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Tumors > Exam 2 (PDF pages 21-42) > Flashcards

Exam 2 (PDF pages 21-42) Flashcards

1
Q

What is the fancy name for lung cancer?

A

bronchogenic carcinoma

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2
Q

What is the #1 leading cause of cancer death in both men and women?

test q

A

lung cancer

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3
Q

What are the 2 major classifications for lung cancer?

A

1) small cell lung cancer (SCLC)
2) non-small cell lung cancer (NSCLC)

note: 85% of lung cancers are non-small cell

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4
Q

What are the 3 subtypes of non-small cell lung cancer (NSCLC)?

A

1) adenocarcinoma
2) squamous cell
3) large cell

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5
Q

What is the location of small cell lung cancers?

A

submucosa of airways

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6
Q

What is the location of non-small cell lung cancer?

A

periphery of lungs

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7
Q

What is the major risk factor for lung cancers?

A

smoking

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8
Q

What are the complications of small cell lung cancer?

A

SVC syndrome or paraneoplastic syndrome

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9
Q

What are some complications of non-small lung cancer?

A

-hemoptysis
-airway obstruction
-pneumonia
-pleuritic involvement with pain
-pleural effusion
-SVC syndrome
-Pancoast’s tumor (first symptom will be shoulder or arm pain)
-hoarseness (due to laryngeal nerve involvement
-neurological symptoms due to brain metastasis
-pathologic fractures due to bone metastasis
-jaundice due to liver metastasis

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10
Q

What is SVC syndrome?

A

-common in patients with small cell lung cancer
-caused by compression of the SVC
-facial and upper extremity edema
-dilated neck and subcutaneous veins over the face and upper trunk
-facial and truncal plethora (flushing)

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11
Q

What is paraneoplastic syndrome?

A

-disease or symptom that is the consequence of the presence of cancer but not due to the local presence of cancer
-it is mediated by hormonal factors like hormones and cytokines excreted by an immune response against the tumor
-this is common in lung cancers, especially hypercalcemia which is the production of parathyroid hormone related protein or syndrome of inappropriate antidiuretic hormone secretion (SIADH)

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12
Q

What are 2 common paraneoplastic syndromes common in lung cancer?

A

hypercalcemia and SIADH

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13
Q

What is another name for pancoast tumor?

A

superior sulcus tumor

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14
Q

What is a pancoast tumor?

boards q

A

-an apical lung cancer, usually a non-small cell lung cancer
-typically found at the apex of the lung unilaterally
-can invade the brachial plexus, pleura, or ribs causing severe shoulder and UE pain and weakness or atrophy of the ipsilateral hand
-may also produce horner’s syndrome (ptosis, miosis, and anhidrosis)
-rarely may cause esophageal compression (dysphagia)

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15
Q

What cancers may produce horner’s syndrome?

A

1) pancoast tumor (superior sulcus tumor)
2) esophageal cancer

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16
Q

What are the 3 symptoms of horner’s syndrome?

A

1) ptosis (drooping eyelid)
2) miosis (pupil constriction)
3) anhidrosis (lack of sweat)

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17
Q

What is mesothelioma/pleural mesothelioma?

A

-malignancy caused by asbestos expsoure (average latency of 30 years)
-can spread locally or metastasize to the pericardium, diaphragm, peritoneum and rarely the testes
-common symptoms are dyspnea and chest pain
-presents on x-ray as diffuse unilateral or bilateral pleural thickening encasing the lungs

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18
Q

What is renal cell carcinoma/adenocarcinoma of the kidneys?

A

-thrombus formation in the renal vein
-can metastasize to the lymph nodes, lungs, adrenal glands, liver, and bone
-hematuria, flank pain, and fever of unknown origin

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19
Q

What is the most common malignant renal tumor?

test q

A

renal cell carcinoma/adenocarcinoma of the kidneys

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20
Q

What are the risk factors for renal cell carcinoma/adenocarcinoma of the kidneys?

