Difference between revisions of "Angiomyolipoma"
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| Micro = smooth muscle, adipose tissue (not always present), abundant blood vessels | | Micro = smooth muscle, adipose tissue (not always present), abundant blood vessels | ||
| Subtypes = conventional, epithelioid angiomyolipoma | | Subtypes = conventional, epithelioid angiomyolipoma | ||
| LMDDx = [[clear cell renal cell carcinoma]] (esp. for epithelioid variant), [[Xp11.2 translocation carcinoma]] (esp. for epithelioid variant), well-differentiated retroperitoneal [[sarcoma]] (esp. [[leiomyosarcoma]] & [[liposarcoma]]) | | LMDDx = [[clear cell renal cell carcinoma]] (esp. for epithelioid variant), [[Xp11.2 translocation carcinoma]] (esp. for epithelioid variant), well-differentiated retroperitoneal [[sarcoma]] (esp. [[leiomyosarcoma]] & [[liposarcoma]]), [[renal cell carcinoma with sarcomatoid differentiation]] | ||
| Stains = | | Stains = | ||
| IHC = HMB-45 +ve, Melan A +ve, SMA +ve | | IHC = HMB-45 +ve, Melan A +ve, SMA +ve, PAX8 -ve, CK (pooled) -ve | ||
| EM = | | EM = | ||
| Molecular = | | Molecular = | ||
Revision as of 16:36, 31 October 2014
| Angiomyolipoma | |
|---|---|
| Diagnosis in short | |
.jpg) Angiomyolipoma. H&E stain. | |
|
| |
| LM | smooth muscle, adipose tissue (not always present), abundant blood vessels |
| Subtypes | conventional, epithelioid angiomyolipoma |
| LM DDx | clear cell renal cell carcinoma (esp. for epithelioid variant), Xp11.2 translocation carcinoma (esp. for epithelioid variant), well-differentiated retroperitoneal sarcoma (esp. leiomyosarcoma & liposarcoma), renal cell carcinoma with sarcomatoid differentiation |
| IHC | HMB-45 +ve, Melan A +ve, SMA +ve, PAX8 -ve, CK (pooled) -ve |
| Site | kidney (see kidney tumours), other sites |
|
| |
| Syndromes | tuberous sclerosis |
|
| |
| Prevalence | uncommon |
| Radiology | classically has regions consistent with fat |
| Prognosis | benign, epithelioid variant may be aggressive |
| Clin. DDx | other kidney tumours |
| Treatment | surgery - esp. if large or imaging characteristics ambiguous |
Angiomyolipoma, abbreviated AML, is a benign mesenchymal tumour that is associated with tuberous sclerosis and belongs to the PEComa group of tumours.
It is typically found in the kidney.
General
- Benign mesenchymal tumour.
- Presentations: flank pain, hematuria, incidentaloma.[1]
- Tumours >4 cm considered a risk for bleeding.[2]
- AMLs occur may be elsewhere in the body, e.g. liver,[3] but are most common in the kidney.
- In the PEComa group of tumours.
Epidemiology
- May be associated with tuberous sclerosis -- 70% have an AML.
- When compared to sporadic cases:
- More often bilateral.
- Usually bigger.
- When compared to sporadic cases:
- There is a suggestion that an epithelioid variant is more worrisome.[4]
- This is not confirmed by all studies.[5]
Microscopic
Features:
- Smooth muscle.
- Adipose tissue - not always present[6] - key feature.
- Abundant blood vessels.
DDx:
- Retroperitoneal sarcoma.
- Renal cell carcinoma with sarcomatoid differentiation.
Images
AML (WC).
AML (WC/KGH).
AML (WC/KGH).
.jpg)
Epithelioid angiomyolipoma
Features:
- Carcinoma-like morphology.
- +/-Spindle cells.
- "High grade" nuclei.
- Pleomorphic nuclei.
DDx:
- Clear cell renal cell carcinoma eosinophilic variant - esp. if epithelioid.
- Xp11.2 translocation carcinoma.
- Renal cell carcinoma with angioleiomyoma-like stroma.[7]
Images:
- Epithelioid AML (birjournals.org).
- Epithelioid AML (rsna.org).
- Atypical epithelioid AML (archivesofpathology.org).[8]
Cytologic
Features[6]
- Nuclei - round/ovoid.
- Chromatin - bland.
IHC
- Melanocytic markers +ve.[9]
- HMB-45 +ve in all cases (15/15).[10]
- Melan A +ve in ~87% of cases (13/15).
- Epithelial markers -ve[9], e.g. EMA and AE1/AE3.
- SMA +ve.
- CD117 +ve/-ve.
- Ki-67:[11]
- Epithelioid variant of AML +ve.
- Conventional AML -ve.
A panel:
- CK (pooled), Desmin, Melan A, HMB-45.
Epithelioid AML versus Xp11.2 translocation carcinoma
- PAX8 -ve.
- Positive in Xp11.2 translocation carcinoma.
