Thank you for the new images Roz – it is interesting to follow this case as it unfolds. After reviewing the new CT images I would agree with Susie that they could indicate an alternative diagnosis of a transitional cell carcinoma (TCC) of the left kidney and ureter.
I would like to investigate a couple of the many treatment options available for TCC and aim to answer the question put forward by Susie – ‘What treatment options are available to the patient?’
Renal transitional cell carcinoma (TCC) is a malignant tumour that stems from theepithelial cells lining the urinary tract. Upper urinary tract TCCs (UUTUC) – in this patient’s case involving the left kidney and ureter - are uncommon. They account for only 5-10% of urothelial carcinomas (Siegel et al, 2012) - although evidence indicates an increase in these malignancies (Jemal et al, 2009). The predisposition UUTUCs have for recurrence, metastases and their multi focal nature mandates aggressive clinical intervention (Cai et al, 2011). Treatment is strongly influenced by tumour stage, which correlates closely with prognosis. Surgical intervention is usually the preferred method of treatment for localised disease.
Radical nephroureterectomy (RNU) with excision of the bladder cuff is the gold standard treatment for UTUC (Margulis et al, 2009). The aim of surgery is to prevent tumour seeding via bypass of the urinary tract during tumour resection. Since the risk of tumour recurrence is considerable, resection of the distal ureter and its orifice is also performed. Recent research by Lughezzani et al, (2010) concluded that this method – removing the distal ureter and bladder cuff - significantly improves survival rates.
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The traditional open surgical approach to RNUs is being challenged by less invasive approaches, e.g. laparoscopic. Simone et al, (2009), a prospective randomised study of 80 patients with non-metastatic UUUC demonstrated no superior effectiveness of laparoscopic surgery over open RNU, whilst the majority of recent research concludes superior outcomes for a laparoscopic versus open surgical approach (Ariane et al, 2012) and (Ni et al, 2012).
Endoscopic ablation is sometimes indicated in patients with a solitary kidney, in bilateral kidney disease and where major surgery is contraindicated. Although now slightly dated, research by Keeley et al, (1997) is commonly cited in recent literature. Their study looked at the ureteroscopic management of 38 patients (41 kidneys) with upper tract urothelial tumours graded 1 - 3. After endoscopic treatment, 16 of the 21 (76%) with grade 1 disease were tumour free - 4 had recurrences at a mean follow-up of 40.3 months. 9 of the 14 (64%) of grade 2 disease were tumour free - 4 had recurrent disease at a mean follow-up of 27.6 months. Finally, 2 of the 5 (40%) grade 3 tumours were tumor free at a mean follow-up of 21 months – no recurrence rates were reported for this group. They concluded that ureteroscopic treatment of the upper urinary tract TCC minimises morbidity and provides excellent success rates in patients with solitary, low-grade tumours.
Despite these findings, the tract recurrence risk is hard to calculate because relatively few endoscopic ablation treatments have been performed. Additionally, there is a reported risk of understating and under grading the disease with endoscopic management alone.
In order to determine the optimal treatment pathway for a patient with TCC – renal function, tumour grade, stage and location must first be evaluated.
I have only examined two of the treatment options available for TCC – would anybody else like to expand upon Susie's question by examiningothers?
I will not add any further questions as there are a few already outstanding.
by Alana McInally - Wednesday, 19 November 2014, 11:51 PM
Thank you for posting this interesting case and uploaded images Susie.
It appears that Noorayen and I have been working on the same topic over the last few days so although this post is likely to overlap in places, I hope to add insight and an alternative slant. I would like to examine the images and a possible diagnosis.
In the right lobe of the liver, there appears to be large 67 x 49 mm, well-defined, heterogeneous mass - predominantly hyperechoic in nature. Appearances are in keeping with a solid, rather than cystic, lesion. In the second ultrasound image, the liver lesion looks more isoechoic in echotexture - with some internal and peripheral vascularity demonstrated when colour Doppler is applied. The sagittal section of the unenhanced CT abdo-pelvis image also highlights this area of low attenuation in the right lobe.
These are unusual findings given the patient’s symptoms - acute LIF pain. It would be interesting to know whether the patient has had any other tests carried out, for example any blood work prior to the scans?
I agree with Noorayen that ultrasound appearances such as these could represent a Focal nodular hyperplasia (FNH). An FNH is a benign hyperplastic process which results in the normal constituents of the liver being arranged in an abnormally organised pattern – this is caused bya response to a 'congenital arteriovenous malformation'(Khan et al, 2013). An FNH is considered the second most common tumour of the liver following hepatic hemangiomas (Kang et al, 2010). In the majority of situations (80-95%), FNH arises as a solitary lesion, however, multiple lesions have been known to present themselves (Khan et al, 2013).
