Wilms tumour affects approximately one person per 10,000 worldwide before the age of 15 years. People of African descent may have slightly higher rates of Wilms tumor. The peak age of Wilms tumour is 3 to 4 years and most cases occur before the age of 10 years.

A genetic predisposition to Wilms Tumor in individuals with aniridia has been established, due to deletions in the p13 band on chromosome 11.

A cystic nephroma, also known as multilocular cystic nephroma, mixed epithelial stromal tumour (MEST) and renal epithelial stromal tumour (REST), is a type of rare benign kidney tumour.

Dr. Sidney Farber, founder of Dana-Farber Cancer Institute, and his colleagues achieved the first remissions in Wilms tumor in the 1950s. By employing the antibiotic actinomycin D in addition to surgery and radiation therapy, they boosted cure rates from 40 to 89 percent.

Cystic nephromas are often asymptomatic. They are typically discovered on medical imaging incidentally (i.e. an incidentaloma).

10-year survival rates for mucinous tumors is excellent in the absence of invasion.

In the case of borderline tumors confined to the ovary and malignant tumors without invasion, the survival rates are 90% or greater. In invasive mucinous cystadenocarcinomas, the survival is approximately 30%

Hemangioblastomas can cause polycythemia due to ectopic production of erythropoietin as a paraneoplastic syndrome.

Congenital mesoblastic nephroma, while rare, is the most common kidney neoplasm diagnosed in the first three months of life and accounts for 3-5% of all childhood renal neoplasms. This neoplasm is generally non-aggressive and amenable to surgical removal. However, a readily identifiable subset of these kidney tumors has a more malignant potential and is capable of causing life-threatening metastases. Congenital mesoblastic nephroma was first named as such in 1967 but was recognized decades before this as fetal renal hamartoma or leiomyomatous renal hamartoma.

Perivascular epithelioid cell tumour, also known as PEComa or PEC tumour, is a family of mesenchymal tumours consisting of perivascular epithelioid cells (PECs). These are rare tumours that can occur in any part of the human body.

The cell type from which these tumours originate remains unknown. Normally, no perivascular epitheloid cells exist; the name refers to the characteristics of the tumour when examined under the microscope.

Establishing the malignant potential of these tumours remains challenging although criteria have been suggested; some PEComas display malignant features whereas others can cautiously be labeled as having 'uncertain malignant potential'. The most common tumours in the PEComa family are renal angiomyolipoma and pulmonary lymphangioleiomyomatosis, both of which are more common in patients with tuberous sclerosis complex. The genes responsible for this multi-system genetic disease have also been implicated in other PEComas.

Many PEComa types shows a female predominance in the sex ratio.

Sertoli cell tumors are known to occur in other species, including domestic ducks, dogs, and horses.

Mucinous cystadenoma is a benign cystic tumor lined by a mucinous epithelium. It is a type of cystic adenoma (cystadenoma).

Mucinous cystadenoma may arise in a number of locations; however, mucinous cystadenoma at different locations are not generally considered to be related to one another.

Based on a survey of >800, surgical removal of the entire involved kidney plus the peri-renal fat appeared curative for the majority of all types of mesoblastic nephroma; the patient overall survival rate was 94%. Of the 4% of non-survivors, half were due to surgical or chemotherapeutic treatments. Another 4% of these patients suffered relapses, primarily in the local area of surgery rare cases of relapse due to lung or bone metastasis.. About 60% of these recurrent cases had a complete remission following further treatment. Recurrent disease was treated with a second surgery, radiation, and/or chemotherapy that often vincristine and actinomycin treatment. Removal of the entire afflicted kidney plus the peri-renal fat appears critical to avoiding local recurrences. In general, patients who were older than 3 months of age at diagnosis or had the cellular form of the disease, stage III disease, or involvement of renal lymph nodes had a higher recurrence rate. Among patients with these risk factors, only those with lymph node involvement are recommended for further therapy.

It has been suggested that mesoblastic nephroma patients with lymph node involvement or recurrent disease might benefit by adding the ALK inhibitor, crizotinib, or a tyrosine kinase inhibitor, either larotrectinib or entrectinib, to surgical, radiation, and/or chemotherapy treatment regimens. These drugs inhibit NTRK3's tyrosine kinase activity. Crizotinib has proven useful in treating certain cases of acute lymphoblastic leukemia that are associated with the "ETV6-NTRK3" fusion gene while larotrectinib and entrectinib have been useful in treating various cancers (e.g. a metastatic sarcoma, papillary thyroid cancer, non-small-cell lung carcinoma, gastrointestinal stromal tumor, mammary analog secretory carcinoma, and colorectal cancer) that are driven by mutated, overly active tyrosine kinases. Relevant to this issue, a 16-month-old girl with infantile fibrosarcoma harboring the "ETV6–NTRK3" fusion gene was successfully trated with larotrectinib. The success of these drugs, howwever, will likely depend on the relative malignancy-promoting roles of ETV6-NTRK3 protein's tyrosine kinase activity, the lose of ETV6-related transcription activity accompanying formation of ETV6-NTRK3 protein, and the various trisomy chromosomes that populate mesoblastic nephroma.

