Path 2021 Flashcards
Which is not true?
Fibrothecoma - good prognosis if ascites present
Brenner tumour - often solid
non-gestational choriocarcinoma - highly radiosensitive
CCF - non-gestational choriocarcinoma - highly radiosensitive (False)
- Rx surgery and chemo, don’t think they are radiosensitive (cant find anything on this and the other two options are true). Non gestational worse prog than gestational.
SCS: agree. Pathoma guy stresses this quite a but in his videos
Fibrothecoma - good prognosis if ascites present T – Ass meigs syndrome – good prog.
Brenner tumour - often solid - T - Transitional cell epithelium solid
**SCS: Meig syndrome = presence of ascites and pleural effusion (tends to be RIGHT sided) with a benign solid ovarian tumour (examples include fibrothecoma, granulosa, Brenner). Less that 1% of ovarian tumours present like this.
Effusion and Ascites resolves after resection.
Downs- least likely?
- moderately associated with alzheimer’s
- moderately associated with epicanthal folds
- moderately associated with myxomatous degeneration of the mitral valve
- moderately associated with mitral valve prolapse
- moderately associated with progressive liver fibrosis
CCF - Mxomatous MV – no assc
Moderately is a subjective term but -
- Alzheimer’s – “virtually all” radiopedia (highly assoc).
- Epicanthal folds – Characteristic phenotype – highly associated
- Mitral valve prolapse – numbers vary (50-60% on Medscape)
- Progressive liver fibrosis -does happen in downs but usually secondary to another associated condition eg fatty liver, autoimmune liver disease, and celiac disease.
**SCS: Myxomatous MV degen = marfans
Cystic fibrosis, most likely?
- minor effect on fertility in males
- diffuse liver fibrosis
- CF is a common cause of childhood pancreatitis
- vitamin B12 deficiency
- focal biliary cirrhosis develops in 1/3 of patients
Answer: Focal biliary cirrhosis develops in 1/3 of patients.
ROBBINS
- most common lethal genetic disease that affects Caucasian populations
- carrier frequency USA 1 in 20.
- lower in AA, asians, hispanics
- AR inheritance pattern, but heterzygote carriers can have respiratory and pancreatic disease.
Clinical features
Pancreas
- 85-90% of patients
- severe cases in older children/adolescents, atrophy of exocrine gland and progressive fibrosis.
- a subset of patients have recurrent bouts of pancreatitis.
Liver
- bile canaliculi plugged by mucous
- ductular proliferation and portal inflamation
- Hepatic steatosis common.
- over time, focal biliary cirrhosis develops in 1/3 of patients
- can eventually involve entire liver, diffuse biliary cirrhosis develops in less than 10% of cases.
Salivary glands
- similar to pancreas, atrophy and fibrosis.
Pulmonary
- most serious complications
- bronchiectasis, lung abscesses
- S. aureus, H. influenzae, P. aeruginosa are 3 most common organisms.
Azoospermia and infertility are found in 95% of males who survive into adulthood.
Congenital bilateral abscence of vas deferens is afrequent finding in these patients.
Meconium ileus
- 5-10% of case present at birht with meconium ileus.
**most common cause of pancreatitis in childhood is trauma.
**malabsorption mainly with Fat-soluble vitamins, not water soluble i.e. B12.
Breast textured implant, which association?
- anaplastic large cell lymphoma
- another lymphoma
- another lymphoma
- another cancer
- another thing
CCF - - anaplastic large cell lymphoma
Polycythemia rubra vera, most likely to get which complication?
- myelofibrosis
- transformation to AML
- transformation to CML
Answer: Myelofibrosis
ROBBINS
Note, “rubra vera” is old term, not used anymore. Just means primary polycythemia vera.
It is a myeloproliferative disorder secondary to a JAK2 point mutation which is found in >95% of cases.
At diagnosis, a moderate to marked increase in reticulin fibers is seen in about 10% of marrows.
Mild organomegaly is common.
Peripheral blood often contains increased numbers of basophils and abnormally large platelets.
High cell turnover gives rise to hyperuricemia, with gout seen in 5-10% of cases.
25% of patients 1st present with DVT, MI or stroke. Budd-Chiari is also prominent.
Late in the course of PCV, often progresses to a spent phase, characterized by extensive marrow fibrosis (myelofibrosis) that displaces hematopoietic cells (15-20% of patients).
This is accompanied by extramedullary hematopoiesis in the spleen and liver with organomegaly.
Transformation to AML is seen in 1-2% of patients.