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Ultrasound characteristics of these lesions can vary, making diagnosis using one modality challenging. Lesions can range from hypoechoic, isoechoic to hyperechoic when compared to that of the surrounding liver tissue (Bates, 2011). Venturi et al, (2007) - as well as other recent research – state that typical lesions usually demonstrate a large, well circumscribed mass, with a central feeding artery and a radiating spoke-wheel pattern of blood flow (Bates, 2011). It could be suggested that the second ultrasound image shows central blood flow within the lesion, in keeping with the typical appearances described.
Although FNHs are normally asymptomatic, which is not in keeping with the patient’s LIF pain, it may indicate that the LIF pain and the liver lesion (located in the RUQ) are unrelated - that the liver lesion is instead an incidental finding; this is consistent with the typical diagnosis of an FNH. Most diagnoses occur when patients undergo cross-sectional imaging or surgery for other problems and / or routine medicals (Palladino et al, 2014).
Although the use of contraceptive agents is not proven to cause FNH, they may have a role in the development of these lesions. Additionally, they can also act as an irritant - causing haemorrhage or infarctions to occur – resulting in symptomatic patients. Malignant transformation of FNH has not been reported (Chung and DeGirolamo, 2011) and FNHs rarely bleed or grow. As a result, the diagnosis of an FNH rarely impacts the patient’s medical management - other than the accurate diagnosis of the lesion to prevent unnecessary biopsies, surgery, and further imaging of the lesion.
Despite advances in medical imaging, it is difficult to discern an FNH from other focal hepatic lesions. As a relatively recent imaging modality, the use of Contrast-Enhanced Ultrasound (CEUS) to identify focal liver lesions is becoming increasingly common (Bartolotta et al, 2009).
I will reiterate one of the three outstanding questions - does anyone have any further differential diagnoses?
by Alana McInally - Tuesday, 18 November 2014, 9:38 PM
Lucy’s post on the function of the spleen - in particular, the implications when it is removed - was interesting to read, especially as the spleen’s function and morphology have, in the past, remained unstudied (Lahey and Norcross, 1948). Although they havebecomeless common, as the spleen'simportance as an organ isrecognised, splenectomies are still performed and I would like to address Lucy’s question ‘What are the indications for a splenectomy?’.
A splenectomy consists of the total or partial surgical removal of the spleen. Literature sources provide a wide spectrum of clinical scenarios for when a splenectomy may be indicated. A general consensus exists for a handful of diagnoses which require a splenectomy. These include: primary cancers of the spleen (very rare), splenic trauma and hematologic diseases.
One blood disorder – Hereditary spherocytosis (HS) – often requires treatment via a splenectomy. It involves the loss of specific proteins in the red blood cell membrane, resulting in fragile cells which are further damaged when they pass through the spleen (Encyclopedia of Surgery). This damage ceases once the spleen is removed. Another hematologic disease - primary immune thrombocytopenia (ITP) - shows the highest cure rate (60-70%) after a splenectomy versus other treatments (Ghanima et al, 2012).
Trauma to the spleen can result from damage or rupture from both blunt and penetrating injuries to the abdomen. Studies suggest 25% of trauma injuries are originally caused by medical intervention to the abdomen (Rull, 2012). Laparoscopic splenectomies are often indicated in splenic trauma to prevent internal hemorrhaging and potentially death (NHS Choices, 2014).
There are also numerous other conditions where a splenectomy may be indicated / recommended - these include:
- Splenic artery aneurysms - surgical resection is often indicated in the presence of large splenic artery aneurysms (over 2 cm in diameter), because the risk of hemorrhage and rupture can be fatal if not treated (Bates, 2011).
- Multiple splenic abscesses - relatively uncommon but have an associated high mortality rate (Provenzale et al, 2012). Some view percutaneous drainage combined with antibiotic therapy as the best management for solitary abscesses (Bates, 2011) whereas other research proposes a splenectomy is the best treatment for multiple abscesses.
- Splenomegaly – an enlarged spleen (more than 12 cm) (Bates, 2011) as an isolated indicator for a splenectomy is often unjustified. Best practice is to now investigate the underlying cause. Surgery is only indicated if the enlarged spleen is causing serious complications and/or if the underlying cause cannot be identified (NHS Choices, 2014).
As Lucy mentioned, there are benefits and risks of a splenectomy. Most research recognises the associated life-long risk of bacterial infection following a splenectomy (Schilling, 2009), combined with the risks of invasive surgery. The general consensus shifts towards a conservative approach – attempting to preserve the spleen as opposed to invasive treatment (Akinkuolie et al, 2010).
In our trust I found it hard to locate hospital guidelines and protocols for when splenectomies were considered appropriate. However, I came across one case where a patient had a partial laparoscopic splenectomy using wedge resection to treat splenic trauma. This allowed him to retain some splenic function and additionally he was fortunate enough to have an accessory spleen (present in 30% of the population) – which has the ability to grow and function when a large portion has been removed (Arra et al, 2013). Consequently his splenic function was comparable to that prior to surgery. This technique supports the recent advance towards a conservative outlook on splenectomies.
It would be interesting if anyone else is aware of the guidelines in their local hospital for when a splenectomy is indicated?
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