Auckland IC path 2022 Flashcards
(100 cards)
Klinefelter syndrome is characterised by all of the following EXCEPT:
Increased risk of germ cell tumours
Sclerosis of seminiferous tubules and Leydig cell nodules
Increased risk of breast cancers
Elevated serum follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels
A karyotype of 47XXY
CCF - Sclerosis of seminiferous tubules and Leydig cell nodules
Klinefelter increased risk mediastinal germ cell tumor, not testicular.
Increased risk of germ cell tumours - yes
Sclerosis of seminiferous tubules and Leydig cell nodules - no
Increased risk of breast cancers - yes
Elevated serum follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels - yes
A karyotype of 47XXY - yes
Official course answer is Increased risk of germ cell tumours but this is BS
Which of the following is FALSE regarding Graft Versus Host Disease?
Marked luminal narrowing of small bowel results in ‘ribbon like’ appearance on enterography
May occur following allogenic liver transplantation
Major clinical manifestations result from involvement of immune system, epithelia of skin, liver and intestines in the acute stage
Mediated by B lymphocytes of donor graft
Biopsy of intestinal mucosa reveals crypt loss and denudation of gut mucosa on histology
CCF -Mediated by B lymphocytes of donor graft - FALSE
-mediated by T lymphocytes
SCS:
Big robbins.
May occur following allogenic liver transplantation. True. GVHD most commonly in HSC transplant. But rarely may occur following transplantation of solid organs, rich in lymphoid cell (ie LIVER, transfusion of unirradiated blood).
Which of the following is least associated with urachal remnants?
Cysts
Abscess
Adenocarcinoma
Squamous cell carcinoma
Sinus tract
- Squamous cell carcinoma
Which of the following is FALSE of Craniopharyngioma?
Is a WHO grade 1 neoplasm
Papillary subtype is found almost exclusively in children
Has a bimodal distribution
Arise from epithelial origins of Rathke pouch
Adamantinomatous and papillary are the two subtypes
-Papillary subtype is found almost exclusively in children
**Adamantinomatous - children
Papillary - adults
What is the MOST likely palpable testicular tumour in a 20 year-old male?
Teratoma
Lymphoma
Embryonal carcinoma
Seminoma
Endodermal sinus tumour
- Seminoma
testicular tumours - 2 major categories
germ cell tumour (95%) - seminomas and nonseminomas
sex cord stromal tumours
Seminoma most common in the thrid decade (20s)
Embryonal mostly occur 20-30 year age group but less common than seminoma
Which of the following is NOT a recognized association of Thymoma?
Graves’ disease
Pernicious anaemia
Cushings syndrome
Myesthenia gravis
Hypergammaglobulinaemia
-Hypergammaglobulinaemia
** its HYPOgammaglobulinemia
Which of the following is NOT a feature of Rheumatic heart disease?
Bulky friable vegetations along the line of closure of the mitral valve
Fibrous thickening and fusion of chordae tendinae.
Microscopic appearance of Aschoff bodies in any of the three layers of the heart
Subependymal lesions called Mac callum plaques usually in the left atrium
Fibrinous bridging across valvular commissures resulting in ‘button hole ‘ or fish mouth stenoses
SCS: bulky friable vegetations- suggestive of infective endocarditis.
-assuming they mean subendocardial - location of MacCallum plaques…
“Bulky”- verrucae in Rh hrt disease is 1-2 mm- SMALL VEGETATIONS Usually along lines of closure which lead to regurgitation.
Subependymal lesions called Mac callum plaques usually in the left atrium.
** subendocardial lesions called MacCallum plaques, usually in the left atrium (Robins 9th edit pg 558).
Which cancer type is most associated with loss of function of the E-cadherin gene?
Invasive ductal carcinoma of the breast
Ewing’s sarcoma
Diffuse brainstem glioma
Diffuse gastric adenocarcinoma
Clear cell renal cell carcinoma
-Diffuse gastric adenocarcinoma (significant reduced expression)
Breast - lobular carcinoma (not listed - loss of e-cadherin)
diffuse gastric adenocarcinom has down regulation of e-cadherin
Which of the following favours acute over subacute bacterial endocarditis?
1 cm large vegetation
Absence of metastatic infection
Slow increase in size of vegetation
Pre-existing damaged valve leaflet
No perforation of leaflets
- 1 cm large vegetation
Which of these are statements are NOT true with regards to Wilms tumour?