Bicuspid valve, with progressive exertional dyspnoea, ejection systolic murmur, most likely finding on CXR
- ascending aorta enlarged
- calcification of the aortic valve
- depression of the cardiac apex
- normal cardiac size
Answer: Calcification of the aortic valve.
EH: Fun fact David Smythe has bicuspid arotic valve.
endometrioid carcinoma of ovary?
- 2% of ovarian tumours are endometrioid type
- 40% are bilateral
- 3% endometrioid are associated with synchronous ovarian endometrial carcinoma
CCF - 40% bilateral – St8 outta robbins p1023 table 22-6
- SCS: additional trivia (StDx and Radiopaedia)
- Endometrioid carcinoma 8-15% of ovarian ca. (second most common).
- About 1/3 are associated with either endometrial carcinoma or hyperplasia.
- 1% of endometriomas (endometriosis) have malignant tx to this subtype of ca.
- Best outcome of all ovarian carcinomas.
Adenomyosis
- invades/infiltrates along the fallopian tubes and forms ovarian masses
- diffuse globular enlargement of the uterus
- well-defined myometrium/endometrium junction
- no thickening of the myometrium
- increased risk of cancer
Answer (most correct): diffuse globular enlargement of the uterus
ROBBINS
Adenomyosis is presence of endometrial tissue within the uterine wall.
Remains in continuity with the endometrium, signifying downgrowth of endometrial tissue into and between the smooth muscle fascicles of the myometrium
Occurs in 20% of uteri.
Clinical symptoms: menometrorrhagia, colicky dysmenorrhea, dyspareunia, pelvic pain.
STATDX
- Firm, large, and globular uterus.
- Rare malignant degeneration to adenocarcinoma.
PATHOUTLINES
Prognosis: Benign, excelling prognosis even if not removed.
Leiomyoma, false?
- diffuse enlargement of the uterus without nodular contour
- gestation implanted into leiomyoma, is bad- associated with something bad
Answer: ?both
No case of pregnancy implanted into leiomyoma, however Leiomyomas in pregnant women increase the frequency of spontaneous abortion, fetal malpresentation, uterine inertia, and post-partum hemorrhage.
Leiomyoma variants include diffuse leiomyomatosis
- diffuse enlargement of the uterus
- gross pathology: innumerable confluent nodules that are less discrete than typical leiomyoma, causing symmetric uterine enlargement.
placental abruption
- acutely presents as enlarged placenta
- echogenic amniotic fluid is only seen with bleeding into the amniotic cavity -
- marginal placental abruption has clot at the internal os
Answer: first and last options correct.
STATDX
Placental abruption = premature separation of placenta.
Etiology
- compromise of vascular structures supporting placenta leads to dissection of blood
- abnormal trophoblast invasion -> rupture of spiral arteries -> premature placental separation
- Intrauterine hypoxia, placental insufficiency.
Associated abnormalities
- Placenta previa (13-14x increase risk for placenta abruption)
- Leiomyoma (2.6x increase risk)
Acute hematoma
- blood often isoechoic to placenta
- may appear as thick placenta (placentomegaly)
- associated echogenic amniotic fluid is common (from clot proteins, large bleed can traverse amniotic fluid).
Marginal Placenta Abruption
- most common type
- hemorrhage from edge of placenta, can see a raised edge in 50% of cases.
- hematoma adjacent to placenta.
- Remote hematoma common, normally infront of os.
CT - blocked Eustachian tube, fluid in mastoid and middle ear, in a middle aged man, next most appropriate test? PET-CT MRI base of skull and neck Some nuclear medicine scan Some ultrasound scan
CCF - MRI base of skull and neck
*SCS: Nasopharyngeal carcinoma until proven otherwise.
Note this question is incorrectly recalled in the 2021 RD section (says a paed)
[Path?] Crazy paving, least likely
- PJP
- PAP
- mucinous adenocarcinoma
- respiratory bronchiolitis -ILD
CCF - RB
(path) which is least common hepatitis to cause cirrhosis Hep B Hep C Hep A Hep E Alcoholic hepatitis NASH Hep D
CCF - Hep A
**SCS: Hep E also does not have a chronic state. Can lead to fulminant hepatitis (liver failure w massive necrosis) in pregnancy especially.
path) Which is least associated with HCC? Hep A Hep B Hep C Haemochromatosis Non-alcoholic hepatitis Wilsons
CCF – Hep A
(path) Which is least likely to cause hepatic deposition of collagen and fibrosis early in its symptomatic course? Budd Chiari Veno-occlusive disease Uncontrolled right heart failure NASH Hep C Hep B
CCF – Favour NASH, fibrosis later /endstage feature- but usually asymptomatic early.
