path recall April 2015 - formatted Flashcards

1
Q
  1. Which of the following cancer is most closely associated with UV ray

a. Sebaceous carcinoma
b. Merkel cell carcinoma
c. Mastocytosis..

A

b. Merkel cell carcinoma as per dermnet

Sebaceous carcinoma is a rare type of cancer that begins in an oil gland in your skin. Sebaceous carcinoma most often affects the eyelids.
Merkel cell carcinoma is a very rare disease in which malignant (cancer) cells form in the skin.
Sun exposure and having a weak immune system can affect the risk of Merkel cell carcinoma.
Merkel cells are found in the top layer of the skin. These cells are very close to the nerve endings that receive the sensation of touch. Merkel cell carcinoma, also called neuroendocrine carcinoma of the skin or trabecular cancer, is a very rare type of skin cancer that forms when Merkel cells grow out of control. Mastocytosis is characterized by excessive accumulation of mast cells in one or more organs. - diffuse sclerosis

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2
Q
  1. Not recognized as risk factor for HCC

a. Lysosomal storage disorder
b. Tyrosinaemia
c. Wilson
d. Hemachromatosis
e. Hepatitis B

A

a. Lysosomal storage disorder not listed on RP2. Not recognized as risk factor for HCC

a. Lysosomal storage disorder not listed on RP
b. Tyrosinaemia listed RP
c. Wilson definitely
d. Hemachromatosis definitely
e. Hepatitis B definitely

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3
Q
  1. Which of the following is not a complication of autoimmune gastritis

a. Subacute combined degeneration
a. Carcinoid
b. Increased gastrin level
c. Gastric cancer
d. Microcytic anaemia

A

e. Microcytic anaemia - Usually macrocytic due to pernicious anaemia

  1. Which of the following is not a complication of autoimmune gastritis
    a. Subacute combined degeneration yes – due to B12 deficiency (pernicious anaemia
    b. Carcinoid - yes
    c. Increased gastrin level - yes. Hypochloridia due to loss of parietal cells means reduced feedback inhibition of gastrin secreting cells.
    d. Gastric cancer - yes
    e. Microcystic anaemia - Usually macrocytic due to pernicious anaemia
    Autoimmune gastritis is a chronic inflammatory disease with destruction of parietal cells of the corpus and fundus of the stomach. The known consequence is vitamin B12 deficiency and, consequently, pernicious anemia.
    Complication- subacute combined degeneration- increased gastrin level - carcinoid- gastric cancer- megaloblastic anaemia
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4
Q
  1. Congenital intra-abdominal hernia, most common

a. Foramen of Winslow
b. Paraduodenal
c. Para-sigmoid
d. Para-cecal
e. Trans mesenteric

A

b. Paraduodenal according to RP – left foramen of Landzert more common than right foramen of Waldyer

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5
Q
  1. Which is an associated complication of pre-eclampsia

a. Hypofibrinogenaemia
b. Thrombocytosis

A

a. Hypofibrinogenaemia

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6
Q
  1. Highest risk of uterine rupture

a. Placenta previa
b. Placenta increta
c. Placenta acreta
d. Placenta percreta

A

d. Planceta percreta

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7
Q
  1. Which disorder affects liver and skin

a. Dermatitis herpetiformis
b. Porphyria cutanea tarda
c. Necrotising erythema migrans
d. Wilson disease
e. Mycosis fungoides

A

b. Porphyria cutanea tarda due to defective enzyme in the liver involved in the synthesis of haem. Although liver function only affected sometimes. Probably most correct.

  1. Which disorder affects liver and skin
    a. Dermatitis herpetiformis can have fatty liver
    b. Porphyria cutanea tarda due to defective enzyme in the liver involved in the synthesis of haem. Although liver function only affected sometimes. Probably most correct.
    c. Necrotising erythema migrans can do
    d. Wilson disease can have increased pigmentation.
    e. Mycosis fungoides no
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8
Q
  1. Regarding retinoblastoma,choose most accurate

a. Contralateral retinocytoma suggest hereditary process
b. Lung is a common site for hematogenous metastasis
c. Autosomal recessive inheritance.
d. Chondrosarcoma is a secondary primary cancer.

A

a. Contralateral retinocytoma suggest hereditary process

  1. Regarding retinoblastoma,choose most accurate
    a. Contralateral retinocytoma suggest hereditary process
    b. Lung is a common site for hematogenous metastasis bone, bone marrow and liver
    c. Autosomal recessive inheritance. AD
    d. Chondrosarcoma is a secondary primary cancer.

Retinocytoma is considered to be a rare benign phenotypic RB1 gene mutation and carries similar genetic implications as germinal retinoblastoma.[1,2,5] In familial cases, various members of a family may present with either retinocytoma or retinoblastoma or even a combination of both between the two eyes.

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9
Q
  1. Adenocarcinoma of the cervix

a. Prone to endometrial invasion
b. Pap smear screening is more sensitive for adenocarcinoma than squamous carcinoma
c. The risk factors for adenocarcinoma carcinoma is same as for squamous cell carcinoma

A

a. Prone to endometrial invasion

  1. Adenocarcinoma of the cervix
    a. Prone to endometrial invasion
    b. Pap smear screening is more sensitive for adenocarcinoma than squamous carcinoma less sensitive
    c. The risk factors for adenocarcinoma carcinoma is same as for squamous cell carcinoma except for smoking
    Cervical cancer - HPVSCC more common
    Adenocarcinoma- serous- mucinous- clear cell- emdometriod- metanephric
    Met
    Lymphoma
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10
Q
  1. Endometrial carcinoma - the most accurate

a. Endometrial intraepithelial carcinoma is a precursor to type I endometrial carcinoma
b. Type I endometrial carcinoma arises from atrophic endometrium
c. Type II endometrial carcinoma is associated infertility
d. Initial nodal spread to para-aortic nodes

A

a. Endometrial intraepithelial carcinoma is a precursor to type I endometrial carcinoma

*AJL - Robbins says Endometrial intraepithelial carcinoma is associated with Type 2
Endometrial hyperplasia is associated with type 1.

