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<div class="noeditbox">Welcome to &lt;a href="Pathophysiology of Complex Patient Problems"&gt;PT 635 Pathophysiology of Complex Patient Problems&lt;/a&gt; This is a wiki created by and for the students in the School of Physical Therapy at Bellarmine University in Louisville KY. Please do not edit unless you are involved in this project, but please come back in the near future to check out new information!!</div><div class="editorbox">
<div class="editorbox">
'''Original Editors '''- Students from Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.  
'''Original Editors '''- Students from Bellarmine University's&nbsp;Pathophysiology of Complex Patient Problems project.  


'''Top Contributors''' - Rachel Flaherty and&nbsp;Allison&nbsp;Hafele&nbsp;  
'''Top Contributors''' - Rachel Flaherty and&nbsp;Allison&nbsp;Hafele&nbsp;  
</div>  
</div>  
== Definition/Description  ==
== Introduction ==
Wilms tumour is a malignant paediatric renal tumour. Also known as nephroblastoma.<ref name=":0">Radiopedia [https://radiopaedia.org/articles/wilms-tumour?lang=gb Wilms tumour] Available:https://radiopaedia.org/articles/wilms-tumour?lang=gb (accessed 27.1.2023)</ref><sup></sup>


Wilms’ Tumor, also known as nephroblastoma, is a solid tumor of the kidney that develops from immature kidney cells.<sup><ref name="1">St. Jude’s Children Research Hospital: Wilms Tumor. Available at http://www.stjude.org/stjude/v/index.jsp?vgnextoid=5ceb061585f70110VgnVCM1000001e0215acRCRD. Accessed 15 March 2014.</ref></sup> It is the most common cancer of the kidneys in children, and the fourth most common type of cancer in children.<sup><ref name="1">St. Jude’s Children Research Hospital: Wilms Tumor. Available at http://www.stjude.org/stjude/v/index.jsp?vgnextoid=5ceb061585f70110VgnVCM1000001e0215acRCRD. Accessed 15 March 2014.</ref><ref name="2">Mayo Clinic: Wilms Tumor. Available at http://www.mayoclinic.org/diseases-conditions/wilms-tumor/basics/definition/con-20043492. Accessed 16 March 2014.</ref></sup> Wilms’ tumors are most often unilateral, affecting only one kidney.<ref name="3">American Cancer Society: Wilms Tumor.  Available at http://www.cancer.org/cancer/wilmstumor/index.  Accessed 16 March 2014.</ref> 5-10% of children with Wilms’ tumors have more than one tumor in the same kidney, and approximately 5% of children with the cancer have bilateral Wilms’ tumors, affecting both kidneys.<ref name="3" />  
== Epidemiology ==
Wilms tumours are the most common [[:Category:Paediatrics|paediatric]]  [[Renal Cancer|renal cancer]] (reportedly 85% of cases) and 7% of all childhood cancers are Wilms tumours. They are most common in children ages 3 to 4 and becomes much less common after the age of 5.<ref name="p2">Mayo Clinic: Wilms Tumor. Available from: http://www.mayoclinic.org/diseases-conditions/wilms-tumor/basics/definition/con-20043492. Accessed 29 October 2022.</ref> Wilms’ tumor affects boys and girls equally, and can also be found in adults, though it is very rare.<sup><ref name="p1">St. Jude’s Children Research Hospital: Wilms Tumor. Available from:http://www.stjude.org/stjude/v/index.jsp?vgnextoid=5ceb061585f70110VgnVCM1000001e0215acRCRD. Accessed 29 October 2022.</ref></sup>
== Etiology ==
The cause of Wilms tumor is not precisely known, but genetic alterations that deal with the normal embryological development of the genitourinary tract are thought to be causative.  Wilms patients with a relative with the disease (typically not a parent) number less thyan 1%.<ref>Leslie SW, Sajjad H, Murphy PB. [https://www.ncbi.nlm.nih.gov/books/NBK442004/ Wilms tumor].Available:https://www.ncbi.nlm.nih.gov/books/NBK442004/ (accessed 27.1.2023)</ref>
Watch this 1 minute video on Wilms tumour.&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;{{#ev:youtube|5Jfh3AxUHz0}}<ref>UCSFPediatricSurgery. What is Wilms Tumor? Available from: https://www.youtube.com/watch?v=5Jfh3AxUHz0
</ref>&nbsp;
== Characteristics/Clinical Presentation ==
Wilms’ tumor is often hard to find early because the tumor can grow large without any signs or symptoms. These children may look and act normally.<sup><ref name="p3">American Cancer Society: Wilms Tumor.  Available from: http://www.cancer.org/cancer/wilmstumor/index.  Accessed 29 October 2022.</ref> </sup>The most common first clinical sign is swelling or a hard mass in the abdomen. It is often firm and large enough to be palpated on both sides of the abdomen, and is usually not painful.<ref name="p3" /> Haematuria occurs in ~20% of cases. [[Hypertension]], due to excessive renin production, is found in up to 1/4 of patients. Acquired [[Von Willebrand Disease|von Willebrand]] disease occurs in 8% of cases. <ref name=":0" />  


&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp; [[Image:Wilms Tumor.jpg]]  
== Diagnosis ==
[[File:Kidney cancer stage.gif|thumb|527x527px|Kidney cancer stage]]
Diagnosis includes:


[http://www.youtube.com/watch?v=5Jfh3AxUHz0 www.youtube.com/watch]
* A physical exam looking for possible signs of Wilms' tumor.
* [[Blood Tests|Blood]] and [[urine]] tests to see how the [[Kidney|kidneys]] are working.
* [[Medical Imaging|Imaging tests]] may include an [[Ultrasound Scans|ultrasound]], [[CT Scans|computerized tomography]] (CT) or magnetic resonance imaging ([[MRI Scans|MRI]]).<ref name="p2" /><ref name="p3" />&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;
== Management ==


<br>  
Management of Wilms’ tumor involves [[Surgery and General Anaesthetic|surgery]], [[Chemotherapy Side Effects and Syndromes|chemotherapy]], and [[Radiation Side Effects and Syndromes|radiation therapy]]. Most children will receive more than one type of treatment. Treatment is based on both the stage and histology of the Wilms’ tumor.<ref name="p6">Dome JS, Huff V. Wilms Tumor Overview. 2003 Dec 19 [Updated 2022]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1294/. (accessed 29 October 2022).</ref>


<sup></sup>
* Unilateral Wilms tumours are, usually, treated by a combination of nephrectomy and chemotherapy.
* Radiotherapy has a small role, but may be used if peritoneal spread or incomplete resection occurs.


== Prevalence  ==
Cure today is possible ~90% of the time. Recurrence can occurs within the tumour area, or distally within the [[Lung Cancer|lungs]] or [[Liver Disease|liver]].<ref name=":0" /> &nbsp;


Wilms’ tumor is most common in children ages 3 to 4 and becomes much less common after the age of 5.<ref name="2" /> Wilms’ tumor affects boys are girls equally, and can also be found in adults, though it is very rare.<sup><ref name="1" /></sup> On average, there are 460 new cases of Wilms’ tumor diagnosed in the United States every year. Approximately 1 in 8,000-10,000 children are affected.<ref name="1" /> About 5% of all cancers in children are Wilms’ tumors.<sup><ref name="3" /></sup>
== Physical Therapy Management ==


== Characteristics/Clinical Presentation  ==
There are several long-term side effects of chemotherapy. Childhood cancers tend to respond better to chemotherapy than adult cancers. <ref name="p3" />Children’s bodies also tend to tolerate it better than adults do. &nbsp;Side effects of chemotherapy can include fatigue or extreme tiredness from having too few red [[blood]] cells.


Wilms’ tumor is often hard to find early because the tumor can grow large without any signs or symptoms. These children may look and act normally.<sup><ref name="3" /> </sup>The most common first clinical sign is swelling or a hard mass in the abdomen. It is often firm and large enough to be palpated on both sides of the abdomen, and is usually not painful.<ref name="3" /><sup></sup><br>  
Musculoskeletal complications that can occur due to radiation therapy include growth abnormalities and hypoplasia.&nbsp;<ref name="p3" />Mild [[scoliosis]] and mild asymmetry of all musculoskeletal structures can occur due to reduced growth of [[bone]] and [[Erector Spinae|paravertebral]] muscles. These effects are more pronounced the younger the patient is at the time of radiation therapy.&nbsp;<ref name="p3" />  


Some children may also experience:<ref name="2" /><ref name="3" /><sup><br></sup>
Physical therapy interventions may include&nbsp;strengthening [[Postural Control|postural muscles]] and also increasing [[Endurance Exercise|endurance]] to promote a higher quality of life. Patients should avoid heavy lifting due to the decreased [[Bone Density|bone density]] and risk of [[osteoporosis]].<ref name="p3" />
== Alternative/Holistic Management  ==


*Fever
Complementary medicine is treatment used in combination with regular medical care. Alternative treatments are used instead of the traditional medical treatment.<ref name="p3" /> Although there&nbsp;are not any alternative medicines to surgery, radiation or chemotherapy, there are complementary treatments including [[Healing Arts and Expressive Therapies in an Interdisciplinary Team|art therapy or play therapy]] to reduce [[Stress and Health|stress]], peppermint tea to relieve nausea, and [[acupuncture]] to help relieve pain.&nbsp;<ref name="p3" /> 
*Loss of appetite
*Shortness of breath
*Constipation
*Nausea
*Blood in the urine
*Abdominal pain


Wilms’ tumors may also cause high blood pressure. The blood pressure can get high enough to cause bleeding in the eye or a change in consciousness in rare cases.<sup><ref name="3" /></sup>
== Differential Diagnosis  ==


<sup></sup>
* - Clear cell sarcoma &nbsp;


<sup></sup><sup><br></sup><br>  
* - Malignant rhaboid tumor &nbsp;
* - Autosomal recessive polycystic kidney disease (ARPKD)
* - Autosomal dominant polycystic kidney disease (ADPKD)
* - Hydronephrosis
* - Renal carbuncles
* - Hemorrhage
* - Neuroblastoma, an embryonal malignancy of the adrenal gland, usually affect the same age group and commonly arise in the same general region of the abdomen&nbsp;<ref name="p9">Huszno J, Starzyczny-Slota D, Jaworska M, Nowara E. Adult Wilms’ tumor-diagnsis and current therapy. Cent Eur J Urol. 2013:39-44 Available from:http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3921847/pdf/CEJU-66-00262.pdf. (accessed 29 October 2022)</ref>


== Associated Co-morbidities  ==
<ref name="p3" />
 
The types of secondary cancers include bone and soft tissue sarcomas, breast cancer, lymphoma, tumors of the digestive tract, melanoma and acute leukaemias. Certain birth defects and also correlated with Wilms tumor including Aniridia, hemihypertrophy, cryptorchidism, hypospadias.<ref name="2" /> There are also several syndromes that are often associated with Wilms tumor including Sotos syndrome, Perlman syndrome, Simpson-Golabi-Behmel syndrome, Bloom syndrome, Frasier syndrome, Beckwith-Wiedemann Syndrome, Denys -Drash syndrome, and WAGR syndrome which are explained in further detail in etiology&nbsp;<ref name="2" />&nbsp;Hypertension is also a common co-morbitity associated with Wilms' tumor.&nbsp;<ref name="3" /><br><br>
 
== Medications  ==
 
The most effective chemotherapeutics in treatment of nephroblastoma are: actinomycin D (ACT), vincristine (VCR), doxorubicin (ADM), cyclophosphamide (ctx), ifosfamide (IFO), etoposide and carboplatin.<ref name="4" /> NWTS recommends polchemotherapy treatment (ACT, VCR, ADM) for a period of 15 weeks in stage III Less aggressive treatment using two medications (VCR and ACT) can be used in cancer stages I and II. <ref name="9" />It is important to note toxicity of such treatment is highger in adults than in children. &nbsp; <ref name="9" />
 
Toxicity: among vinicristine, dactinomycin and Adriamycin, the main acue toxicity was neuropathy due to vincristine. During chemotherapy with ICE (ifosfamide, carboplatin, etoposide) all patients had hematological toxicity such as neutropenia and thrombocytopenia in stage IV toxicity. The type, timing, and dosage of chemotherapy have been major risk factors in combined therapy. <ref name="9" />
 
Side Effects: Late side effects include cardiotoxicity, reproductive problems, renal dysfunction and the development of benign and malignant second tumors.<ref name="9" /> In some cases fertility may be impaired.&nbsp; <ref name="4" />Other side effects of chemotherapy include: hair loss , mouth sores , loss of appetite , nausea and vomiting , diarrhea or constipation , increased chance of infections (from having too few white blood cells), Easy bruising or bleeding (from having too few blood platelets), Fatigue or extreme tiredness (from having too few red blood cells)&nbsp;<ref name="3" /><br><br>
 
Below are treatment protocols for the National Wilms' Tumor Study Group (NWTSG) and Society of Pediatric Oncology ( SIOP):<ref name="9" />
 
[[Image:Picture 1.png]]<br>
 
== Diagnostic Tests/Lab Tests/Lab Values  ==
 
'''Physical Examination:''' Usually first symptoms in adults include pain and haematuria, while children experience palpation detectable, painless, rapidly increasing size, abdominal mass.<ref name="9">Huszno J, Starzyczny-Slota D, Jaworska M, Nowara E. Adult Wilms’ tumor-diagnsis and current therapy. Cent Eur J Urol;2013. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3921847/pdf/CEJU-66-00262.pdf. (accessed 14 March 2014)</ref>&nbsp;&nbsp;Blood and Urine Tests: A urinalysis may also be performed to look for blood and other substances like catecholamines to rule out adrenal issues<ref name="10">Zhang LJ, Liu W, Gao YM, Qin YJ, Wu RD. The expression of IL-6 and STAT3 might predict progression and unfavorable prognosis in Wilms' tumor. Biocehm Biophys Res Commun. 2013 Jun 7; 435(3).http://www.ncbi.nlm.nih.gov/pubmed/23665320. (accessed 24 March 2014)</ref>. Wilms tumor can not be diagnosed by a blood or urine test, but it can provide an overall assessment of health.<ref name="2" /><br>
 
'''Imaging Tests:'''
 
This may include ultrasound, Magentic resonance imaging (MRI), and Computerized tomogrpahy (CT) scan.&nbsp;<ref name="2" />
 
Abdominal Ultrasound: High frequency sound waves create an image of internal organs, blood vessels and tissues. <ref name="12">The Children's Hospital of Philadelphia. Wilms' Tumor ( kidney) in Children. http://www.chop.edu/service/oncology/cancers-explained/wilms-tumor-diagnosing-and-treating-wilms-tumor-in-children.html. Accessed March 24 2014.</ref>&nbsp;Ultrasonography is the first choice for diagnostic imaging because it creates a panoramic image of the abdomen.&nbsp;<ref name="12">Dome J, Huff V. Wilms' Tumor Overview. 2003. Available at:http://www.ncbi.nlm.nih.gov/books/NBK1294/.Accessed March 24 2014.</ref>
 
Bone scan: detects decreased bone density <ref name="12" />
 
Chest X-ray: produces images of the heart, lungs, and bones <ref name="12" /><br>
 
Biopsy: This helps confirm the diagnosis and aids in developing a treatment plan <ref name="12" />
 
Definite diagnosis can only be made by surgical resection or biopsy.<ref name="12">Dome J, Huff V. Wilms' Tumor Overview. 2003. Available at:http://www.ncbi.nlm.nih.gov/books/NBK1294/.Accessed March 24 2014.</ref>
 
There are several stages in Wilms' Tumor which help to determine treatment protocols mentioned above in medications.
 
- Stage I. The cancer is found only in one kidney, and generally can be completely removed with surgery.<br>- Stage II. The cancer has spread to the tissues and structures near the affected kidney, such as fat or blood vessels, but it can still be&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp; completely removed by surgery.<br>&nbsp;-Stage III. The cancer has spread beyond the kidney area to nearby lymph nodes or other structures within the abdomen, and it may not be&nbsp; completely removed by surgery.<br>&nbsp;-Stage IV. The cancer has spread to distant structures, such as the lungs, liver, bones or brain.<br>&nbsp;-Stage V. Cancer cells are found in both kidneys. <ref name="2" />
 
'''Other Lab values:''' A small percentage of Wilms tumors are caused by changes in the WT1 and WT2 genes, which are tumor suppressor genes on chromosome 11. Pathological diagnosis of adult nephroblastoma is based on criteria including: the presence of primitive blastemic spindle or round cell component; abortive or embryonal tubules or glomerular structures.<ref name="10" /> Cytokeratin, vimentin,desmin,actin, and WTI allows to distinguish between other rare cancer types. An elevated serum IL-6 level has already been demonstrated to correlate with poor survival and unfavorable clinical outcome in some solid tissue cancers.&nbsp; <ref name="10" />
 
<br>
 
<br>
 
<br>
 
<br>
 
<br>
 
<br>
 
<br>
 
== Etiology/Causes  ==
 
Wilms’ tumor is typically seen in otherwise healthy children, and the exact cause in most children is unknown.<ref name="4">National Cancer Institute: Wilms Tumor and Other Childhood Kidney Tumors Treatment.  Available at http://www.cancer.gov/cancertopics/pdq/treatment/wilms/HealthProfessional/page1.  Accessed 16 March 2014.</ref><ref name="5">Medline Plus Encyclopedia: Wilms Tumor.  National Institutes of Health.  Available at http://www.nlm.nih.gov/medlineplus/ency/article/001575.htm.  Accessed 15 March 2014.</ref> However, approximately 10% of patients with the tumor have a congenital anomaly, including certain birth defect syndromes and genetic changes.<ref name="5" /> Wilms’ tumor, whether hereditary or sporadic, shows evidence of changes in one or more of at least ten different genes.<ref name="4" />
 
<br>Kidneys are developed early in fetal growth. Gene mutations in early kidney cells may lead to undeveloped mature kidney cells. These early kidney cells may remain after birth. If the cells do not mature, they may grow out of proportion, forming a Wilms’ tumor. Genes WT1 and WT2 on chromosome 11 are tumor suppressor genes, and mutations of these cause a small number of Wilms’ tumors.<ref name="3" />
 
<br>Common syndromes that are associated with Wilms’ tumor include the following: <br>''WAGR Syndrome'': WAGR stands for Wilms tumor, Aniridia (lack of the iris of the eyes), Genitourinary tract abnormalities, and mental retardation. Children who have WAGR syndrome have approximately 30-50% chance of developing a Wilms’ tumor, and often have them bilaterally. In this syndrome, cells are missing part of chromosome 11, which involves the WT1 gene.<ref name="3" /><ref name="6">Dome JS, Huff V. Wilms Tumor Overview. 2003 Dec 19 [Updated 2013 Sep 19]. Available at http://www.ncbi.nlm.nih.gov/books/NBK1294/. Accessed 15 March 2014.</ref><br>''Beckwith-Wiedemann sydrome'': These children are at a 5% risk of having a Wilms’ tumor. This syndrome is caused by a malformation of chromosome 11.<ref name="3" /> <br>''Denys-Drash Syndrome'': This syndrome is also caused by mutations to the WT1 gene. The risk for Wilms’ tumor in these patients is greater than 90%.<ref name="6" /> In this disease, the kidneys become diseased and no longer function at a very young age, leading to a Wilms’ tumor.<ref name="3" /><br>
 
Less common syndromes linked to Wilms’ tumor:<ref name="3" /><ref name="4" /><br>
 
*Perlman sydrome
*Sotos syndrome
*Simpson-Golabi-Behmel Syndrome
*Bloom syndrome
*Li-Fraumeni Syndrome
*Frasier Syndrome
*Trisomy 18
*Alagille syndrome
 
== