Quiz Summary
0 of 5 Questions completed
Questions:
Information
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading…
You must sign in or sign up to start the quiz.
You must first complete the following:
Results
Results
0 of 5 Questions answered correctly
Your time:
Time has elapsed
You have reached 0 of 0 point(s), (0)
Earned Point(s): 0 of 0, (0)
0 Essay(s) Pending (Possible Point(s): 0)
Average score |
|
Your score |
|
Categories
- Abdominal 0%
-
Gold standard case presentation
Background
This case demonstrates the need to develop a differential diagnosis. In this case, there is a left sided, flank laparotomy scar. There are a number of reasons for this and you should have these in your mind not only when carrying out the rest of the examination, but also when you talk to the examiners. In this case, there has clearly been an open, rather than laparoscopic method used to approach the left kidney. Given this woman’s age, and the clearly old nature of the operative site, you cannot presume the reason was because of a large tumour (although this would be at the top of your differential list).
Differential diagnosis
Nephrectomy
- Partial nephrectomy
- Incidental, low-stage tumours
- Higher risk of peri-operative complications
- Higher risk of recurrence
- Preservation of renal function
- Total nephrectomy (lower complication rate, but loss of renal function on that side)
- Standard treatment for renal cell carcinoma
- Lower risk of peri-operative complications
- Lower risk of recurrence
- Loss of renal function
Renal cell carcinoma (RCC)
RCC makes up, up to 3% of all adult malignancies and is the 9th most common malignancy worldwide. It has a 2:1 preponderance for men over women. It presents generally between 30-50 years of age. It is not obvious why it has this leaning towards men, and developed countries. Other risk factors include smoking, obesity, hypertension, exposure to petrol and related chemicals as well as heavy metals and other renal diseases (cystic kidney disease, chronic kidney disease, dialysis and immunosuppression such as HIV).
Classification
- Clear cell RCC (up to 70%, originating in the proximal tubule with a 3p deletion)
- Papillary RCC (2nd most common)
- Chromophobe RCC
- Nephron and collecting system tumours (rare) – <5% of RCC
- Benign or pre-malignant tumours
Around 75% of RCC have localised disease rather than disseminated and nephrectomy, either partial or total is still the gold standard treatment.
Clinical features
- Asymptomatic and found incidentally
- Haematuria
- Flank mass
- Loin tenderness
The above classic symptoms are found only in around 10% of patients. Other symptoms are non-specific, such as anorexia, fever of unknown origin and metastases (most commonly lung).
Other features
- Polycythaemia (raised haemoglobin; raised platelet count; leucocytosis/eosinophilia)
- Iron deficiency anaemia
Investigations
Bloods
- Full blood count
- Urea and electrolytes
- Bone profile (especially calcium)
- Liver function tests
- Thyroid function tests
- Clotting profile
Urine
- Urine dip for haematuria and proteinuria
- Culture to ensure no infection
Imaging
- Staging computer tomography scan of the chest, abdomen and pelvis
- If concerns about bone metastases, then magnetic resonance imaging of the brain and spine and/or nuclear medicine bone scan
Histopathology
- Biopsy of identified mass
Staging
I T1N0M0 (Tumour ≤ 7cm) II T2N0M0 or T3N0Mo (Tumour ≥ 7cm) III T1-T3N1M0 (Not spread beyond Gerota’s fascia) IV T4AnyNM0 or AnyTAnyNM1 (Spread beyond Gerota’s fascia)
Management
Supportive care for the patient is key along with education and pastoral support. Surgery is the primary curative management option, if available. Metastases is not a contra-indication for surgery, as symptoms of recede even if the malignancy has spread beyond the kidney. There are a number of models used to predict the success after surgery including SSIGN (developed by the Mayo clinic and aka Leibovich Score) which claissifies patient as having low-, intermediate- and high-risk of recurrence (based upon Staging, tumour Size, Nuclear Grade and the absence/presence of tumour Necrosis). If surgical removal, or metastases remain, then targeted treatments are available:
- Tyrosine kinase inhibitors (sunitinib and pazopanib)
- Multikinase inhibitor (sorafenib)
- Anti-vascular endothelial growth factor monoclonal antibody (bevacizumab)
- Mammalian target of rapamycin inhibitors (temsirolimus and everolimus)
For a detailed review of current and future treatments, visit here.
