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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).
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).
Around 75% of RCC have localised disease rather than disseminated and nephrectomy, either partial or total is still the gold standard treatment.
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).
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)
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:
For a detailed review of current and future treatments, visit here.
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:
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:
Management of Cushing’s disease requires a team of experts and treatment options available for the disease include:
For a review of different laparotomy scars, this Review Article is very good.
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What scar did this patient have?
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?
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?
What mode of inheritance is seen with the commonest form of polycystic kidney disease?