Renal angiomyoadenomatous tumour

From Libre Pathology
Jump to: navigation, search
Renal angiomyoadenomatous tumour
Diagnosis in short

Renal cell carcinoma with clear cells and leiomyomatous bands. H&E stain.

Synonyms renal cell carcinoma with angioleiomyoma-like stroma, renal cell carcinoma associated with prominent angioleiomyoma-like proliferation

LM leiomyomatous capsule of variable thickness, tubular structures with (1) apical snouts, and (2) clear cells with basal nuclei and mild nuclear atypia
LM DDx clear cell renal cell carcinoma, papillary renal cell carcinoma, (epithelioid) angiomyolipoma, mixed epithelial and stromal tumour, tuberous sclerosis-associated renal cell carcinoma
IHC CK7 +ve, CK20 +ve, CAM5.2 +ve
Gross tan-brown mass +/-cystic component
Grossing notes partial nephrectomy grossing, total nephrectomy for tumour grossing
Site kidney - see kidney tumours

Associated Dx possibly related to clear cell papillary renal cell carcinoma - the WHO lumps this entity with it
Prevalence extremely rare
Prognosis good

Renal angiomyoadenomatous tumour, abbreviated RAT, is an uncommon kidney tumour not recognized by the WHO classification of renal neoplasia.

The Vancouver modification of the WHO classification[1] lumps it with clear cell papillary renal cell carcinoma (also known as clear cell tubulopapillary renal cell carcinoma).

Synonyms are renal cell carcinoma with angioleiomyoma-like stroma and renal cell carcinoma associated with prominent angioleiomyoma-like proliferation.[2]

It may be one and the same with TCEB1-mutated renal cell carcinoma.

General

Clincal:

  • Typical age (years): 50s.[4]
    • Age range: 40-93 years.

Gross

Features:[4]

  • Tan-brown renal mass +/-cystic component.

Microscopic

Features:[4][5]

  • Leiomyomatous capsule of variable thickness - key feature.
  • Tubular structures with:

DDx:[4][6]

Images

IHC

Features:[5]

  • CK7 +ve.
  • CK20 +ve.
  • CAM5.2 +ve.
  • AE1/AE3 +ve.

Molecular

See also

References

  1. 1.0 1.1 Srigley, JR.; Delahunt, B.; Eble, JN.; Egevad, L.; Epstein, JI.; Grignon, D.; Hes, O.; Moch, H. et al. (Oct 2013). "The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia.". Am J Surg Pathol 37 (10): 1469-89. doi:10.1097/PAS.0b013e318299f2d1. PMID 24025519.
  2. Williamson, SR.; Cheng, L.; Eble, JN.; True, LD.; Gupta, NS.; Wang, M.; Zhang, S.; Grignon, DJ. (Feb 2015). "Renal cell carcinoma with angioleiomyoma-like stroma: clinicopathological, immunohistochemical, and molecular features supporting classification as a distinct entity.". Mod Pathol 28 (2): 279-94. doi:10.1038/modpathol.2014.105. PMID 25189644.
  3. Petersson, F.; Grossmann, P.; Hora, M.; Sperga, M.; Montiel, DP.; Martinek, P.; Gutierrez, ME.; Bulimbasic, S. et al. (Jul 2013). "Renal cell carcinoma with areas mimicking renal angiomyoadenomatous tumor/clear cell papillary renal cell carcinoma.". Hum Pathol 44 (7): 1412-20. doi:10.1016/j.humpath.2012.11.019. PMID 23434146.
  4. 4.0 4.1 4.2 4.3 Singh, C.; Kendi, AT.; Manivel, JC.; Pambuccian, SE. (Dec 2012). "Renal angiomyoadenomatous tumor.". Ann Diagn Pathol 16 (6): 470-6. doi:10.1016/j.anndiagpath.2012.01.006. PMID 22534244.
  5. 5.0 5.1 Michal, M.; Hes, O.; Nemcova, J.; Sima, R.; Kuroda, N.; Bulimbasic, S.; Franco, M.; Sakaida, N. et al. (Jan 2009). "Renal angiomyoadenomatous tumor: morphologic, immunohistochemical, and molecular genetic study of a distinct entity.". Virchows Arch 454 (1): 89-99. doi:10.1007/s00428-008-0697-3. PMID 19020896.
  6. Petersson, F.; Branzovsky, J.; Martinek, P.; Korabecna, M.; Kruslin, B.; Hora, M.; Peckova, K.; Bauleth, K. et al. (Jul 2014). "The leiomyomatous stroma in renal cell carcinomas is polyclonal and not part of the neoplastic process.". Virchows Arch 465 (1): 89-96. doi:10.1007/s00428-014-1591-9. PMID 24838683.
  7. Guo, J.; Tretiakova, MS.; Troxell, ML.; Osunkoya, AO.; Fadare, O.; Sangoi, AR.; Shen, SS.; Lopez-Beltran, A. et al. (Nov 2014). "Tuberous Sclerosis-associated Renal Cell Carcinoma: A Clinicopathologic Study of 57 Separate Carcinomas in 18 Patients.". Am J Surg Pathol 38 (11): 1457-67. doi:10.1097/PAS.0000000000000248. PMID 25093518.