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Mesoblastic nephroma

mesoblastic nephroma, mesoblastic nephroma radiology
Congenital mesoblastic nephroma, while rare, is the most common kidney neoplasm diagnosed in the first three months of life and accounts for 3-5% of all childhood renal neoplasms12 This neoplasm is generally non-aggressive and amenable to surgical removal However, a readily identifiable subset of these kidney tumors has a more malignant potential and is capable of causing life-threatening metastases Congenital mesoblastic nephroma was first named as such in 1967 but was recognized decades before this as fetal renal hamartoma or leiomyomatous renal hamartoma3


  • 1 Presentation
  • 2 Tumor pathology
  • 3 Genetics
  • 4 Diagnosis
  • 5 Treatment and prognosis
  • 6 References
  • 7 External links


Congenital mesoblastic nephroma typically 76% of cases presents as an abdominal mass which is detected prenatally 16% of cases by ultrasound or by clinical inspection 84% of cases either at birth or by 38 years of age median age ~1 month The neoplasm shows a slight male preference Concurrent findings include hypertension 19% of cases, polyhydramnios ie excess of amniotic fluid in the amniotic sac 15%, hematuria 11%, hypercalcemia 4%, and elevated serum levels of the kidney-secreted, hypertension-inducing enzyme, renin 1% Congenital anomalies have been reported in 11 patients: 6 with genitourinary anomalies, 2 with gastrointestinal anomalies, 1 with hydrocephalus, and 1 with the Beckwith–Wiedemann syndrome The vast majority of patients present with localized ie non-metastatic disease14 Most patients' disease is classified at presentation as stage I or II ie localized, few patients present with stage III ie locally advanced/infiltrating, and virtually no patients present with stage IV metastases present or V ie tumors in both kidneys disease see staging of renal cancer1

Tumor pathologyedit

Congenital Mesoblastic nephroma in a malignant tumorous growth of the kidney's mesenchyme ie connective tissue cells Histologic examination of these tumors provides critical information on their prognoses This examination divides congenital mesoblastic nephroma into three types:

  • 1 The classic type occurs in ~39% of patients Its tissues show interlaced spindle-shaped smooth muscle cells evidencing low mitotic activity with no evidence of tumor encapsulation; and infiltration into and entrapment of normal kidney tissue1
  • 2 The cellular type occurs in ~42% of patients1 Its tissues show densely packed fibrosarcoma-like cells evidencing high rates of mitosis, less infiltration of normal kidney tissue, and multiple areas of hemorrhage and cysts13
  • 3 The mixed type occurs in ~19% of patients It shows a mixture of the classic and cellular types in different areas of the neoplasm1


A study conducted in 1998 found that congenital mesoblastic nephroma tissues taken from some patients contained an acquired mutation, the ETV6-NTRK3 fusion gene This gene results from a translocation of genetic material from the ETV6 gene located on the short arm designated p of chromosome 12 at position p132 ie 12p132 to the NTRK3 gene located on the long arm designated q of chromosome 15 at position q253 ie 15q253 This ETV6-NTRK3 gene fusion is notated as t12;15p13;q25 and consists of the 5' end of ETV6 fused to the 3' end of NTRK33 In consequence, the chimeric protein product of this gene lacks ETV6 protein's transcription factor activity while having NTRK3 protein's tyrosine kinase in an unregulated and continuously active form Either event can drive the malignant growth of cells but in most cases the chimeric protein's tyrosine kinase activity appears responsible for doing so5 Based on a limited number of genetic studies a total of 65 patients, the ETY6-NTRK3 fusion gene appears to occur in most cases of the cellular and some cases of the mixed but no cases of the classical types of congenital mesolastic nephroma136 However, a more recent study of 19 patients detected the fused gene in all 8 cases of cellular, 5 of 6 cases of mixed, and 0 of 5 cases of classic mesoblastic nephroma This suggests that expression of this fused gene may be more common in cellar and mixed mesoblastic nephroma than previously appreciated3

Trisomy, ie pathological presence of an extra chromosome, also occurs in these neoplasms Trisomy of chromosome 11 eg trisomy 11 appears to be the most commonly found trisomy in this disease, being detected in 7 of 13 genetically studied cases1 Individual case reports have also found trisomy 8 9 cases, 17 4 cases, 20 4 cases, 7 3 cases, 10 3 cases, 18 2 cases, 2 2 cases, and 9 2 cases associated with the disease13 The contribution of these trisomies to the development of mesoblastic nephroma is unclear


Diagnosis of mesoblastic nephroma and its particular type ie classic, mixed, or cellular is made by histological examination of tissues obtained at surgery Besides its histological appearance, various features of this disease aid in making a differential diagnosis that distinguish it from the following childhood neoplasms:

