Multilocular cystic renal neoplasm of low malignant potential

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Multilocular cystic renal neoplasm of low malignant potential
Diagnosis in short
Synonyms multilocular clear cell renal cell carcinoma, multilocular cystic renal cell neoplasm of low malignant potential (proposed name)
LM thin septa with (polygonal) cells with a clear cytoplasm, fibrous capsule (surrounds tumour), +/-calcification
LM DDx cystic renal disease with macrophages in the septa, cystic clear cell renal cell carcinoma, tubulocystic carcinoma
Gross completely cystic - no solid components, well-circumscribed
Site kidney - see kidney tumours
Prevalence rare (case reports)
Prognosis very good
Clin. DDx other cystic renal tumours (e.g. cystic clear cell renal cell carcinoma), cystic renal disease

Multilocular cystic renal cell carcinoma, abbreviated MCRCC, is a rare kidney tumour.

It is also known as multilocular clear cell renal cell carcinoma.[1]

As the behaviour of the tumour is very good, it has be proposed that this entity be renamed multilocular cystic renal cell neoplasm of low malignant potential.[2]

Multicystic renal cell neoplasm of low malignant potential[3] redirects here.

General

Gross

Features:[4]

  • Completely cystic with thin septa.
    • No solid nodules - required for diagnosis.[7]
  • Well circumscribed.

Note:

  • This tumour, radiologically, can often be separated from other cystic tumours.[8]

Microscopic

Features:[4][7]

  • Thin septa with (polygonal) cells with a clear cytoplasm - required.
  • Fibrous capsule (surrounds tumour) - required.
  • +/-Calcification (common).

DDx:

Images

www:

IHC

  • EMA +ve.
  • Keratins +ve.
  • CD68 -ve.

Note:

See also

References

  1. ↑ 1.0 1.1 Chowdhury, AR.; Chakraborty, D.; Bhattacharya, P.; Dey, RK. (Apr 2013). "Multilocular cystic renal cell carcinoma a diagnostic dilemma: A case report in a 30-year-old woman.". Urol Ann 5 (2): 119-21. doi:10.4103/0974-7796.110012. PMID 23798872.
  2. ↑ Montironi, R.; Mazzucchelli, R.; Scarpelli, M.; Lopez-Beltran, A.; Cheng, L. (Dec 2013). "Update on selected renal cell tumors with clear cell features. With emphasis on multilocular cystic clear cell renal cell carcinoma.". Histol Histopathol 28 (12): 1555-66. PMID 23893851.
  3. ↑ Epstein, Jonathan I.; Netto, George J. (2014). Differential Diagnoses in Surgical Pathology: Genitourinary System (1st ed.). Wolters Kluwer. pp. 200. ISBN 978-1451189582.
  4. ↑ 4.0 4.1 4.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 292. ISBN 978-0781765275.
  5. ↑ Agarwal, S.; Agrawal, U.; Mohanty, NK.; Saxena, S. (Mar 2011). "Multilocular cystic renal cell carcinoma: a case report of a rare entity.". Arch Pathol Lab Med 135 (3): 290-2. doi:10.1043/2010-0243-LE.1. PMID 21366448.
  6. ↑ Halat, S.; Eble, JN.; Grignon, DJ.; Lopez-Beltran, A.; Montironi, R.; Tan, PH.; Wang, M.; Zhang, S. et al. (Jul 2010). "Multilocular cystic renal cell carcinoma is a subtype of clear cell renal cell carcinoma.". Mod Pathol 23 (7): 931-6. doi:10.1038/modpathol.2010.78. PMID 20348877.
  7. ↑ 7.0 7.1 Eble, JN.; Bonsib, SM. (Feb 1998). "Extensively cystic renal neoplasms: cystic nephroma, cystic partially differentiated nephroblastoma, multilocular cystic renal cell carcinoma, and cystic hamartoma of renal pelvis.". Semin Diagn Pathol 15 (1): 2-20. PMID 9503503.
  8. ↑ You, D.; Shim, M.; Jeong, IG.; Song, C.; Kim, JK.; Ro, JY.; Hong, JH.; Ahn, H. et al. (Jul 2011). "Multilocular cystic renal cell carcinoma: clinicopathological features and preoperative prediction using multiphase computed tomography.". BJU Int. doi:10.1111/j.1464-410X.2011.10247.x. PMID 21722289.
  9. ↑ Epstein, Jonathan I.; Netto, George J. (2014). Differential Diagnoses in Surgical Pathology: Genitourinary System (1st ed.). Wolters Kluwer. pp. 197. ISBN 978-1451189582.
  10. ↑ 10.0 10.1 Tantisattamo, E.; Elam, A.; Cobb, J.; Ellis, CL.; Bailey, JL. (2014). "No tumor-free waiting period after treatment of multilocular cystic renal cell carcinoma: a new case and review of the literature.". Am J Nephrol 40 (2): 151-6. doi:10.1159/000365201. PMID 25171490.
  11. ↑ Williamson, SR.; Halat, S.; Eble, JN.; Grignon, DJ.; Lopez-Beltran, A.; Montironi, R.; Tan, PH.; Wang, M. et al. (Oct 2012). "Multilocular cystic renal cell carcinoma: similarities and differences in immunoprofile compared with clear cell renal cell carcinoma.". Am J Surg Pathol 36 (10): 1425-33. doi:10.1097/PAS.0b013e31825b37f0. PMID 22982885.
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