Mesoblastic nephroma overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
Overview
Mesoblastic nephroma is a type of kidney tumor. Mesoblastic nephroma is a rare disease that tends to affect children under 3 years of age, especially neonates.[1][2] The male to female ratio is approximately 2 to 1.[3] Mesoblastic nephroma may be classified according to pathology into three subtypes: classic, cellular, and mixed mesoblastic nephroma.[4] Mesoblastic nephroma may be caused by translocation t(12:15)(p13;q25) that generates the fusion of ETV6/NTRK3 gene.[5] On gross pathology, a solid nonencapsulated mass that often occurs near the renal hilum is the characteristic finding of mesoblastic nephroma. On microscopic histopathological analysis, spindle cell is the characteristic finding of classic mesoblastic nephroma; whereas, plump cells with vesicular nuclei is the characteristic finding of cellular mesoblastic nephroma.[6] Mesoblastic nephroma must be differentiated from rhabdoid tumor, clear cell sarcoma of the kidney, and Wilm’s tumor. Symptoms of mesoblastic nephroma include polyhydramnios, hematuria, and abdominal distension. Biopsy is helpful in the diagnosis of mesoblastic nephroma. The predominant therapy for mesoblastic nephroma is surgical resection. Adjunctive chemotherapy may be required for cellular mesoblastic nephroma.[4] Prognosis is generally excellent, and the 5-year survival rate of patients with mesoblastic nephroma is approximately 96%.[7]
Classification
Classification
Mesoblastic nephroma may be classified according to pathology into three subtypes: classic, cellular, and mixed mesoblastic nephroma.[4]
Pathophysiology
Pathophysiology
On gross pathology, a solid nonencapsulated mass that often occurs near the renal hilum is the characteristic finding of mesoblastic nephroma. On microscopic histopathological analysis, spindle cell is the characteristic finding of classic mesoblastic nephroma; whereas, plump cells with vesicular nuclei is the characteristic finding of cellular mesoblastic nephroma.[8]
Causes
Causes
Mesoblastic nephroma may be caused by translocation t(12:15)(p13;q25) that generates the fusion of ETV6/NTRK3 gene.[5]
Differential diagnosis
Differential diagnosis
Mesoblastic nephroma must be differentiated from rhabdoid tumor, clear cell sarcoma of the kidney, and Wilm’s tumor.
Epidemiology and Demographics
Epidemiology and Demographics
Mesoblastic nephroma is a rare disease that tends to affect children under 3 years of age, especially neonates. [1][9] The male to female ratio is approximately 2 to 1.[3]
Risk Factors
Risk Factors
There are no established risk factors for mesoblastic nephroma.
Screening
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for mesoblastic nephroma.[10]
Complications and Prognosis
Complications and Prognosis
Complications of mesoblastic nephroma include stillbirth, shoulder dystocia at birth, hydrops fetalis, and respiratory distress syndrome. Prognosis is generally excellent, and the 5-year survival rate of patients with mesoblastic nephroma is approximately 96%.[7]
Staging
Staging
There is no established system for the staging of mesoblastic nephroma.
Diagnosis
Diagnosis
Symptoms
Symptoms of mesoblastic nephroma include polyhydramnios, hematuria, and abdominal distension.
Physical Examination
Common physical examination findings of mesoblastic nephroma include pallor and an palpated abdominal mass.
Laboratory Findings
There are no laboratory findings associated with mesoblastic nephroma.
X Ray
Abdominal X-ray may be helpful in the diagnosis of mesoblastic nephroma.
CT Scan
CT scan may be helpful in the diagnosis of mesoblastic nephroma.
MRI
MRI may be helpful in the diagnosis of mesoblastic nephroma.
Ultrasound
Ultrasound may be helpful in the diagnosis of mesoblastic nephroma.
Other Imaging Findings
There are no other imaging findings associated with mesoblastic nephroma.
Other Diagnostic Studies
There are no other diagnostic study findings associated with mesoblastic nephroma.
Biopsy
Biopsy is helpful in the diagnosis of mesoblastic nephroma.
Treatment
Treatment
Medical Therapy
The predominant therapy for mesoblastic nephroma is surgical resection. Adjunctive chemotherapy may be required for cellular mesoblastic nephroma.[4]
Surgery
Surgery is the mainstay of treatment for mesoblastic nephroma.
Primary Prevention
There is no established method for prevention of mesoblastic nephroma.
Secondary Prevention
There are no secondary preventive measures available for mesoblastic nephroma.
References
References
- ↑ 1.0 1.1 Mesoblastic nephroma.Dr Ayush Goel and Dr Yuranga Weerakkody et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/mesoblastic-nephroma
- ↑ Tavassoli, Fattaneh (2003). Pathology and genetics of tumours of the breast and female genital organs. Lyon: IAPS Press. ISBN 9283224124.
- ↑ 3.0 3.1 Furtwaengler R, Reinhard H, Leuschner I, Schenk JP, Goebel U, Claviez A; et al. (2006). “Mesoblastic nephroma–a report from the Gesellschaft fur Pädiatrische Onkologie und Hämatologie (GPOH)”. Cancer. 106 (10): 2275–83. doi:10.1002/cncr.21836. PMID 16596620.
- ↑ 4.0 4.1 4.2 4.3 Malkan AD, Loh A, Bahrami A, et al. (2015). “An approach to renal masses in pediatrics”. Pediatrics. 135 (1): 142–58. doi:10.1542/peds.2014-1011. PMID 25452658.
- ↑ 5.0 5.1 Knezevich SR, McFadden DE, Tao W, Lim JF, Sorensen PH (1998). “A novel ETV6-NTRK3 gene fusion in congenital fibrosarcoma”. Nature Genetics. 18 (2): 184–7. doi:10.1038/ng0298-184. PMID 9462753.
- ↑ Humphrey, Peter (2008). The Washington manual of surgical pathology. Philadelphia: Lippincott Williams & Wilkins. ISBN 978-0781765275.
- ↑ 7.0 7.1 van den Heuvel-Eibrink MM, Grundy P, Graf N, Pritchard-Jones K, Bergeron C, Patte C; et al. (2008). “Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: A collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups”. Pediatr Blood Cancer. 50 (6): 1130–4. doi:10.1002/pbc.21389. PMID 18095319.
- ↑ Humphrey, Peter (2008). The Washington manual of surgical pathology. Philadelphia: Lippincott Williams & Wilkins. ISBN 978-0781765275.
- ↑ Tavassoli, Fattaneh (2003). Pathology and genetics of tumours of the breast and female genital organs. Lyon: IAPS Press. ISBN 9283224124.
- ↑ Mesoblastic nephroma. U.S. Preventive Service Task Force (USPSTF) 2015. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=mesoblastic+nephroma Accessed on November, 3, 2015
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