Table 1.
Clinical Features.
| Patie nt No. |
Prior Diagnosis & Year |
Age at Prior Diagnosis (y) |
Therapy | Renal Neoplasm |
Age at Renal Neoplasm Diagnosis (y) |
Time Interval (y) |
Other Associated Neoplasia |
Age at Last Follow Up (f/u) |
|---|---|---|---|---|---|---|---|---|
| 1 | Wilms Tumor (1956) | 1y | Radiation | Clear Cell RCC | 58y | 57y | - | NED (64y) |
| 2 | Wilms Tumor (1964) | 2y | Actinomycin D and Radiation | Clear Cell RCC | 54y | 52y | Papillary thyroid carcinoma | AWD (58y); Metastatic RcC on f/u |
| 3 | Wilms Tumor (1949) | 2y | Radiation | Tumor1: Clear Cell RCC; Tumor2: Oncocytoma (Synchronous) | 56y | 54y | - | AWD (72y); Metastatic RcC on f/u |
| 4 | Wilms Tumor (1957) | 5y | Radiation | Clear Cell RCC | 57y | 52y | Breast ductal carcinoma in situ | DoD (59y); Metastatic RCC on f/u |
| 5 | Wilms Tumor (1986) | 6y | Chemotherapy (Regimen unknown) and Radiation | Metanephric Adenoma | 27y | 21y | Esophageal squamous cell carcinoma | NED (40y) |
| 6 | Wilms Tumor (1988) | 20y | Chemotherapy (Regimen unknown) and Radiation | Tumor1 (left): Metanephric Adenoma; Tumor2 (left): Oncocytoma (Synchronous) | 39y | 19y | Ossifying fibromas (jaw), Uterine adenofibroma; Parathyroid adenoma (Familial Hyperparathyroidism) | NED (51y) |
| Tumor3 (left): Metanephric Adenoma (biopsy/cryoablation) | 51y | 32y | - | |||||
| Tumor4 (left): Unknown (cryoablation) | 51y | 32y | - | |||||
| 7 | Neuroblastoma (2005, *Previously Reported) | <1y | Carboplatin, Etoposide, Cyclophospha mide and Doxorubicin | t(6;11) RCC | 12y | 11y | - | - |
| 8 | Neuroblastoma (1972) | 1y | Radiation (1800 cGy in 17 fractions; Recurrence: 2500 cGy in 30 fractions and cyclophospha mide) | Clear Cell RCC | 26y | 25y | Basal cell carcinoma (vulva); Hepatic adenoma | NED (49y) |
| 9 | Neuroblastoma (1980) | 2y | Radiation (2500 cGy) | Clear Cell RCC | 27y | 25y | Ganglioneuroma at L1 (6y); Ganglioneuroma at the time of partial nephrectomy (27y) | NED (41y) |
| 10 | Neuroblastoma (1994) | 5y | Chemotherapy (Regimen unknown) and Radiation | Clear Cell RCC | 27y | 22y | - | NED (30y) |
| 11 | Neuroblastoma (1975) | 6y | Chemotherapy (Regimen unknown) and Radiation | Clear Cell RCC | 50y | 44y | Urothelial carcinoma (pT3bN0), Prostatic adenocarcinoma (Gleason score 3+3=6, grade group1; pT2N0); Pancreatic intraductal papillary mucinous neoplasm | AWD (50y) |
| 12 | ALL (1973) | 3y | Chemotherapy (Regimen unknown) and Radiation | FH-Deficient RCC (no family history significant for HLRCC) | 43y | 40y | Hurthle Cell thyroid carcinoma; Follicular thyroid carcinoma; Schwannoma | DoD (45y); Metastatic RCC on f/u |
| 13 | ALL (2001) | 3y | Chemotherapy (Regimen unknown) | Clear Cell RCC | 22y | 19y | - | NED (22y) |
| 14 | SLE (**Previously Reported) | 9y | Cyclophosphamide and Prednisone | Tumor1 (right): ASPL-TFE3 rearranged RCC2 | 17y | 8y | - | - |
| Tumor2 (left): PRCC-TFE3 rearranged RCC | 22y | 13y | - | AWD (22y); Metastatic RCC |
cGy: centigray; ALL: acute lymphoblastic leukemia; SLE: systemic lupus erythematosus; y: years; RCC: renal cell carcinoma; FH: fumarate hydratase; HLRCC: hereditary leiomyomatosis and renal cell cancer; NED: no evidence of disease; AWD: alive with disease; DoD: dead of disease.
*
Patient 7 was reported previously by Gupta et al (2019)
**
Patient 14 was reported previously by Argani et al (2001 and 2006).