The most accurate answer is Type 2 associated with infertility as they are usually post-menopausal. Weird way to phrase it/ask it but seems the most correct.

**LJS - agree. The way it’s written, c. is most correct. Though type 1 is perimenopausal, typically 50-60yr, so would class them as infertile too.

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11
Q
  1. Adenocarcinoma of bladder risk factor

a. Bladder extrophy
b. Urachal remnant
c. Schistosomiasis
d. Smoking

A

b. Urachal remnant

*LW:
TCC:
 - Most common
 - Risk factors:
     - cyclophosphamide 
     - aromatic amines in tobacco smoke
     - arylamines
     - polycyclic aromatic hydrocarbons.

SCC:

  • 5%
  • RF:
    • Schistosomiasis
    • chronic irritation, e.g. IDC, calculi, infection
    • intravesical BCG (Bacillus Calmette-Guerin)

Adenocarcinoma:

  • 1%
  • RF:
    • persistent urachal remnant (most common)
    • cystitis glandularis (itself secondary to bladder outlet obstruction, chronic infection and/or bladder calculi)
    • associated with bladder exstrophy

Previous answers:
Schisotosomiasis, smoking - SCC
Types:TCC: 90% begin in urothelial cells- age- male- race : white- previous history of cancer- chemical: smoking, cyclophosphamide- cancer syndrome: LynchSCC: 5%- schistosomiasis,- exostrophy
Adenocarcinoma: 1%- urachal remnant Sarcoma : rare

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12
Q
  1. Which tumor is not associated with seizures

a. Central neurocytoma
b. Ganglioglioma
c. Pilocytic astrocytoma
d. DNET
e. Oligodendroglioma

A

pilocytic astrocytoma

LJS agree - pilocytic astrocytoma is circumscribed astrocytoma, posterior fossa, most likely to present with hydrocephalus. All others known to cause seizures

  1. Which tumor is not associated with seizures
    a. Central neurocytoma yes according to RP (common according statdx)
    b. Ganglioglioma most common tumoral cause of temporal lobe epilepsy
    c. Pilocytic astrocytoma seems to be the cortically based lesions that cause seizures.
    d. DNET second behind GG
    e. Oligodendroglioma yes.
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13
Q
  1. Which cancer metastasized to dura without metastasis to brain

a. Prostate
b. Lung
c. Colorectal
d. TCC
e. Breast

A

a. Prostate

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14
Q
  1. Which of the following do not have peptidoglycan cell wall.

a. Mycoplasma
b. Poliovirus
c. CMV
d. Chlamydia trichomatis
e. Mycobacterium tuberculosis

A
  • LW:
  • LW: likely poor recall as no clear answer:

Quoting Robbins / literature review:

No mention of peptidoglycan cell wall in viruses.

Mycoplasma lacks a cell well.

Basic understanding is that Chlamydia doesn’t have standard peptidoglycan cell wall, at least by standard methods of detection, and likely use different assembly / elements of peptidoglycan cell wall (I would favour in this question the simple answer would be they do not have the standard cell wall).

TB: The cell wall is complex and does contain peptidoglycan, but otherwise it is composed of complex lipids. Over 60% of the mycobacterial cell wall is lipid. The lipid fraction of MTB’s cell wall consists of three major components, mycolic acids, cord factor, and wax-D.

  • *LJS - I spent way too much time on this.
  • Bacteria are prokaryotes which have a cell wall (but no membrane bound organelles/nucleus) This cell wall generally contains peptidoglycan. Differences in the peptidoglycan wall determines whether they stain with gram stain. This can be thick (gram +ve) or thin (gram -ve)
  • Mycoplasma is the only bacteria lacking a cell wall (bound only by a cell membrane). Can’t be rx by abx that target cell wall synthesis (many abx types)
  • Chlamydia has a cell wall but doesn’t contain peptidoglycan
  • Viruses do not have cell walls (they are genetic material surrounded by a protein coat). They are not cells or even considered living
  • Mycobacterium tuberculosis has a cell wall containing peptidoglycans but is unique because it otherwise is composed mainly of lipids. Is Gram unclassifiable (can be +ve or -ve, not helpful in dx)

Therefore: this must be a poor recall, as the only option that has a peptidoglycan cell wall is myobacterium tuberculosis.

Old answer:
a. Mycoplasma - the only bacteria that lacks peptidoglycan cell wall

  1. Which of the following do not have peptidoglycan cell wall.
    a. Mycoplasma
    b. Poliovirus yes
    c. CMV yes
    d. Chlamydia trichomatis
    e. Mycobacterium tuberculosis Does. Could this be a which of the following does have a peptidoglycan cell wall.apparently virus also have peptidoglycan cell wallgram positive- thick peptidoglycan cell wallgram negative bacteria- outer and inner membrane- thin peptidoglycan cell wall
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15
Q
  1. Harlequin eyes in craniosynostosis

a. Coronal
b. Sagittal
c. Metopic
d. Lambdoid

A

a. Coronal

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16
Q
  1. Most common retroperitoneal cancer

a. Liposarcoma
b. Leiomyosarcoma
c. Malignant nerve sheath tumor

A

liposarcoma in adults anyway

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17
Q
  1. Leiomyosarcoma of the uterus, which is true

a. Haemtogenous spread is a common form of metastasis
b. Leiomyoma is a precursor to leiomyosarcoma

A

a. Haemtogenous spread is a common form of metastasis - yes
b. Leiomyoma is a precursor to leiomyosarcoma ( this is very rarely true)

LJS - Robbins and Pathoma say does not arise in leiomyoma, always de novo

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18
Q
  1. Most common paratesticular malignancy

a. Angiosarcoma
b. Leiomyosarcoma
c. Fibrosarcoma
d. Lymphoma
e. Liposarcoma

A

*AJL (from Robbins 10e)
Most common benign paratesticular tumour is adenomatoid tumour.
Most common malignant is rhabdomyosarcoma (children), liposarcoma (adults)

I have added liposarcoma to the question.

