Wilms' tumor

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Wilms' Tumor

 

Definition

Wilms' tumor is a cancerous tumor of the kidney that usually occurs in young children. It is named for Max Wilms, a German surgeon (1867–1918) and is also known as a nephroblastoma.

Description

When an unborn baby is developing, the kidneys are formed from primitive cells. Over time, these cells become more specialized. The cells mature and organize into the normal kidney structure. Sometimes, clumps of these cells remain in their original, primitive form. If these cells begin to multiply after birth, they may ultimately form a large mass of abnormal cells. This is known as a Wilms' tumor.
Wilms' tumor is a type of malignant tumor. This means that it is made up of cells that are significantly immature and abnormal. These cells are also capable of invading nearby structures within the kidney and traveling out of the kidney into other structures. Malignant cells can even travel through the body to invade other organ systems, most commonly the lungs and brain. These features of Wilms' tumor make it a type of cancer that, without treatment, would eventually cause death. However, advances in medicine during the last 20 years have made Wilms' tumor a very treatable form of cancer.
Wilms' tumor occurs almost exclusively in young children. The average patient is about three years old, although cases have been reported in infants younger than six months and adults in their early twenties. Females are only slightly more likely than males to develop Wilms' tumors. In the United States, Wilms' tumor occurs in 8.3 individuals per million in white children under the age of 15 years. The rate is higher among African-Americans and lower among Asian-Americans. Wilms' tumors are found more commonly in patients with other types of birth defects. These defects include:
  • absence of the colored part (the iris) of the eye (aniridia)
  • enlargement of one arm, one leg, or half of the face (hemihypertrophy)
  • certain birth defects of the urinary system or genitals
  • certain genetic syndromes (WAGR syndrome, Denys-Drash syndrome, and Beckwith-Wiedemann syndrome)

Causes and symptoms

The cause of Wilms' tumor is not completely understood. Because 15% of all patients with this type of tumor have other heritable defects, it seems clear that at least some cases of Wilms' tumor are due to an inherited alteration. A genetic defect known as WT1, the Wilms' tumor suppressor gene, has been identified in some patients on chromosome 11. It
Wilm's tumor.
Wilm's tumor.
(Illustration by Argosy, Inc.)
appears that the tendency to develop a Wilms' tumor can run in families. In fact, about 1.5% of all children with a Wilms' tumor have family members who have also had a Wilms' tumor. The genetic mechanisms associated with the disease are unusually complex; it is thought as of 2004 that the tumor develops because the defective WT1 gene fails to stop its growth. Other genes that have been linked to Wilms' tumor are located on chromosomes 16q, 7p15, and 17q12.
Some patients with Wilms' tumor experience abdominal pain, nausea, vomiting, high blood pressure, or blood in the urine. However, the parents of many children with this type of tumor are the first to notice a firm, rounded mass in their child's abdomen. This discovery is often made while bathing or dressing the child and frequently occurs before any other symptoms appear. Rarely, a Wilms' tumor is diagnosed after there has been bleeding into the tumor, resulting in sudden swelling of the abdomen and a low red blood cell count (anemia).
About 4-5% of Wilms' tumor cases involve both kidneys during the initial evaluation. The tumor appears on either side equally. When pathologists look at these tumor cells under the microscope, they see great diversity in the types of cells. Some types of cells are associated with a more favorable outcome in the patient than others. In about 15% of cases, physicians find some degree of cancer spread (metastasis). The most common sites in the body where metastasis occurs are the liver and lungs.
Researchers have found evidence that certain types of lesions occur before the development of the Wilms' tumor. These lesions usually appear in the form of stromal, tubule, or blastemal cells.

Diagnosis

Children with Wilms' tumor generally first present to physicians with a swollen abdomen or with an obvious abdominal mass. The physician may also find that the child has fever, bloody urine, or abdominal pain. The physician will order a variety of tests before imaging is performed. These tests mostly involve blood analysis in the form of a white blood cell count, complete blood count, platelet count, and serum calcium evaluation. Liver and kidney function testing will also be performed as well as a urinalysis.
Initial diagnosis of Wilms' tumor is made by looking at the tumor using various imaging techniques. Ultrasound and computed tomography scans (CT scans) are helpful in diagnosing Wilms' tumor. Intravenous pyelography, where a dye injected into a vein helps show the structures of the kidney, can also be used in diagnosing this type of tumor. Final diagnosis, however, depends on obtaining a tissue sample from the mass (biopsy), and examining it under a microscope in order to verify that it has the characteristics of a Wilms' tumor. This biopsy is usually done during surgery to remove or decrease the size of the tumor. Other studies (chest x rays, CT scan of the lungs, bone marrow biopsy) may also be done in order to see if the tumor has spread to other locations.

