Case
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Diagnosis: Congenital Mesoblastic Nephroma, Cellular Type
Congenital renal tumors comprise 2.5-7% of all perinatal tumors. Congenital renal neoplasms include, in decreasing order of frequency, congenital mesoblastic nephroma, Wilms tumor, rhabdoid tumor, clear cell sarcoma, hamartomas, and ossifying tumor of infancy. Congenital mesoblastic nephrom (or simply mesoblastic nephroma) represents 3-10% of all pediatric renal tumors. This tumor was first described as a separate entity by Bolande et al in 1967. Prior to this, it was erroneously confused with congenital Wilms tumor (Singh SP. Mesoblastic Nephroma. In: e-Medicine; visited February 24th, 2010 [Link]
There are two pathologic variants: classic and cellular. The cellular type is characterized by a high mitotic index, hypercellularity, and an atypical growth pattern with necrosis, hemorrhage, and invasion of adjacent structures. The cellular type accounts for 42-63% of cases.
The cellular type has been shown to bear the t(12;15)(p13;q25) and ETV6 (chromosome 12)-NTRK3 (chromosome 15) gene fusion. These combined genes are thought to activate tyrosine kinase growth signaling. This gene fusion transcript is also reported in congenital or infantile fibrosarcoma (Singh SP. Mesoblastic Nephroma. In: e-Medicine; visited August 24th, 2017 [Link]).
See the chapter: Renal Neoplasms of our Tutorial (only Spanish version).
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References
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Tripathy PK, Behera S, K Mohanty H. Cellular Congenital Mesoblastic Nephroma in a Newborn. J Neonatal Surg. 2017;15;6(2):45. [PubMe link].
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Laetsch TW, Nagasubramanian R, Casanova M. Targeting NTRK fusions for the treatment of congenital mesoblastic nephroma. Pediatr Blood Cancer. 2017 Apr 25. doi: 10.1002/pbc.26593. [Epub ahead of print] [PubMed link]
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Daniel J, Ruzic A, Dalland J, Miller V, Hanna M. Management of mixed type congenital mesoblastic nephroma: Case series and review of the literature. J Neonatal Perinatal Med. 2017;10(1):113-118. [PubMed link]
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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. Congenital mesoblastic nephroma 50 years after its recognition: A narrative review. Pediatr Blood Cancer. 2017;64(7). [PubMed link]