A

-smoking
-obesity
-excess use of phenacetin (fever reducer/pain relief medication that is no longer sold in the market)
-acquired cystic kidney disease in dialysis patients
-adult polycystic kidney disease
-exposure to radiopaque dyes and asbestos, cadmium, and leather tanning and petrolatum products
-excess Tylenol use

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21
Q

What is wilm’s tumor/nephroblastoma?

A

-embryonal cancer of the kidney usually presenting in children under 5 y/o
-associated with chromosomal delation of WT1, the Wilms’ tumor suppressor gene, often associated genetic abnormalities include deletion of WT2 (Wilms’ tumor suppressor gene 2), deletion of chromosome 16, and duplication of chromosome 12
-may be associated with WAGR syndrome
-painless, palpable abdominal mass, hematuria, fever, anorexia, nausea, vomiting

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22
Q

What is WAGR syndrome?

A

W= wilms’ tumor
A= aniridia (born without iris in eye, makes then photophobic)
G= genitourinary malformations
R= mental retardation

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23
Q

Which cancer is 90% of all transitional cell carcinomas?

test q

A

bladder cancer

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24
Q

What are the risk factors for bladder cancer?

A

-smoking
-excess phenacetin use
-chronic irritation
-exposure to hydrocarbons and aromatic amines (aniline dyes)

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25
Q

What are the symptoms of bladder cancer?

A

-unexplained hematuria (gross or microscopic)
-anemia
-irritable voiding symptoms (dysuria/difficulty urinating, binding, frequency)
-pyuria (pus in urine)
-advanced stages may have pelvic pain and palpable pelvic mass

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26
Q

Which cancer has this treatment plan?
-transurethral resection (for early superficial cancers) and BCG (bacillus calmette guerin) instillation and chemo
-more advanced cancer will require radical cystectomy with concomitant urinary diversion
-note: BCG is a vaccine used to prevent TB but can also have anti-cancer properties

test q

A

bladder cancer

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27
Q

What is the most common non-dermatologic cancer in men greater than 50 y/o in the US?

A

prostate cancer/adenocarcinoma of the prostate

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28
Q

What are the risk factors for prostate cancer/adenocarcinoma of the prostate?

A

-age
-black men
-1st degree family hx

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29
Q

What are the symptoms of prostate cancer/adenocarcinoma of the prostate?

A

**usually not until advanced
-hematuria
-bladder outlet obstruction (hesitancy, straining, weak and/or intermittent urine stream, a sense of incomplete emptying)
-bone pain if metastasized

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30
Q

What enzyme is elevated if prostate cancer/adenocarcinoma of the prostate has metastasized to bone?

A

acid phosphatase

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31
Q

Who would have a high PSA test (prostate specific antigen)?

A

-patients with prostate cancer/adenocarcinoma of the prostate
-patients with BPH (benign prostatic hypertrophy)
-smokers

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32
Q

What would confirm prostate cancer/adenocarcinoma of the prostate?

A

TRUS (a guided transrectal needle biopsy)

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33
Q

What is the 3rd most common gynecological cancer?

test q

A

cervical cancer

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34
Q

Cervical cancer is linked to what virus?

A

HPV

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35
Q

What are most of the cervical cancers (80-85%)?

A

squamous cell carcinomas (the other 15-20% are adenocarcinomas)

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36
Q

What are the risk factors for cervical cancers?

test q

A

-early age of intercourse
-a high lifetime number of sex partners
-immunodeficiency
-smoking

note: these are the same risk factors for HPV

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37
Q

What are the signs and symptoms of cervical cancer?

