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.

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