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Kidney medullary carcinoma

MedGen UID:
888108
•Concept ID:
C4049328
•
Neoplastic Process
Synonyms: carcinoma of renal medulla; Kidney Medullary Carcinoma; kidney medullary carcinoma; renal medulla carcinoma; Renal Medullary Carcinoma; Renal medullary carcinoma; renal medullary carcinoma
SNOMED CT: Renal medullary carcinoma (765094003); Renal medullary carcinoma (765095002)
 
Monarch Initiative: MONDO:0006260
Orphanet: ORPHA319319

Definition

Renal medullary carcinoma is a rare, aggressive subtype of renal cell carcinoma characterized by a large, white or tan, firm, infiltrative tumor with microabscess-like foci centered in the renal medulla, typically presenting with hematuria, abdominal/flank pain, weight loss and fever. It is associated with sickle cell trait and disease and metastasis to the bones and lungs is common at time of diagnosis. [from ORDO]

Professional guidelines

PubMed

New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
The Differential Diagnosis of Medullary-Based Renal Masses.
Baniak N, Tsai H, Hirsch MS
Arch Pathol Lab Med 2021 Sep 1;145(9):1148-1170. doi: 10.5858/arpa.2020-0464-RA. PMID: 33406251
Updates on the Management of Thyroid Cancer.
Araque KA, Gubbi S, Klubo-Gwiezdzinska J
Horm Metab Res 2020 Aug;52(8):562-577. Epub 2020 Feb 10 doi: 10.1055/a-1089-7870. PMID: 32040962Free PMC Article

Recent clinical studies

Etiology

Obesity and renal cell carcinoma: Biological mechanisms and perspectives.
Venkatesh N, Martini A, McQuade JL, Msaouel P, Hahn AW
Semin Cancer Biol 2023 Sep;94:21-33. Epub 2023 Jun 5 doi: 10.1016/j.semcancer.2023.06.001. PMID: 37286114Free PMC Article
Renal Cell Carcinoma of Variant Histology: Biology and Therapies.
Msaouel P, Genovese G, Tannir NM
Hematol Oncol Clin North Am 2023 Oct;37(5):977-992. Epub 2023 May 25 doi: 10.1016/j.hoc.2023.04.019. PMID: 37244822
The 2022 World Health Organization Classification of Tumours of the Urinary System and Male Genital Organs-Part A: Renal, Penile, and Testicular Tumours.
Moch H, Amin MB, Berney DM, Compérat EM, Gill AJ, Hartmann A, Menon S, Raspollini MR, Rubin MA, Srigley JR, Hoon Tan P, Tickoo SK, Tsuzuki T, Turajlic S, Cree I, Netto GJ
Eur Urol 2022 Nov;82(5):458-468. Epub 2022 Jul 16 doi: 10.1016/j.eururo.2022.06.016. PMID: 35853783
Safety and efficacy of nivolumab plus ipilimumab in patients with advanced non-clear cell renal cell carcinoma: results from the phase 3b/4 CheckMate 920 trial.
Tykodi SS, Gordan LN, Alter RS, Arrowsmith E, Harrison MR, Percent I, Singal R, Van Veldhuizen P, George DJ, Hutson T, Zhang J, Zoco J, Johansen JL, Rezazadeh Kalebasty A
J Immunother Cancer 2022 Feb;10(2) doi: 10.1136/jitc-2021-003844. PMID: 35210307Free PMC Article
Updates on the Management of Thyroid Cancer.
Araque KA, Gubbi S, Klubo-Gwiezdzinska J
Horm Metab Res 2020 Aug;52(8):562-577. Epub 2020 Feb 10 doi: 10.1055/a-1089-7870. PMID: 32040962Free PMC Article

