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114 Cards in this Set

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What is MEN 1?
familial multiglandular endocrine tumor syndrome
affects 2-10 in 100,000
may present in childhood or adulthood
presentation varies
hyperparathyroidism

may include pituitary, parathyroid, endopancreatic, and non-endocrine tumors

gene testing
hypercalcemia
What is a giant cell tumor?
benign vascular lesion in bone but may transform into malignancy
Orthopedics p386
What are multiple endocrine neoplasias?
autosomal dominant genetic disorders causing a predisposition in the development of tumors, especially tumors involving endocrine glands
Define oncogene.
a gene that normally codes for proteins that up-regulate cell growth and differentiation; if mutated or over-expressed, can cause normal cells to become cancer cells
What characterizes MEN 2A?
medullary thyroid cancers, pheochromocytomas, Hirschsprung disease
What is the etiology of giant cell tumor?
spontaneous
associated with hyperparathyroidism
rare
usually occurs between 20-40y/o
rare in children and elderly
What characterizes MEN 2B?
medullary thyroid cancers, pheochromocytomas, Marfan-like habitus, mucosal neuromas, intestinal ganglioneuroma, delayed puberty
Define tumor suppressor gene.
a gene that normally codes for proteins that down-regulate cell growth or cause apoptosis; if mutated or under-expressed, can cause normal cells to become cancer cells
What characterizes MEN 1?
tumors of the parathyroid glands, endocrine pancreas and duodenum, pituitary, adrenal, thyroid; lipomas and facial angiofibromas
What is the clinical presentation of a giant cell tumor?
bone pain usually affecting distal metaphysis and epiphysis of femur
possible pathologic fracture
Define hypertrophy.
enlargement due to an increase in the size of cells
What is the diagnostic workup of a giant cell tumor?
AP and lateral radiographs of femur → "soap bubble" appearance
Orthopedics p386
Define hyperplasia.
enlargement due to an increase in the number of cells
What is the management of a giant cell tumor?
surgical removal
Define dysplasia.
partially reversible change from a mature cell to cell with abnormalities in both differentiation and maturation
List types of bone tumors and their characteristic radiographic appearance.
osteosarcoma → sunburst appearance
ewing's sarcoma → onion skin appearance
giant cell tumor → soap bubble appearance
Define anaplasia.
regression of cells to an undifferentiated state
Benign and malignant lesions typically develop in which part of a long bone?
metaphysis
Define aplasia.
congenital absence or defective development of a tissue or organ
What percent of 2° bone tumors affect the spine?
40%

*spine = most common site for bone metastasis
Orthopedics p356
Define metaplasia.
reversible change from one type of mature cell to another type of mature cell that is normally not found in the tissue involved
What is an enchondroma?
benign bone tumor originating from cartilage (i.e. cartilage cyst)
Orthopedics p446
Define neoplasm.
tissue that grows by cell proliferation more rapidly than normal and continues to grow after stimuli that initiated the new growth cease; usually forms a distinct mass; either benign or malignant
What is the clinical presentation of enchondroma?
usually occurs between 10-50 y/o
nonspecific symptoms
no pain
usually in small bones of hands and feet
discovered incidentally or following pathologic fracture
enlarged fingers
deformities
Orthopedics p446
Define neoplasia.
pathological process that results in a neoplasm
Where do enchondromas most commonly occur?
small bones of hands and feet
Orthopedics p446
Define carcinoma.
any malignant neoplasm derived from epithelial cells
What is the diagnostic workup of enchondroma?
radiograph
usually located centrally in bone
What are examples of carcinoma?
squamous cell carcinoma
basal cell carcinoma
melanoma
adenocarcinoma
What is the management of enchondroma?
1. observation → periodic radiographs
2. if diagnosis uncertain → surgery to R/O chondrosarcoma
3. if recurrent fracture → surgery
Orthopedics p446
Define carcinoma in situ.
localized carcinoma; precursor of metastatic carcinoma
How do you distinguish between enchondroma and chondrosarcoma?
enchondroma → painless
chondrosarcoma → painful, bone erosion
Define metastatic/invasive carcinoma.
carcinoma that has spread beyond epithelium into adjacent structures
enchondroma (of 5th metacarpal)