Difficult to find time course vs RHF - so not entirely sure - Thoughts?
**SCS: also unsure… this is what I could find:
Budd Chiari: Robbins 10th Ed (small one): “centrilobular fibrosis develops in instances in which the thrombosis is more slowly developing”
Venoocclusive definitely does this.
I Favour RHF (same Robbins as above): “uncommonly with sustained chronic severe CHF, centrilobular fibrosis … develops”
WJI:
additionally HBV not always cirrhotic, HCV develop cirrhosis >6months post infection. Hard to know what this Q is getting at
[PATH] Sarcoid, which is least likely a manifestation?
- Mikulicz syndrome
- lacrimal gland inflammation
- uveitis
- non caseating epithelioid granulomas in the liver
- segmental glomerulonephritis
CCF – Mikulicz syndrome – v. rare thought to be IGG4 disease with sarcoid tangentially related.
All others direct manifestations.
** SCS: Sarcoid causes granulomatous interstitial nephritis.
Pagets disease of the nipple
- is a non-specific eczematous reaction caused by many different breast conditions
- is associated with DCIS in 50%
- is associated with IDC in 50%
- is a paraneoplastic syndrome associated with breast cancer that can be seen in either breast
CCF -
Associated with IDC in 50% (“[ in pagets ]palpable mass present in 50-60% almost all of these have invasive carcinoma” – my guy Robbins”. “quik maffs” -Big Shaq
(DICS higher than 50% (90%))
Stat dx, robbins orange box pg 1057.
Thymoma, not associated with? Red cell aplasia low T cells Hypogammaglobulinanaemia (this is how it was spelled) Myaesthenia gravis Graves disease
CCF
low T cells
All below listed on radiopeida Red cell aplasia – Yes 5% Hypogammaglobulinanaemia (this is how it was spelled) - Yes Myaesthenia gravis - Yes Graves disease Yes
[path] Giant cell arteritis, true?
- rarely involves MCA
- rarely involves ophthalmic artery
- commonly shows continuous involvement of temporal artery
- causes stenosis of the branches of aortic arch
- commonly seen in 30-50yo people
CCF – Multiple true
- rarely involves MCA – T (COW rare)
- rarely involves ophthalmic artery – F (v. common – blindness)
- commonly shows continuous involvement of temporal artery – F (focal involvement with long segments of relatively normal artery interposed – robbins pg 508). Prob why false negatives are common when we biopsy.
- causes stenosis of the branches of aortic arch - T – Yes (radiopedia and https://pubmed.ncbi.nlm.nih.gov/14674004/).
- commonly seen in 30-50yo people – F- Older patients over 50, peak 70-80
Renal cystic disease, which is most likely hereditary? (repeat)
- nephronopthisis
- medullary sponge kidney
- adult polycystic kidney disease
- autosomal recessive PKD
CCF
- AR PKD
- If question say least likely then MSK.
**SCS: prior recall said NOT hereditary.
Therefore MSK.
Which of the following is most likely to cause bilateral ovarian enlargement? (repeat, but with different wording from recalls)
- serous ovarian tumours
- mucinous ovarian tumours
- Brenner
- dermoid
- fibrothecoma
CCF – Serous ovarian
Pg 1023 robbins
Hypertrophic cardiomyopathy, most likely associated with? (repeat)
- assymetric thickening of the left ventricle free walls
- hypertension
- diastolic dysfunction
- normal mitral valve
CCF - - diastolic dysfunction - True
- assymetric thickening of the left ventricle free walls – No, subtypes are Asymmetric (most common) of the septum, Symemtrical, apical, mass like.
- hypertension - No
- normal mitral valve - Regurg
MEN1 don’t get which thing? (this question was duplicated in the exam)
- carcinoid
- pituitary adenoma
- islet cell tumour
- phaeochromocytoma
- parathyroid adenoma
CCF – pheochromocytoma.
Paraganglioma, least likely associated with (this was in the same exam as the question above)?
- succinate dehydrogenase deficiency
- Carney triad
- VHL
- MEN1
CCF – MEN1
**SCS: The most common genetic cause of hereditary paragangliomas are mutations in the succinate dehydrogenase (SDH) subunit (SDHB, SDHD, SDHA or SDHAF2) (radiopaedia)