Nephrogenic rests are identified in 100% of bilateral Wilms tumour
Children with WAGR syndrome have a 33% chance of developing Wilms tumour
Pulmonary metastases are uncommon at the time of diagnosis
In 10% of cases, there are bilateral or multicentric tumours at the time of diagnosis
The peak age for diagnosis of Wilms tumour is 2-5 years of age
Answer: Pulmonary metastases are uncommon at the time of diagnosis (FALSE)
FROM ROBBINS
- Most children with Wilms tumors present with a large abdominal mass. Hematuria, pain in the abdomen after some traumatic incident, intestinal obstruction, and appearance of hypertension are other patterns of presentation. in a considerable number of these patients, pulmonary metastases are present at the time of primary diagnosis.
- Nephrogenic rests are putative precursor lesions of Wills tumors, and are seen in the renal parenchyma adjacent to approximately 25-40% of unilateral tumors; this frequency rises to nearly 100% in cases of bilateral Wilms tutors.
- The risk of Wilms tumor is increased with a least three recognisable groups of congenital malformations associated with distinct chromosomal loci; WAGR syndrome, Denys-Drash syndrome, and Beckwith-Wiedemann syndrome
- WAGR syndrome is characterised by Wilms tumor, aniridia, genital anomalies, and mental retardation. The lifetime risk of developing Wilms tumor is approximately 33%.
- Grossly, Wilms tumor tends to present as a large, solitary, well-circumscribed mass, although 10% are either bilateral or multi centric at the time of diagnosis.
- IN approximately 5-10% of Wilms tumors involve both kidneys, either simultaneously (synchronous) or one after the other (metachronous).
- The peak incidence for Wilms tumor is between 2 and 5 years of age, and 95% occur before the age of 10 years old.
- Approximately 5% of tumors reveal anaplasia. The presecence of anaplasia correlates with the presence of TP53 mutations, and the emergence of resistance to chemotherapy.
Which of the following is MOST likely to be associated with Rheumatoid arthritis?
HLA B27 antigen
IgM anti IgG antibodies
Anti - DNase B
Urate crystals and neutrophils in synovial fluid
Antibodies to double stranded DNA
Answer: IgM anti IgG antibodies.
FROM ROBBINS
- 80% of patient has serum IgM or IgA autoantibodies that bind to the Fc portions of their own IgG. These autoantibodies are call Rheumatoid Factor.
- 50% of the risk developing RA is related to inherited genetic susceptibility. Specific HLA-DRB1 alleles are linked to RA. (HLA-DRB1 is the most common allele of HLA-DR4).
- The synovial of RA contains germinal centres with secondary follicles and plasma cells which produce antibodies. Many of the autoantibodies produced are specific for citrullinated peptides (CCPs).
FROM RADIOPAEDIA
- RF is a traditional marker but is non-specific, associated with several autoimmune and chronic infectious diseases
- Anti-CCP/anti-citrullinated plasma antibody (ACPA) is more than 80% sensitive and more than 95% specific.
What is the MOST common location of an intralobar sequestration?
Right upper lobe
Left upper lobe
Right middle lobe
Left lower lobe
Right lower lobe
CCF - Left lower
All of the following conditions affect the terminal duct lobular unit except:
Carcinoma
Breast cyst
Sclerosing adenosis
Atypical hyperplasia
Fibroadenoma
-Fibroadenoma
Which lesion is MOST likely to be epiphyseal?
Unicameral bone cyst
Clear cell chondrosarcoma
Chondromyxoid Fibroma
Conventional osteosarcoma
Aneurysmal bone cyst
Answer: Clear cell chondrosarcoma
FROM RADIOPEDIA
DDX for epiphyseal lesions
- Chrondroblastoma
- GCT
- Geode
- Intraosseous ganglion
- Osteomyelitis
- Clear cell chondrosarcoma
- Others that occur rarely: ABC, osteosarcoma and osteoblastoma, brown tumour, enchondroma, osteoid osteoma.
Hypoparathyroidism can be caused by all of the following except:
Congenital parathyroid absence
Autoimmune mediated
Parathyroid removal
Chronic hypocalcaemia
End organ resistance to parathyroid hormone (PTH)
Official answer: chronic hypocalcaemia. Round one goes to Saj.