A

-can be asymptomatic early on
-irregular bleeding (postcoital or spontaneously between menses)
-advanced stages= obstructive uropathy, back pain, leg swelling due to venous or lymphatic obstruction

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38
Q

How is cervical cancer diagnosed?

test q

A

papaniccolaou (pap) test and colposcopy directed biopsy or cone biopsy (conization) which is where a cone of tissue is removed using a loop of electrical procedure (LEEP), laser or cold knife

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39
Q

What is the most common gynecological cancer in US?

test q

A

endometrial cancer

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40
Q

What are the risk factors of endometrial cancer?

test q

A

-obesity
-diabetes
-HTN
-tamoxifen use for over 5 years (anti-estrogen med used for breast cancer patients)
-previous pelvic radiation therapy
-personal or family hx of breast or ovarian cancer
-unopposed estrogen (high circulating levels with none or low levels of progesterone, can be associated with obesity, PCOS, nulliparity, late menopause, and an-ovulation)
-hereditary (especially families with hereditary nonpolyposis colorectal cancer (HNPCC) syndrome, also called lynch syndrome)

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41
Q

What is the symptom of endometrial cancer?

A

abnormal uterine bleeding postmenopausally

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42
Q

What is the 2nd most common gynecological cancer?

test q

A

ovarian cancer

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43
Q

What is the deadliest gynecological cancer?

test q

A

ovarian cancer

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44
Q

What are the risk factors for ovarian cancer?

test a

A

-nulliparity
-delayed childbearing
-personal or family hx of endometrial, breast, or colon cancer
-BRCA1 gene

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45
Q

What are the signs and symptoms for ovarian cancer?

A

-early cancer is usually asymptomatic
-may present with a solid, palpable mass
-advanced cancer presents with nonspecific symptoms such as dyspepsia (indigestion), bloating, early satiety (fullness), gas pains, backache, unintended weight loss, change in bowel habits

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46
Q

How is ovarian cancer diagnosed?

A

-CA125 is elevated in 80% of cases
-ultrasound, CT scan, MRI

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47
Q

95% of fallopian tube cancers are….

A

papillary serous adenocarcinomas

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48
Q

What are the risk factors for fallopian tube cancer?

test q

A

-chronic salpingitis (inflammation of the fallopian tubes)
-inflammatory disorders
-infertility

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49
Q

What are the signs and symptoms of fallopian tube cancer?

A

adnexal mass (near ovary or uterus) or vague abdominal or pelvic symptoms like discomfort and bloating

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50
Q

What is the rarest gynecological cancer in the US?

test q

A

vaginal cancer (1%)

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51
Q

What are the risk factors of vaginal cancer?

test q

A

-HPV infection
-cervical or vulvar cancer
-exposure to diethylstilbestrol (DES) in utero predisposes to clear cell adenocarcinoma of vagina which is rare and average diagnosis is 19 y/o
-note: most vaginal cancers (95%) are squamous cell carcinomas

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52
Q

Where specifically is vaginal cancer found?

test q

A

upper 1/3 of the posterior vaginal wall

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53
Q

What are the signs and symptoms of vaginal cancer?

A

-abnormal vaginal bleeding (postmenopausal, postcoital (after sex), or intermenstrual)
-watery vaginal discharge
-dyspareunia (pain during sexual intercourse)

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54
Q

What are the risk factors for vulvar cancer?

test q

A

-vulvar intraepithelial neoplasia (VIN)
-HPV infection
-heavy cig smoking
-lichen sclerosis (patchy skin condition)
-squamous carcinoma of vagina or cervix

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55
Q

What are the signs and symptoms of vulvar cancer?

A

palpable vulvar lesion with history of pruritis

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56
Q

Which tumors are neoplasms of trophoblastic origin that can follow intra- or extra-uterine pregnancy and be benign or malignant?

A

gestational trophoblastic tumors
-benign= hydatidiform mole
-malignant= choriocarcinoma

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57
Q

What is a hydatidiform mole?