Previous answer:
b. Leiomyosarcoma I think

No rhadomyosarcoma in the options - if present most common.

Lipoma is most common paratesticular mass, 
adenomatoid tumour (benign) is the most common epididymal mass.

adult - benign : lipoma, leomyoma, fibroma, epidermoid inclusion cyst

Spermatic cord lesions:
- malignant : -> rhabdo, -> spermatic PUS-> spermatic leiomyosarcoma-> spermatic liposarcoma

  • children
    • > malignant: embryonal rhabdomyosarcoma

Epididymal lesion:

  • adenomatous tumour
  • spermatocele, epididymal cyst
  • met, leukaemia, leomyosarcoma
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19
Q
  1. Regarding chondrosarcoma, what is least likely

a. Clear cell is most commonly seen in the pelvis
b. Myxoid subtype is found in soft tissue of the lower limb
c. Hyaline variant is seen in the ribs
d. Mesenchymal chondrosarcoma is commonly found around the mandible

A

a. Clear cell is most commonly seen in the pelvis - long bones

  1. Regarding chondrosarcoma, what is least likely
    a. Clear cell is most commonly seen in the pelvis - long bones
    * *LJS - epiphyses of long bones (mimics chrondroblastoma)
    b. Myxoid subtype is found in soft tissue of the lower limb - true
    c. Hyaline variant is seen in the ribs - never heard of this as a variant. **LJS - probably just means conventional (hyaline producing) - true, occurs in ribs
    d. Mesenchymal chondrosarcoma is commonly found around the mandible - spine, ribs and jaw.
Chondrosarcoma types: 
conventional - low, medium, high grade
juxtacortical
extra skeletal
clear cell - slow growing, better prognosis
myxoid - soft tissue > bone
mesenchymal - soft tissue or bone
dedifferentiated

**LJS - according to big Robbins (9th ed p 1202), subtypes as follows:
-Conventional, divided into central/intramedullary (most common 90% of all chondrosarcomas) and juxtacortical
-Mesenchymal - rare, can occur in young adults
-Clear cell - rare, can occur in young adults
Originates in the epiphyses of long tubular bones and can mimic chondroblastoma
-Dedifferentiated - second high grade non-cartilaginous sarcoma arising within lower grade chondrosarcoma.

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20
Q
  1. PUJ - least common

a. Occurs more commonly in the inferior moeity in the duplex kidneys as oppose to upper moeity.
b. Occurs more commonly in male than female
c. Occurs more commonly on the right than left
d. Associated with contralateral renal agensis

A

c. Occurs more commonly on the right than left - left side in 2/3rds.

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21
Q
  1. Regarding vulval pathology, most accurate

a. Lichen simplex chronicus is a precursor to squamous cell carcinoma
b. Vulval melanoma frequently invasive on presentation
c. Lichen sclerosis……

A

b. Vulval melanoma frequently invasive on presentation

  1. Regarding vulval pathology, most accurate
    a. Lichen simplex chronicus is a precursor to squamous cell carcinoma nope. Lichen sclerosis is. (think of oral lichen planus)
    b. Vulval melanoma frequently invasive on presentation
    c. Lichen sclerosis……
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22
Q
  1. Regarding anal cancer

a. Anal melanoma often familial
b. Vulval carcinoma is a risk factor

A

b. Vulval carcinoma is a risk factor

anal cancer risk factor

  • HPV / HIV infection
  • immunosuppression
  • number of lifetime sexual partners, and receptive anal intercourse
  • smoking
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23
Q
  1. Regarding carcinoid, which is associated with lowest risk for metastasis

a. Oesophagus
b. Stomach
c. Ileal
d. Colon
e. Appendix

A

e. Appendix

Carcinoid location- SR CASP- small intestine- rectum- colon- appendix- stomach- pancreas

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24
Q
  1. Phthisis bulbi, least likely

a. Ciliochoroidal effusion
b. Metaplastic bone
c. Retinal detachment

A

Features include all of these as well as cyclitic membrane, optic nerve atrophy, thickening sclera and micropthia.

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25
Q
  1. Regarding pulmonary alveolar proteinosis, least correct

a. Congential PAP is fatal without transplant
b. Adult PAP is associated with recurrent infection
c. Adult PAP is associated with smoking
d. Adult PAP is associated with immunodeficiency
e. Adult PAP is associated with silicosis

A
  1. Regarding pulmonary alveolar proteinosis, least correct

a. Congential PAP is fatal without transplant - yes
b. Adult PAP is associated with recurrent infection - yes susceptible to recurrent infection eg nocardia
c. Adult PAP is associated with smoking - strong association
d. Adult PAP is associated with immunodeficiency - yes
e. Adult PAP is associated with silicosis - yes
Alveolar proteinosis- autoimmune : IgG antibodies to GM-CSF- secondary : silica dust, haematological malignancy, infection ( nocardia)- congenital

