Wilms' tumor
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| Wilms' tumor Classification and external resources | |
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| ICD-10 | C64. |
| ICD-9 | 189.0 |
| ICD-O: | M8960/3 |
| OMIM | 194070 607102 |
| DiseasesDB | 8896 |
| eMedicine | med/3093 ped/2440 |
| MeSH | D009396 |
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Editor-In-Chief: Steven C. Campbell, M.D., Ph.D., Professor of Surgery, Residency Program Director, Section of Urologic Oncology, Glickman Urological and Kidney Institute, Cleveland Clinic. You can email Dr. Campbell by clicking here. Office phone: 216-444-5595.
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Overview
Wilms' tumor or nephroblastoma is a tumor of the kidneys that typically occurs in children, rarely in adults. Its common name is an eponym, referring to Dr. Max Wilms, the German surgeon (1867-1918) who first described this kind of tumor.[1]
Approximately 500 cases are diagnosed in the U.S. annually. The majority (75%) occur in otherwise normal children; a minority (25%) is associated with other developmental abnormalities. It is highly responsive to treatment, with about 90% of patients surviving at least five years.
Pathology
Pathologically, a triphasic nephroblastoma comprises three elements:
Wilms' tumor is a malignant tumor containing metanephric blastema, stromal and epithelial derivatives. Characteristic is the presence of abortive tubules and glomeruli surrounded by a spindled cell stroma. The stroma may include striated muscle, cartilage, bone, fat tissue, fibrous tissue. The tumor is compressing the normal kidney parenchyma. Pathology images
The mesenchymal component may include cells showing rhabdomyoid differentiation. The rhabdomyoid component may itself show features of malignancy (rhabdomyosarcomatous Wilms).
Wilms tumor may be separated into 2 prognostic groups based on pathologic characteristics:
- Favorable - Contains well developed components mentioned above
- Anaplastic - Contains diffuse anaplasia (poorly developed cells)
Molecular biology
Mutations of the WT1 gene on chromosome 11 are observed in approximately 20% of Wilms' tumors.[2][3] At least half of the Wilms' tumors with mutations in WT1 also carry mutations in CTNNB1, the gene encoding the proto-oncogene beta-catenin.[4]
A gene on the X chromosome, WTX, is inactivated in up to 30% of Wilms' tumor cases, according to research published in 2007.[5]
Diagnosis
CT
Staging and treatment
Staging is determined by combination of imaging studies, and pathologic findings if the tumor is operable (adapted from www.cancer.gov). Treatment strategy is determined by the stage:
Stage I (43% of patients)
For stage I Wilms' tumor, 1 or more of the following criteria must be met:
- Tumor is limited to the kidney and is completely excised.
- The surface of the renal capsule is intact.
- The tumor is not ruptured or biopsied (open or needle) prior to removal.
- No involvement of renal sinus vessels.
- No residual tumor apparent beyond the margins of excision.
Treatment: Nephrectomy + 18 weeks of chemotherapy
Outcome: 98% 4-year survival; 85% 4-year survival if anaplastic
Stage II (23% of patients)
For Stage II Wilms' tumor, 1 or more of the following criteria must be met:
- Tumor extends beyond the kidney but is completely excised.
- No residual tumor apparent at or beyond the margins of excision.
- Any of the following conditions may also exist:
- Tumor involvement of the blood vessels of the renal sinus and/or outside the renal parenchyma.
- The tumor has been biopsied prior to removal or there is local spillage of tumor during surgery, confined to the flank.
Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy
Outcome: 96% 4-year survival; 70% 4-year survival if anaplastic
Stage III (23% of patients)
For Stage III Wilms' tumor, 1 or more of the following criteria must be met:
- Unresectable primary tumor.
- Lymph node metastasis.
- Positive surgical margins.
- Tumor spillage involving peritoneal surfaces either before or during surgery, or transected tumor thrombus.
Treatment: Abdominal radiation + 24 weeks of chemotherapy + nephrectomy after tumor shrinkage
Outcome: 95% 4-year survival; 56% 4-year survival if anaplastic
Stage IV (10% of patients)
Stage IV Wilms' tumor is defined as the presence of hematogenous metastases (lung, liver, bone, or brain), or lymph node metastases outside the abdomenopelvic region.
Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy + radiation of metastatic site as appropriate
Outcome: 90% 4-year survival; 17% 4-year survival if anaplastic
Stage V (5% of patients)
Stage V Wilms’ tumor is defined as bilateral renal involvement at the time of initial diagnosis. Note: For patients with bilateral involvement, an attempt should be made to stage each side according to the above criteria (stage I to III) on the basis of extent of disease prior to biopsy. The 4-year survival was 94% for those patients whose most advanced lesion was stage I or stage II; 76% for those whose most advanced lesion was stage III.
Treatment: Individualized thereapy based on tumor burden
Stage I-IV Anaplasia
Children with stage I anaplastic tumors have an excellent prognosis (80-90% five-year survival). They can be managed with the same regimen given to stage I favorable histology patients.
Children with stage II through stage IV diffuse anaplasia, however, represent a higher-risk group. These tumors are more resistant to the chemotherapy traditionally used in children with Wilms’ tumor (favorable histology), and require more aggressive regimens.
Treatment
Once a kidney tumor is found, surgery can find out whether or not the tumor is cancer. A sample of tissue from the tumor is sent to a pathologist, who looks at it under a microscope to check for signs of cancer. If the tumor is only in the kidney, it can be removed along with the whole kidney (a process called nephrectomy). If there are tumors in both kidneys or if the tumor has spread outside the kidney, a piece of the tumor will be removed.
