Difference between revisions of "SMARCB1-deficient renal medullary carcinoma"

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*[[Collecting duct carcinoma]].
*[[Collecting duct carcinoma]].


===Image===
===Images===
*[http://www.nature.com/modpathol/journal/v20/n9/fig_tab/3800934f3.html#figure-title RMC (nature.com)].
*[http://www.nature.com/modpathol/journal/v20/n9/fig_tab/3800934f3.html#figure-title RMC (nature.com)].
*[http://www.nature.com/modpathol/journal/v22/n2s/fig_tab/modpathol200970f6.html#figure-title RMC (nature.com)].<ref name=pmid19494850>{{Cite journal  | last1 = Srigley | first1 = JR. | last2 = Delahunt | first2 = B. | title = Uncommon and recently described renal carcinomas. | journal = Mod Pathol | volume = 22 Suppl 2 | issue =  | pages = S2-S23 | month = Jun | year = 2009 | doi = 10.1038/modpathol.2009.70 | PMID = 19494850 }}</ref>
*[http://www.nature.com/modpathol/journal/v22/n2s/fig_tab/modpathol200970f7.html#figure-title RMC with yolk sac-like morphology (nature.com)].<ref name=pmid19494850/>


==IHC==
==IHC==

Revision as of 21:01, 22 August 2014

SMARCB1-deficient renal medullary carcinoma
Diagnosis in short
LM DDx collecting duct carcinoma, yolk sac tumour
IHC INI1 -ve, vimentin +ve, EMA +ve
Gross well-circumscribed mass in renal medulla
Site kidney, medulla - see kidney tumours
Associated Dx sickle cell disease or sickle cell trait
Clinical history usu. young adults
Prevalence rare
Prognosis poor

Renal medullary carcinoma, abbreviated RMC, is a rare malignant kidney tumour associated with sickle cell trait and a poor prognosis.

General

Aside:

Gross

Features:[3]

  • Well circumscribed.
  • Renal medulla.

Microscopic

Features:[1][3]

  • Variable architecture:
  • Desmoplastic stroma - prominent.
  • Inflammation:
    • Lymphocytes.
    • Neutrophils - margination in vessels.

DDx:

Images

IHC

Features:[2]

  • SMARCB1 (INI1) -ve.[6]
  • Vimentin +ve.
  • EMA +ve.
  • CAM5.2 +ve.
  • AE1/AE3 +ve.

See also

References

  1. ↑ 1.0 1.1 1.2 Davis CJ, Mostofi FK, Sesterhenn IA (January 1995). "Renal medullary carcinoma. The seventh sickle cell nephropathy". Am. J. Surg. Pathol. 19 (1): 1–11. PMID 7528470.
  2. ↑ 2.0 2.1 Cheng JX, Tretiakova M, Gong C, Mandal S, Krausz T, Taxy JB (June 2008). "Renal medullary carcinoma: rhabdoid features and the absence of INI1 expression as markers of aggressive behavior". Mod. Pathol. 21 (6): 647–52. doi:10.1038/modpathol.2008.44. PMID 18327209.
  3. ↑ 3.0 3.1 3.2 Watanabe, IC.; Billis, A.; Guimarães, MS.; Alvarenga, M.; de Matos, AC.; Cardinalli, IA.; Filippi, RZ.; de Castro, MG. et al. (Sep 2007). "Renal medullary carcinoma: report of seven cases from Brazil.". Mod Pathol 20 (9): 914-20. doi:10.1038/modpathol.3800934. PMID 17643096. http://www.nature.com/modpathol/journal/v20/n9/full/3800934a.html. Cite error: Invalid <ref> tag; name "pmid17643096" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid17643096" defined multiple times with different content
  4. ↑ Calderaro J, Moroch J, Pierron G, et al. (September 2012). "SMARCB1/INI1 inactivation in renal medullary carcinoma". Histopathology 61 (3): 428–35. doi:10.1111/j.1365-2559.2012.04228.x. PMID 22686875.
  5. ↑ 5.0 5.1 Srigley, JR.; Delahunt, B. (Jun 2009). "Uncommon and recently described renal carcinomas.". Mod Pathol 22 Suppl 2: S2-S23. doi:10.1038/modpathol.2009.70. PMID 19494850.
  6. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 286. ISBN 978-0781765275.
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