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26
Q

Smoking related lung disease

A
  • copd, bronchitis, emphysema- alveolar proteinosis- CEP- UIP, DIP, RBILD- PLHC- cancer
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27
Q
  1. What is the most common profile for lobular carcinoma

a. ER + PR + HER2 –ve
b. ER –ve PR-ve HER2 –ve
c. Different combinations of ER, PR, HER 2

A

a. ER + PR + HER2 –ve

**LJS agree:
Oestrogen and progesterone expression more common in ILC than IDC:
	95% ER+ve
	65% PR+ve
5% HER2+ve (rare cf IDC)
Robbins says no HER2 overexpression
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28
Q
  1. Which is not a prion associated with spongiform encephalopathy

a. CJD
b. Variant CJD
c. Kuru
d. Familial fatal insomnia
e. Some syndrome……

A

e. Some syndrome……spongiform encephalopathy = prion disease

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29
Q
  1. Regarding anaemia, least likely association

a. Spherocyte anaemia and prosthetic heart valve

A

yes

30
Q
  1. Which congenital anaemia most likely results in splenic infarcts

a. Sickle cell
b. Thalasemia
c. Spherocytosis
d. G6PD deficiency

A

a. Sickle cell

31
Q
  1. Which of the following has the least malignant potential

a. Pacnreatic mucinous cystadenoma
b. IPMN
c. Solid papillary tumor
d. Insulinoma
e. Pancreatic epithelial adenoma`

A

e. Pancreatic epithelial adenoma` - alt name for benign epithelial neoplasms EG serous cystadenoma

LJS - not sure about answer e.
Robbins doesn’t use this term, and neither does anything on google. “Pancreatic adenoma” appears to be used for variety of pathologies, including cystic neoplasms and NET. Possibly poor recall. However, all others have some malignant potential (insulinoma least)

  1. Which of the following has the least malignant potential
    a. Pacnreatic mucinous cystadenoma
    b. IPMN 60% main duct, 5 percent side branch
    c. Solid papillary tumor 15 % malignant. EJB thinks this one
    d. Insulinoma 10% malignant
    e. Pancreatic epithelial adenoma` alt name for benign epithelial neoplasms EG serous cystadenoma
32
Q
  1. IPMN, most accurate

a. Rarely present with jaundice
b. More common in female
c. No malignant potential
d. More common in body and tail..
e. Malignant change is associated with increase in serum amylase.

A

e. Malignant change is associated with increase in serum amylase. True, in the absence of pancreatitis. From radiopaedia.

  1. IPMN, most accurate
    a. Rarely present with jaundice - can do.
    b. More common in female - male predilection
    c. No malignant potential 60% main duct, 5% sidebranch
    d. More common in body and tail. Side branch are most common in head.
    e. Malignant change is associated with increase in serum amylase. True, in the absence of pancreatitis. From radiopaedia.
33
Q
  1. Most likely infection in testis, without infection of epididymis

a. TB
b. Syphilis
c. Gonorrhoea
d. Chlamydia
e. E.Coli

A

b. Syphilis its in robbins

34
Q
  1. Ovarian neoplasm, false option

a. Endosalpingiosus is associated with borderline serous cyastadenoma
b. Mucinous cystadenoma is common postmenopausal women

A

b. Mucinous cystadenoma is common postmenopausal women middle age. Rare before puberty and after menopause.
* LW: peak age 30-50yrs.

*AJL - agree. Additional info below on the other one.
“Endosalpingiosis” refers to the presence of fallopian tube-like epithelium outside fallopian tubes. Has a close relationship with serous carcinoma, especially low grade.
https://link.springer.com/article/10.1007/s40944-016-0072-2

  1. Ovarian neoplasm, false option
    a. Endosalpingiosus is associated with borderline serous cyastadenoma Yes but thought not to be causes
    b. Mucinous cystadenoma is common postmenopausal women middle age. Rare before puberty and after menopause.
35
Q
  1. Ameloblastoma, false option

a. When found in suprasellar region, they are called craniopharyngioma
b. More commonly solid than cystic
c. Associated with unerupted tooth
d. More commonly found in mandible than maxilla

A

b. More commonly solid than cystic

*LW:
Ameloblastoma:
Approximately 20% of cases are associated with dentigerous cysts and unerupted teeth.
There are no specific histological features to differentiate between ameloblastomas and craniopharyngiomas, with only location differentiating between two tumours
Commonly mandible.

36
Q
  1. Difference between RA and JRA

a. Ankylosis
b. Uveitis
c. Irits
d. Oligoarthritis

A

Robbins: JRA - oligoarthritis more common- large joints more common- systemic disease more common- no rheumatoid nodules and RF- ANA positive

37
Q
  1. 14 year old with painful knee presents for xray. He already has two normal knee xray previously. What is the next appropriate next steop.

a. Oblique projection of the knee.
b. Xray the hip
c. MRI
d. Repeat knee xray

A

b. Xray the hip right age for a SUFe, can have knee pain. Good to start with, then consider MRI

38
Q
  1. Regarding gestation trophoblastic disease, most accurate

a. Complete moles are triploid
b. Partial moles do not become invasive moles
c. Partial moles increases risk of choriocarcinoma
d. Partial moles are completely paternal
e. Complete moles contain fetal parts.

A

LW: All answers incorrect - likely poor recall.

  1. Regarding gestation trophoblastic disease, most accurate
    a. Complete moles are triploid F - are diploid
    b. Partial moles do not become invasive moles: False, low / partial risk of becoming invasive. Invasive moles moles are complete moles that are more invasive locally.
    c. Partial moles increases risk of choriocarcinoma: False - despite pathoma stating “partial has partial risk of choriocarcinoma”, RObbins and UpToDate state choriocarcinoma occurs only rarely if ever after a partial mole.

