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Kidney: Metanephric adenoma

Written2012-09Chin-Chen Pan
Department of Pathology, Taipei Veterans General Hospital, Taipei, Taiwan
Updated2016-11Kelsey McIntyre, Michelle S. Hirsch
Department of Pathology, Brigham and Women's Hospital, Boston, MA. pdalcin@partners.org

(Note : for Links provided by Atlas : click)

Abstract

Abstract Review on Kidney metanephric adenoma a, with data on clinics, and the genes involved.

Identity

ICD-Topo C649 KIDNEY
ICD-Morpho 8325/0 Metanephric adenoma
Atlas_Id 5210
Phylum Urinary system: Kidney::Metanephric adenoma
Other namesEmbryonal adenoma
Nephrogenic nephroma
Note A benign renal epithelial tumor resembling differentiation toward early embryonic metanephric tubules.
Figure 1A Metanephric adenomas are typical, small, well circumscribed with a fleshy tan cut surface (Kelsey McIntyre and Michelle S. Hirsch).

Classification

Note Metanephric adenoma belongs to the category of Metanephric tumor in the WHO histological classification of tumors of the kidney.

Clinics and Pathology

Etiology Although previously suggested to be derived from persistent blastema, metanephric adenoma is currently thought to develop from maturation of nephroblastoma.
Epidemiology Metanephric adenomas are rare (<1% of renal epithelial neoplasms) but occur across a wide range of patients, from infants to older individuals.. The peak age frequency is 40 to 69 years. There is a female predominance (2.3:1). Ten percent of patients present with polycythemia.
Clinics Mostly metanephric adenomas are incidentally discovered. Some patients present with hematuria and abdominal or flank pain.
Pathology Grossly, metanephric adenomas demonstrate a tan to fleshy white homogeneous cut surface (Fig. 1A) (Grignon et al., 2016). Most tumors are well-demarcated but lack a true capsule and are typically unilateral. Microscopically, the tumor is highly cellular and chiefly composed of tightly packed primitive tubules; papillary or glomeruloid structures may also be seen. The individual tumor cells are small, bland and cuboidal with scant cytoplasm, and round-to-oval nuclei with smooth chromatin, and inconspicuous nucleoli (Fig. 1B). Stromal hyalinization, dystrophic calcification and psammoma bodies are occasionally seen. Metanephric adenomas may resemble nephroblastoma or papillary renal cell carcinoma microscopically. Mitotic figures are rare or absent; necrosis is not seen. Tumor cells are immunoreactive for WT1, PAX8 and BRAF, and are typically negative for CK7 and AMACR.
 
Figure 1B Histologically, the tumor cells are small, monomorphic and cuboidal. Architecturally, the tumors can demonstrate tubular (predominant), papillary and glomeruloid growth patterns (courtesy Chin-Chen Pan).
Treatment Surgical excision.
Prognosis The vast majority of metanephric adenomas behave in a benign fashion. Cases of regional lymph node metastasis and sarcomatoid changes have been individually reported.

Cytogenetics

Note No consistent cytogenetic abnormalities have been identified in metanephric adenoma. The gains of chromosomes 7 and 17 noted in papillary renal cell carcinoma, as well as the chromosome gains of 1q, 7q, and 12, and losses of 11p and 16q commonly seen in Wilms' tumor have not been detected in metanephric adenoma.

Genes involved and Proteins

Note Approximately 90% of metanephric adenomas contain a V600E activating mutation in exon 15 of BRAF (Choueiri et al., 2012). BRAF-mutated metanephric adenomas stain positive for V600E mutation-specific immunohistochemistry (Pinto et al., 2015; Udager et al., 2015). BRAF mutations are very rare (<1%) or absent in other renal tumor subtypes.
Gene NameBRAF (v-raf murine sarcoma viral oncogene homolog B1)
Location 7q34
Note BRAF V600E mutations are found in approximately 90% of metanephric adenomas.

Bibliography

World Health Organization Classification of.Tumours of the Urinary Systems and Male Genital Organs
Moch, H., Humphrey, P.A., Ulbright, T.M., Reuter, V.E. eds (4th edition); IARC Press, Lyon 2016; 11-76.
 