A

-an abnormal pregnancy in which chorionic vili become edematous and trophoblastic tissue proliferates
-most common in women less than 17 or greater than 35
-over 80% of hydatidiform moles are benign and regress spontaneously
-uterus becomes larger than expected within 10-16 weeks of gestation
-vaginal bleeding
-lack of fetal movement
-absent fetal heart sounds
-severe vomiting
-excessive amounts of human chorionic gonadotropin (HCG)
-passage of grape-like tissue (fluid filled chorionic tissue) strongly suggests the diagnosis
-tx involves evacuation by suction curettage

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58
Q

If hydatidiform moles persist and become invasive what are they called (note this is very rare)?

A

choriocarcinoma

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59
Q

What is choriocarcinoma?

A

-invasive, usually widely metastasis tumor composed of malignant trophoblastic cells
-usually manifests symptoms due to metastasis in brain and liver, increasing levels of HCG, and persistent bleeding
-tx is chemo and hysterectomy

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60
Q

What is a common cancer for males 15-35 y/o?

test q

A

testicular cancer

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61
Q

Who is high risk for testicular cancer?

test q

A

patients with cryptorchidism (undescended testicle (even if its surgically corrected, the patient is still high risk)

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62
Q

What are the signs and symptoms of testicular cancer?

A

scrotal mass (painless or associated with dull aching pain)

note: most testicular masses are cancerous but most extra testicular masses are not so scrotal ultrasound can confirm origin and nature of mass

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63
Q

What are the serum markers for testicular cancer?

test q

A

elevated alpha fetoprotein and HCG

64
Q

Most penile cancers are squamous cell carcinomas and originate….

A

on the glans, in the coronal sulcus, or under the foreskin

65
Q

Which cancer is this?
-usually begins as a small erythematous lesion confined to the skin and can become ulcerative and infiltrative or a sore that has not healed with subtle induration of the skin

A

penile cancer

66
Q

What are the risk factors for penile cancer?

A

-age (elderly)
-uncircumcised males
-HPV

67
Q

What are the 2 common types of esophageal cancer?

A

1) squamous cell carcinoma
2) adenocarcinoma

68
Q

What are the risk factors for esophageal squamous cell carcinoma?

test q

A

-alcohol ingestion
-tobacco use
-HPV
-irradiation of the esophagus
-lye ingestion
-plummer-vinson syndrome
-tylosis (autosomal dominant disorder)

69
Q

Where is esophageal adenocarcinoma found?

A

distal esophagus

70
Q

Which cancer is associated with barrett’s esophagus (results from chronic irritation from GERD)?

test q

A

esophageal adenocarcinoma

71
Q

What are the signs and symptoms of esophageal cancer?

A

-dysphagia
-chest pain radiating to the back
-weight loss
-vocal cord paralysis (compression of recurrent meningeal nerve)
-hoarseness
-horner’s syndrome (compression of sympathetic nerves)
-spinal pain
-hiccups
-vomiting
-hematemesis (vomiting blood)
-cough

72
Q

What are the risk factors for stomach cancer?

A

-H. pylori infections
-autoimmune atrophic gastritis
-genetics

73
Q

Which cancer risk is DECREASED in patients with duodenal ulcers?

test q

A

stomach cancers

74
Q

Stomach cancer is classified based on appearance. What are the 5 categories?

A

1) protruding (tumor is polypoid or fungating)
2) penetrating (ulcerating on the surface)
3) superifical spreading (tumor spreads along the mucosa)
4) linitus plastica (tumor infiltrates stomach wall with an associated fibrous reaction that produces a leather bottle stomach (no rugae on stomach so it looks leathery))
5) miscellaneous (tumor demonstrates 2+ characteristics of other types)

75
Q

How is stomach cancer diagnosed?

A

-endoscopy and biopsy
-CT scan of the chest and abdomen
-elevated CEA (carcinoembryonic antigen) levels

76
Q

70% of colorectal cancer cases are in the….

A

rectum and sigmoid colon

77
Q

95% of colorectal cancer cases are what type of cancer?