**LJS - agree that the answer is false. But partial mole has NO choriocarcinoma risk (Robbins). Do have risk of invasive mole (though lower than complete mole)

d. Partial moles are completely paternal: False, are usually triploid with diploid paternal, single maternal. Complete moles are completely paternal and diploid.
e. Complete moles contain fetal parts: False - complete moles completely paternal / diploid, thus no ability to form fatal parts. Partial mole may show some fetal parts.
37. Regarding gestation trophoblastic disease, most accurate

a. Complete moles are triploid - diploid
b. Partial moles do not become invasive moles - yes they can
c. Partial moles increases risk of choriocarcinoma - no the dont
d. Partial moles are completely paternal - no
e. Complete moles contain fetal parts. No they don’t.
All are incorrect. ? recall

39
Q
  1. Complication of complete occlusion of left circumflex artery –

a. VSD
b. Apical thrombus
c. Mitral regurgitation from papillary muscle necrosis
d. Bundle branch block
e. Ventricular tachyarrhythmia

A

c. Mitral regurgitation from papillary muscle necrosis

  • *LJS
    a. VSD - septum supplied by RCA/LAD
    b. Apical thrombus - apex supplied by LAD
    c. Mitral regurgitation from papillary muscle necrosis - true, supplies part of anterior papillary muscle, recognised complication of RCA and LCx occlusion
    d. Bundle branch block - bundle of His in inferolateral septum, ?supplied by RCA
    e. Ventricular tachyarrhythmia
40
Q
  1. Ependymoma, true option

a. Myxopapillary is WHO GRADE I/IV
b. Subependymoma is most common in 3rd ventricle
c. Most common in NF 1

A
  • LW:
    a. Myxopapillary is WHO GRADE I/IV: WHO grade 1 only.

b. Subependymoma is most common in 3rd ventricle: False - inferior 4th ventricle typical (60%) > Lateral > 3rd ventricle > spinal cord
c. Most common in NF 1: false - NF2
a. Myxopapillary is WHO GRADE I/IV

41
Q
  1. Regarding CML, false option

a. Myelofibrosis
b. Progression to AML
c. Philedelphia chromosome

A

a. Myelofibrosis

a. Myelofibrosis
b. Progression to AML kind of. Eventually have a blast crisis
c. Philedelphia chromosome true.

42
Q
  1. Regarding carcinoid, most accurate

a. Often presents in the elderly population
b. Associated with MEN 1 in the stomach
c. Is a precursor to large cell neuroendocrine tumor
d. Associated with smoking
e. Often presents with carcinoid syndrome

A

b. Associated with MEN 1 in the stomach (pg 774 9th ed Robbins)
41. Regarding carcinoid, most accurate

a. Often presents in the elderly population - 50-65 according to Robbins
b. Associated with MEN 1 in the stomach (pg 774 9th ed Robbins)
c. Is a precursor to large cell neuroendocrine tumor -don’t think so
d. Associated with smoking - don’t think so
e. Often presents with carcinoid syndrome <10%

43
Q
  1. Regarding osteogenesis imperfecta, which is correct

a. Type I has kyphoscoliosis
b. Type IV has blue sclera
c. Type II is compatible with life
d. All type have dental problems

A

*LW:
Based on Big Robbins Table, I would favor this to be an incomplete recall:

a. Type I has kyphoscoliosis: unlikely as near normal stature, with bone deformity minor. Progressive kyphoscoliosis listed under OI 3.

b. Type IV has blue sclera: variable
Robbins states type 1 and 2 have blue sclera, while type 3 have blue sclera at birth that become white.
Robbins states OI IV have normal sclera.

Donnelly Paeds book states type I and III have blue sclera.

RP states type 1 has blue sclera, while type IV sclerae are often light blue in infancy, but the colour intensity varies - the sclerae may lighten to white later in childhood or early adulthood

c. Type II is compatible with life: false INcompatible / lethal.

d. All type have dental problems: Likely false.
Type II neonatal lethal, thus they don’t have teeth, while all the other forms do report dental problems…confusing. Type 2 does not have dentinogenesis imperfecta listed in Robbins table, while types 1, 2, and sometimes 4 are listed as having it.

**LJS - agree. Though primary dentition development starts at 6-8 weeks in utero and permanent dentition at 20 weeks. Dentin formation seems to be a relatively early event, so type 2 OI possibly do have dentinogenesis imperfecta, but never seen clinically as they die before the teeth erupt. I agree, probably poor recall, but this seems the least false of those listed.

Prior answers:
42. Regarding osteogenesis imperfecta, which is correct

a. Type I has kyphoscoliosis - could do but minimal bone deformity, more typical of III – progressive deforming.
b. Type IV has blue sclera - variable
c. Type II is compatible with life - no
d. All type have dental problems - I think all have

44
Q
  1. Regarding valvular heart disease

a. Rheumatic heart disease is due to direct invasion of Strep A into heart valves
b. Liebman Sack endocarditis - sterile vegetations in the tricuspid valve
c. Heart disease in carcinoid syndrome is from tumor cell invasion into right heart.
d. Ankylosing spondylitis associated with mitral valve prolapsed

A

b. Liebman Sack endocarditis - sterile vegetations in the tricuspid valve mitral and tricuspid valves

  1. Regarding valvular heart disease
    a. Rheumatic heart disease is due to direct invasion of Strep A into heart valves (type 2 hypersensitivity)
    b. Liebman Sack endocarditis - sterile vegetations in the tricuspid valve mitral and tricuspid valves
    c. Heart disease in carcinoid syndrome is from tumor cell invasion into right heart.
    d. Ankylosing spondylitis associated with mitral valve prolapsed (aortic)
45
Q
  1. Pick the correct association for thoracic aneurysm

a. Bicuspid valve
b. Syphilis
c. Hypertension
d. Loeys Dietz Syndrome
e. Ankylosing spondylitis

A
  • LW: all of listed options are known associations with thoracic aneursyms.
  • AJL agree

Previous
d. Loeys Dietz Syndrome similar to marfans

  1. Pick the correct association for thoracic aneurysm
    a. Bicuspid valve
    b. Syphilis
    c. Hypertension
    d. Loeys Dietz Syndrome - similar to marfans
    e. Ankylosing spondylitis - aortitis

Everything is associated - bad recall?