CCF - End organ resistance to parathyroid hormone (PTH)
as this is describes pseudohypothyroidism and is ass w HyperPTH.
Chronic hypocalcaemia - is also wrong (Hyper PTH like in secodary hyperpara)).
^SCS agree; two technically incorrect answers. The body is essentially running as hypoparathyroid in pseudoHPT so i would go with chronic hypocalcaemia if i had a gun to my head….
Also hypomagnesaemia can do it.
Good discussions all around. Pity about the ambiguity in the question.
Disagree - Chronic hypocalcaemia will give your HYPERparathyroidism.
End organ resistance to parathyroid hormone (PTH) - yes this is pseudo but still results in hypoparathyroidism which is what the question is asking]
^ Disagree - Psudo is end organ resistance to PTH, therefore to try and compensate the PTH is HIGH (feedback loops).
*** see robins pg 1105 (9th edition) PTH levels can be normal in pseudospeudphypo
**WJI - a lot of argument here. I think it is important to note that the question asks for causes of hypoparathyroidism which is the clinical syndrome. It does not ask for causes of raised parathyroid hormone. In end organ resistance the patient may have a raised PTH (irrelevant) but will have the syndrome of hypoparathyroidism (or pseudohypoparathyroidism). Agree with chronic hypocalcaemia as this results in hyperparathyroidism.
**Pathlads: we concur.
BRCA2 oncogene is associated with the following cancers EXCEPT:
Renal cancer
Melanoma
Male breast cancer
Ovarian cancer
Gastric cancer
CCF - Renal
Facts below from robbins:
Both BRCA 1 and 2
Breast (3% total, 1>2)
Ovary
Male breast (>in BRCA 2)
Prostate
Pancreas
BRCA1
-Breast cancer tends trip negative, poor diff w “medullary features”
-Ovary tends to be serous type
-Fallopian tube- can remember this as also serous (serous ovarian cancer cells are related to fallopian epithelium embryologically-pathoma lecture)
BRCA2
-Stomach
-Melanoma
-GB, bile duct
Pharynx
Breast also poor diff but ER +Ve
**Pathlads: Just FYI, BRCA2 is a tumour supressor gene. duh.
Phaeochromocyoma is NOT found in:
Organ of Zuckerkandl
Adrenal medulla
Mediastinum
Adrenal cortex
Bladder
CCF - Cortex, wrong cell type in this location.
- Adrenal cortex - site of Adrenocortical carcinoma
Medulla is pheochromocytoma
can also be found in bladder, mediastinum, Organ of Zuckerkandl
Adenomatous polyps are NOT a feature of:
Peutz-Jeghers Syndrome
Gardners Syndrome
Villous adenomas
Turcot’s Syndrome
Familial Polyposis
CCF - Peutz-Jeghers Syndrome (hamartmatous)
Which of the following is LEAST likely to be true of pulmonary embolism?
Pulmonary infarction usually occurs when there is pre-existing cardiac or lung disease
Haemorrhage can be a sequelae without infarction
Has a higher incidence in patients with burns
The thrombi typically show lines of Zahn
Has a 10% chance of recurrence
CCF -Has a 10% chance of recurrence (false - robbins says 30% page 699)
Pulmonary infarction usually occurs when there is pre-existing cardiac or lung disease - T
Haemorrhage can be a sequelae without infarction - T
Has a higher incidence in patients with burns - T (burns - inflammation - hypercoag state)
The thrombi typically show lines of Zahn - T
Additional facts from robbins
-50-80% of emboli are clinically silent; in these cases bronchial artery sustains the viability of the affected lung,
-severe consequences (sudden death, RIght heart failure, shock (pump collapse), typically occur when more thst 60% of the total pulmonary vasculature is obstructed)
Which of the following is NOT TRUE of Rheumatoid nodules?
Centre of the nodule consists of an area of coagulative necrosis
May have one or more necrotic centres
Radially arranged cellular palisades of macrophages and fibroblasts is characteristic
Rheumatic skin nodules and pulmonary nodules are identical histologically
Have an outer fibrous shell
Answer: Centre of nodule consists of an read of coagulative necrosis (NOT TRUE)
FROM ROBBINS
- Rheumatoid nodules: necrotising granulomas with a central zone of fibrinoid necrosis surrounded by a prominent rm of activated macrophages and numerous lymphocytes and plasma cells.