A

adenocarcinomas

78
Q

Is colon cancer more common in women or men?

A

women

79
Q

Is rectal cancer more common in women or men?

A

men

80
Q

What are the hereditary risk factors for colorectal cancer?

A

Hereditary nonpolyposis colorectal carcinoma (HNPCC, an autosomal dominant disorder) and familial adenomatoua polyposis (FAP)

81
Q

What are the signs and symptoms of colorectal cancer?

A

-anemia
-bleeding with defecation
-ascites
-hepatomegaly
-lymphadenopathy

82
Q

How is colorectal cancer diagnosed?

Test q

A

-fecal occult blood (FOB) testing
-colonoscopy or sigmoidoscopy
-biopsy
-elevated CEA levels

83
Q

Most commonly, pancreatic cancers are _____________ tumors that develop from ductal/acinar cells and the most common location is the _________________________

Test q

A

exocrine, head of the pancreas

84
Q

What are the symptoms of pancreatic cancer?

A

**symptoms only appear in late stages
-severe upper abdominal pain radiating to the back (pain can be relieved by bending forward or assuming a fetal position)
-weight loss
-obstructive jaundice
-splenic vein obstruction
-splenomegaly
-gastric and esophageal varices
-GI hemorrhage
-diabetes

85
Q

How do you diagnose pancreatic cancer?

Test q

A

-CT scan or MRI
-biopsy
-elevated CA19-9

86
Q

How do you treat pancreatic cancer?

A

A whipple operation (pancreaticoduodenectomy, partial pancreas and duodenum removal) + chemotherapy and external beam radiation

87
Q

Pancreatic endocrine tumors arise from where?

A

Islet or gastrin producing cells

88
Q

What are some examples of pancreatic endocrine tumors?

A

-ACTHoma
-gastrinoma
-glucogonoma
-GFRoma
-insulinoma
-somatostatinoma
-vipoma

89
Q

What syndrome is associated with ACTHoma (an endocrine pancreatic tumor that produces excess ACTH)?

A

Cushing syndrome (high levels of cortisol)

90
Q

What are the signs and symptoms of a gastrinoma (endocrine pancreatic tumor with excess gastrin)?

A

-abdominal pain
-peptic ulcer
-diarrhea

91
Q

What are the signs and symptoms of glucagonoma (an endocrine pancreatic tumor with excess glucagon)?

A

-glucose intolerance
-rash
-weight loss
-anemia

92
Q

What hormone is overproduced with a GRFoma?

A

growth hormone releasing factor

93
Q

Where is a GRFoma tumor found?

A

usually the lungs

94
Q

Acromegaly is associated with which tumor?

A

GRFoma

95
Q

What sign/symptom is associated with an insulinoma (pancreatic endocrine tumor)?

A

fasting hypoglycemia

96
Q

What are the signs and symptoms of a somatostatinoma (pancreatic endocrine tumor)?

A

-glucose intolerance
-diarrhea
-gallstones

97
Q

Which hormone is overproduced in vipoma (pancreatic endocrine tumor)?

A

vasoactive intestinal peptidase

98
Q

What are the signs and symptoms of a vipoma (pancreatic endocrine tumor)?

A

-severe watery diarrhea
-hypokalemia
-flushing

99
Q

What are the risk factors for liver cancer?

test q

A

-hep B infection (100 fold)
-cirrhosis from chronic hep C
-hemochromatosis
-alcoholic cirrhosis

100
Q

What is the fancy name for liver cancer?

A

hepatocellular carcinoma

101
Q

What are the signs and symptoms for liver cancer?

A

-abdominal pain
-weight loss
-RUQ mass
-ascites
-hypoglycemia
-hypercalcemia
-hyperlipidemia

102
Q

How is liver cancer diagnosed?

test q

A

-elevated levels of alpha fetoprotein (AFP)
-CT scan, ultrasound, MRI
-biopsy

103
Q

Metastatic cancer in the liver is the MOST COMMON hepatic malignancy- commonly originating in the….