- CAIX -ve.
- AE1/AE3 -ve.
- EMA -ve.
Sign out
RIGHT KIDNEY, PARTIAL NEPHRECTOMY: - ANGIOMYOLIPOMA.
Micro
The section show a circumscribed tumour composed of muscle-like tissue and adipose tissue with prominent muscular blood vessels. Rare nuclear enlargement is seen. No signficant nuclear atypia is appreciated. Mitotic activity is not apparent.
Biopsy
MASS OF LEFT KIDNEY, CORE BIOPSY: - COMPATIBLE WITH ANGIOMYOLIPOMA. COMMENT: The tumour stains as follows: POSITIVE: Melan A, HMB-45, desmin. NEGATIVE: CK (pooled).
Micro
The sections show a tumour composed of spindle cells in a fasicular arrangement with scant adipose tissue and prominent muscular blood vessels. Rare nuclear enlargement is seen. No signficant nuclear atypia is appreciated. Mitotic activity is not apparent. No renal parenchyma is identified.
See also
References
- ↑ Seyam, RM.; Bissada, NK.; Kattan, SA.; Mokhtar, AA.; Aslam, M.; Fahmy, WE.; Mourad, WA.; Binmahfouz, AA. et al. (Nov 2008). "Changing trends in presentation, diagnosis and management of renal angiomyolipoma: comparison of sporadic and tuberous sclerosis complex-associated forms.". Urology 72 (5): 1077-82. doi:10.1016/j.urology.2008.07.049. PMID 18805573.
- ↑ Abrams, J.; Yee, DC.; Clark, TW. (Jul 2011). "Transradial embolization of a bleeding renal angiomyolipoma.". Vasc Endovascular Surg 45 (5): 470-3. doi:10.1177/1538574411408352. PMID 21571778.
- ↑ Zhang, SH.; Cong, WM.; Xian, ZH.; Wu, WQ.; Dong, H.; Wu, MC. (Oct 2004). "[Morphologic variants and immunohistochemical features of hepatic angiomyolipoma.]". Zhonghua Bing Li Xue Za Zhi 33 (5): 437-40. PMID 15498214.
- ↑ Nelson, CP.; Sanda, MG. (Oct 2002). "Contemporary diagnosis and management of renal angiomyolipoma.". J Urol 168 (4 Pt 1): 1315-25. doi:10.1097/01.ju.0000028200.86216.b2. PMID 12352384.
- ↑ Aydin, H.; Magi-Galluzzi, C.; Lane, BR.; Sercia, L.; Lopez, JI.; Rini, BI.; Zhou, M. (Feb 2009). "Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association.". Am J Surg Pathol 33 (2): 289-97. doi:10.1097/PAS.0b013e31817ed7a6. PMID 18852677.
- ↑ 6.0 6.1 Crapanzano, JP. (Jan 2005). "Fine-needle aspiration of renal angiomyolipoma: cytological findings and diagnostic pitfalls in a series of five cases.". Diagn Cytopathol 32 (1): 53-7. doi:10.1002/dc.20179. PMID 15584043.
- ↑ Williamson, SR.; Cheng, L.; Eble, JN.; True, LD.; Gupta, NS.; Wang, M.; Zhang, S.; Grignon, DJ. (Sep 2014). "Renal cell carcinoma with angioleiomyoma-like stroma: clinicopathological, immunohistochemical, and molecular features supporting classification as a distinct entity.". Mod Pathol. doi:10.1038/modpathol.2014.105. PMID 25189644.
- ↑ Aljerian, K.; Evans, AJ. (Oct 2004). "Pathologic quiz case: a 44-year-old woman with an incidental asymptomatic renal mass. Atypical epithelioid angiomyolipoma.". Arch Pathol Lab Med 128 (10): 1176-8. doi:10.1043/1543-2165(2004)1281176:PQCAYW2.0.CO;2. PMID 15387699.
- ↑ 9.0 9.1 Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 324. ISBN 978-0443066771.
- ↑ Esheba, Gel S.; Esheba, Nel S. (Sep 2013). "Angiomyolipoma of the kidney: clinicopathological and immunohistochemical study.". J Egypt Natl Canc Inst 25 (3): 125-34. doi:10.1016/j.jnci.2013.05.002. PMID 23932749.
- ↑ Ooi, SM.; Vivian, JB.; Cohen, RJ. (2009). "The use of the Ki-67 marker in the pathological diagnosis of the epithelioid variant of renal angiomyolipoma.". Int Urol Nephrol 41 (3): 559-65. doi:10.1007/s11255-008-9473-1. PMID 18839327.
- ↑ Amin MB, Epstein JI, Ulbright TM, et al. (August 2014). "Best practices recommendations in the application of immunohistochemistry in urologic pathology: report from the international society of urological pathology consensus conference". Am. J. Surg. Pathol. 38 (8): 1017–22. doi:10.1097/PAS.0000000000000254. PMID 25025364.