Treatment

In the United States, treatment for Wilms' tumor almost always begins with surgery to remove or decrease the size of the kidney tumor. Except in patients who have tumors in both kidneys, this surgery usually will require complete removal of the affected kidney. During surgery, the surrounding lymph nodes, the area around the kidneys, and the entire abdomen will also be examined. While the tumor can spread to these surrounding areas, it is less likely to do so compared to other types of cancer. In cases where the tumor affects both kidneys, surgeons will try to preserve the kidney with the smaller tumor by removing only a portion of the kidney, if possible. Additional biopsies of these areas may be done to see if the cancer has spread. The next treatment steps depend on whether/where the cancer has spread. Samples of the tumor are also examined under a microscope to determine particular characteristics of the cells making up the tumor.
Information about the tumor cell type and the spread of the tumor is used to decide the best kind of treatment for a particular patient. Treatment is usually a combination of surgery, medications used to kill cancer cells (chemotherapy), and x rays or other high-energy rays used to kill cancer cells (radiation therapy). These therapies are called adjuvant therapies, and this type of combination therapy has been shown to substantially improve outcome in patients with Wilms' tumor. It has long been known that Wilms' tumors respond to radiation therapy. Likewise, some types of chemotherapy have been found to be effective in treating Wilms' tumor. These effective drugs include dactinomycin, doxorubicin, vincristine, and cyclophosphamide. In rare cases, bone marrow transplantation may be used.
The National Wilms' Tumor Study Group (NWTSG) has developed a staging system to describe Wilms' tumors. All of the stages assume that surgical removal of the tumor has occurred. Stage I involves "favorable" Wilms' tumor cells and is usually treated successfully with combination chemotherapy involving dactinomycin and vincristine and without abdominal radiation therapy. Stage II tumors involving a favorable histology (cell characteristics) are usually treated with the same therapy as Stage I. Stage III tumors with favorable histology are usually treated with a combination chemotherapy with doxorubicin, dactinomycin, and vincristine along with radiation therapy to the abdomen. Stage IV disease with a favorable histology is generally treated with combination chemotherapy with dactinomycin, doxorubicin, and vincristine. These patients usually receive abdominal radiation therapy and lung radiation therapy if the tumor has spread to the lungs.
In the case of Stage II through IV tumors with unfavorable, or anaplastic, cells, then the previously-mentioned combination chemotherapy is used along with the drug cyclophosphamide. These patients also receive lung radiation therapy if the tumor has spread to the lungs. Another type of tumor cell can be present in Stages I through IV. This cell type is called clear cell sarcoma of the kidney. If this type of cell is present, then patients receive combination therapy with vincristine, doxorubicin, and dactinomycin. All of these patients receive abdominal radiation therapy and lung radiation therapy if the tumor has spread to the lungs.
As of 2004, there are significant differences between the treatment protocols of the NWTSG and its European counterpart, the Société Internationale d'Oncologie Pédiatrique (SIOP). Whereas American practice favors surgery followed by chemotherapy, European oncologists use preoperative chemotherapy and stage the tumor at the time of surgery rather than at the point of initial imaging studies.

Key terms

Biopsy — A procedure in which a small sample of tissue is removed, prepared, and examined with a microscope to determine the characteristics of the tissue's cells.
Blastemal — An immature material from which cells and tissues develop.
Cancer — A process where abnormal cells within the body begin to grow out of control, acquire the ability to invade nearby structures, and travel through the bloodstream in order to invade distant structures.
Malignant — Refers to cancer or cancer cells.
Sarcoma — A type of cancer that originates from connective tissue such as bone or muscle.
Stromal — A type of tissue that is associated with the support of an organ.
Tubule — Tissues and cells associated with the structures that connect the renal pelvis to the glomeruli.