A

-GI tract
-breast
-lung
-pancreas

104
Q

Which cancer is this?
-incidental finding at a cholecystectomy done for biliary pain and cholelithiasis (gallstones)
-advanced stages will have pain, weight loss, and abdominal mass

A

gallbladder cancer

105
Q

Which cancer is this?
-develop from neuroendocrine cells in the GI tract (appendix, ileum, and rectum), pancreas, and pulmonary bronchi
-can be endocrinologically inert or produce a variety of hormones
-the most common endocrinologic symptom is a syndrome (same name as the tumor)

test q

A

carcinoid tumor

106
Q

What is carcinoid syndrome?

test q

A

-characterized by cutaneous flushing, abdominal cramps and diarrhea resulting from vasoactive substances including serotonin, bradykinin, histamine, and prostaglandins
-diagnosis is confirmed by increased urinary excretion of the serotonin metabolite 5-hydroxyindolacetic acid (5-HIAA)

107
Q

What is a krukenberg tumor?

test q

A

-rare 1-2% of ovarian tumors
-metastatic signet cell adenocarcinoma of the ovary, usually bilateral
-metastasizes to the ovary from the stomach, colon, appendix, or breast (ILC), note: this is called transcoelomic spread because the cancer is being spread across cavities
-abdominal pain and distension
-may have increased CA125 levels

108
Q

What is a neurofibromatosis?

A

-benign fibrous tumor
-inherited
-autosomal dominat
-has 2 clinical forms

109
Q

What are the 2 clinical forms of neurofibromatosis?

A

1) neurofibromatosis 1 (von Recklinghausen’s disease, cafe au lait spots, neurofibromas, bone changes)
2) neurofibromatosis 2 (acoustic nerve tumors)

110
Q

What are the signs and symptoms of neurofibromatosis?

A

-if on the optic nerve= blurred vision and scotoma
-if on the posterior superior orbital wall= dropping eyelid and exophthalmos
-if on spinal nerves= localized pain and motor symptoms

111
Q

What are the cutaneous abnormalities in neurofibromatosis?

test q

A

-6+ cafe au lait spots
-fibroma molluscum
-elephantasis neuromatosa

112
Q

What are cafe au lait spots?

A

-non-elevated cutaneous tan macules
-most commonly found on the back, chest, and abdomen
-smooth margins= coast of Cali appearance*
-jagged irregular margins= coast of Maine appearance*

113
Q

What is the most frequent diagnostic feature of neurofibromatosis?

A

fibroma molluscum (multiple asymptomatic elevated cutaneous nodules)

114
Q

What is elephantiasis neuromatosa?

A

large soft tissue masses creating skin folds

115
Q

What are the radiological features of neurofibromatosis?

A

-scoliosis with kyphoscoliosis in the lower thoracic spine (most common), may cause paraplegia
-cervical kyphosis with posterior vertebral body scalloping
-enlargement of IVF due to localized, dumbbell neurofibroma and erosion of the posterior body and pedicle
-agenesis of the posterior wall of the orbit, wings of the sphenoid and the orbital plate of the frontal bone
-macrocranium and macroencephaly
-scalloping irregularities of the ribs creating a twisted ribbon appearance

116
Q

What is a fibrous cortical defect (FCD)?

A

-most common in 4-8 y/o
-more common in females
-incidental finding
-bones of the LE are most effected (distal femur is most common)
-lesion is solitary, eccentric, radiolucent or bubbly ovoid, usually located in the metaphysis

117
Q

What is non-ossifying fibroma?

A

-similar lesion to FCD but different age range
-FCD is 4-8 y/o whereas non-ossifying fibroma is 8-20 y/o
-most commonly affects bones of the LE (distal femur is most common)
-lesions are diametaphyseal and eccentric (off to the side) in position
-lesion is solitary, radiolucent, generally ovoid and there is often scalloped margins and a multilocular, bubbly appearance

118
Q

What are the 3 forms of fibrous dysplasia?

