Hora Milan, Urge Tomáš, Trávníček Ivan, Ferda Jiří, Chudáček Zdeněk, Vaněček Tomáš, Michal Michal, Petersson Fredrik, Kuroda Naoto, Hes Ondřej
Department of Urology Faculty Hospital, E. Beneše 13, Pilsen, 305 99 Czech Republic ; Faculty of Medicine in Pilsen, Charles University in Prague, Prague, Czech Republic.
Department of Radiology, Pilsen, Czech Republic ; Faculty of Medicine in Pilsen, Charles University in Prague, Prague, Czech Republic.
Springerplus. 2014 May 13;3:245. doi: 10.1186/2193-1801-3-245. eCollection 2014.
MiT translocation renal cell carcinomas (TRCC) predominantly occur in younger patients with only 25% of patients being over 40 years. TRCC contains two main subgroups with translocations involving 6p21 or Xp11.2. Herein we present 10 cases.
Eight cases were treated at main author's institution (identified among 1653 (0.48%) cases of kidney tumours in adults). Two cases were retrieved from the Pilsen (CZ) Tumour Registry.
Six cases were type Xp11.2 and four 6p21; 7 female, 3 male patients; Xp11.2 4:2, 6p21 3:1. The mean age 49 years (range: 21-80), 5 patients (50%) over 40 years. The mean age of the group with Xp11.2 TRCCs was 55 (median 51) and 6p21 41 (32) years. One female with a 6p21 tumour (24 years) underwent nephrectomy at 4 months of pregnancy. Stage (UICC, 7th ed. 2009) was 5xI, 3xIII, 2xIV. The mean size of tumour was 80 (40-165) mm. The mean follow-up was 33.2 (1-92) months. In patients with 6p21 tumours, one (25%) died after 3 months due to widely metastatic disease. In patients with Xp11.2 tumours, 3 (50%) succumbed due to metastatic disease (range 1-8 months). Three patients with Xp11.2 are alive at 7, 52 and 92 months of follow-up, were diagnosed at early stage (T1a).
TRCCs were more common in females. Patient with 6p21 tumours were younger than those with Xp11.2. Both types have definitive malignant potential Type Xp11.2 seems to be a more aggressive neoplasm than 6p21. The case with metastatic 6p21 tumour is the 4th case described in the English literature.
MiT 易位性肾细胞癌（TRCC）主要发生于较年轻患者，仅有 25%的患者年龄超过 40 岁。TRCC 包含两个主要亚组，其易位涉及 6p21 或 Xp11.2。在此我们报告 10 例病例。
8 例在主要作者所在机构接受治疗（在 1653 例成人肾肿瘤病例中识别出，占 0.48%）。2 例从比尔森（捷克）肿瘤登记处获取。
6 例为 Xp11.2 型，4 例为 6p21 型；7 例女性，3 例男性患者；Xp11.2 型 4:2，6p21 型 3:1。平均年龄 49 岁（范围：21 - 80 岁），5 例患者（50%）年龄超过 40 岁。Xp11.2 型 TRCC 组的平均年龄为 55 岁（中位数 51 岁），6p21 型为 41 岁（32 岁）。1 例患有 6p21 肿瘤的女性（24 岁）在妊娠 4 个月时接受了肾切除术。分期（UICC，2009 年第 7 版）为 5 例 I 期，3 例 III 期，2 例 IV 期。肿瘤平均大小为 80（40 - 165）mm。平均随访时间为 33.2（1 - 92）个月。患有 6p21 肿瘤的患者中，1 例（25%）在 3 个月后因广泛转移疾病死亡。患有 Xp11.2 肿瘤的患者中，3 例（50%）因转移疾病死亡（范围 1 - 8 个月）。3 例 Xp11.2 型患者在随访 7、52 和 92 个月时存活，诊断时为早期（T1a）。
TRCC 在女性中更常见。患有 6p21 肿瘤的患者比患有 Xp11.2 肿瘤的患者更年轻。两种类型都具有明确的恶性潜能。Xp11.2 型似乎比 6p21 型更具侵袭性。转移性 6p21 肿瘤病例是英文文献中描述的第 4 例。
