Kidney tumours

The most common renal tumor in childhood, nephroblastoma was first classified as a renal neoplasm by Rance in 1814. The German surgeon Max Wilms (1867 - 1918) described the tumor in detail in his monograph "Die Mischgeschwülste der Niere" in 1899. Since then, nephroblastoma has also been referred to as Wilms' tumor (Graf 2006).

A renal tumor is a growth in the area of the kidney that can be benign or malignant, unilateral or bilateral.

Benign renal tumors:

• Mesenchymal tumors such as:
• Lipomas
• Fibromas
• Myomas
• epithelial such as:
• epithelial adenoma
• oncocytoma (Herold 2020)
• Mixed tumors such as:
• Angiomyolipoma (Staehler 1997)

Malignant renal tumors (Herold 2020 / Kasper 2015 / Doehn 2017 / Pokop 2007)

• Renal cell carcinoma
• clear cell
• papillary
• chromophobe
• oncocytoma (considered rather benign)
• ductal Bellini carcinoma
• tubulocystic
• clear cell papillary
• translocation-associated
• associated with acquired cystic kidney disease
• associated with hereditary leiomyomatosis
• Nephroblastoma
• Renal metastases

Age-related tumors (occurring in childhood -von Schweinitz 2019):

• Nephroblastoma
• Clear cell sarcoma
• Renal cell carcinoma
• Rhabdoid tumor
• mesoblastic nephroma

Renal cell carcinoma: Renal cell carcinoma is the most common renal tumor in adults, accounting for 90%. The incidence of renal cell carcinoma increases with age. The peak incidence of the disease is between 50 - 75 years Kasper 2015).

Hereditary papillary renal cell carcinoma (HPRCC): HPRCC is a very rare renal tumor, more detailed figures are not available (Doehn 2017).

Nephroblastoma: Nephroblastoma is a rare but typical tumor of childhood. The incidence is 1 in 100,000 children < 15 years of age (Graf 2016). Nephroblastoma accounts for approximately 6% of all malignancies in children < 15 years of age (von Schweinitz 2019). The age of onset is between 1 - 4 years of age. In adults, the tumor occurs extremely rarely (von Schweinitz 2019). According to the guideline (Graf 2016), girls develop the disease more frequently than boys, while Manski (2020) describes the gender ratio as balanced.

Rhabdoid tumor: This typically occurs in children < 1 year of age (von Schweinitz 2019).

Mesoblastic nephroma: Mesoblastic nephroma occurs predominantly in the first 3 months of life (von Schweinitz 2019).

Oncocytoma: Oncocytoma is found rather rarely with 5% - 10% (Kasper 2015) and occurs predominantly in older age (Vogl 2011).

Angiomyolipoma: Angiomyolipoma occurs in 0.3 % - 0.1 % of cases and predominantly affects women. The peak age is between 50 - 60 years. In patients with tuberous cerebral sclerosis (an autosomal-dominantly inherited phakomatosis [Pantelis 2007]), bilateral angiomyolipoma is found with a frequency of 45 % - 80 % (Manski 2020).

The vast majority of renal tumors are found unilaterally. Bilateral tumours can occur with an incidence of 1.6 % - 6 % (Siemer 2001).

Nephroblastoma: Nephroblastoma usually occurs unilaterally, but in 5 % - 10 % also bilaterally (von Schweinitz 2019).

Clear cell sarcoma: Clear cell sarcoma is almost always found unilaterally (von Schweinitz 2019).

Hereditary papillary renal cell carcinoma (HPRCC): HPRCC always occurs bilaterally (Doehn 2017).

Renal metastases: Metastases to the kidneys occur bilaterally in 50%. They originate in decreasing frequency from the following primary tumors (Prokop 2007):

• Bronchial CA
• Mamma- CA
• Colon CA
• gastric CA
• Cervical CA
• Ovarian CA
• Pancreas- CA
• Prostate CA
• Skin tumor
• Tumor of the contralateral kidney

Further details regarding clinical symptoms, diagnosis, differential diagnosis, therapy and prognosis of the individual tumors differ greatly and will not be discussed further here (see corresponding clinical picture).

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