Other renal tumours
Key points
Mesoblastic nephroma
Also called Congenital Mesoblastic Nephroma (CMN)
Most common renal tumour <6 months age
Mesenchymal
Can diagnose on antenatal USS
Polyhydramnios, hydrops
Paraneoplastic syndromes - Hypertension, hypercalcaemia (PTH related)
USS - ring sign
Histology
Type | Frequency | Histologic and Molecular Features | Clinical Behaviour |
Classical | 66% | Spindle cells, similar to infantile myofibromatosis | Smaller, benign, rare recurrence |
Cellular | 24% | Sarcoma-like, blue cells, ETV6-NTRK gene fusion | Larger, local recurrence, rare distant spread |
Mixed | 10% | Combined features of classical and cellular types | Clinical implications uncertain |
Radical nephrectomy alone often sufficient
No set chemo regimen - can use sarcoma protocols - vincristine, actinomycin, cyclophosphamide
Post op - monitor and supplement Vit D, Ca, Phos
96% survival in <7 months age
Investigations for non-Wilms renal tumour
Clear cell sarcoma of kidney: MRI brain + Whole body MRI/PET/Bone scan
Rhabdoid: MRI Brain
RCC: Whole body MRI/PET/Bone scan
Renal Cell Carcinoma
Average Age: Older than 15 years
Symptoms: Haematuria
Diagnostic Workup: Calcifications on CT more likely than in Wilms tumour
Genetics/Syndromes: Xp11 (TFE3) translocation, von Hippel-Lindau, tuberous sclerosis, familial renal cell carcinoma
Features: Partial nephrectomy considered if margins are negative, chemotherapy/radiotherapy not effective, lymph node involvement not prognostic, 87% 5-year overall survival
Rhabdoid Tumour of the Kidney
Average Age: 11 months (range: 0-4 years)
Symptoms: Fever, haematuria, advanced stage
Diagnostic Workup: Brain CT or MRI
Genetics/Syndromes: Loss of function in SMARCB1
Features: Upfront resection required, similar chemotherapy to Wilms, all receive radiotherapy, hypercalcaemia common, no survival without complete resection, early follow-up necessary due to high relapse, 33% 5-year overall survival
Clear Cell Sarcoma of the Kidney
Average Age: 36 months (range: 2 months to 14 years)
Diagnostic Workup: PET or bone scan, consider brain CT or MRI
Genetics/Syndromes: t(10;17) translocation, 14q deletion
Features: Spreads to lung, bone, brain, and soft tissue, brain frequent site of recurrent disease
Cystic Nephroma
Average Age: Infants/young children or adult females
Multilocular renal cysts
Genetics/Syndromes: DICER1 mutation (paediatric cases)
Benign
Renal Medullary Carcinoma
Average Age: Young adults
Almost exclusive to sickle cell trait
Near 100% mortality due to late presentation
References
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 Jul;64(7). doi: 10.1002/pbc.26437. Epub 2017 Jan 26. PMID: 28124468.
Ray S, Jones R, Pritchard-Jones K, Dzhuma K, van den Heuvel-Eibrink M, Tytgat G, van der Beek J, Oades G, Murphy D. Pediatric and young adult renal cell carcinoma. Pediatr Blood Cancer. 2020 Nov;67(11):e28675. doi: 10.1002/pbc.28675. Epub 2020 Sep 1. PMID: 32869954.
Amar AM, Tomlinson G, Green DM, Breslow NE, de Alarcon PA. Clinical presentation of rhabdoid tumors of the kidney. J Pediatr Hematol Oncol. 2001 Feb;23(2):105-8. doi: 10.1097/00043426-200102000-00007. PMID: 11216700.
Aldera AP, Pillay K. Clear Cell Sarcoma of the Kidney. Arch Pathol Lab Med. 2020 Jan;144(1):119-123. doi: 10.5858/arpa.2018-0353-RS. Epub 2019 Mar 27. PMID: 30917048.
https://radiopaedia.org/articles/pediatric-cystic-nephroma?lang=gb
Su Y, Hong AL. Recent Advances in Renal Medullary Carcinoma. Int J Mol Sci. 2022 Jun 26;23(13):7097. doi: 10.3390/ijms23137097. PMID: 35806102; PMCID: PMC9266801.