Pediatric Tumors
Rate of 2nd malignancy from childhood cancer is 3.6-6.4x rate of general population. A study looking at SEER data for >1million patients with malignancies showed a 2nd malignancy rate in 15-39yo of 12.4% at 30 years for those who did not receive radiation to 17.6% for those who did. (1) Testicular microlithiasis may be associated with cancer. In 4.6% of boys with microlithiasis tumors were identified vs. 0.3% in those without. (2)
RENAL TUMORS
CONGENITAL MESOBLASTIC NEPHROMA
WILMS
NEUROBLASTOMA
RHABDOMYOSARCOMA
RENAL MEDULLARY CARCINOMA
MULTILOCULAR CYSTIC NEPHROMA (MLCN)
CLEAR CELL SARCOMA OF THE KIDNEY
- Most common tumor 3-6mo
- Tx=nephrectomy (b/c cannot differentiate from Wilm's)
WILMS
- Mean age diagnosis is 3.5 years
- Does not cross midline
- Often smooth, less fixed (than neuroblastoma)
- Patients appear well
- Increased association w/ Wilm's tumor
- BEST - blastema, epithelium, stroma (triphasic pattern), because high cure rate
- Associated w:
- Deny's Drash - gonadal dysgenesis (cryptorchidism, proximal hypospadias), nephropathy (diffuse mesangial sclerosis)
- WAGR syndrome - Wilms tumor, Aniridia, Genitourinary abnormality (hypospadias/UDT), Retardation (mental)
- Beckwith-Wiedeman - macroglossia, gigantism, abdominal wall defects, Wilm's tumor
NEUROBLASTOMA
- Originate from neural crest cells
- Abdominal mass crosses midline
- 50% occur in <2yrs age
- Often fixed in place, ie. nonmobile
- Elevated norepinephrine (lack PMMNT which is found in adrenal medulla or pheochromocytomas)
- Patients appear ill (compare to Wilms Tumor)
RHABDOMYOSARCOMA
- Bimodal distribution 6-10yrs, then 18-20yrs
- Small, blue, round cells
- grapelike clusters (botryoid)
RENAL MEDULLARY CARCINOMA
- Collecting duct origin
- Mostly in sickle cell patients
- Survival ~1yr
MULTILOCULAR CYSTIC NEPHROMA (MLCN)
- Benign
- Bimodal 3mo-4yrs males and middle aged females
- Nephrectomy (b/c unable to differentiate malignant cystic tumors on imaging)
CLEAR CELL SARCOMA OF THE KIDNEY
- Mets to bone
- Nephrectomy, radiation, chemo
- Worse than wilms
- Lee, Jean S., et al. "Increased risk of second malignant neoplasms in adolescents and young adults with cancer." Cancer 122.1 (2016): 116-123.
- Trout, Andrew T., et al. “Association between Testicular Microlithiasis and Testicular Neoplasia: Large Multicenter Study in a Pediatric Population.” Radiology, vol. 285, no. 2, 2017, pp. 576–583.