Metanephric adenoma lacks the gains of chromosomes 7 and 17 and loss of Y that are typical of papillary renal cell carcinoma and papillary adenoma.
Brunelli M, Eble JN, Zhang S, Martignoni G, Cheng L.
Mod Pathol. 2003 Oct;16(10):1060-3.
PMID 14559991
 
Metanephric adenoma of the kidney: a clinicopathological and molecular study of two cases.
Burger M, Junker K, Denzinger S, Schubert T, Stoehr R, Wieland WF, Hartmann A.
J Clin Pathol. 2007 Jul;60(7):832-3. Epub 2006 Nov 10.
PMID 17098885
 
BRAF mutations in metanephric adenoma of the kidney
Choueiri TK, Cheville J, Palescandolo E, Fay AP, Kantoff PW, Atkins MB, McKenney JK, Brown V, Lampron ME, Zhou M, Hirsch MS, Signoretti S
Eur Urol 2012 Nov;62(5):917-22
PMID 22727996
 
Metanephric adenoma: histology, immunophenotype, cytogenetics, ultrastructure.
Gatalica Z, Grujic S, Kovatich A, Petersen RO.
Mod Pathol. 1996 Mar;9(3):329-33.
PMID 8685236
 
Cytologic and cytogenetic analysis of metanephric adenoma of the kidney: a report of two cases.
Granter SR, Fletcher JA, Renshaw AA.
Am J Clin Pathol. 1997 Nov;108(5):544-9.
PMID 9353093
 
Metanephric adenoma of the kidney. A clinicopathological, immunohistochemical, flow cytometric, cytogenetic, and electron microscopic study of seven cases.
Jones EC, Pins M, Dickersin GR, Young RH.
Am J Surg Pathol. 1995 Jun;19(6):615-26.
PMID 7755148
 
Metanephric adenoma, nephrogenic rests, and Wilms' tumor: a histologic and immunophenotypic comparison.
Muir TE, Cheville JC, Lager DJ.
Am J Surg Pathol. 2001 Oct;25(10):1290-6.
PMID 11688464
 
Alpha-methylacyl-CoA racemase as a marker in the differential diagnosis of metanephric adenoma.
Olgac S, Hutchinson B, Tickoo SK, Reuter VE.
Mod Pathol. 2006 Feb;19(2):218-24.
PMID 16424894
 
Detection of chromosome copy number alterations in metanephric adenomas by array comparative genomic hybridization
Pan CC, Epstein JI
Mod Pathol 2010 Dec;23(12):1634-40
PMID 20802469
 
Metanephric adenosarcoma in a young adult: morphologic, immunophenotypic, ultrastructural, and fluorescence in situ hybridization analyses: a case report and review of the literature.
Picken MM, Curry JL, Lindgren V, Clark JI, Eble JN.
Am J Surg Pathol. 2001 Nov;25(11):1451-7.
PMID 11684965
 
Immunohistochemical staining for BRAF V600E supports the diagnosis of metanephric adenoma
Pinto A, Signoretti S, Hirsch MS, Barletta JA
Histopathology 2015 May;66(6):901-4
PMID 25130952
 
Metastatic metanephric adenoma in a child.
Renshaw AA, Freyer DR, Hammers YA.
Am J Surg Pathol. 2000 Apr;24(4):570-4.
PMID 10757405
 
High-resolution array CGH of metanephric adenomas: lack of DNA copy number changes.
Szponar A, Yusenko MV, Kovacs G.
Histopathology. 2010 Jan;56(2):212-6.
PMID 20102400
 
Molecular and immunohistochemical characterization reveals novel BRAF mutations in metanephric adenoma
Udager AM, Pan J, Magers MJ, Palapattu GS, Morgan TM, Montgomery JS, Weizer AZ, Hafez KS, Miller DC, Wolf JS Jr, McHugh JB, Chinnaiyan AM, Dhanasekaran SM, Mehra R
Am J Surg Pathol 2015 Apr;39(4):549-57
PMID 25602792
 

Citation

This paper should be referenced as such :
McIntyre K, Hirsch MS
Kidney: Metanephric adenom
Atlas Genet Cytogenet Oncol Haematol. in press
On line version : http://AtlasGeneticsOncology.org/Tumors/MetanephricAdenomaID5210.html
History of this paper:
Pan, CC. Kidney: Metanephric adenoma. Atlas Genet Cytogenet Oncol Haematol. 2013;17(2):135-137.
http://documents.irevues.inist.fr/bitstream/handle/2042/48500/09-2012-MetanephricAdenomaID5210.pdf


Other genes implicated (Data extracted from papers in the Atlas) [ 2 ]

Genes CEACAM1 NKX2-1

External links

arrayMap Topo ( C64) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
Disease databaseKidney: Metanephric adenoma
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed


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