Angiomyolipoma of the Kidney

Review literature: (Oesterling et al, 1986).

Definition of Angiomyolipoma of the
Kidney
Angiomyolipoma of the kidney is a rare benign renal tumor with a
high fat content.
Epidemiology of Renal Angiomyolipoma
Prevalence:
The prevalence in autopsies is 0.3%, with ultrasound screening
0.1%. Women are much more frequently affected than men. Age
peak 5060 years.
Tuberous sclerosis:
4580% of patients with tuberous sclerosis have (bilateral)
asymptomatic angiomyolipomas. In tuberous sclerosis, there is an
equal distribution between men and women, age peak 30 years.
Etiology and Pathology of
Angiomyolipoma
Etiology:
Angiomyolipomas derive from perivascular epithelioid cells and grow
probably hormone-dependent.
Gross Pathology of Angiomyolipoma:
Angiomyolipomas are grey-yellow lesions without a tumor capsule,
round to oval. Sometimes, angiomyolipomas show multi-center
growth with involvement of lymph nodes without metastatic
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potential.
Histology of Angiomyolipoma:
Mature fat cells, smooth muscle cells, (atypical) blood vessels,
occasional mitoses.
Signs and Symptoms of Angiomyolipoma
of the Kidney
Flank pain
Potentially life-threatening hemorrhage due to spontaneous
rupture into the retroperitoneum (Wunderlich's syndrome).
Pregnancy increases the risk of rupture.
Diagnosis of Renal Angiomyolipoma
Sonography of the Kidney:
Sonography of the kidneys shows an echogenic mass in the kidney,
caused by the high fat content.
CT-Abdomen:
The fat content typically causes a hypodense mass in the kidney
with -20 to -80 HU, this enables the differentiation to a renal cell
carcinoma [fig. CT of angiomyolipoma]. Calcifications are rare in
renal angiomyolipoma.

Fig. CT abdomen of
renal angiomyolipoma:
visible are hypodense
fatty portions of the
kidney tumor. By
courtesy, Dr. G. Antes,
Kempten.
MRI Abdomen:
MRI enables the reliable detection of fat, which is typical for
angiomyolipoma, and allows the differentiation to a renal cell
carcinoma.
























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Angiography:
Neovascularization, similar to renal cell carcinoma, are possible.
Angiography has no role in the differential diagnosis of renal tumors.
Treatment of Renal Angiomyolipomas
Conservative Treatment of
Angiomyolipomas:
Annual imaging controls are possible with angiomyolipomas less
than 4 cm and mild symptoms. The annual growth rate is about 5%,
in tuberous sclerosis up to 20%.
Partial Nephrectomy:
Partial nephrectomy is indicated in angiomyolipomas of >4 cm,
severe symptoms or in tumors of uncertain diagnosis [see chapter
partial nephrectomy].
Selective embolization:
Selective embolization is a possible minimally invasive treatment
option. Sometimes, a relapse is possible after embolization. Rarely,
percutaneous drainage of necrosis is necessary.
Emergency nephrectomy:
Nephrectomy is necessary in rupture of angiomyolipoma with
life-threatening bleeding.
Renal oncocytoma Index Renal diseases
Index: 19 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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References
Deutsche Version:
Angiomyolipom der Nieren
Ads by Google Symptoms Kidney Cancer Kidney Pain Kidney Tumor
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Oesterling u.a. 1986 OESTERLING, J. E. ; FISHMAN, E. K. ; GOLDMAN,
S. M. ; MARSHALL, F. F.:
The management of renal angiomyolipoma.
In: J Urol
135 (1986), Nr. 6, S. 11214
Last update 13.3.2012
Dr. med. Dirk Manski
man...@urologielehrbuch.de
Angiomyolipoma of the Kidney www.urology-textbook.com http://www.urology-textbook.com/angiomyolipoma-kidney.html
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