TFEB-rearranged renal cell carcinoma

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Renal tumour with t(6;11) translocation, also t(6;11) renal cell carcinoma, is a rare kidney tumour seen primarily in children.

It also known as TFEB RCC.[1]

General

  • Very rare - approximately 2 dozen reported cases as of 2014.[1][2]
  • Lymph node metastases are common.
  • Traditionally thought of as a pediatric tumour... but cases in adults are reported.[1][3]

Microscopic

Features:

  • Hyaline material between nests.
  • Large cells with clear to eosinophilic cytoplasm.

Images

IHC

  • TFEB +ve (nuclear staining).
  • HMB-45 +ve[1] (focal[5]).

Molecular

  • t(6;11)(p21;q12) MALAT1[6]/TFEB.[7]

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 Hora, M.; Urge, T.; Trávníček, I.; Ferda, J.; Chudáček, Z.; Vaněček, T.; Michal, M.; Petersson, F. et al. (2014). "MiT translocation renal cell carcinomas: two subgroups of tumours with translocations involving 6p21 [t (6; 11)] and Xp11.2 [t (X;1 or X or 17)].". Springerplus 3: 245. doi:10.1186/2193-1801-3-245. PMID 24877033.
  2. ↑ Argani, P.; Yonescu, R.; Morsberger, L.; Morris, K.; Netto, GJ.; Smith, N.; Gonzalez, N.; Illei, PB. et al. (Oct 2012). "Molecular confirmation of t(6;11)(p21;q12) renal cell carcinoma in archival paraffin-embedded material using a break-apart TFEB FISH assay expands its clinicopathologic spectrum.". Am J Surg Pathol 36 (10): 1516-26. doi:10.1097/PAS.0b013e3182613d8f. PMID 22892601.
  3. ↑ Ishihara, A.; Yamashita, Y.; Takamori, H.; Kuroda, N. (Sep 2011). "Renal carcinoma with (6;11)(p21;q12) translocation: Report of an adult case.". Pathol Int 61 (9): 539-45. doi:10.1111/j.1440-1827.2011.02711.x. PMID 21884304.
  4. ↑ URL: http://atlasgeneticsoncology.org/Tumors/RenalCellt0611ID5011.html. Accessed on: December 29, 2014.
  5. ↑ 5.0 5.1 Davis, IJ.; Hsi, BL.; Arroyo, JD.; Vargas, SO.; Yeh, YA.; Motyckova, G.; Valencia, P.; Perez-Atayde, AR. et al. (May 2003). "Cloning of an Alpha-TFEB fusion in renal tumors harboring the t(6;11)(p21;q13) chromosome translocation.". Proc Natl Acad Sci U S A 100 (10): 6051-6. doi:10.1073/pnas.0931430100. PMID 12719541.
  6. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 607924
  7. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 281. ISBN 978-0781765275.
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