46
Q
  1. Regarding parathyroid disease

a. Hypoparathyroidism can result in subcapsular calcification in the lens
b. Vit D deficiency can exacerbate secondary hyperparathyroidism
c. Tertiary hyperparathyroidism can result in dental hypoplasia

A

b. Vit D deficiency can exacerbate secondary hyperparathyroidism

47
Q
  1. What is not commonly seen in Type 1 diabetes but is seen in Type 2 diabetes

a. Amyloidosis
b. Glaucoma
c. Neuropathy
d. Gastroparesis
e. Nephropathy

A

Amyloid is seen more commonly in T2.`

**LJS -
Glaucoma in both.
Neuropathy and nephropathy in both
Gastropareisis occurs in both but more common in type 1

Can’t find anything on absolute differences between types 1 and 2 for anything except amyloid - localised amyloid in type 2 DM

*AJL - Agree with above. I have changed the question to reflect that, and left the ridiculous english that makes it hard to understand. (Maybe this is all training to help us understand the poorly written clinical details)

48
Q
  1. Pick the correct option

a. Polyarteritis nodosa is a type IV reaction
b. Goodpasture is a type III reaction
c. Insulin resistance diabetes mellitus is a type II reaction
d. Myasthenia gravis is a type III reaction.

A
  • LW: Favoured answer is C - insulin resistance DM is a type II hypersensitivity reaction.
  • *LJS - agree, though caution as latest Rob has type 1 DM listed as type 4 (direct cytotoxicity) and “insulin resistant DM” as type 2. All others incorrect

a. Polyarteritis nodosa is a type IV reaction: incorrect, is a type III immune complex mediated.
b. Goodpasture is a type III reaction: incorrect, is a type II anti body mediated.
c. Insulin resistance diabetes mellitus is a type II reaction: Correct - Robbins has this listed as a type II antibody mediated hypersensitivity reaction.
d. Myasthenia gravis is a type III reaction. Incorrect, type II anti body mediated reaction.

Previous Answers:
All false

  1. Pick the correct option
    a. Polyarteritis nodosa is a type IV reaction (type III) pANCA
    b. Goodpasture is a type III reaction. (Type II)
    c. Insulin resistance diabetes mellitus is a type II reaction (nope)
    d. Myasthenia gravis is a type III reaction. (Type II)
49
Q
  1. Regarding phaeochromocytoma, most accurate

a. Laboratory diagnosis via VMA
b. Paroxysmal hypertension in minority
c. Bilateral disease associated with MEN 1

A

a. Laboratory diagnosis via VMA

50
Q
  1. Which renal tumor is closely associated with cardiac tumor

a. Angiomyolipoma
b. RCCc. ……

A

a. Angiomyolipomacardiac rhabdomyoma in TS

51
Q
  1. Regarding thymic tumor

a. Hyperplasia is the most common cause to thymic mass in young population
b. Thymolipoma can reach massive sizes before presentation

A

b. Thymolipoma can reach massive sizes before presentation

a. Hyperplasia is the most common cause to thymic mass in young population - possibly true
b. Thymolipoma can reach massive sizes before presentation

52
Q
  1. Regarding Meig syndrome

a. Bilateral haemorrhagic pleural effusion
b. Right sided hydrothorax
c. Left sided chylothorax
d. Left sided pneumothorax

A

b. Right sided hydrothorax

**LJS - right sided pleural effusion in 70%

53
Q
  1. Which is true of tubular carcinoma

a. 70% 5 year survival
b. Can remain stable for many years
c. Presents in elderly female

A

b. Can remain stable for many years

  1. Which is true of tubular carcinoma
    a. 70% 5 year survival (97% 10 yr survival)
    b. Can remain stable for many years
    c. Presents in elderly female (tends to present younger)
54
Q
  1. What is the correct association

a. Enteropathic T cell lymphoma and stomach
b. Mantle cell lymphoma and cecum
c. GIST in rectum
d. Leiomyoma in duodenum
e. .. the correct answer…..anyone?

A

c. GIST in rectum (probably)

55
Q
  1. Which organ is least likely to be biopsied for Graft vs Host disease

a. Kidney
b. Liver
c. Sigmoid colon
d. Skin

A

*LW:
Favoured answer is A: Kidney:
Acute GCHD causes epithelial cell necrosis in liver, skin, gut, with chronic GVHD following similar patterns.
Thus, kidney not affected by GVHD hence not biopsied.

**LJS agree - GVHD rarely affects the kidney

a. Kidney
GVHD:The skin, gastrointestinal tract (especially small bowel), and liver are the principal affected organs.

Histologic confirmation may be helpful to corroborate a clinical impression of possible acute GVHD. The skin and gastrointestinal tract are relatively easy to biopsy.

56
Q
  1. Another question on Graft vs Host disease, one of the option is
    a. can result in organizing pneumonia.
A

yes

57
Q
  1. Regarding fibrous dysplasia

a. McCune Albright is associated with hypercorticolism
b. Monoostotic presents earlier than polyostotic without endocrinopathy.
c. Monoostotic is associated with bilateral cafe-au-lait spots
d. Monootototic can be a precursor to osteosarcoma
e. Monoostotoic can progress to polyosototic.