- Cutaneous rheumatoid nodules are the most common. They occur in 25% of affected individuals, usually those with severe disease. Less commonly, the form in the lungs, spleen, pericardium, myocardium, heart valves, and aorta.
FROM RADIOPAEDIA/STATDX
- On histology, rheumatoid pulmonary nodules appear identical to nodules found in subcutaneous tissue.
- Rheumatoid nodule is a soft tissue mass with 3 histological zones; central fibrinoid necrosis, intermediate zone palisading, histiocyte-like macrophages, peripheral granulation/fibrous tissue.
**May have one or more necrotic centres: Not mentioned in Robbins/Statdx/Radiopaedia. Probably true.
Post menopausal PV bleeding and endometrial hyperplasia is associated with which of the following ovarian neoplasms?
Granulosa cell tumour
Serous cystadenocarcinoma
Teratoma
Krukenberg tumour
Dysgerminoma
Answer: Granulosa cell tumour
FROM ROBBINS
- Granulosa cell tumors are of clinical importance for two reasons: (1) they may elaborate large amounts of extrogen, and (2) they may behave like low-grade malignancies.
- In prepubertal girls (juvenile granulosa cell tumors) they may produce precocious sexual development.
- In adult women, they may be associated with proliferative breast disease, endometrial hyperplasia, and endometrial carcinoma, which eventually develops in 10-15% of women.
- Occasionally, granulos cell tumors produce androgens, masculizing the patient.,
- Histologically, Call-Exner Bodies are seen.
OTHER (From Robbins)
- Serous cystadenocarcinoma is a primary ovarian neoplasms arising from mullerian epithelium.
- Krukenberg tumor is a classic metastatic gastrointestinal carcinoma involving the ovaries, characterised by bilateral metastases composed of mucin-producing signet-ring cancer cells, most often of gastric origin.
- Teratomas, often referred to as dermoid cysts, are tumors of germ cell origin can can be divided into three categories. 1) mature (benign), 2) immatures (malignant), 3) monodermal or highly specialised.
- Dysgerminoma is the ovarian counterpart of testicular seminoma. Most of these tumors have no endocrine function. A few produce elevated levels of chorionic gonadotropin. They express OCT-3, OCT4, and NANOG transcription factors. They also express receptor tyrosine kinase KIT, with 1/3 having activating mutations in the KIT gene.
Which of the following combinations is FALSE?
Cystic medial degeneration and Marfan syndrome
Gamna Gandy nodules and spleen in portal hypertension
Homer – Wright pseudorosettes and Nephroblastoma
Rasmussen aneurysm and cavitary pulmonary tuberculosis
Obliterative endarteritis of vasa vasorum and Syphylitic aortitis
Homer – Wright pseudorosettes and Nephroblastoma -INCORRECT
**SCS: Radiopaedia/Pathoma. These are associated with neuronal origin tumours (medullo, PNET, esthesioneuroblastoma, pineoblastoma)
What is the most common tumour of the parotid gland?
Warthin tumour
Adenocarcinoma
Pleomorphic adenoma
Squamous cell carcinoma
Acinic cell carcinoma
Answer: Pleomorphic adenoma.
FROM ROBBINS
- Pleomorphic adenomas represent about 60% of tumors in the parotid, and are less common in the submandibular glands, and relatively rare in the minor salivary glands.
- Warthin Tumor arises almost exclusively in the parotid gland (the only tumor virtually restricted to the parotid) and occurs more commonly in males than females in the 5th-7th decade of life. Smokers have 8 times the risk of non-smokers.
EXTRA (From robbins)
Benign Salivary tumours
- 50%: Pleomorphic adenoma
- 5-10%: Warthins
- 5-10%: other (basal cell adenoma, canalicular adenoma)
- 1%: oncocytoma
Malignant Salivary tumours
- 15%: mucoepidermoid carcinoma
- 10%: adenocarcinoma
- 5%: acinic cell carcinioma
- 5%: adenoid cystic carcinoma
- 3-5%: Malignant mixed tumours
- 1%: Squamous cell carcinoma
What is the most likely cause for siderotic nodules (Gamna-Gandy bodies) in spleen?
Portal hypertension
Chronic lymphocytic leukemia (CLL)
Cardiac failure
Myelofibrosis
Polycythaemia vera
Answer: Portal hypertension