Diagnosis

Obesity and renal cell carcinoma: Biological mechanisms and perspectives.
Venkatesh N, Martini A, McQuade JL, Msaouel P, Hahn AW
Semin Cancer Biol 2023 Sep;94:21-33. Epub 2023 Jun 5 doi: 10.1016/j.semcancer.2023.06.001. PMID: 37286114Free PMC Article
Renal Cell Carcinoma of Variant Histology: Biology and Therapies.
Msaouel P, Genovese G, Tannir NM
Hematol Oncol Clin North Am 2023 Oct;37(5):977-992. Epub 2023 May 25 doi: 10.1016/j.hoc.2023.04.019. PMID: 37244822
The 2022 World Health Organization Classification of Tumours of the Urinary System and Male Genital Organs-Part A: Renal, Penile, and Testicular Tumours.
Moch H, Amin MB, Berney DM, Compérat EM, Gill AJ, Hartmann A, Menon S, Raspollini MR, Rubin MA, Srigley JR, Hoon Tan P, Tickoo SK, Tsuzuki T, Turajlic S, Cree I, Netto GJ
Eur Urol 2022 Nov;82(5):458-468. Epub 2022 Jul 16 doi: 10.1016/j.eururo.2022.06.016. PMID: 35853783
New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
Updates on the Management of Thyroid Cancer.
Araque KA, Gubbi S, Klubo-Gwiezdzinska J
Horm Metab Res 2020 Aug;52(8):562-577. Epub 2020 Feb 10 doi: 10.1055/a-1089-7870. PMID: 32040962Free PMC Article

Therapy

Research Progress on Mechanism and Management of Adverse Drug Reactions of Anlotinib.
Li S, Wang H
Drug Des Devel Ther 2023;17:3429-3437. Epub 2023 Nov 15 doi: 10.2147/DDDT.S426898. PMID: 38024530Free PMC Article
Peptide Receptor Radionuclide Therapy in Thyroid Cancer.
Gubbi S, Koch CA, Klubo-Gwiezdzinska J
Front Endocrinol (Lausanne) 2022;13:896287. Epub 2022 May 30 doi: 10.3389/fendo.2022.896287. PMID: 35712243Free PMC Article
Safety and efficacy of nivolumab plus ipilimumab in patients with advanced non-clear cell renal cell carcinoma: results from the phase 3b/4 CheckMate 920 trial.
Tykodi SS, Gordan LN, Alter RS, Arrowsmith E, Harrison MR, Percent I, Singal R, Van Veldhuizen P, George DJ, Hutson T, Zhang J, Zoco J, Johansen JL, Rezazadeh Kalebasty A
J Immunother Cancer 2022 Feb;10(2) doi: 10.1136/jitc-2021-003844. PMID: 35210307Free PMC Article
Lenvatinib in Management of Solid Tumors.
Hao Z, Wang P
Oncologist 2020 Feb;25(2):e302-e310. Epub 2019 Oct 14 doi: 10.1634/theoncologist.2019-0407. PMID: 32043789Free PMC Article
Updates on the Management of Thyroid Cancer.
Araque KA, Gubbi S, Klubo-Gwiezdzinska J
Horm Metab Res 2020 Aug;52(8):562-577. Epub 2020 Feb 10 doi: 10.1055/a-1089-7870. PMID: 32040962Free PMC Article

Prognosis

Renal Medullary Carcinoma: A Surveillance, Epidemiology, and End Results (SEER) Analysis.
Haupt T, Akinyemi O, Raju RA, Wadley AE, Nnorom S, Aponte V, Thelus J, Tonkin JB, Coleman PW, Metwalli AR
J Surg Res 2023 Dec;292:1-6. Epub 2023 Aug 9 doi: 10.1016/j.jss.2023.06.019. PMID: 37567029
SWI/SNF-deficient neoplasms of the genitourinary tract.
Sirohi D MD, Ohe C, Smith SC, Amin MB
Semin Diagn Pathol 2021 May;38(3):212-221. Epub 2021 Apr 3 doi: 10.1053/j.semdp.2021.03.007. PMID: 33840529
New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427
Comprehensive Molecular Characterization Identifies Distinct Genomic and Immune Hallmarks of Renal Medullary Carcinoma.
Msaouel P, Malouf GG, Su X, Yao H, Tripathi DN, Soeung M, Gao J, Rao P, Coarfa C, Creighton CJ, Bertocchio JP, Kunnimalaiyaan S, Multani AS, Blando J, He R, Shapiro DD, Perelli L, Srinivasan S, Carbone F, Pilié PG, Karki M, Seervai RNH, Vokshi BH, Lopez-Terrada D, Cheng EH, Tang X, Lu W, Wistuba II, Thompson TC, Davidson I, Giuliani V, Schlacher K, Carugo A, Heffernan TP, Sharma P, Karam JA, Wood CG, Walker CL, Genovese G, Tannir NM
Cancer Cell 2020 May 11;37(5):720-734.e13. Epub 2020 Apr 30 doi: 10.1016/j.ccell.2020.04.002. PMID: 32359397Free PMC Article
Updates on the Management of Thyroid Cancer.
Araque KA, Gubbi S, Klubo-Gwiezdzinska J
Horm Metab Res 2020 Aug;52(8):562-577. Epub 2020 Feb 10 doi: 10.1055/a-1089-7870. PMID: 32040962Free PMC Article