  • Wilm's tumor is the most common childhood kidney neoplasm, representing some %85 of cases Unlike mesoblastic nephroma, <2% of Wilm's tumor patients present at under 3 months of age and most present in patients of >3 years of age Bilateral kidney tumors, concurrent birth defects, and/or metastatic disease at presentation favor a diagnosis of Wilm's tumor4
  • congenital infantile sarcoma is a rare aggressive sarcoma typically presenting in the lower extremities, head, or neck of infants during their first year of life The histology, association with the ETV6-NRTK3 fusion gene along with certain chromosome trisomies, and the distribution of markers for cell type ie cyclin D1 and Beta-catenin within this tumor are the same as those found in cellular mesoblastic nephroma Mesoblastic nephroma and congenital infantile sarcoma appear to be the same diseases with mesoblastic lymphoma originating in the kidney and congenital infantile sarcoma originating in non-renal tissues3478
  • Rhabdoid tumor, which accounts for 5-510% of childhood kidney neoplasms, occurs predominantly in children from 1 to 2 years of age Unlike mesoblastic nephroma, rhabdoid tumors may present with tumors in other tissues including in ~13% of cases, the brain Rhabdoid tumors have a distinctive histology and abnormalities ie loss of heterozygosity, single nucleotide polymorphism, and deletions in chromosome 229
  • Clear cell sarcoma of the kidney, which is responsible for 5-10 of childhood pediatric tumors, occurs predominantly in children from 2 to 3 years of age Unlike meoblastic nephorma, clear cell sarcoma of the kidney presents with metastasis, particularly to bone, in 5-6% of cases; it histology is diverse and has been mistaken for mesoblastic nephroma One chromosomal translocations t,10;17q22;p13, has been repeatedly reported to be associated with clear cell sarcoma of the kidney1011
  • Infantile myofibromatosis is a fibrous tumor of infancy and childhood most commonly presenting during the first 2 years of life as a single subcutaneous nodule of the head and neck region or less commonly as multiple lesions of skin, muscle, bone, and in ~33% of these latter cases, visceral organs All of these lesions have an excellent prognosis and can regress spontaneously except for those in which there is visceral involvement where the prognosis is poor12 While infantile myofibromatosis and classic mesoblastic nephroma have been suggested to be the same diseases because of their very similar histology, studies on the distribution of cell-type markers ie cyclin D1 and Beta-catenin indicate that they have different cellular origins34

Treatment and prognosisedit

Based on a survey of >800, surgical removal of the entire involved kidney plus the peri-renal fat appeared curative for the majority of all types of mesoblastic nephroma; the patient overall survival rate was 94% Of the 4% of non-survivors, half were due to surgical or chemotherapeutic treatments Another 4% of these patients suffered relapses, primarily in the local area of surgery rare cases of relapse due to lung or bone metastasis About 60% of these recurrent cases had a complete remission following further treatment Recurrent disease was treated with a second surgery, radiation, and/or chemotherapy that often vincristine and actinomycin treatment1 Removal of the entire afflicted kidney plus the peri-renal fat appears critical to avoiding local recurrences In general, patients who were older than 3 months of age at diagnosis or had the cellular form of the disease, stage III disease, or involvement of renal lymph nodes had a higher recurrence rate Among patients with these risk factors, only those with lymph node involvement are recommended for further therapy4

It has been suggested that mesoblastic nephroma patients with lymph node involvement or recurrent disease might benefit by adding the ALK inhibitor, crizotinib, or a tyrosine kinase inhibitor, either larotrectinib or entrectinib, to surgical, radiation, and/or chemotherapy treatment regimens These drugs inhibit NTRK3's tyrosine kinase activity1 Crizotinib has proven useful in treating certain cases of acute lymphoblastic leukemia that are associated with the ETV6-NTRK3 fusion gene while larotrectinib and entrectinib have been useful in treating various cancers eg a metastatic sarcoma, papillary thyroid cancer, non-small-cell lung carcinoma, gastrointestinal stromal tumor, mammary analog secretory carcinoma, and colorectal cancer that are driven by mutated, overly active tyrosine kinases Relevant to this issue, a 16-month-old girl with infantile fibrosarcoma harboring the ETV6–NTRK3 fusion gene was successfully trated with larotrectinib113 The success of these drugs, howwever, will likely depend on the relative malignancy-promoting roles of ETV6-NTRK3 protein's tyrosine kinase activity, the lose of ETV6-related transcription activity accompanying formation of ETV6-NTRK3 protein, and the various trisomy chromosomes that populate mesoblastic nephroma