A

a. McCune Albright is associated with hypercorticolism

**LJS - agree. Classic triad is:
Polyostotic FD
Precocious puberty
Cafe au lait spots

But now recognised to have other hyperfunctioning endocrine manifestations - Cushing syndrome, hyperthyroidism, GH excess, hyperprolactinaemia

Osteosarcoma uncommonly arises in FD, but can occur in mono or polyostotic. ?less common than a.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4063639/

58
Q
  1. Inclusion body myositis

a. Predominantly affects flexor muscles of the hand as well as proximal thigh and upper arm muscles.
b. Associated with malignancy
c. ????????

A

a. Predominantly affects flexor muscles of the hand as well as proximal thigh and upper arm muscles.

Inclusion body myositis (IBM) is a type of inflammatory myopathy. It is often considered is the most common acquired myopathy in patients older than 50.
Pathognomonic histologic feature of this condition is the presence of inclusion bodies in the nucleus and cytoplasm of affected muscle cells.Inclusion body myositis can affect both proximal (close to the trunk of the body) and distal (further away from the trunk) muscles. It is usually bilateral although muscle weakness may affect only one side of the body. A/W- diabetes- autoimmune condition
No treatment

59
Q
  1. Which is not a complication of breast cancer treatment

a. Ovarian carcinoma
b. Aplastic anaemia
c. Myelodysplastic syndrome
d. Angiosarcoma from lymphoedema

A

a. Ovarian carcinoma

  • *LJS - agree. Despite inducing hyperoestrogenism, tamoxifen seems NOT to be ass/w incr risk of ovarian ca (though other causes of oestrogen excess e.g. oestrogen only HRCT are ass/w ovarian carcinoma)
    https: //www.ncbi.nlm.nih.gov/pmc/articles/PMC3756313/
60
Q
  1. Regarding fibromatosis, least correct.

a. Associated with FAP
b. Occurs in ventral aspect of the penis
c. Plantar fibromatosis rarely cause contractures
d. Extra-abdominal form commonly occurs in musculature of the shoulder
e. Can be associated with caesarean scar

A

**LJS - disagree with previous answer.
I think b. is false
b. Occurs in ventral aspect of the penis - Peyronie disease causes induration/mass on dorsolateral aspect of the penis (Robbins p 1222).

Interesting factoid: anatomical position of penis is flaccid lying flat against abdomen. Dorsal surface is against abdominal wall, ventral is anterior (i.e. the underside when dangly).

a. Associated with FAP - true (Gardner syndrome)
c. Plantar fibromatosis rarely cause contractures - true (unlike palmar)
d. Extra-abdominal form commonly occurs in musculature of the shoulder - true (Robbins p 1222). Common locations for extra-abdominal deep fibromatosis:
- Extremities (esp limb girdle)
- Abdominal wall
e. Can be associated with caesarean scar - true, often occurs at sites of trauma/incisions

Previous answer:
d. Extra-abdominal form commonly occurs in musculature of the shoulder

  1. Regarding fibromatosis, least correct.
    a. Associated with FAP true
    b. Occurs in ventral aspect of the penis payronies
    c. Plantar fibromatosis rarely cause contractures true
    d. Extra-abdominal form commonly occurs in musculature of the shoulder
    e. Can be associated with caesarean scar. true
    Peyronies disease:Peyronie disease is the most common cause of painful penile induration. Fibrous tissue plaques form within the tunica albuginea, causing painful deformity and shortening of the penis.
61
Q
  1. Which is true

a. Superficial DVT in Behcets
b. Trousseaus sign is due to metastatic microthrombi
c. Lower limb DVT in 60 year old need to be investigated for Factor V Leiden.
d. Middle cerebral artery thrombosis is associated with prothrombin 20210 mutation

A

a. Superficial DVT in Behcets

The most common vascular complaints are secondary to venous thrombosis, often of the superficial veins. This can occur after venipuncture. Patients who develop superficial thrombophlebitis are more at risk than other patients with Behçet syndrome for the development of deep vein thrombosis and arterial disease.

Behct:- inflammatory vasculitis of unknown etiology- usually in young patient 20-30 yo- oral /genital ulceration, ocular abnormality

*LW:
Trousseau’s sign is the induction of carpal spasm by inflation of a sphygmomanometer above systolic blood pressure for three minutes and indicates hypocalceami. Carpal spasm, as indicated above, is characterized by adduction of the thumb, flexion of the metacarpophalangeal joints, extension of the interphalangeal joints, and flexion of the wrist

Trousseau syndrome (not to be confused with Trousseau sign) represents the association between migratory thrombophlebitis and malignancy, particularly mucin-producing tumours; hence one of its other names: cancer-associated thromboembolism.
--> so sneaky!
62
Q
  1. Petechiae in the oral mucosa, most likely

a. Vitamin K deficiency
b. Myelodysplastic syndrome
c. Christmas disease
d. Antiphospholipid syndrome

A

a. Vitamin K deficiency

63
Q
  1. What is not a risk factor for gallbladder carcinoma

a. PBC
b. Crohn’s
c. Cholelthiasis
d. Procelain gallbladder

A

a. PBC

64
Q
  1. What is associated with PSC but not for PBC

a. Antimitochondrial antibody
b. Female
c. Crohn’s disease
d. Lymphadenopathy (not sure about this recall)

A

c. Crohn’s disease

**LJS agree. PSC is ass/w IBD (UC>Crohn), PBC ass/w other AI diseases (Sjogrens, scleroderma thyroiditis etc)

65
Q
  1. Most likely association

a. NSAID and diffuse renal cortical necrosis
b. Multiple myeloma with recurrent pyelonephritis