L: radiograph
R: MRI
What are the most common sites of metastasis for lung cancer?
brain, bones
http://www.cancer.gov/cancertopics/factsheet/Sites-Types/metastatic
What is an osteochondroma?
classified as benign bone tumor but thought to be a developmental abnormility where part of physis forms outgrowth on bone surface
Orthopedics p446
What are the most common sites of metastasis for colon cancer?
liver
http://www.cancer.gov/cancertopics/factsheet/Sites-Types/metastatic
What is the clinical presentation of osteochondroma?
painless mass
usually occurs at distal metaphysis of femur or proximal metaphysis of tibia
growth ceases with skeletal maturity
Orthopedics p446
What are the most common sites of metastasis for breast cancer?
brain, lungs, liver, bones
http://www.cancer.gov/cancertopics/factsheet/Sites-Types/metastatic
What is the management of osteochondroma?
1. observation → periodic radiographs → often regresses once reach skeletal maturity
2. if symptoms (pain, blood vessel or nerve compromise, large cartilage cap) → surgical removal once reach skeletal maturity
Orthopedics p446
What are the most common sites of metastasis for prostate cancer?
bones
http://www.cancer.gov/cancertopics/factsheet/Sites-Types/metastatic
What is the diagnostic workup of osteochondroma?
radiograph →
sessile or pedunculated lesion
continuous with cortex and marrow cavity
small cartilage cap
List the 5 most common sites of metastasis and their associated symptoms.
brain → HA, vertigo, seizures
lungs → dyspnea, cough, hemoptysis
liver → hepatomegaly, jaundice
bones → pain, fracture
lymph nodes → lymphadenopathy
pedunculated osteochondroma of distal femur + associated fracture
Define sarcoma.
any malignant neoplasm derived from connective tissue cells
What is chondrosarcoma?
malignant cartilage tumor
primary or secondary from enchondroma
Orthopedics p448
Define leukemia.
malignancy derived from hematopoietic cells
What is the clinical presentation of chondrosarcoma?
occurs >40 y/o
shoulder girdle, pelvis, proximal femur
painful swelling
Orthopedics p448
What does ALL stand for?
acute lymphocytic leukemia
What bone tumor presents at the diaphysis?
Ewing's sarcoma
Orthopedics p448
What does AML stand for?
acute myeloid leukemia
What is the diagnostic workup of chondrosarcoma?
radiograph → speckled calcifications in destructive radiolucent lesion
Orthopedics p449
What is the difference between acute and chronic leukemias?
acute → predominance of immature WBCs

