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Review on Angiomyolipoma (AML) Dr KP Wong Urology, PYNEH Joint Surgical Grand Round.

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Presentation on theme: "Review on Angiomyolipoma (AML) Dr KP Wong Urology, PYNEH Joint Surgical Grand Round."— Presentation transcript:

1 Review on Angiomyolipoma (AML) Dr KP Wong Urology, PYNEH Joint Surgical Grand Round

2 What is angiomyolipoma?  2 nd commonest benign neoplasm in kidney vessel fat smooth muscle

3 Imaging

4 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 males (0.1%)  11 out of 4971 females (0.22%)

5 pain mass haematuria shock haemorrhage Lenk’s triad 1. Oesterling et al. The management of renal angiomyolipoma.J Urol, 135:121, 1986 Presentation

6 ~59% symptomic on presentation Presentation. 2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315, 2002.

7 W - who is at risk ?

8 More common in Female ~86% W - Who at AML 2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315, 2002.

9 Tuberous Sclerosis Complex Cortical tubers Ash-leaf spots

10 W - Who at AML 2. NELSON et al. Contemporary diagnosis and management of renal angiomyolipoma.J Uro 168:1315, ~20% associated with Tuberous Sclerosis

11 W- who is at risk ? Am I different from sporadic AML ? In Which Way I was in difference?

12 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.

13 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

14 W - When to intervent? Symptomic vs Asymptomic

15 Intervention  Asymptomic AML  no RCT available  Limit prospective data, retrospective data:  symptomic or haemorrhage => likely larger tumors  Larger tumor => become symptomic with time Large?

16 Tumor size → symptom  Oesterling et al.  retrospective review (1948~ 1985)  253 patient: 8% from autopsy, 20% were TSC  stratified into 4cm (70%) 1. Oesterling et al. The management of renal angiomyolipoma.J Urol, 135:121, 1986

17 Tumor size → treatment 43%95% 1. Oesterling et al. The management of renal angiomyolipoma.J Urol, 135:121, 1986

18 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.

19  ↑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.

20 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

21 W - How to intervene?  Nephrectomy  total  Nephron sparing approach  Partial nephrectomy  Enucleation of AML  Cryotherapy (open or lap.)  Trans-arterial embolization

22 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.

23 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

24 end

25 Q & A

26

27 More information  Presentation  Histology  Association  TSC  LAM  Malignant variant  Imaging  Intervention  Consideration  Surgical intervention  Lap cryotherapy  Embolization  Hormonal potentiation  Molecular biology and recent advance

28 Presentation

29  Classical triad  Flank pain, tender palpable mass, gross haematuria  Incidental finding  Microscopic haematuria  Others:  Renal failure, UTI, anemia

30 Presentation  Palpable mass – 20%  Retroperitoneal hemorrhage – 15%  Hypovolemic shock – 30%  Renal insufficiency

31 Haemorrhagic aetiology  Wunderlich’s syndrome  Spontaneous retroperitoneal haemorrhage  Trauma  During pregnancy

32 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, WEBB et al. A population study of renal disease in patients with tuberous sclerosis. Br J Urol 74:151–4,1994

33 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

34 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

35 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

36 Histology

37 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

38 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

39 vascular fat smooth muscle

40 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

41 histological stain  anti-smooth muscle stain: spindle and epithelioid cell  S-100 antibodies: fat cell  HMB-45 : perivascular epithelioid cell derived lesion

42 Histological examination  Percutaneous biopsy  Tumor spread  Complication  Subsequent management  FNAC  Correctly Dx: 5/8  Melanosome associated protein HMB-45: 7/8

43 Association of AML

44 Association  Association  Tuberous Sclerosis complex  Lymphangionleiomyomatosis (LAM)  40 % of TSC (young female)  Rare for sporadic case - 60% associated with AML

45 Tuberous Sclerosis complex  Autosomonal dominant  Mutation in TSC1 or TSC 2 gene  Incidence of AML in TSC – 55~75%

46 Tuberous Sclerosis Complex

47  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

48 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

49 Growth Characteristic - TSC TSCsporadic 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

50 Growth Characteristic - TSC  Ewalt et al.  60 children with TSC: 4 year FU  50% 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, De Luca et al. Management of renal angiomyolipoma: a report of 53 cases. BJU Int, 83: 215

51 Am I different from sporadic AML ? In Which Way I was in difference? When will disease be present?

52 Lymphangioleiomyomatosis (LAM)

53  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

54 Lymphangioleiomyomatosis (LAM)  Pathology  Smooth muscle infiltration  Cystic destruction of lung  Symptoms:  Progressive SOBOE  Recurrent pneumothorax  Abdominal & thoracic lymphadenopathy  Abdominal tumor, e.g. AML, lymphangiomyoma

55 Lymphangioleiomyomatosis (LAM)  Treatment – antagonism of estrogen action  Progesterone im  Oral progrestin  GnRH agonist  Reversible Airflow limitation  Bronchodilator  Lung transplantation

56 epithelioid subtype

57 histological classification  classical  vascular, smooth muscle and adipose  epithelioid variant of angiomyolipoma  large component of epithelioid cell

58 epithelioid subtype  more aggressive  recur after resection  fatal

59 diagnostic imaging

60 Imaging - US  Mark hyperechoic signal and acoustic shadowing  8~47% RCC also hyperechoic  Some proportion of AML not highly hyperechoic

61 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)

62 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

63 Imaging – MRI T1 image T2 image

64 Consideration on intervention

65 Selective of intervention  Multiple factor  symptom  Lesion size  Tuberous sclerosis  Pregnancy plan  Occupation, activity  Renal reserve  Co-morbidity  Reliability and compliance

66 TSC and associated feature Symptomic Choice of treatment Observation & Fu

67 Stenier et al  Sporadic  < 4cm  Yearly imaging  > 4cm  Asymptomic or mild: semiannual US  Growth: surgery  Severe symptoms: surgery  TSC  < 4cm  Semiannual US  Growth: Surgery  > 4cm  Surgery

68 Surgical management

69 Intervention Pre-embolization post-embolization

70 Surgical management  Indication  Significant haemorrhage  Haematuria  Symptoms not responsive to conservative Tx  Local tissure or vascular invasion  Suspicion of malignancy after imaging

71 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

72 laparoscopic cryoablation

73 Gregory et al. Laparoscopic Cryoablation of Renal Angiomyolipoma. J Uro. 176, , 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

74 Gregory et al. Laparoscopic Cryoablation of Renal Angiomyolipoma THE JOURNAL OF UROLOGY Vol. 176, , October 2006

75 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

76 Angiographic embolization Pre-embolization post-embolization

77 Angiographic embolization Pre-embolization post-embolization

78 Angiographic embolization Pre-embolization post-embolization

79 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.

80 Kothary et al Renal 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)

81 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.

82 Angiographic embolization  Pros  Option in emergency setting  Nephron sparing  Cons  no tumor diagnosis  Difficult in Follow up  Possibility of revascularization Pre-embolization post-embolization

83 Angiographic embolization  Adipose tissue  Not sign affected  Angiomyogenic component  Responsed  Size  Decrease is variable  Enhancement  Decreased or absent Pre-embolization Post-embolization

84 Durability of embolization ?  Efficacy  Durability  Re-embolization rate

85 Conservative Treatment  Avoid contact activities which flank/abdominal impact is likely  Repeat yearly or half yearly CT or US

86 Horomal potentiation

87 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

88 Molecular biology recent advance

89 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

90 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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