Shemssie Shewmollo Bushira*
Senior Surgical Resident at Saint Paul’s Teaching Referral Hospital, Addis Ababa, Ethiopia
Received Date: May 26, 2017; Accepted Date: May 30, 2017; Published Date: June 05, 2017
Citation: Bushira SS. Bilateral Renal Sngiomyolipoma: What is the Role of Conservative Management. J Clin Epigenet. 2017, 3:2. doi: 10.21767/2472-1158.100050
Angiomyolipoma of the kidney is a benign neoplasm composed of variable amount of adipose tissue, smooth muscle and thick walled blood vessels. It is the most common cause of spontaneous renal hemorrhage often presenting with classic triads of abdominal pain, palpable mass and hypovolemic shock called Wunderlich syndrome. Once the diagnosis of ruptured angiomyolipoma with unstable hemodynamic state is made, the treatment recommendation is either surgery or therapeutic embolization. Here we present review of the current literature for management of bilateral AML with rupture including conservative treatment options.
Angiomyolipoma of the kidney is a benign neoplasm composed of variable amount of adipose tissue, smooth muscle and thick walled blood vessels . These tumors account for 0.3% to 3% of renal masses and 1% of surgically resected tumors [2,3]. The chance of malignant transformation is exceedingly rare. Tuberous sclerosis-associated angiomyolipomas have a tendency to be large, multiple, and are more likely to bleed than their sporadic counterpart. CT or MRI is usually adequate for diagnosis and biopsy is rarely needed.
Patients with AML may present with fl ank pain, hematuria or palpable mass that may result from bleeding or large size tumor. Spontaneous renal bleeding secondary to an AML usually occurs when the tumor is larger than 4 cm .
Hemorrhagic AMLs need differentiation from a RCC or other vascular entities . Considering this a careful search must be done during CT evaluation in order to detect fat . Low grade retroperitoneal or capsular liposarcoma and RCC encasing perirenal fat are lesions that may mimic renal AML. The presence of typical internal tortuous aneurysmal vessels and a renal parenchyma defects [findings not often seen in liposarcomas] are used to differentiate AML from liposarcoma on CT scans . All renal mass with detectable fat should be considered an AML .
The primary reasons for intervention is symptomatic conditions such as pain or bleeding whereas prophylactic treatment is warranted for large tumors . Active surveillance is strongly recommended for asymptomatic tumors more than 4 cm, while angioembolization or renal sparing surgical interventions such as partial nephrectomy, enucleation, or wedge resection should be alternative options for symptomatic tumors ≥4 cm. Though some studies recommended a prophylactic radical nephrectomy for solitary tumors ≥8 cm or those refractory to angioembolization, others found angioembolization and nephron sparing surgery to offer long-term renal function preservation with low complication rates [8-12]. However, characteristics of TSC-associated AMLs such as large size and multiplicity have limited the role of nephron sparing surgery and are one of the reasons for increased number of angioembolization for this group of patients. Even though solitary lesions can successfully be treated by embolization and nephron sparing surgical therapies, the much more frustrating clinical problem of multiple renal angiomyolipomas that replace renal parenchyma has remained largely unaddressed. When bleeding occurs in this circumstance, it can be impossible to identify which lesion is the source . For patients with large, multiple bilateral AML with rupture, shock, and deranged renal function, surgical intervention in the form of nephron sparing nephrectomy and radical surgery is virtually impossible. In the absence of angioembolization service [not best for this kind of lesions as pointed out above], conservative management in the form of transfusion, fluid resuscitation and aggressive monitoring of clinical, laboratory and serial abdominal ultrasound seems the only option for caring such patients.
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