Table 1. Clinicopathologic Features of TC-PD RCCs.
| Case # | Classification∼ | Age [y] | Sex | Side | HLRCC?# | Size [cm] | pT Stage& | Node Status$ | Metastasis@ | Progression [mo]ˆ | Status% | Follow-Up (mo) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| TC-D 1 | FH-D | 18 | M | R | - | 10 | - | - | - | - | - | - |
| TC-D 2 | FH-D | 20 | M | L | No | 12.5 | pT3b | Neg | Li | 1 | DOD | 25 |
| TC-D 3 | FH-D | 25 | M | R | No | 15.5 | pT3a | Neg | Li, Lu, Bn, Adr | P | DOD | 8 |
| TC-D 4 | FH-D | 40 | M | L | Fam, RCC | 3.7 | pT3a | - | Li | P | AWD | 2 |
| TC-D 5 | FH-D | 42 | M | L | No | 5 | pT3a | I | Bn | P | AWD | 16 |
| TC-D 6 | FH-D | 42 | F | R | No | 6 | pT3a | R | Li, R | P | DOD | 2 |
| TC-D 7 | FH-D | 42 | F | L | UM | 4.1 | pT3a | Neg | Lu, E, CW | P | DOD | 3 |
| TC-D 8 | FH-D | 44 | M | R | No | 10.5 | pT3a | Neg | - | - | NED | 2 |
| TC-D 9 | FH-D | 45 | F | R | No | 5.5 | pT3 | Neg | Lu, Li, R, Bn, M, O | 10 | DOD | 43 |
| TC-D 10 | FH-D | 46 | M | L | No | 8.5 | pT3a | A | Bn, Lu, Li, T, A | P | DOD | - |
| TC-D 11 | FH-D | 46 | M | R | No | 10 | pT3c | Neg | Li | 4 | DOD | 25 |
| TC-D 12 | FH-D | 52 | M | L | - | 12 | pT4 | Neg | - | - | LTF | - |
| TC-D 13 | FH-D | 53 | M | R | Fam, MCL | 6 | pT3a | C | - | - | LTF | 4 |
| TC-D 14 | FH-D | 59 | M | L | No | 16 | pT2b | Neg | None | - | LTF | - |
| TC-D 15 | FH-D | 61 | M | L | - | 14 | pT2 | Neg | Li, Bn, C | 2 | AWD | 68 |
| TC-D 16 | FH-D | 67 | M | R | No | 10 | pT3a | R | Li, Lu | 6 | DOD | 6 |
| TC-D 17 | FH-D* | 34 | F | L | No | 3.7 | pT1a | - | Lu | 4 | AWD | 10 |
| TC-D 18 | FH-D* | 40 | F | L | No | 6 | pT1b | Neg | - | - | DNOS | - |
| TC-D 19 | FH-D* | 71 | M | R | No | 8 | pT3a | U | - | - | DOD | 16 |
| TC-D 20 | FH-S | 16 | F | R | No | 6.5 | pT1b | Neg | None | - | NED | 126 |
| TC-D 21 | FH-S | 35 | M | L | No | 14 | pT3 | A | Adr, Bn, E | P | DOD | 9 |
| TC-D 22 | FH-S | 50 | M | R | No | 21 | pT2b | Neg | Lu | 8 | DOD | 10 |
| TC-D 23 | FH-S | 53 | M | R | No | 13 | pT3 | Neg | P, R | - | AWD | 5 |
| TC-D 24 | FH-S | 53 | F | L | No | 3.3 | pT3a | - | - | - | AWD | 2 |
| TC-D 25 | FH-R | 70 | M | R | No | 11.7 | pT3a | - | Adr | P | AWD | 0 |
| TC-D 26 | FH-R | 71 | M | R | No | 8.5 | pT3a | U | Lu, Li | P | AWD | 0 |
| TC-D 27 | FH-R | 74 | M | L | No | 4.5 | pT3a | - | Lu , Bn | - | LTF | - |
| TC-D 28 | FH-R | 80 | F | R | No | 5.5 | pT3a | A | Lu , Bn | 8 | DOD | 15 |
| TC-D 29 | FH-R | 86 | M | R | No | 3 | pT3a | - | None | 0 | NED | 7 |
Abbreviations: ‘-’, no data; TC-PD, tubulocystic carcinoma with poorly differentiated foci; FH, fumarate hydratase; RCC, renal cell carcinoma; IHC, immunohistochemistry;
Classification – Final integrating IHC and molecular findings; FH-D, FH-deficient RCC; FH-S, FH-suspicious RCC; FH-R, FH-retained RCC, * indicates IHC suspicious case reclassified as FH-D based on NGS findings
HLRCC: includes any evidence of HLRCC, whether stigmata apparent clinically (UM-uterine leiomyomatosis) or family history (Fam), RCC, renal cell carcinoma; MCL, multiple cutaneous leiomomas) reported, see Table 3.
pTNM: Classification by AJCC 2010 TNM Staging System
Lymph Node Metastasis: nodal apparent at surgical staging; -, no data; Neg, negative; I, positive iliac node; R, positive retroperitoneal node; AC, positive aortic/caval lymph node; S, sinus; Re, positive “regional” lymph node; U, positive unspecified node; lymph node metastasis evolving during follow-up is included only in “Metastatc Site”
Metastatic Site: metastatic sites apparent either at presentation or evolving during follow-up; Li, liver; Lu, lung; Bn, bone; R, retroperitoneal node; E, malignant effusion; CW, chest wall; M, mediastinal node; O, ovary; T, thoracic LN; Ab, abdominal LN; C, cervical LN; Sp, spleen; P, pancreas;
Progression: time to first metastatic progression, months; P, metastatic at presentation
Status: last available status at followup; DOD, dead of disease; NED, no evidence of disease; LTF, lost to follow-up; AWD, alive with disease; DNOS, dead, not otherwise specified