Prognosis

The prognosis for patients with Wilms' tumor is quite good, compared to the prognosis for most types of cancer. One German study reported the overall five-year survival rate to be 89.5%. The patients who have the best prognosis are usually those who have a small-sized tumor, a favorable cell type, are young (especially under two years old), and have an early stage of cancer that has not spread. Modern treatments have been especially effective in the treatment of this cancer. Patients with the favorable type of cell have a long-term survival rate of 93%, whereas those with anaplasia have a long-term survival rate of 43% and those with the sarcoma form have a survival rate of 36%.

Prevention

There are no known ways to prevent a Wilms' tumor, although it is important that children with birth defects associated with Wilms' tumor be carefully monitored.

Resources

Books

Beers, Mark H., MD, and Robert Berkow, MD., editors. "Wilms' Tumor (Nephroblastoma)." In The Merck Manual of Diagnosis and Therapy. Whitehouse Station, NJ: Merck Research Laboratories, 2004.
Black, Timothy L. "Wilms' Tumor." In Dambro: Griffith's 5-Minute Clinical Consult. Philadelphia: J.B. Lippincott, 1999.
DeVita, Vincent J., et al., editors. "Wilms' Tumor." In Cancer: Principles and Practice of Oncology. Philadelphia: J. B. Lippincott Company, 2001.

Periodicals

Boglino, C., A. Inserra, S. Madafferi, et al. "A Single-Institution Wilms' Tumor and Localized Neuroblastoma Series." Acta Paediatrica Supplementum 93 (May 2004): 74-77.
Coppes, Max J., and Kathy Pritchard-Jones. "Principles of Wilms' Tumor Biology." Urologic Clinics of North America 27 (August 2000).
Emerson, R. E., T. M. Ulbright, S. Zhang, et al. "Nephroblastoma Arising in a Germ Cell Tumor of Testicular Origin." American Journal of Surgical Pathology 28 (May 2004): 687-692.
Glick, R. D., M. J. Hicks, J. G. Nuchtern, et al. "Renal Tumors in Infants Less Than 6 Months of Age." Journal of Pediatric Surgery 39 (April 2004): 522-525.
Neville, Holly L., and Michael L. Ritchey. "Wilms' Tumor." Urologic Clinics of North America 27 (August 2000).
Weirich, A., R. Ludwig, N. Graf, et al. "Survival in Nephroblastoma Treated According to the Trial and Study SIOP-9/GPOH with Respect to Relapse and Morbidity." Annals of Oncology 15 (May 2004): 808-820.

Organizations

American Cancer Society. 1515 Clifton Rd. NE, Atlanta, GA 30329. (800) 227-2345. http://www.cancer.org.
March of Dimes Birth Defects Foundation, National Office. 1275 Mamaroneck Ave., White Plains, NY 10605. http://www.modimes.org.
National Cancer Institute (National Institutes of Health). 9000 Rockville Pike, Bethesda, MD 20892. (800) 422-6237. 〈http://www.nci.nih.gov\〉.
National Wilms Tumor Study Group (NWTSG). Fred Hutchinson Cancer Research Center, 1100 Fairview Avenue North, P. O. Box 19024, Seattle, WA 98109-1024. (800) 553-4878. Fax: (206) 667-6623. http://www.nwtsg.org.

Other

Online Mendelian Inheritance in Man (OMIM). #194070. "Wilms Tumor 1; WT1." http://www3.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=194070.

tumor

 [too´mor]
1. swelling or morbid enlargement; this is one of the cardinal signs of inflammation.
2. a new growth of tissue in which cell multiplication is uncontrolled and progressive. Tumors are also called neoplasms, which means that they are composed of new and actively growing tissue. Their growth is faster than that of normal tissue, continuing after cessation of the stimuli that evoked the growth, and serving no useful physiologic purpose. adj., adj tu´morous.

