sx: anemia & leukocytosis, pain, mass –> Small Round Blue cells!!
Tx: Chemo + surgery or radiation (if not resectable)
Chondrosarcoma
Malignant! Cartilage tumor in trunk or prox. femur/humerus.
- primary (#1): 40+
- secondary: 20-40, osteochondroma or enchondroma (–> mets!)
Path: myxoid w/ soft tissue mass (“popcorn” on Xray)
Tx: aggressive resection – No response to chemo or radiation :(
Myeloma
bone marrow tumor of axial bones (esp. pelvis & legs);
Sx: pain, fracture, fatigue, anemia. Age 20-80.
Dx: high ESR & Ca, serum protein spike
abnormal plasma proteins (K & lambda light chains)
Tx: radiation (#1, but recurs), chemo (steroids), bone marrow transplant *poor prognosis :(
osteoid osteoma
bone-forming tumor (F*
Sx: night pain, profound relief w/ NSAIDs (prostaglandins)
Path: sclerotic w/ small nidus
Tx: 1. Radio-ablation, 2. resection
Osteochondroma
Benign cartilage & bone tumor (normal marrow), in knee, shoulder, or hip metaphyses; age 10-35, M>F.
Path: pedunculated or sessile.
Sx: may be asymptomatic. stops growing when pt. stops growing
Tx: resect if Sxs (elbow bursitis, vascular or nerve compression)
*50% spontaneous, 50% hereditary.
Enchondroma
benign cartilage tumor in hand, calcifies & may erode bone cortex;
Path: endosteal calcification
Sx: pain, fracture (esp. finger joints), age 15-40.
Tx: curettage & graft if Sxs, *be wary of malignant conversion!
Non-ossifying fibroma
latent or active, benign medullary bone tumor; eccentric.
*most common tumor in children, goes away by age 30;
Path: storiform spindle cells, hemosiderin, cortical expansion.
= wild sclerosis from growth plate (“firing blanks”).
Tx: curettage & graft if painful or fractures.
Simple bone cyst
latent or active, in bone metaphysis (80% in prox. humerus & femur), grow away from joint. Age F.
Sx: fracture (fragile bones!)
Tx: no competitive sports until adult, steroid/marrow/graft injection.
giant cell tumor
benign, aggressive bone tumor in epiphysis/metaphysis/subchondr.
Age 20s/30s, M>F,
Sx: large mass, pain (late, from bone lysis), NO inflammation.
Tx: 1. scrape out, 2. cement sides +/- graft, recurrence common
* may metastasize to lungs! –> Tx = RANK ligand Ab
Hemangioma
latent/active benign tumor in spinal bodies (#1) or femur/humerus.
Dx: age 40-60, F>M(!), coarse vertebral striations
* neurologic if 2nd to spinal fracture
Rhabdomyosarcoma
malignant soft tissue tumor in head, neck, & GU tract;
** assoc. w/Li Fraumeni (p53 mut) **
Most: age < 10 (“embryonal” 11p15 translocation) - head.
Fewer: age 10-25 (“alveolar” t(1 or 2, 13) translocation) - deep mm.
Tx: chemotherapy sensitive
Synovial Sarcoma
malignant soft tissue tumor, unknown origin; *slow growing.
Sx: calcify/ossify/cysts, biphasic on histology.
* t(x:18) –> SYT/SSX1 fusion.
Esp. Men 30-50 yrs, around knee/joints.
Lipoma vs. Liposarcoma
Lipoma - benign soft tissue tumor (fatty mass)
Liposarcoma - malignant soft tissue tumor
*Dx: lipoblasts (w/ indented nuclei) in histopath.
Sebhorreic keratosis
benign cutaneous growth/plaque.
actinic keratosis
pre-cancerous skin growth, plaque.
warts (verruca…)
benign skin growths due to virus.
Fibrosarcoma
Rare, deep soft tissue tumor;
penetrating, highly cellular lesion w/ hemorrhage & necrosis.
(Spindle cells in herringbone pattern)
Malignant fibrous histiocytoma (MFH)
MOST common soft tissue cancer in adults,
in muscles of proximal extremities,
De-differentiated –> = undifferentiated sarcoma.
* high rates of metastasis & recurrence :(