Adrenalectomy
This scar could also be due to an adrenalectomy rather than nephrectomy, which would relate to Cushing’s Disease (not syndrome). It is characterized by an abnormally high level of cortisol in blood due to an ACTH secreting pituitary tumor. ACTH producing tumors if grow elsewhere in the body, they are called ectopic tumors. Clinical features of Cushing disease include the symptoms resulting from excess hormone in blood as well as compressive effects of the pituitary tumor (if large enough). Common clinical features in the disease are:
- Change in physical appearance
- Moon facies
- Buffalo hump
- Easy bruising of skin
- Abdominal striae (purplish stretch marks)
- Weight gain (in abdominal area only. Limbs tend to be thin)
- Plethora (red cheeks)
- Increased hair growth on legs, face, neck and chest
- Generalized weakness and fatigue
- Muscle wasting in upper thigh making getting up from chair difficult
- Menstrual disorders
- Reduced sex drive
- Reduced fertility
- Hypertension (uncontrollable)
- Diabetes mellitus (severe)
- Behavioral changes and mood disturbances
- Vision loss (macroadenomas)
- Loss of acuity
- Color perception affected
- Hypopituiarism (macroadenoma)
- Reduced sex hormones, LH and FSH
- Elevated Prolactin levels (stalk effect)
Due to slow changes, it’s often difficult to diagnose the disease in early stages. Furthermore, elevations in hormones occur in cycles and periods making diagnosis complicated. A detailed history, a thorough physical examination and tests are crucial to reach a diagnosis. Investigations that can be carried out include:
- Hormone testing
- 24 hour urine free cortisol measurement
- Cortisol saliva testing
- Blood ACTH
- MRI- detects adenoma in 70% of cases
- Inferior petrosal sinus sampling (IPSS)-if MRI fails then IPSS is conducted. It distinguishes ACTH producing tumor in pituitary from a tumor elsewhere in the body
Management of Cushing’s disease requires a team of experts and treatment options available for the disease include:
- Surgery
- Surgical removal is a long-term solution
- Cure rate for small tumors- 80-85%
- Endoscopic technique is very effective
- Medication
- Drugs don’t lower ACTH production and don’t shrink tumor but inhibit cortisol production by adrenals
- Necessary before surgery (ill patients)
- Indicated if surgery fails
- Radiation therapy
- Effective for controlling growth if surgery fails
- Stereotactic radiosurgery
- Delayed pituitary failure may occur several years later as a consequence
- Other options
- Cortisol replacement by hydrocortisone or prednisolone after surgery.
For a review of different laparotomy scars, this Review Article is very good.
Summary
We hope you’ve enjoyed the first part of our free trial content.
If you already like what you’ve seen, you can get instant access right away by subscribing. Benefit from this entire free trial and many more complete examination carousels instantly just by purchasing a subscription from our website or downloading our mobile app.
Need longer to decide?
We have an entire free carousel just waiting for our mailing list subscribers – simply sign up using the form below and we’ll send you exclusive free access to the remaining stations!
We highly recommend subscribers consider sourcing a reasonable quality pair of over ear headphones to maximise the value of our heart murmur content. If you sign up to our mailing list via our website you’ll get a free video straight to your email inbox in a few days to sample this!
Simply sign up for our mailing list from our home page.
Click here to continue
- Partial nephrectomy
- 1
- 2
- 3
- 4
- 5
- Current
- Review / Skip
- Answered
- Correct
- Incorrect
-
Question 1 of 5
1. Question
1 point(s)What scar did this patient have?
CorrectIncorrect -
Question 2 of 5
2. Question
1 point(s)CorrectIncorrect -
Question 3 of 5
3. Question
1 point(s)On examination you identify a readily ballotable mass on the right side that moves posteriorly with respiration. What is the most likely reason this patient had a nephrectomy?
CorrectIncorrect -
Question 4 of 5
4. Question
1 point(s)You are taking your PACES and see a young patient with bilaterally enlarged kidneys. You suspect polycystic kidney disease.
You notice that they also have a hemiparesis. What is the likely cause of their neurological deficit?
CorrectIncorrect -
-
Question 5 of 5
5. Question
1 point(s)What mode of inheritance is seen with the commonest form of polycystic kidney disease?
CorrectIncorrect