review on angiomyolipoma (aml) www@aml dr kp wong urology, pyneh joint surgical grand round
TRANSCRIPT
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Review on Angiomyolipoma (AML)www@AML
Dr KP WongUrology, PYNEH
Joint Surgical Grand Round
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What is angiomyolipoma?
2nd commonest benign neoplasm in kidney
vessel
fat
smooth muscle
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Imaging
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How common? 1969: 8501 Autopsies without tuberous sclerosis
complex (TSC) 2 males (0.02%) 25 females (0.29%)
1995: Population based USG screening (Japanese Study) 13 out of 12970 males (0.1%) 11 out of 4971 females (0.22%)
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pain
mass
haematuria
shock
haemorrhage
Lenk’s triad
1. Oesterling et al. The management of renal angiomyolipoma.J Urol, 135:121, 1986
Presentation
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~59% symptomic on presentation
Presentation.
2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315,
2002.
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W - who is at risk ?
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More common in Female ~86%
W - Who at risk @ AML
2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315,
2002.
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Tuberous Sclerosis Complex
Cortical tubers
Ash-leaf spots
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W - Who at risk @ AML
2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315,
2002.
~20% associated with Tuberous Sclerosis
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W- who is at risk ?
Am I different from sporadic AML ?
In Which Way I was in difference?
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Nature history - TSC
Young, Large, Multiple, more acute bleeding
2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315,
2002.
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Growth Characteristic - TSC Steiner et al.
35 patient: Follow up: 4 year
3. Steiner, et al. The natural history of renal angiomyolipoma. J Urol,150: 1782, 1993
67%
50%
21%28%
0%
20%
40%
60%
80%
100%
No. tumors thatgrow (%)
requir surgicalintervention
TSC
sporadic
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W - When to intervent?
Symptomic vs Asymptomic
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Intervention
Asymptomic AML no RCT available Limit prospective data, retrospective data:
symptomic or haemorrhage => likely larger tumorsLarger tumor => become symptomic with time
Large?
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Tumor size → symptom Oesterling et al.
retrospective review (1948~ 1985) 253 patient: 8% from autopsy, 20% were TSC stratified into < 4cm (30%), > 4cm (70%)
1. Oesterling et al. The management of renal angiomyolipoma.J Urol, 135:121, 1986
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Tumor size → treatment
43% 95%
1. Oesterling et al. The management of renal angiomyolipoma.J Urol, 135:121, 1986
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Tumor size → symptoms Nelson et al.
Large tumors (>4cm) more likely symptomic
2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315,
2002.
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↑Need to intervene with larger tumor (>4cm)
Tumor size → treatment
2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315,
2002.
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Tumor size → Growth Steiner et al.
35 patient: Follow up: 4 year
3. Steiner, et al. The natural history of renal angiomyolipoma. J Urol,150: 1782, 1993
27%
46%
0%
10%
20%
30%
40%
50%
No. tumors that grow (%)
< 4cm
> 4cm
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W - How to intervene?
Nephrectomy total
Nephron sparing approach Partial nephrectomy Enucleation of AML Cryotherapy (open or lap.) Trans-arterial embolization
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Angiographic embolization
Emergency Avoid total nephrectomy Nephron sparing
Elective Reduce size
Efficacy Re-embolization ~14% Surgical intervention ~16%
Pre-embolization
Post-embolization
2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315,
2002.
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Take Home Message W – Who at risk
female Tuberous sclerosis complex
W - When to intervene Size:
W - How to intervene Nephron sparing approach would be choice of
management in AML
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Q & A
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More information
Presentation Histology Association
TSC LAM Malignant variant
Imaging
Intervention Consideration Surgical intervention Lap cryotherapy Embolization
Hormonal potentiation Molecular biology and
recent advance
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Presentation
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Presentation Classical triad
Flank pain, tender palpable mass, gross haematuria
Incidental finding Microscopic haematuria Others:
Renal failure, UTI, anemia
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Presentation
Palpable mass – 20% Retroperitoneal hemorrhage – 15% Hypovolemic shock – 30% Renal insufficiency
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Haemorrhagic aetiology
Wunderlich’s syndrome Spontaneous retroperitoneal haemorrhage
Trauma During pregnancy
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haemorrhage
risk or haemorrhage: 25%~50% female 18%, male: 8%
6.KESSLER et al. Management of renal angiomyolipoma: Analysis of 15 cases. Eur Urol 33:572–5, 1998
7.WEBB et al. A population study of renal disease in patients with tuberous sclerosis. Br J Urol 74:151–4,1994
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Growth Characteristic - Size Steiner et al.