A

1) monostotic
2) polyostotic w/o endocrine involvement
3) polyostotic w/ endocrine involvement (McCune-Albright syndrome)

119
Q

What is McCune-Albright syndrome?

test q

A

-polyostotic fibrous dysplasia with endocrine involvement
-skin pigmentation
-precocious (premature) sexual development

120
Q

What is the most common sign/symptom of fibrous dysplasia?

test q

A

bowing deformities and pathologic fractures

121
Q

What is the cutaneous abnormality for fibrous dysplasia?

A

cafe au lait spots with jagged irregular margins (coast of Maine) appearance

122
Q

What is cherubism?

A

familial fibrous dysplasia in the jaw

123
Q

What are the radiological features of fibrous dysplasia?

test q

A

-most lesions are radiolucent, locuated or trabeculated in appearance and may have a “ground glass” or “smoky” appearance
-most lesions have a sclerotic border “rind of sclerosis”
-shepherd’s crook deformity of the femur (reduction of the femoral angle to near 90 degrees when normal is 120-130 degrees, creating a coxa vara deformity)

124
Q

What is a shepherd’s crook deformity?

A

reduction of the femoral angle to near 90 degrees when normal is 120-130 degrees, creating a coxa vara deformity

125
Q

What is a chondroma/periosteal chondroma?

A

-slowly growing benign cartilaginous tumor
-most commonly found in small bones of hands and feet, humerus, femur, tibia, radius, ulna, and fibula
-commonly metaphyseal in location, appears as an area of cortical indentation (scalloped or saucerized depression of the outer cortex)

126
Q

What is a chondroblastoma?

A

-rare, benign cartilaginous tumor
-appears as an oval or round lytic lesion, most are eccentric

127
Q

What is the most common benign tumor of the hand?

A

enchondroma (usually asymptomatic until trauma)

128
Q

The presence of multiple enchondromas is referred to as….

A

enchondromatosis or Ollier’s disease

129
Q

What is the most common benign skeletal growth/tumor?

A

osteochondroma

130
Q

What is an osteochondroma?

test q

A

-a bony exostosis projecting from the external (metaphyseal) surface of a bone
-usually affects long bones
-grows away from the joint
-2 types

131
Q

What are the 2 types of osteochondroma?

A

1) sessile (flat broad based protuberance (less common)
2) pedunculated (elongated bony stalk within a cartilaginous cap (coat hanger exostosis) projecting away from a joint

132
Q

What is a hereditary multiple exostosis (HME)?

A

-an inherited autosomal dominant metaphyseal overgrowth
-bones aren’t growing properly (2-10 y/o)
-painless lumpy joints
-xray will show a bayonet hand deformity which is a shortening of the ulna and bowing of the radius as a result of retardation of bone growth and also thickened medial neck of the femur

133
Q

What is the most common benign tumor of the nose and paranasal sinuses (most lesions occur in the frontal and ethmoid sinuses)?

test q

A

osteoma

134
Q

Which tumor is associated with Gardner’s syndrome (multiple benign tumors, colonic polyposis, and soft tissue fibromas)?

A

osteoma

note: most common location of osteoma with Gardner’s syndrome is the mandible NOT THE SINUSES like it would be with normal osteoma

135
Q

What is an endosteoma/bone island?

A

-solitary discrete area of sclerosis commonly found in the ischium, ilium, sacrum, and proximal femur (can be found elsewhere but NEVER in the skull)
-usually an incidental finding

136
Q

What is another name for paget’s disease?

A

osteitis deformans

137
Q

What is the most common symptom for paget’s disease?

test q

A

dull pain, present at rest and at night, not aggravated by exertion

138
Q

What are the 4 stages of paget’s disease?

