Renal cell carcinoma
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Steven C. Campbell, M.D., Ph.D.
Contributors: C. Michael Gibson, M.S., M.D., Cafer Zorkun M.D., PhD. Michael Maddaleni, B.S.
Synonyms and key Words: RCC, renal cell CA, kidney cancer, kidney carcinoma, kidney CA, Grawitz tumor, hypernephroma
Overview
Historical Perspective
Renal cell carcinoma was first described in 1826. Following contradictory hypotheses regarding the origin of renal tumors, it was not until 1960 that Oberling and colleagues showed that renal carcinomas originate from renal cells.
Classification
Classification of renal cell carcinomas according to histopathological subtypes is based on the 1997 classification by Heidelberg and colleagues. Classification may also be according to the stage of renal cell carcinoma, which often is based on Robson classification system or the tumor-lymph node-metastasis (TNM) system.
Pathophysiology
The pathophysiology of renal cell carcinomas plays an important role in differentiating different types of renal cell carcinomas and in choosing appropriate targeted medical therapies. Sporadic forms of clear cell renal carcinomas, the most common form of renal cell carcinomas, have similar pathophysiological mechanisms to those of von Hippel Lindau (VHL) disease. On the other hand, MET proto-oncogene seems to play a role in the disease pathogenesis of papillary forms of renal cell carcinoma. Uniquely also, oncocytomas are benign tumors that arise from type A intercalated cells, whereas chromophobe renal cell carcinoma arises from type B intercalated cells.
Causes
Most renal cell carcinomas develop sporadically. Only a few have genetic predisposition and are associated with known syndromes, such as von Hippel Lindau (VHL), hereditary paragangliomas, leiomyomatosis, and Birt-Hogg-Dube (BHD) syndrome, among several others.
Differentiating Renal cell carcinoma from other Diseases
The differential diagnosis of renal cell carcinomas includes metastastic disease, cysts, abscesses, lymphomas, and other benign and malignant tumors, and associated syndromes.
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
The projected 5-year survival is significantly associated with the stage of the tumor at diagnosis. Stage I tumors are associated with more than 90% 5-year survival vs. less than 20% survival for tumors of stage IV. The potential aggression of a tumor has been shown to be associated with tumor size, grade of tumor, and histopathological subtype. High grade tumors greater than 7 cm of clear cell type generally have a higher aggressive potential than low grade tumors smaller than 3 cm of papillary type. Finally, complications vary according to the local extension of the tumor, and the presence of paraneoplastic syndromes and/or metastases.
Diagnosis
Staging | History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Medical Therapy | Surgery | Primary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
Case Studies
Related Chapters
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