35 patient: Follow up: 4 year
3. Steiner, et al. The natural history of renal angiomyolipoma. J Urol,150: 1782, 1993
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Growth Characteristic - Size
< 4cm > 4cm
No. tumors that grow (%) 27% 46%
Require surgical intervention (%)
7% 53.8%
Large AML (>4cm) enlarged more likely than small AML
Steiner et al. 35 patient: Follow up: 4 year
3. Steiner, et al. The natural history of renal angiomyolipoma. J Urol,150: 1782, 1993
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Growth Characteristic - Size Steiner et al.
35 patient: Follow up: 4 year
3. Steiner, et al. The natural history of renal angiomyolipoma. J Urol,150: 1782, 1993
27%
7%
46%
53.80%
0%
10%
20%
30%
40%
50%
60%
No. tumors thatgrow (%)
Require surgicalintervention (%)
< 4cm
> 4cm
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Histology
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Classification
Hamartoma? Fat and smooth muscle not normal found in kidney
Choristoma? Neoplastic?
Clonal expansion LN invasion
Extra-renal AML Adrenal gland, Liver, ovary, fallopian tube, spermatic cord,
colon
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History
1900: Grawitz a large renal tumor comprised of fat, muscle and blood
vessels
1911: Fisher Histopathology: Contains fat cell, smooth muscle cell,
blood vessel in different proportions
1951: Morgan Name: angiomyolipoma
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vascular
fat
smooth muscle
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Pathological evaluation Fatty – normal Vascular – thicken
wall, lower elastin content, surround by cuff of smooth muscle cell
Smooth muscle – normal spindle cell or rounded epithelioid cells
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histological stain
anti-smooth muscle stain: spindle and epithelioid cell
S-100 antibodies: fat cell
HMB-45 : perivascular epithelioid cell derived lesion
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Histological examination
Percutaneous biopsy Tumor spread Complication Subsequent management
FNAC Correctly Dx: 5/8 Melanosome associated protein HMB-45: 7/8
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Association of AML
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Association
Association Tuberous Sclerosis complex Lymphangionleiomyomatosis (LAM)
40 % of TSC (young female)Rare for sporadic case - 60% associated with AML
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Tuberous Sclerosis complex
Autosomonal dominant Mutation in TSC1 or
TSC 2 gene Incidence of AML in
TSC – 55~75%
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Tuberous Sclerosis Complex
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Tuberous Sclerosis Complex
Dermatological Ash-leaf spots Shagreen patch Adenoma sebaceum Ungal fibromas
Neurological Cerebral cortical tubers Epilepsy, infantile spasm Neruobehavorial disorder
Renal AML epithelial cyst, polycystic
kidney, RCC
Pulmonary Lymphangio-leiomyomatosis
Cardiac Rhabdomyomas
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Growth Characteristic - TSC Steiner et al.
35 patient: Follow up: 4 year
3. Steiner, et al. The natural history of renal angiomyolipoma. J Urol,150: 1782, 1993
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Growth Characteristic - TSC
TSC sporadic
No. tumors that grow (%)
67% 21%
requir surgical intervention
50% 28%
Steiner et al. 35 patient: Follow up: 4 year
3. Steiner, et al. The natural history of renal angiomyolipoma. J Urol,150: 1782, 1993
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Growth Characteristic - TSC Ewalt et al.
60 children with TSC: 4 year FU50% had renal AML at initial stage During Fu, 40.7 % of children without AML at initial stage had
AML
De Luca et al. 51 patient with sporadic AML: 5 year Fu
92% without growth
AML with TSC enlarged more likely than sporadic AML
4. Ewalt, et al. Renal lesion growth in children with tuberous sclerosis complex. J Urol, 160: 141, 1998
5. De Luca et al. Management of renal angiomyolipoma: a report of 53 cases. BJU Int, 83: 215
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Am I different from sporadic AML ?
In Which Way I was in difference?
When will disease be present?
