Ossifying renal tumor of infancy
2021-07-07 Rita Alaggio, MD , Paola Dal Cin, PhD Affiliation1.IRCCS Ospedale Bambino Gesú , Roma (Italy)
2.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)
Keywords
Pediatric renal tumor, Benign renal tumor, Trisomy 4Classification
Definition
Ossifying renal tumor of infancy (ORTI) is an extremely rare benign pediatric renal tumor.
Clinics and Pathology
Epidemiology
Less than 25 cases have been reported, mostly within the first year of life (age range 6d-30mths, mean 6.7m). There is a predilection for males (male to female ratio 6:1).
Clinical features
Presentation symptoms include painless gross hematuria and rarely with an abdominal mass. Unilateral mass located in the renal pelvis and calyces with occasional extension into the collecting ducts, leading to hydronephrosis or pelvicalyceal dilatation 1. Rarely coexistence with other malignant tumors has been reported.
Macroscopic apperances
Nodular, often calcified mass with firm, grey-white, cut surface.
Histopathology
The tumor consists of core surrounded by osteoblasts blends at the periphery with a blastemal-like spindle cell component with infiltrative growth. The osteoid matures with the age of the patient.
Immunohistochemistry
EMA, Vimentin and STAB2 were positive in osteoblasts and WT-1 in spindle cells; CK, PAX8 and PAX2 negative.
Cytogenetics
Prognosis and treatment
The tumor is benign, and so far, reported patients remain well without recurrence or metastasis after operation.2
Genetics
Cytogenetics
Trisomy 4 has been reported in a small group of tumors.2-4
Article Bibliography
| Reference Number | Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|---|
| 1 | 24633911 | 2014 | The impact of race, age, and sex in follicular lymphoma: A comprehensive SEER analysis across consecutive treatment eras. | Nabhan C et al |
| 2 | 27633911 | 2016 | Ossifying renal tumor of infancy (ORIT): The clinicopathological and cytogenetic feature of two cases and literature review. | Guan W et al |
| 3 | 22976287 | 2013 | Clonal trisomy 4 cells detected in the ossifying renal tumor of infancy: study of 3 cases. | Liu J et al |
| 4 | 29187024 | 2017 | Ossifying Renal Tumor of Infancy: Report of a Case With Positive WT1 Immunohistochemistry and High Mitotic Index and Review of the Literature. | Vaillancourt B et al |
Citation
Rita Alaggio, MD ; Paola Dal Cin, PhD
Ossifying renal tumor of infancy
Atlas Genet Cytogenet Oncol Haematol. 2021-07-07
Online version: http://atlasgeneticsoncology.org/solid-tumor/208918/ossifying-renal-tumor-of-infancy