Tumors are classified in a number of ways, one of the simplest being according to their origin and whether they are malignant or benign. Tumors of mesenchymal origin include fibroelastic tumors and those of bone, fat, blood vessels, and lymphoid tissue; they may be benign or malignant (sarcoma). Tumors of epithelial origin are found in glandular tissue and such organs as the breast, stomach, uterus, or skin; they also may be either benign or malignant (carcinoma). Mixed tumors contain different types of cells derived from the same primary germ layer, and teratomas contain cells derived from more than one germ layer; both kinds may be benign or malignant.
Benign Tumors. Benign tumors do not endanger life unless they interfere with normal functions of other organs or affect a vital organ. They grow slowly, pushing aside normal tissue but not invading it. They are usually encapsulated, well demarcated growths. They are not metastatic; that is, they do not form secondary tumors in other organs. Benign tumors usually respond favorably to surgical treatment and some forms of radiation therapy.
Malignant Tumors. These tumors are composed of embryonic, primitive, or poorly differentiated cells. They grow in a disorganized manner and so rapidly that nutrition of the cells becomes a problem. For this reason necrosis and ulceration are characteristic of malignant tumors. They also invade surrounding tissues and are metastatic, initiating the growth of similar tumors in distant organs. (See also cancer.)
Gross appearance of benign (A) and malignant (B) tumors. From Damjanov, 2000.
benign tumor one that lacks the properties of invasiveness and metastasis and that is usually surrounded by a fibrous capsule; its cells also show a lesser degree of anaplasia than those of a malignant tumor do.
bladder tumor a tumor of the urinary bladder; see also bladder cancer.
brain tumor see brain tumor.
brown tumor a giant-cell granuloma produced in and replacing bone, occurring in osteitis fibrosa cystica and due to hyperparathyroidism.
Burkitt's tumor Burkitt's lymphoma.
Buschke-Löwenstein tumor a slow-growing mass of warts found usually in the prepuce but sometimes elsewhere in the perianal region; it starts as a plaque and may grow into a large cauliflowerlike mass. Called also giant condyloma.
carcinoid tumor carcinoid (def. 1).
carotid body tumor a chemodectoma of a carotid body, found as a firm round mass at the bifurcation of the common carotid artery.
connective tissue tumor any tumor arising from a connective tissue structure, such as a fibroma or sarcoma.
desmoid tumor desmoid (def. 1).
endodermal sinus tumor yolk sac tumor.
erectile tumor cavernous hemangioma.
Ewing's tumor Ewing's sarcoma.
false tumor pseudotumor.
fibroid tumor
germ cell tumor any of a group of tumors arising from primitive germ cells, usually of the testis or ovum; they range from benign to highly malignant. Types include germinoma, yolk sac tumor, teratoma, embryonal carcinoma, and some types of choriocarcinoma; many tumors are mixtures of types.
giant cell tumor
1. a benign or malignant tumor containing giant cells; see under carcinoma, granuloma, and sarcoma.
2. a bone tumor, ranging from benign to frankly malignant, composed of cellular spindle cell stroma containing multinucleated giant cells resembling osteoclasts.
3. a small yellow benign tumorlike nodule of tendon sheath origin, usually of the wrist and fingers or ankle and toes, laden with lipophages and containing multinucleated giant cells.
glomus tumor
1. a blue-red, extremely painful chemodectoma involving an arteriovenous anastomosis or cluster of blood cells, which may be found anywhere in the skin, most often in the distal portion of the fingers and toes, especially beneath the nail. Such tumors may also occur in the stomach and nasal cavity.
granular cell tumor a relatively common neoplasm whose cells have a granular appearance by light microscopy; it is usually benign but occasionally malignant, and multiple tumors may occur. It can be found anywhere but is most often seen in the oral cavity, especially in the tongue.
granulosa tumor (granulosa cell tumor) see granulosa cell tumor.
granulosa-theca cell tumor see granulosa-theca cell tumor.
heterologous tumor one made up of tissue differing from that in which it grows.
homoiotypic tumor (homologous tumor) one made up of tissue resembling that in which it grows.
Hürthle cell tumor see hürthle cell tumor.
islet cell tumor a tumor of the islands of Langerhans; many secrete excessive amounts of hormones. Types include gastrinoma, glucagonoma, insulinoma, somatostatinoma, and vipoma.
Krukenberg's tumor see krukenberg's tumor.
lipoid cell tumor of ovary a usually benign ovarian tumor composed of eosinophilic cells or cells with lipoid vacuoles; it causes masculinization.
tumor lysis syndrome severe hyperphosphatemia, hyperkalemia, hyperuricemia, and hypocalcemia occurring after effective induction chemotherapy of rapidly growing malignant neoplasms; thought to be due to release of intracellular products after cell lysis.
malignant tumor one that has the properties of invasiveness and metastasis and that shows a greater degree of anaplasia than a benign tumor does.
mast cell tumor mastocytoma.
melanotic neuroectodermal tumor a benign, rapidly growing, dark tumor of the jaw or occasionally some other site, almost always seen in infants; called also melanoameloblastoma.
mixed tumor one composed of more than one type of neoplastic tissue.
tumor necrosis factor receptor–associated periodic syndrome (TRAPS) familial periodic fever.
organoid tumor teratoma.
peripheral neuroectodermal tumor (PNET) any of a heterogeneous group of neoplasms originating in supporting structures or neuronal tissue, primarily of the extremities, pelvis, or chest wall; seen most often in adolescents and young adults and frequently having widespread metastases.
plasma cell tumor
1. plasma cell dyscrasias.
sand tumor psammoma.
Sertoli-Leydig cell tumor androblastoma (def. 1).
theca cell tumor a fibroidlike tumor of the ovary containing yellow areas of fatty material derived from theca cells.
turban t's multiple cylindromas of the scalp that are grouped together so as to cover the entire scalp.
Wilms' tumor a rapidly developing malignant mixed tumor of the kidneys, made up of embryonal elements, occurring chiefly in children before the seventh year; a genetic component is suspected in its etiology. It may be accompanied by congenital defects such as urinary tract abnormalities, absent iris of the eye, and asymmetry of parts. With treatment, the prognosis is excellent. Called also embryonal carcinosarcoma and nephroblastoma.
yolk sac tumor a malignant germ cell tumor of children that represents a proliferation of both yolk sac endoderm and extraembryonic mesenchyme. It produces α-fetoprotein and most often occurs in the testes, but is also seen in the ovaries and some extragonadal sites. Called also endodermal sinus tumor.

Wilms' tumor

(wĭlmz)
n.
A malignant tumor of the kidney that is associated with certain genetic abnormalities and chiefly affects young children. Also called nephroblastoma.

Wilms' tumor

[vilms]
Etymology: Max Wilms, German surgeon, 1867-1918
a malignant neoplasm of the kidney occurring in young children before the fifth year in 75% of the cases. It is slightly more common among females than males and among African-American children than Caucasian children. The most frequent early signs are hypertension, a palpable mass, pain, and hematuria. Diagnosis can be established by an excretory urogram with tomography. The tumor, an embryonal adenomyosarcoma, is well encapsulated in the early stage, but it may extend into lymph nodes, the renal vein, or the vena cava and metastasize to the lungs or other sites. Removal of resectable tumors by transperitoneal nephrectomy is recommended. Radiotherapy is used before or after surgery or palliatively in inoperable cases. Chemotherapy combined with surgery and irradiation is proving highly effective. Also called adenomyosarcoma, nephroblastoma. See also kidney cancer.
enlarge picture
Wilms' tumor

Wilms' tumor

Nephroblastoma Oncology An embryonal renal tumor that arises in aberrant mesenchymal renal stem cell lines, coupled with loss of functioning tumor suppressor genes; WT comprises 92% of 1º childhood renal CAs–frequency: 1:10,000–the rest are sarcomas Clinical WT presents as a mass, often with HTN 60%, hematuria 24%, nephritis–Wilms' nephritis, serosal effusions–ascites, pleural effusions; 5% are bilateral; anaplasia connotes a poor prognosis. See WAGR syndrome.
Wilms' tumor staging
  I  CA in kidney only; can be completely removed by surgery
 II  CA spread to tissue near kidney, to blood vessels, or to the renal sinus–a part of the kidney through which blood and fluid enter and exit; CA can be completely removed by surgery
III CA has spread to tissues near the kidney; cannot be completely removed by surgery. CA may have spread to blood vessels or organs near the kidney or throughout the abdomen and/or to regional lymph nodes
IV CA spread to organs distant from kidney–eg, lungs, liver, bone, brain
 V CA in both kidneys

Wilms' tumor

a rapidly developing malignant mixed tumor of the kidneys, made up of embryonal elements, and occurring chiefly in young pigs and chickens; called also embryonal nephroma, nephroblastoma.