  1. ^ a b c d e f g h i j k l m Gooskens SL, Houwing ME, Vujanic GM, Dome JS, Diertens T, Coulomb-l'Herminé A, Godzinski J, Pritchard-Jones K, Graf N, van den Heuvel-Eibrink MM 2017 "Congenital mesoblastic nephroma 50 years after its recognition: A narrative review" Pediatric Blood & Cancer 64 7 PMID 28124468 doi:101002/pbc26437 
  2. ^ Lamb MG, Aldrink JH, O'Brien SH, Yin H, Arnold MA, Ranalli MA 2017 "Renal Tumors in Children Younger Than 12 Months of Age: A 65-Year Single Institution Review" Journal of Pediatric Hematology/oncology 39 2: 103–107 PMID 27820132 doi:101097/MPH0000000000000698 
  3. ^ a b c d e f g h El Demellawy D, Cundiff CA, Nasr A, Ozolek JA, Elawabdeh N, Caltharp SA, Masoudian P, Sullivan KJ, de Nanassy J, Shehata BM 2016 "Congenital mesoblastic nephroma: a study of 19 cases using immunohistochemistry and ETV6-NTRK3 fusion gene rearrangement" Pathology 48 1: 47–50 PMID 27020209 doi:101016/jpathol201511007 
  4. ^ a b c d e Wang ZP, Li K, Dong KR, Xiao XM, Zheng S 2014 "Congenital mesoblastic nephroma: Clinical analysis of eight cases and a review of the literature" Oncology Letters 8 5: 2007–2011 PMC 4186628  PMID 25295083 doi:103892/ol20142489 
  5. ^ Kar A, Gutierrez-Hartmann A 2013 "Molecular mechanisms of ETS transcription factor-mediated tumorigenesis" Critical Reviews in Biochemistry and Molecular Biology 48 6: 522–43 PMC 4086824  PMID 24066765 doi:103109/104092382013838202 
  6. ^ Anderson J, Gibson S, Sebire NJ 2006 "Expression of ETV6-NTRK in classical, cellular and mixed subtypes of congenital mesoblastic nephroma" Histopathology 48 6: 748–53 PMID 16681692 doi:101111/j1365-2559200602400x 
  7. ^ Ud Din N, Minhas K, Shamim MS, Mushtaq N, Fadoo Z 2015 "Congenital infantile fibrosarcoma of the scalp: a case series and review of literature" Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery 31 11: 2145–9 PMID 26206116 doi:101007/s00381-015-2824-1 
  8. ^ Walther C, Nilsson J, von Steyern FV, Wiebe T, Bauer HC, Nord KH, Gisselsson D, Domanski HA, Mandahl N, Mertens F 2013 "Cytogenetic and single nucleotide polymorphism array findings in soft tissue tumors in infants" Cancer Genetics 206 7-8: 299–303 PMID 23938179 doi:101016/jcancergen201306004 
  9. ^ Jackson EM, Sievert AJ, Gai X, Hakonarson H, Judkins AR, Tooke L, Perin JC, Xie H, Shaikh TH, Biegel JA 2009 "Genomic analysis using high-density single nucleotide polymorphism-based oligonucleotide arrays and multiplex ligation-dependent probe amplification provides a comprehensive analysis of INI1/SMARCB1 in malignant rhabdoid tumors" Clinical Cancer Research : an Official Journal of the American Association for Cancer Research 15 6: 1923–30 PMC 2668138  PMID 19276269 doi:101158/1078-0432CCR-08-2091 
  10. ^ Gooskens SL, Furtwängler R, Vujanic GM, Dome JS, Graf N, van den Heuvel-Eibrink MM 2012 "Clear cell sarcoma of the kidney: a review" European Journal of Cancer Oxford, England : 1990 48 14: 2219–26 PMID 22579455 doi:101016/jejca201204009 
  11. ^ Alavi S, Khoddami M, Yazdi MK, Dehghanian P, Esteghamati S 2013 "Clear cell sarcoma of the kidney misdiagnosed as mesoblastic nephroma: a case report and review of the literature" Ecancermedicalscience 7: 311 PMC 3634723  PMID 23634181 doi:103332/ecancer2013311 
  12. ^ https://wwwncbinlmnihgov/books/NBK65923/
  13. ^ Laetsch TW, Nagasubramanian R, Casanova M 2017 "Targeting NTRK fusions for the treatment of congenital mesoblastic nephroma" Pediatric Blood & Cancer PMID 28440051 doi:101002/pbc26593 

External linksedit

  • Congenital mesoblastic nephroma entry in the public domain NCI Dictionary of Cancer Terms

 This article incorporates public domain material from the US National Cancer Institute document "Dictionary of Cancer Terms"

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