chronic → bone marrow functional → differentiation → predominance of mature WBCs
Pathology p84
bone destruction + sunburst pattern → osteosarcoma (of proximal fibula)
Who is most commonly affected by chronic leukemia, children or adults?
adults
Pathology p84
chondrosarcoma of inferior ramus of pubis
Who is most commonly affected by acute leukemia, children or adults?
children
Pathology p84
Ewing's sarcoma (of femur)
What is the etiology of AML?
malignant proliferation of myeloid precursor cells; commonly occurs in young adults 15-39y/o
What is the management for chondrosarcoma?
1. refer to orthopedic specialist
2. surgical resection
3. NOT sensitive to chemotherapy or radiation
Orthopedics p449
What is the clinical presentation of acute leukemia?
acute onset
pallor
bleeding → petechiae, purpura, gingival bleeding, epistaxis, menorrhagia
infection → pneumonia, cellulits, perirectal fissures
gingival hypertrophy
hepatosplenomegaly variable
lymphadenopathy variable
bone tenderness → sternum, femur, tibia
joint pain
hyperleukocytosis → headache, confusion, blurred vision, dyspnea, priapism
Current p464
What is the most common tumor of the hand?
enchondroma
What is the diagnostic work-up of acute leukemia?
CBCDP → pancytopenia
blood smear → blasts
bone marrow biopsy → hypercellular, >20% blasts
flow cytometry to determine type of leukemia
Current p464
What is multiple myeloma?
neoplastic proliferation of plasma cells of bone marrow
Orthopedics p168
Which is more difficult to treat, AML or ALL?
AML
Pathology p85
What is the etiology of ALL?
leukemia characterized by malignant proliferation of lymphoid precursor cells; commonly occurs in young adults
What is the ddx for leukemia?
idiopathic
chromosomal abnormalities
myeloproliferative diseases
myelodysplasia
toxins → benzene
chemotherapy
radiation
Current p464
ALL comprises _% of childhood leukemias and _% of adult leukemias?
80% of childhood leukemias
20% of adult leukemias
Current p464
What are auer rods?
needle-like clumps in cytoplasm of myeloid blasts
auer rods → AML
What is the management for acute leukemia?
1. refer to hematologist
2. initial chemotherapy → dependent on leukemia type
3. postremission therapy → chemotherapy, transplantation → dependent on age, risk-factors, status
Current p467
What is the prognosis for AML?
if <60y/o, complete remission for 70-80%; 35-60% cure rate dependent on postremission therapy
if >60y/o, complete remission for 50% but only 10-15% cure rate
Current p467
What does CLL stand for?
chronic lymphocytic leukemia
What is CLL?
leukemia characterized by malignant proliferation of B lymphocytes
What is the etiology of CLL?
malignant proliferation of B lymphocytes; commonly occurs >50y/o
Current p467
What is the clinical presentation of CLL?
fatigue
lymphadenopathy
hepatosplenomegaly
Current p467
What is the diagnostic work-up of CLL?
CBCDP → lymphocytosis
FISH
Current p467
What is the management of CLL?
1. refer to hematologist
2. if early → observation
3. if progressive fatigue, symptomatic lymphadenopathy, anemia, or thrombocytopenia → treatment
4. chemotherapery → fludarabine
5. antibody → rituximab
6. if refractory → allogenic transplantation
Current p467
What are the complications of CLL?
autoimmune hemolytic anemia
immune thrombocytopenia
Current p467
What is the prognosis of CLL?
if early, 10-15 years
if late, 2 years
Current p467
What are the USPSTF screening recommendations for bladder cancer?
do not screen (grade D)

*encourage smoking cessation since 50% of all bladder cancer occurs in current or former smokers
USPSTF Handbook 2009
What are the USPSTF screening recommendations for prostate cancer?
1. men <75y/o → current evidence insufficient to recommend screening (grade I)
2. >75y/o → do not screen (grade D)

