Fine Samson W, Humphrey Peter A, Dehner Louis P, Amin Mahul B, Epstein Jonathan I
Department of Pathology, Johns Hopkins Hospital, Baltimore, Maryland, USA.
J Urol. 2005 Nov;174(5):1976-80. doi: 10.1097/01.ju.0000176801.16827.82.
Urothelial neoplasms in patients younger than 20 years are rare, with conflicting data regarding clinical outcomes.
We identified 23 patients 4 to 20 years old with urothelial neoplasms, reclassified the microscopic diagnoses using the 2004 WHO/International Society of Urologic Pathology grading classification and collected data on presentation, risk factors and outcomes.
Pathological grading revealed 2 urothelial papillomas, 10 papillary urothelial neoplasms of low malignant potential (PUNLMPs), and 8 low grade and 3 high grade papillary urothelial cancers, all without invasion. Mean patient age was 13.2 years (range 4 to 20), 19 patients were male and 19 presented with gross hematuria. All lesions were solitary and measured 0.1 to 6 cm. One patient had a history of smoking and 1 had parents who smoked. Three patients (13%) had recurrences classified as either urothelial papilloma (1) or PUNLMP (2). All patients were alive with no evidence of disease after a mean followup of 4.5 years (range 6 months to 13 years).
Urothelial neoplasms in individuals younger than 20 years more commonly occur in males and are predominantly low grade with a favorable clinical outcome. Before the current classification system the 10 patients with a diagnosis of PUNLMP would have been classified as having papillary carcinoma. Thus, the diagnostic category of PUNLMP allowed 43.5% of patients in this series to avoid being labeled with "cancer" at a young age.
20岁以下患者的尿路上皮肿瘤罕见,关于临床结果的数据存在矛盾。
我们确定了23例年龄在4至20岁的尿路上皮肿瘤患者,使用2004年世界卫生组织/国际泌尿病理学会分级分类对显微镜诊断进行重新分类,并收集了有关临床表现、危险因素和结果的数据。
病理分级显示2例尿路上皮乳头状瘤、10例低恶性潜能乳头状尿路上皮肿瘤(PUNLMP)、8例低级别和3例高级别乳头状尿路上皮癌,均无浸润。患者平均年龄为13.2岁(范围4至20岁),19例为男性,19例表现为肉眼血尿。所有病变均为单发,大小为0.1至6厘米。1例患者有吸烟史,1例患者父母吸烟。3例患者(13%)复发,分类为尿路上皮乳头状瘤(1例)或PUNLMP(2例)。平均随访4.5年(范围6个月至13年)后,所有患者均存活,无疾病证据。
20岁以下个体的尿路上皮肿瘤更常见于男性,主要为低级别,临床结果良好。在当前分类系统之前,10例诊断为PUNLMP的患者会被分类为乳头状癌。因此,PUNLMP这一诊断类别使本系列中43.5%的患者在年轻时避免被贴上“癌症”标签。