A

**LJS - agree
NSAIDs cause tubulointerstitial nephritis and renal papillary necrosis (not cortical - usually due to systemic hypotension)

b. Multiple myeloma with recurrent pyelonephritis ??

a. NSAID and diffuse renal cortical necrosis Acute kidney injury, hypersensitivity interstitial nephritis, minimal change disease and membranous nephropathy
b. Multiple myeloma with recurrent pyelonephritis

66
Q
  1. Regarding Pagets, which is least accurate

a. Extramedullary hematopoiesis in polyostotis disease
b. Fibular involvement is rare
c. Weakening of the bone results in skull base invagination and compression of the posterior fossa structures
d. Can result in high output cardiac failure

A
  • LW:
    65. Regarding Pagets, which is least accurate

a. Extramedullary hematopoiesis in polyostotis disease: Does occur, but not always with present in polyostotic cases, so this is least accurate.
b. Fibular involvement is rare: true
c. Weakening of the bone results in skull base invagination and compression of the posterior fossa structures: true
d. Can result in high output cardiac failure: true (acts as a AV shunt)

Previous answer:
a. Extramedullary hematopoiesis in polyostotis disease can do but rare. Probably least accdurate

67
Q
  1. Most common ovarian tumor in children resulting in precocious puberty

a. juvenile granulosa cell tumor
b. immature teratoma
c. yolk sac tumor

A

a. juvenile granulosa cell tumor

68
Q
  1. Which is not an IgG4 disease

a. Subacute granulomatous thyroiditis
b. Orbital pseudotumor
c. Retroperitoneal fibrosis
d. Chronic sclerosing parotitis
e. Tubulointerstitial nephritis

A

a. Subacute granulomatous : de quirvaine thyroiditis

  1. Which is not an IgG4 disease
    a. Subacute granulomatous thyroiditis - reidels
    b. Orbital pseudotumor yes
    c. Retroperitoneal fibrosis yes
    d. Chronic sclerosing parotitis yes
    e. Tubulointerstitial nephritis
69
Q
  1. Regarding toxicity, which is not true

a. Alcohol and cerebellum
b. Methanol and hippocampus
c. Polyvinvyl and hemangiopericytoma
d. some toxin and angiosarcoma

A

b. Methanol and hippocampus - False: Methanol classically effects the putamen bilaterally.

70
Q
  1. Grave’s disease, which is true

a. Opthalmopathy can persist even after treatment
b. Antibody can reliably distinguish Grave’s from toxic adenoma.

A

a. Opthalmopathy can persist even after treatment

71
Q
  1. Prostate cancer, which is true

a. Urethral invasion occurs early in the disease
b. Invasion of rectum is rare due to Denonvilliers fascia
c. Spread to lung is more common than liver

A
  • LW: b. Invasion of rectum is rare due to Denonvilliers fascia: True - word for word from Robbins, hence Gospel and favoured correct answer.
    71. Prostate cancer, which is true
    a. Urethral invasion occurs early in the disease: less likely, as Ca commonly occurs in peripheral zone, thus early disease less likely to extend into central zone and then into urethra.
    b. Invasion of rectum is rare due to Denonvilliers fascia: True - word for word from Robbins, hence Gospel and favoured correct answer.
    c. Spread to lung is more common than liver: Unsure, but probable, as most drainage via baton plexus, an vertebral veins, and pelvic nodal drainage, however Recta invasion is felt more correct.
    b. Invasion of rectum is rare due to Denonvilliers fascia
  1. Prostate cancer, which is true
    a. Urethral invasion occurs early in the disease
    b. Invasion of rectum is rare due to Denonvilliers fascia J Urol. 1993 Apr;149(4):793-8.Invasion of Denonvilliers’ fascia in radical prostatectomy specimens.Villers A1, McNeal JE, Freiha FS, Boccon-Gibod L, Stamey TA.
    c. Spread to lung is more common than liver according to RP. Plausible given limited portal drainage. Probably the most concreted. Para-aortic nodes is the initial place for lymphatic spreadDenonvilliers fascia - fascia that separates the prostate from the rectum
72
Q
  1. Regarding patients with dementia and parkinsonism, least true

a. Lewy body dementia
b. Multisystem atrophy
c. Huntingtons’s disease
d. Supranuclear palsy

A

c. Huntingtons’s disease
Parkinson:
- neurodegenerative disorder
- loss of dopaminergic neuron in substantia nigra
- absent swallow tail sign : loss of nigrosome
-1 which is responsible for high signal on T2 star.

Lewy body dementia:

  • neurodegenerative disorder related to parkinson
  • 2nd most commmon cause of dementia (after Alzheimers)
  • accumulation of Lewy bodies throughout the brain ( greatest concentrations in the midbrain, hypothalamus, basal ganglia, inferior olives, brainstem reticular formation, and dentate nuclei of the cerebellum)
  • Neurofibrillary tangles (no amyloid core)
  • imaging:-> non-specific-> generalised atrophy-> normal hippocampus

PSA:

  • neurodegenerative disorder
  • supranuclear palsy, cognitive decline, falls, parkinson features
  • MR: MIDBRAIN atrophy!!!-> hummingbird sign : small midbrain with concave superior midbrain-> mickey mouse appearance : decreased AP diameter-> morning glory sign : loss of convex lateral margin

MSA: 50 yo

  • neurodegenerative disease
  • parkisonism like symptoms, autonomic dysfunction. corticospinal dysfunction

MSA-C: predominance of cerebellar symptoms (olivopontocerebellar atrophy).MSA-P: predominance of parkinsonian signs and symptoms (striatonigral degeneration).