A

1) osteolytic/destructive (aggressive bone resorption)
2) combined/mixed
3) sclerotic or ivory
4) malignant degeneration

139
Q

What are the most common bones affected in paget’s disease?

test q

A

-pelvis
-vertebrae
-clavicle
-humerus
-ribs
-skull

140
Q

What are the radiologic features of paget’s disease?

A

-thickened cortex (bones becoming bigger slowly overtime)
-coarsened trabeculae pattern
-bone expansion
-increased or decreased density
-deformity

141
Q

What are the radiological signs of paget’s disease?

test q

A

-osteoporosis circumscripta (decreased density in the skull/lytic stage in frontal and occipital region)
-cotton wool appearance in skull
-picture frame vertebra (vertebral body is enlarged with thickened cortex)
-ivory vertebra (enlarged vertebral body is radiopaque)
-brim/rim sign (thickening of the iliopectineal line/obliteration of kohler’s teardrop)
-blade of grass appearance (sharply demarcated radiolucency usually V shaped in long bones)

142
Q

What are the 3 most common causes of an ivory vertebra?

A

1) osteoblastic metastatic carcinoma
2) paget’s disease
3) hodgkin’s lymphoma (anterior scallop on vertebral body)

143
Q

What are the 4 most common causes of portrusio acetubuli?

A

1) paget’s disease
2) osteomalacia
3) RA
4) trauma

“PORT”

144
Q

What are the common complications/deformities of paget’s disease?

A

-shepherd’s crook deformity of the proximal femur (coxa vara)
-saber shin deformity (anterior tibial bowing)
-protrusio acetubuli (weakened pelvic bones)
-basilar invagination (the weight of the enlarged skull leads to molding of the skull base which may lead to brain stem compression, obstructive hydrocephalus and cranial nerve palsies
-nerve deafness (complicated by severe auditory canal stenosis from bone expansion)
-pathologic fracture
-pseudofractures/looser’s lines (areas of demineralization within bone and may predispose to complete fracture
-spinal stenosis (due to expansion of vertebral body and neural arch)
-malignant degeneration

145
Q

What is the most common complication of paget’s disease?

test q

A

pathologic fracture (and the most common affected site is the proximal 1/3 of femur)

146
Q

What are the lab findings for paget’s disease?

test q

A

-high alkaline phosphatase (20x higher than normal)
-high serum/urinary hydroxyproline

note: both of these indicate bone lysis/damage

147
Q

What is the treatment for paget’s disease?

test q

A

calcitonin or biphosphonates to inhibit bone resorption

148
Q

When would you see a fallen fragment sign after pathologic fracture (cortical fragment of bone falling in the lesion)?

A

simple bone cyst/unicameral bone cyst/solitary bone cyst

149
Q

What is the CC for giant cell tumor/osteoclastoma?

A

intermittent aching pain with localized swelling and tenderness and restricted ROM

150
Q

What is the most common benign tumor of the clavicle?

A

aneurysmal bone cyst (ABC)

151
Q

What is the only benign tumor known to cross the epiphyseal plate?

A

aneurysmal bone cyst (ABC)

152
Q

Which cyst has a cortical ballooning termed as “blown out” appearance with a very thin cortical outline?

A

aneurysmal bone cyst (ABC)

153
Q

What is the most common benign tumor of the spine?

A

hemangioma

154
Q

Where is a hemangioma found?

A

lower thoracic/upper lumbars in the vertebral body or the frontal bone of skull

155
Q

What is the classic presentation of osteoid osteoma?

A

night pain relieved by aspirin accompanied by vasomotor disturbances (profuse sweating and increased skin temp)

156
Q

Which tumor shows up on xray with an oval or round lucent nidus?

test q

A

osteoid osteoma

157
Q

Which tumor has a ghost like appearance (cloud calcifications within muscle)?

A

myositis ossificans

Tumors (3 decks)

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