Clinical prediction guides

Renal Medullary Carcinoma: A Surveillance, Epidemiology, and End Results (SEER) Analysis.
Haupt T, Akinyemi O, Raju RA, Wadley AE, Nnorom S, Aponte V, Thelus J, Tonkin JB, Coleman PW, Metwalli AR
J Surg Res 2023 Dec;292:1-6. Epub 2023 Aug 9 doi: 10.1016/j.jss.2023.06.019. PMID: 37567029
Obesity and renal cell carcinoma: Biological mechanisms and perspectives.
Venkatesh N, Martini A, McQuade JL, Msaouel P, Hahn AW
Semin Cancer Biol 2023 Sep;94:21-33. Epub 2023 Jun 5 doi: 10.1016/j.semcancer.2023.06.001. PMID: 37286114Free PMC Article
SMARCB1 regulates the hypoxic stress response in sickle cell trait.
Soeung M, Perelli L, Chen Z, Dondossola E, Ho IL, Carbone F, Zhang L, Khan H, Le CN, Zhu C, Peoples MD, Feng N, Jiang S, Zacharias NM, Minelli R, Shapiro DD, Deem AK, Gao S, Cheng EH, Lucchetti D, Walker CL, Carugo A, Giuliani V, Heffernan TP, Viale A, Tannir NM, Draetta GF, Msaouel P, Genovese G
Proc Natl Acad Sci U S A 2023 May 23;120(21):e2209639120. Epub 2023 May 15 doi: 10.1073/pnas.2209639120. PMID: 37186844Free PMC Article
SWI/SNF-deficient neoplasms of the genitourinary tract.
Sirohi D MD, Ohe C, Smith SC, Amin MB
Semin Diagn Pathol 2021 May;38(3):212-221. Epub 2021 Apr 3 doi: 10.1053/j.semdp.2021.03.007. PMID: 33840529
New developments in existing WHO entities and evolving molecular concepts: The Genitourinary Pathology Society (GUPS) update on renal neoplasia.
Trpkov K, Hes O, Williamson SR, Adeniran AJ, Agaimy A, Alaghehbandan R, Amin MB, Argani P, Chen YB, Cheng L, Epstein JI, Cheville JC, Comperat E, da Cunha IW, Gordetsky JB, Gupta S, He H, Hirsch MS, Humphrey PA, Kapur P, Kojima F, Lopez JI, Maclean F, Magi-Galluzzi C, McKenney JK, Mehra R, Menon S, Netto GJ, Przybycin CG, Rao P, Rao Q, Reuter VE, Saleeb RM, Shah RB, Smith SC, Tickoo S, Tretiakova MS, True L, Verkarre V, Wobker SE, Zhou M, Gill AJ
Mod Pathol 2021 Jul;34(7):1392-1424. Epub 2021 Mar 4 doi: 10.1038/s41379-021-00779-w. PMID: 33664427

Recent systematic reviews

Renal medullary carcinoma and sickle cell trait: A systematic review.
Alvarez O, Rodriguez MM, Jordan L, Sarnaik S
Pediatr Blood Cancer 2015 Oct;62(10):1694-9. Epub 2015 Jun 5 doi: 10.1002/pbc.25592. PMID: 26053587
Understanding pathologic variants of renal cell carcinoma: distilling therapeutic opportunities from biologic complexity.
Shuch B, Amin A, Armstrong AJ, Eble JN, Ficarra V, Lopez-Beltran A, Martignoni G, Rini BI, Kutikov A
Eur Urol 2015 Jan;67(1):85-97. Epub 2014 May 21 doi: 10.1016/j.eururo.2014.04.029. PMID: 24857407

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