The precursor cell of PEComas is currently unknown; there is no normal counterpart "perivascular epitheloid cell". Genetically, PECs are linked to the tuberous sclerosis genes TSC1 and TSC2, although this link is stronger for angiomyolipoma and lymphangioleiomyomatosis than for other members of the PEComa family.

Variants with edema can be associated with Meigs' syndrome. They may be a part of nevoid basal cell carcinoma syndrome (Gorlin syndrome).

Clear cell ovarian tumors are part of the surface epithelial-stromal tumor group of ovarian cancers, accounting for 6% of these cancers. Clear cell tumors are also associated with the pancreas and salivary glands.

The United States' NIH estimates for 2013 around 64,770 new cases of kidney cancer and 13,570 deaths from the disease.

The incidence of kidney cancer is also increasing in the United States. This is thought to be a real increase, not only due to changes in the way the disease is diagnosed.

The most recent estimates of incidence of kidney cancer suggest that there are 63,300 new cases annually in the EU25. In Europe, kidney cancer accounts for nearly 3% of all cancer cases.

A stromal tumor is a tumor that arises in the supporting connective tissue of an organ.

Fibroepithelial neoplasms (or tumors) are biphasic tumors. This means they consist of epithelial tissue, and stromal or mesenchymal tissue. They may be benign or malignant.

Examples include:

- Brenner tumor of the Ovary

- Fibroadenoma of the Breast

- Phyllodes tumor of the Breast

Phyllodes tumors are considered to be on a spectrum of disease that consists of fibroadenoma, fibroadenoma variant and benign phyllodes. Some would extend the spectrum to include malignant phyllodes tumors and frank sarcoma.

Serous tumours are part of the surface epithelial-stromal tumour group of ovarian neoplasms, which derive from Mullerian epithelium.

They are common neoplasms with a strong tendency to bilaterality, and they account for 50% of all ovarian tumours.

Sixty percent are benign (cystadenoma), 10% are borderline and 30% are malignant (cystadenocarcinoma).

The outcome for hemangioblastoma is very good, if surgical extraction of the tumor can be achieved; excision is possible in most cases and permanent neurologic deficit is uncommon and can be avoided altogether if the tumor is diagnosed and treated early. Persons with VHL syndrome have a bleaker prognosis than those who have sporadic tumors since those with VHL syndrome usually have more than one lesion.

Thecomas or theca cell tumors are benign ovarian neoplasms composed only of theca cells. Histogenetically they are classified as sex cord-stromal tumours.

They are typically estrogen-producing and they occur in older women (mean age 59; 84% after menopause). (They can, however, appear before menopause.)

60% of patients present with abnormal uterine bleeding, and 20% have endometrial carcinoma.

Mucinous tumors are part of the surface epithelial-stromal tumor group of ovarian neoplasms, and account for approximately 36% of all ovarian tumors.

Approximately 75% are benign, 10% are borderline and 15% are malignant.

Rarely, the tumor is seen bilaterally; approximately 5% of primary mucinous tumors are bilateral.

"Benign" mucinous tumors are typically multilocular (have several lobes), and the cysts have a smooth lining of epithelium that resembles endocervical epithelial cells with small numbers of gastrointestinal-type epithelial cells.

"Borderline" and "malignant" mucinous tumors often have papillae and solid areas.

There may also be hemorrhage and necrosis.

It is well documented that malignancy may be only focally present in mucinous neoplasms of the ovary, so thorough sampling is imperative.

The major distinguishing features of mucinous tumors are that the tumors are filled with a mucus-like material, which gives them their name; this mucus is produced by mucus-secreting goblet cells very similar to the cells lining normal intestine.

These tumors may become very large, some have been weighed as large as 25 kilograms.

Cystadenocarcinomas (malignant tumors) contain a more solid growth pattern with the hallmarks of malignancy: cellular atypia and stratification, loss of the normal architecture of the tissue, and necrosis. The appearance can look similar to colonic cancer.

Clear stromal invasion is used to differentiate borderline tumors from malignant tumors.

Pseudomyxoma peritonei may present as a result of an ovarian mucinous tumor, however this is a rare cause of this condition, which is a rare condition. A more common cause of pseudomyxoma peritonei is a mucin-producing tumor of the appendix.

Since mucinous tumors arising from the ovary usually only involve one ovary, the presence of involvement in both ovaries with a mucinous tumor suggests that the tumor may have arisen in another location, and further study is warranted.

The risk of mucinous tumors is significantly associated with smoking: relative risk for current smokers 2.22 (2.22 times the risk for non-smokers) and 2.02 for past smokers. Risk is also associated with smoking duration: relative risk per 20 years was 1.44. See article by Tworoger SS in Cancer March 1, 2008 using data from the Nurses Health Study.

A Sertoli cell tumour, also Sertoli cell tumor (US spelling), is a sex cord-gonadal stromal tumor of a Sertoli cells. They are very rare and generally peak between the ages of 35 and 50. They are typically well-differentiated, and are commonly misdiagnosed as seminomas as they often appear very similar.

A tumor that produces both Sertoli cells and Leydig cells is known as a Sertoli-Leydig cell tumor.

A prospective study of ovarian sex cord–stromal tumours in children and adolescents began enrolling participants in 2005.