泌尿系统肿瘤

1. 泌尿系统肿瘤 Zhu keqing 竺可青 Pathology Department Zhejiang University School of Medicine 2013-4-9

2. TUMORS BENIGN Papillary Adenoma Fibroma/Hamartoma Angiomyolipoma Oncocytoma MALIGNANT Renal Cell Carcinoma (Clear Cell Carcinoma, Adenocarcinoma) Urothelial (Transitional)

3. Three most common forms Clear Cell Carcinomas Papillary Renal Cell Carcinomas Chromophobe Renal Carcinomas

4. RENAL CELL CARCINOMA TOBACCO RELATED, STRONGLY SOME HEREDITARY/FAMILIAL MOST are “CLEAR CELL”, a few PAPILLARY YELLOW grossly, “CLEAR” cells microscopically STRONGLY tend to invade the renal VEIN early, in preference to lymphatics.

5. These tumors are derived from the renal tubular epithelium, and hence they are located predominantly in the cortex. Renal carcinomas represent 80% to 85% of all primary malignant tumors of the kidney, and 2% to 3% of all cancers in adults.

6. Most renal cancer is sporadic, but unusual forms of autosomal-dominant familial cancers occur, usually in younger individuals. Although they account for only 4% of renal cancers, familial variants have been enormously instructive in studying renal carcinogenesis.

7. Von Hippel-Lindau (VHL) syndrome: Half to two-thirds of patients with VHL, characterized by hemangioblastomas of the cerebellum and retina, develop renal cysts and bilateral, often multiple, renal cell carcinomas (nearly all, if they live long enough). Current studies implicate the VHL gene in the development of both familial and sporadic clear cell tumors.

8. Three most common forms Clear cell carcinomasare the most common; associated with homozygous loss of the VHL tumor suppressor protein; tumors frequently invade the renal vein.

9. Three most common forms Papillary renal cell carcinomasare frequently associated with increased expression and activating mutations of the MET oncogene; tend to be bilateral and multiple, and show varyingpapilla formation.

10. Three most common forms Chromophobe renal cell carcinomasare lesscommon; tumor cells are not as clear as in theother renal cell carcinomas.

11. Clinically, patients may show hematuria, fever, constitutional symptoms, and /or a paraneoplastic syndrome. Renal cell carcinoma has a tendency to metastasize widely before giving rise to any local symptoms.

12. 肾细胞癌，肾腺癌 • 本癌不太多见，男性中年较多，临床较隐蔽，难早期发现。 • 肉眼：结节状，可有假包膜，在肾上极较多，色带土黄，易出血坏死 • 镜检：由含类脂及糖原的透明细胞构成，核不大，核浆比例小。腺体样结构（现认为其来自肾小管），亦可呈巢状；间质较少。 • 肾癌临床病理特点： • (1)无痛性血尿；但常为隐匿性； • (2)血道转移； • (3)常有意外的远隔转移（如转至鼻尖）； • (4)逆行转移：男性左肾癌可侵入左肾静脉→左精索内静脉→逆行引起左附睾发生转移。（右侧精索内静脉直接流入下腔V，故无此现象）； • (5)异位内分泌现象较其它癌为多。

13. Nephroblastoma(Wilms’ Tumor)

14. The most common primary renal tumor of childhood, usually diagnosed between the age of 2 and 5 years.

15. Grossly, Wilms’ tumor tends to present as a large, solitary, well-circumscribed mass, although 10% are either bilateral or multicentric. On cut section, the tumor is soft, homogeneous, and tan to gray with occasional foci of hemorrhage, cyst formation, and necrosis.

16. Microscopically, Wilms’ tumors are characterized by recognizable attempts to recapitulate different stages of nephrogenesis. The classic triphasic combination of blastemic, stromal, and eiphelial cell types is observed in the vast majority of lesions, although the percentage of each component is variable.

17. Most patients present with a large abdominal mass that may be unilateral or, when very large, may extend across the midline and down into the pelvis. Hematuria, pain in the abdomen after some traumatic incident, intestinal obstruction, and appearance of hypertension are other patterns of presentation.

18. 肾母细胞瘤 Nephroblastoma • 亦名：肾胚胎性腺肉瘤，Wilm氏瘤 • 来源：胚胎性肾母细胞，可分化为腺体及纤维、软骨等间叶组织；细胞分化差。其发生与抑癌基因WT1异常有关。一般见于3岁左右儿童。 • 肉眼：大结节状，境界清楚，灰白，肾组织受压萎缩，肾盂肾盏变形。 • 镜检：有腺癌和纤维肉瘤两种成份，二者均有恶性特征，有时移行。瘤细胞有时可分化成类似肾小球或肾小管的结构，有时见分化较好的软骨。

19. Urothelial (Transitional Cell) Tumorsof the Bladder

20. UROTHELIAL (TRANSITIONAL)RENAL CARCINOMAS) In renal pelvis. 1/10 as common as renal cell carcinomas EXACTLY the same appearance as lower urinary tract carcinomas. MUCH more likely to obstruct and cause hematuria early than renal (clear) cell carcinomas. Associated with ureter and bladder carcinomas.

21. Urothelial tumors represent about 90% of all bladder tumors and run the gamut from small benign lesions that may never recur to aggressive cancers associated with a high risk of death. Many of these tumors are multifocal at presentation.

22. The gross patterns vary from purely papillary to nodular or flat to mixed papillary and nodular. The tumors may also be invasive or noninvasive.

23. Grading of Urothelial (Transitional Cell) Tumors(WHO/ISUP Grades ) Urothelial papiloma Urothelial neoplasm of low malignant potential Papillary urothelial carcinoma, low grade Papillary urothelial carcinoma, high grade

24. 膀胱癌 Carcinoma of bladder • 膀胱移行细胞癌比较常见，临床上发生无痛性血尿。 • 肉眼：多呈乳头状突出表面。检查标本时需特别注意乳头基底宽或细，切面有无向下浸润。由于乳头很脆，常折断脱落，尿沉淀涂片镜检可查出癌细胞。 • 镜检：移行上皮癌，可分Ⅰ～Ⅲ级。Ⅰ级为低度恶性，Ⅱ级细胞层次明显增多，有一定异型性。Ⅲ级可不形成乳头，而是癌巢。移行上皮乳头状瘤，细胞几乎无异型性，但一般认为其极易恶变，须按癌处理。 • 临床特点：无痛性血尿;膀胱刺激症状;尿路阻塞。

25. Review Questions for Tumors of the Urinary System Describe the gross and histological characteristics of renal cell carcinoam, nephroblastoma and urothelial (transitional cell) tumors Based on renal cell carcinoma or nephroblastoma, discuss the importance of hereditary (familial) cases in the study of oncogenesis

26. Review Questions for Tumors of the Urinary Systmen Based on histological characteristics of urothelial tumor, review the concepts of atypia and grading of neoplasms