*discuss benefits and harms of prostate cancer screening and treatment with patients
USPSTF Handbook 2009
What are the USPSTF screening recommendations for testicular cancer?
do not screen in asymptomatic adolescent and adult males (grade D)
USPSTF Handbook 2009
What are the USPSTF screening recommendations for renal cell carcinoma?
no recommendations
USPSTF Handbook 2009
What are the USPSTF screening recommendations for penile cancer?
no recommendations
USPSTF Handbook 2009
What are the USPSTF screening recommendations for Wilms' tumor?
no recommendations
USPSTF Handbook 2009
What do the USPSTF screening grades mean?
A = recommend screening (high benefit)
B = recommend screening (moderate benefit)
C = recommend against screening (but may be useful in certain patients)
D = recommend against screening (no benefit or potential harm)
I = insufficient evidence to recommend for or against screening
What is the controvery of PSA screening?
prostate cancer is slow-growing
may be lethal or cause no harm
controvery over overdiagnosis and overtreatment
controvery over whether PSA screening save lives or just subjects patients to unecessary radiation, surgery, and side effects
What are the risk factors of prostate cancer?
black
high fat diet
FH of prostate cancer
Current ch39
What is the clinical presentation of prostate cancer?
prostate → focal nodules or areas of induration
(prostate may feel normal but PSA elevated)
if large → obstructive voiding symptoms
Current ch39
What is the diagnostic workup of prostate cancer?
elevated PSA
prostate biopsy
transrectal ultrasonanography for staging
bone scan for bone metastasis
MRI for lymph metastasis
Current ch39
What is the management of prostate cancer?
controversial
management based on age, comorbidities, staging
observation → serial rectal exams, PSAs, and biopsies
if >10 year life expectancy → consider treatment with prostatectomy or radiation therapy
Current ch39
What is the pattern of metastasis for prostate cancer?
axial skeleton → back pain, pathologic fractures
lymph nodes → LE lymphedema
Current ch39
What are the risk factors for bladder cancer?
smoking
environmental exposure to solvents or dyes
Current ch39
What is the clinical presentation of bladder cancer?
hematuria
irritative voiding symptoms → frequency, urgency
Current ch39
What is the diagnostic workup of bladder cancer?
UA → hematuria
cytology
US, CT, or MRI → filling defects
biopsy → staging
Current ch39
What is the pattern of metastasis for bladder cancer?
liver → hepatomegaly
lymph → LE lymphedema
Current ch39
What is the management of bladder cancer?
1. if superficial → resection +/-local chemotherapy
2. if invasive but localized → cystectomy, irradiation, chemotherapy
3. if invasive → systemic chemotherapy followed by cystectomy
Current ch39
What are the risk factors for renal cell carcinoma?
smoking
familial and genetic factors
dialysis
What is the clinical presentation of renal cell carcinoma?
fever, weight loss
hematuria
flank pain
palpable mass in abdomen
What is the diagnosic workup of renal cell carcinoma?
erythrocytosis 5% though anemia more common
hypercalcemia 10%
UA → hematuria 60%
KUB or CT → renal masses
What is the management of renal cell carcinoma?
1. if localized → partial or complete nephrectomy
2. no effective chemotherapy
What is the pattern of metastasis for renal cell carcinoma?
◦Lung (75%)
◦Soft tissues (36%)
◦Bone (20%)
◦Liver (18%)
◦Cutaneous sites (8%)
◦Central nervous system (8%)
What are the risk factors for testicular cancer?
most common 20-35y/o and >60y/o
cryptochorism


*trauma and infertility DO NOT increase risk
What is the clinical presentation of testicular cancer?
usually asymptomatic
painless swelling or nodule of testicle
sensation of heaviness
nodule cannot be separated from testicle
secondary hydrocele
if advanced,
What is the diagnostic workup of testicular cancer?
HCG
AFP (alpha-fetoprotein)
LD (lactate dehydrogenase)
scrotal ultrasound → to distinguish between testicular vs non-testicular
inguinal orchiectomy → establishes diagnosis
chest, abdominal, and pelvic CT → staging
What is the management of testicular cancer?
1. inguinal exploration
2. if testicular cancer cannot be excluded → orchiectomy
3. avoid testicular biospy
4. further therapy dependent on histology and staging → irradiation, chemotherapy
5. FUV monthly x 2 years, then bimonthly x 1 year, obtaining tumor markers at each visit and CXR/CT every 3 months
What is the pattern of metastasis of testicular cancer?
lymph
retroperitoneal
lung
brain
What are the risk factors of penile cancer?
5th or 6th decade
uncircumsized
smoking
balanitis
What is the clinical presentation of penile cancer?
non-tender ulcer or warty growth under foreskin
itchy or burning sensation beneath foreskin
hidden behind erythematous phimosis
What is the diagnostic workup of penile cancer?
ultrasonagraphy or MRI
biospy
What is the management of penile cancer?
1. circumcision
2. resection
3. radiation if young male + noninvasive cancer, resection refused, metastasis
4. chemotherapy if metastasis
What is the pattern of metastasis of penile cancer?
lymph
What is the best treatment for metastatic prostate cancer?
decreasing testosterone