TFE3-rearranged renal cell carcinoma

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Renal tumour with Xp11.2 translocation, also Xp11.2 translocation carcinoma, is an uncommon malignant kidney tumour.

TFE3-rearranged renal cell carcinoma
Diagnosis in short

Xp11.2 translocation carcinoma. H&E stain.
LM large cells with clear or eosinophilic cytoplasm, calcification (classic histomorphologic feature), +/-papillae, +/-nests, +/-psammoma bodies (common), +/-hyaline bodies (common)
LM DDx clear cell renal cell carcinoma, papillary renal cell carcinoma, epithelioid angiomyolipoma, clear cell papillary renal cell carcinoma
IHC TFE3 +ve (nucleus), CD10 +ve, vimentin +ve, CK7 -ve (usu.)
Molecular translocation involving TFE3, e.g. t(X;1)(p11.2;q21)
Site kidney - see kidney tumours
Prevalence rare
Prognosis poor
Clin. DDx other kidney tumours

General

  • Defined by the presence of a fusion gene formed with TFE3 @ Xp11.2.
  • TFE3 is the gene involved in the translocation seen in alveolar soft part sarcoma (ASPS).
  • Poor prognosis ~ 50% present at stage IV, majority of lymph node metastases.
  • ~1/3 of childhood RCC.[1]

Microscopic

Features:[2]

  • Large cells.
  • Clear or eosinophilic cytoplasm.
  • Papillae or nests.
  • Psammoma bodies - common.[3]
    • Calcification is considered the classic histomorphologic feature.
  • Hyaline bodies - common.

Notes:

DDx:

Images

  • Xp11.2 translocation RCC - intermed. mag. (WC/Nephron)

  • Xp11.2 translocation RCC - high mag. (WC/Nephron)

  • Xp11.2 translocation RCC - very high mag. (WC/Nephron)

www:

IHC

  • TFE3 +ve (nucleus) - key feature.[2]
  • CD10 +ve.
  • Vimentin +ve.
  • CK7 -ve.
    • Positive in ~20% of cases.[4]

Others:

Molecular

See also

References

  1. ↑ Argani, P.; Olgac, S.; Tickoo, SK.; Goldfischer, M.; Moch, H.; Chan, DY.; Eble, JN.; Bonsib, SM. et al. (Aug 2007). "Xp11 translocation renal cell carcinoma in adults: expanded clinical, pathologic, and genetic spectrum.". Am J Surg Pathol 31 (8): 1149-60. doi:10.1097/PAS.0b013e318031ffff. PMID 17667536.
  2. ↑ 2.0 2.1 2.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 285. ISBN 978-0781765275.
  3. ↑ Prasad, SR.; Humphrey, PA.; Catena, JR.; Narra, VR.; Srigley, JR.; Cortez, AD.; Dalrymple, NC.; Chintapalli, KN.. "Common and uncommon histologic subtypes of renal cell carcinoma: imaging spectrum with pathologic correlation.". Radiographics 26 (6): 1795-806; discussion 1806-10. doi:10.1148/rg.266065010. PMID 17102051.
  4. ↑ He, H.; Zhou, GX.; Zhou, M.; Chen, L. (Sep 2011). "The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray.". Int J Gynecol Pathol 30 (5): 425-30. doi:10.1097/PGP.0b013e318214dd4f. PMID 21804394.
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