Renal cell carcinoma

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Renal cell carcinoma Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Renal cell carcinoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural history, Complications and Prognosis

Diagnosis

Staging

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Case #1

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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

Renal cell carcinoma is considered the 7th most common cancer in men and 9th most common cancer in women, accounting for approximately 2-3% of malignant tumors in adults. Men are twice more likely to be affected than women with an mean age of presentation at 60 years. Most renal cell carcinomas develop sporadically, while only a small number of cases are of genetic diseases. Clear cell carcinomas are the most common renal cell carcinomas and collecting-duct carcinomas are the least common. Renal cell carcinomas are mostly found incidentally during radiological abdominal work-up.

Risk Factors

Established risk factors for renal cell carcinoma include cigarette smoking, hypertension, end-stage renal failure, analgesic drug use, environmental exposure to carcinogens, and some genetic syndromes.

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

Case #1

Related Chapters

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