Punga-Maole M L, Hubert J, Grignon Y, Floquet J, Mangin P
Service d'Urologie, CHU de Nancy-Brabois, Vandoeuvre-lès-Nancy.
Prog Urol. 1994 Dec;4(6):977-86.
Between 1987 and 1992, we operated on 15 patients with tubulopapillary tumours (TPT) of the kidney, corresponding to 10% of all renal cancers operated during this 5-year period, which brings the total number of published cases to 418. TPT differ from non-tubulopapillary tumours in several ways. The medical imaging findings may differ when the tumour is not necrotic (20 to 30% of cases). The histology differs not only in terms of the tumour architecture, but also by the frequent association (9/15 cases) with other ipsilateral or contralateral renal anomalies: 2 bilateral tumours, 3 tumours associated with multiple cysts and 4 multifocal tumours. These characteristics, combined with the finding of 12 familial cases suggest a genetic origin for TPT, especially as the genetic abnormalities detected (tri-or tetrasomy 17) differ from the anomalies usually detected in non-tubulopapillary tumours (anomalies of 3p and 5q). This study is the first to report familial cases of TPT. The prognosis of these tumours, generally better than that of non-tubulopapillary tumours, suggests the possibility of conservative surgery when the diagnosis can be established preoperative or at operation.
1987年至1992年间，我们对15例肾管乳头状瘤（TPT）患者进行了手术，这占该5年期间所有接受手术的肾癌患者的10%，使得已发表病例总数达到418例。TPT在几个方面与非管乳头状瘤不同。当肿瘤未发生坏死时（20%至30%的病例），医学影像表现可能有所不同。其组织学不仅在肿瘤结构方面存在差异，而且常与其他同侧或对侧肾脏异常相关（9/15例）：2例双侧肿瘤、3例与多发囊肿相关的肿瘤以及4例多灶性肿瘤。这些特征，再加上发现12例家族性病例，提示TPT有遗传起源，特别是因为检测到的基因异常（17号染色体三体或四体）与非管乳头状瘤中通常检测到的异常（3p和5q异常）不同。本研究首次报告了TPT的家族性病例。这些肿瘤的预后通常优于非管乳头状瘤，这表明在术前或手术时能够确诊的情况下，有可能进行保守手术。