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Lymphangioleiomyomatosis (LAM)
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Lymphangioleiomyomatosis (LAM)
Male Biopsy document LAM: 3 with TSC, 1 without TSC
Tuberous sclerosis complex 40% of TSC: had LAM 1% of TSC: symptoms
Sporadic Sporadic: rare < 1000 case 60% of LAM: had AML
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Lymphangioleiomyomatosis (LAM)
Pathology Smooth muscle infiltration Cystic destruction of lung
Symptoms: Progressive SOBOE Recurrent pneumothorax Abdominal & thoracic
lymphadenopathy Abdominal tumor, e.g. AML,
lymphangiomyoma
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Lymphangioleiomyomatosis (LAM)
Treatment – antagonism of estrogen action Progesterone im Oral progrestin GnRH agonist
Reversible Airflow limitation Bronchodilator
Lung transplantation
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epithelioid subtype
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histological classification
classical vascular, smooth muscle and adipose
epithelioid variant of angiomyolipoma large component of epithelioid cell
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epithelioid subtype
more aggressive
recur after resection fatal
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diagnostic imaging
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Imaging - US
Mark hyperechoic signal and acoustic shadowing 8~47% RCC also
hyperechoic Some proportion of
AML not highly hyperechoic
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Imaging - CT AML:
Fat contain renal lesion (< -15 HU)
Some lesion contain minimal fat
Malignant tumor (RCC): Tissue necrosis with lipid
formation, intratumoral bone metaplasia => areas of calcification (rare in AML)
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Imaging – MRI AML
High signal intensity on T1 image
Low intensity on T2 image
RCC Low signal intensity
on T1 image High intensity on T2 Contrast MRI
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Imaging – MRI
T1 imageT2 image
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Consideration on intervention
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Selective of intervention Multiple factor
symptom Lesion size Tuberous sclerosis Pregnancy plan Occupation, activity Renal reserve Co-morbidity Reliability and compliance
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TSC and associated feature
Symptomic
Choice of treatment
Observation & Fu
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Stenier et al
Sporadic < 4cm
Yearly imaging
> 4cm Asymptomic or mild:
semiannual USGrowth: surgerySevere symptoms:
surgery
TSC < 4cm
Semiannual USGrowth: Surgery
> 4cm Surgery
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Surgical management
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Intervention
Pre-embolization post-embolization
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Surgical management
Indication Significant haemorrhage Haematuria Symptoms not responsive to conservative Tx Local tissure or vascular invasion Suspicion of malignancy after imaging
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Total nephrectomy
Indication: Whole kidney replaced by AML Solitary AML near hilum or risk for partial than
complete nephrectomy Suspicion of malignancy Reperitoneal haemorrhage, unsuccessful or
unavailable embolization
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laparoscopic cryoablation
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Gregory et al.Laparoscopic Cryoablation of Renal Angiomyolipoma. J Uro. 176, 1512-1516, 2006
7 patient Average size – 4.2cm 3 minor complication in 2 patient Operative time – 170 miniutes, Blood loss of
116 cc Average decreased in CrCl 8.6 ml/min
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Gregory et al.Laparoscopic Cryoablation of Renal Angiomyolipoma THE JOURNAL OF UROLOGY
Vol. 176, 1512-1516, October 2006
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Angiographic embolization
Indication: Acute hemorrhage Symptomic tumor with limit renal reserve or
poor operative candidate Prophylatic treatment for asymptomic tumor
Materials: Gelatin sponge, alcohol, iodized oil, polyvinyl
alcohol particles, metal coils
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Angiographic embolization
Pre-embolization post-embolization
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Angiographic embolization
Pre-embolization post-embolization
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Angiographic embolization
Pre-embolization post-embolization
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Angiographic embolization Post embolization syndrome: 85% Complication: 10% of case
Abscess formation 5% Pleural effusion 3%
2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315,
2002.
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Kothary et alRenal Angiomyolipoma: Long-term Results after Arterial Embolization J Vasc Interv
Radiol 2005; 16:45–50
Retrospective review, mean Fu 51.5 months 19 patient (TSC: 10/19) 30 renal AML (TSC:21/30)
Recurrence 31.6% of patient (6/19) 30% of AML (9/30) – all from TSC group (9/21)
Time from embolization to recurrence Mean: 81.3 months (range: 36~132 months)
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Angiographic embolization Efficacy
Difficult to assess in absence of controlled studies
Re-embolization ~14% Surgical intervention ~16%
2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315,
2002.
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Angiographic embolization
Pros Option in emergency
setting Nephron sparing
Cons no tumor diagnosis Difficult in Follow up Possibility of
revascularization
Pre-embolization
post-embolization
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Angiographic embolization
Adipose tissue Not sign affected
Angiomyogenic component Responsed
Size Decrease is variable
Enhancement Decreased or absent
Pre-embolization
Post-embolization
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Durability of embolization ?
Efficacy Durability Re-embolization rate
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Conservative Treatment
Avoid contact activities which flank/abdominal impact is likely
Repeat yearly or half yearly CT or US
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Horomal potentiation
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Hormonal potentiation
Progestrone and estrogen receptor in smooth muscle cell nuclei of some AML
Cases of rapid growth during pregnancy
Hormonal therapy: Early stage => not well defined
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Molecular biology recent advance
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Molecular biology Tuberous sclerosis complex
TSC1 on chromosome 9q34 TSC2 on chromosome 16p13 => produce Tuberin
Tumor suppressor Gene AML tissue with TSC2 mutation: lack tuberin expression Heterozyogosity at ½ of TSC loci: consistent finding at TSC
associated AML
Other genes Loss of TSC2 heterozygosity in only 10% of sporadic AML
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Animal modal in AML
Akt singaling pathway in tuberous sclerosis Rat with hepatic tumor (HMB45 positive)
Rapamycin: Quenches S6K phosphorylation Shrinkage of inappropriate cell growth Shrinkage of renal tumor in mice
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