Kidney tumor
Childhood neoplasms
MiT family translocation carcinoma

Author: Mandolin Ziadie, M.D. (see Reviewers page) (see Authors page)

Revised: 17 January 2017, last major update January 2012

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PubMed Search: MiT family translocation carcinoma kidney

Cite this page: MiT family translocation carcinoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/kidneytumortranslocationchildren.html. Accessed April 24th, 2017.
Definition / General
  • Pediatric renal cell carcinoma with translocations involving TFE3 transcription factor gene (Xp11.2) or TFEB protein (11q12)
  • Also called MiT translocation subgroup carcinomas
Epidemiology
Diagrams / Tables

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Ideogram and karyotype

Clinical Features
Gross Description
  • Tan yellow, frequent hemorrhage and necrosis
Gross Images

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Translocation carcinoma of the kidney

Micro Description
  • Cells with abundant clear or granular, eosinophilic cytoplasm with sharp borders and irregular nuclei with vesicular chromatin and small nucleoli
  • Cells are arranged in septa separated by thin walled vessels in papilla or nests
  • Calcifications are frequent
  • A tubular / nested architecture is more common in t(6;11) tumors
  • Some may show solid / acinar / alveolar / tubular patterns
  • Mitoses are rare / absent
  • Cases with TFE translocations rarely have melanin pigment and morphologically resemble PEComas (Am J Surg Pathol 2009;33:609)
Micro Images

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Various images

t(6;11) tumors

Virtual Slides

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Xp11.2 translocation associated carcinoma

Positive Stains
Negative Stains
Molecular / Cytogenetics Description
  • t(X;17)(p11.2;q25) - balanced translocation of TFE3 gene at Xp11.2 and ASPL gene at 17q25; also found in alveolar soft part sarcoma, but unbalanced
  • t(X;1)(p11.2;q21) - TFE3 and PRCC genes
  • t(6;11)(p21;q12) - TFEB and alpha genes; appear to have low malignant potential in contrast to other translocation carcinomas (Am J Surg Pathol 2005;29:230)
  • Hereditary papillary carcinoma has t(X;1)(p11.2; p34) - fusion of TFE3 and PSF
Electron Microscopy Description
  • Features of clear cell carcinoma, including cell junctions, numerous mitochondria, microvilli, basement membrane and abundant glycogen
Differential Diagnosis