Tumors

Question 0

Glioblastoma multiforme

Answer 0

= 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

Question 1

fibrillary astrocytoma

Answer 1

= glioma in younger ppl, solid or cystic, grade II or III;

path: poorly demarcated, diffuse infiltration throughout brain, w/ fibrillary astrocytes & GFAP

Question 2

pilocytic astrocytoma

Answer 2

~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

Question 3

Oligodendroglioma

Answer 3

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)**

Question 4

Ependymoma

Answer 4

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.

Question 5

primary tumors in cerebral hemispheres (6)

Answer 5

1. Glioblastoma 4. Astrocytoma
2. Oligodendroglioma 5. Ependymoma
3. CNS lymphoma 6. meningioma - benign

Question 6

primary tumors in cerebellum, brainstem, etc. (posterior fossa) (7)

Answer 6

1. Medulloblastoma 5. hemangiomablastoma
2. pilocytic astrocytoma 6. schwannoma
3. ependymoma 7. meningioma
4. (diffuse) astrocytoma

Question 7

primary tumors in the sellar region (4)

Answer 7

1. craniolpharyngioma
2. pituitary adenoma
3. Germ cell neoplasms (esp. pineal region)
4. pilocytic astrocytoma (esp. optic n, hypothalamus)

Question 8

primary tumors in the spinal canal (5)

Answer 8

1. astrocytoma (diffuse or pilocytic)
2. ependymoma
   
   • myxopapillary
3. schwannoma
4. meningioma
5. neurofibrola

Question 9

Meduloblastoma

Answer 9

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

Question 10

meningioma

Answer 10

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.

Question 11

hemangiopericytoma

Answer 11

a sarcoma, vascular tumor in meninges or elsewhere;
Path: “stag horn” blood vessels
Tx: resect, frequently recurs, aggressive

Question 12

schwannoma

Answer 12

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!

Question 13

Neurofibroma

Answer 13

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.

Question 14

von Rcklinghausen’s disease (NF-1)

Answer 14

type 1 neurofibromatosis,
= peripheral, w/ cafe au lait spots & cutaneous neurofibromas, optic gliomas, etc.
Mut: neurofibromin on chrom. 17

Question 15

NF-2 neurofibromatosis

Answer 15

central form w/ >1 CN & spinal root schwannomas (ie: bilateral acoustic schwannomas) & gliomas, but NOT usually neurofibromas.
Mut: merlin (chrom 22)

Question 16

Metastatic brain tumor characteristics

Answer 16

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

Question 17

Primary CNS Lymphoma

Answer 17

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.

Question 18

Secondary lymphomas

Answer 18

= metastatic from systemic lymphomas, not 1st presentation;

mostly in leptomeninges & dura, multiple.

Question 19

Craniopharyngioma

Answer 19

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

Question 20

germinoma

Answer 20

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

Question 21

Teratoma

Answer 21

differentiated germ cell tumor, in pineal region.

• ecto, meso, & endodermal parts
• in children, 90+% M, benign OR w/ malignant parts

Question 22

pituitary adenoma

Answer 22

hormone-producing tumors in sella (expand to brain parenchyma),
Sxs: visual field defects, + sudden severe headache if infarction or hemorrhage of tumor

Question 23

Metastatic bone tumors

Answer 23

*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!

Question 24

Primary osteosarcoma

Age? Path? Bad?

Answer 24

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!

Question 25

Ewing’s Sarcoma

Where? What gene mut? How bad?

Answer 25

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)

Question 26

Chondrosarcoma

Answer 26

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 :(

Question 27

Myeloma

Answer 27

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 :(

Question 28

osteoid osteoma

Answer 28

bone-forming tumor (F*
Sx: night pain, profound relief w/ NSAIDs (prostaglandins)
Path: sclerotic w/ small nidus
Tx: 1. Radio-ablation, 2. resection

Question 29

Osteochondroma

Answer 29

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.

Question 30

Enchondroma

Answer 30

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!

Question 31

Non-ossifying fibroma

Answer 31

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.

Question 32

Simple bone cyst

Answer 32

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.

Question 33

giant cell tumor

Answer 33

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

Question 34

Hemangioma

Answer 34

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

Question 35

Rhabdomyosarcoma

Answer 35

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

Question 36

Synovial Sarcoma

Answer 36

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.

Question 37

Lipoma vs. Liposarcoma

Answer 37

Lipoma - benign soft tissue tumor (fatty mass)
Liposarcoma - malignant soft tissue tumor
*Dx: lipoblasts (w/ indented nuclei) in histopath.

Question 38

Sebhorreic keratosis

Answer 38

benign cutaneous growth/plaque.

Question 39

actinic keratosis

Answer 39

pre-cancerous skin growth, plaque.

Question 40

warts (verruca…)

Answer 40

benign skin growths due to virus.

Question 41

Fibrosarcoma

Answer 41

Rare, deep soft tissue tumor;
penetrating, highly cellular lesion w/ hemorrhage & necrosis.
(Spindle cells in herringbone pattern)

Question 42

Malignant fibrous histiocytoma (MFH)

Answer 42

MOST common soft tissue cancer in adults,
in muscles of proximal extremities,
De-differentiated –> = undifferentiated sarcoma.
* high rates of metastasis & recurrence :(