See also
External links
- Metzger ML, Dome JS (2005). "Current therapy for Wilms' tumor". Oncologist 10 (10): 815–26. doi:10.1634/theoncologist.10-10-815. PMID 16314292.
- Information from National Cancer Institute
- Information from WebMD
- Information from the Mayo Clinic
- Information from Sydney Children's Hospital (Australia)
- Photos from Atlas of Pathology
- List of additional resources compiled by the NIH
- Wilms' tumor at the Open Directory Project
References
- ↑ WhoNamedIt.com: Max Wilms
- ↑ Call K, Glaser T, Ito C, Buckler A, Pelletier J, Haber D, Rose E, Kral A, Yeger H, Lewis W (1990). "Isolation and characterization of a zinc finger polypeptide gene at the human chromosome 11 Wilms' tumor locus". Cell 60 (3): 509-20. PMID 2154335.
- ↑ Huff V (1998). "Wilms tumor genetics". Am J Med Genet 79 (4): 260-7. PMID 9781905.
- ↑ Maiti S, Alam R, Amos CI, Huff V (2000). "Frequent association of beta-catenin and WT1 mutations in Wilms tumors". Cancer Res 60 (22): 6288-92. PMID 11103785.
- ↑ Rivera M, Kim W, Wells J, Driscoll D, Brannigan B, Han M, Kim J, Feinberg A, Gerald W, Vargas S, Chin L, Iafrate A, Bell D, Haber D (2007). "An X chromosome gene, WTX, is commonly inactivated in Wilms tumor". Science 315 (5812): 642-5. PMID 17204608.
Pathology: Cancer, Tumors, Neoplasms, and oncology (C00-D48, 140-239) | |
|---|---|
| Benign tumors | Hyperplasia - Cyst - Pseudocyst - Hamartoma - Benign tumor |
| Malignant progression | Dysplasia - Carcinoma in situ - Cancer - Metastasis |
| Topography | lip, oral cavity and pharynx: Oral - Head/Neck - Nasopharyngeal
digestive system: tract (Esophagus, Stomach, Small intestine, Colon/rectum, Appendix, Anus) - glands (Liver, Bile duct, Gallbladder, Pancreas) respiratory system: Larynx - Lung bone, articular cartilage, skin, and connective tissue: Bone (Tailbone) - Skin - Blood urogenital: breast and female genital organs (Breast, Vagina, Cervix, Uterus, Endometrium, Ovaries) - male genital organs (Penis, Prostate, Testicles) - urinary organs (Kidney, Bladder) nervous system: Eye (Uvea) - Brain (Choroid plexus) endocrine system: Thyroid (Papillary, Follicular, Medullary, Anaplastic) - Adrenal tumor (Adrenocortical carcinoma, Pheochromocytoma) - Pituitary |
| Misc. | Tumor suppressor genes/oncogenes - Staging/grading - Carcinogenesis - Carcinogen - Research - Paraneoplastic syndrome - List of oncology-related terms |
Soft tissue tumors and sarcomas (ICD-O 8800-9349) | |
|---|---|
| Not otherwise specified (8800-8809) | Soft tissue sarcoma - Desmoplastic small round cell tumor |
| Fibromatous (8810-8839) | Fibroma/fibrosarcoma - Malignant fibrous histiocytoma - Dermatofibroma/dermatofibrosarcoma - Dermatofibrosarcoma protuberans |
| Myxomatous (8840-8849) | Myxoma - Ossifying fibromyxoid tumour |
| Lipomatous (8850-8889) | Lipoma/liposarcoma (Angiomyolipoma) |
| Myomatous (8890-8929) | Leiomyoma/leiomyosarcoma - Myoma - Rhabdomyoma/rhabdomyosarcoma - Sarcoma botryoides |
| Complex Mixed And Stromal (8930-8999) | Adenomyoma - Pleomorphic adenoma - Mixed Mullerian tumor - Mesoblastic nephroma - Wilms' tumor - Rhabdoid tumour - Clear cell sarcoma of the kidney - Hepatoblastoma - Carcinosarcoma |
| Fibroepithelial (9000-9039) | Brenner tumour - Fibroadenoma - Phyllodes tumor |
| Synovial-like (9040-9049) | Synovial sarcoma - Clear cell sarcoma, NOS |
| Mesothelial (9050-9059) | Mesothelioma - Adenomatoid tumor |
| Germ cell tumors (9060-9119) | germinomatous germ cell tumors: Dysgerminoma - Germinoma - Seminoma nongerminomatous germ cell tumors: Embryonal carcinoma - Endodermal sinus tumor / Yolk sac tumor - Teratoma/Fetus in fetu / Dermoid cyst/Struma ovarii - Gestational trophoblastic disease (Hydatidiform mole) - Choriocarcinoma - Polyembryoma - Gonadoblastoma |
| Vascular (9120-9179) | blood vessels: Hemangioma/hemangiosarcoma - Angioma/angiosarcoma - Blue rubber bleb nevus syndrome - Hemangioendothelioma - Kaposi's sarcoma - Hemangiopericytoma lymphatic vessels: Lymphangioma/lymphangiosarcoma - Lymphangioleiomyomatosis |
| Osseous and chondromatous (9180-9349) | Osteoma/osteosarcoma - Osteochondroma - Chondroma/enchondroma/chondrosarcoma - Chondroblastoma - Giant cell tumor of bone - Ewing's sarcoma - Chordoma
teeth/odontogenic: (Cementoblastoma, Cementoma, Odontoma, Adenomatoid odontogenic tumor, Ameloblastoma) Adamantinoma |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
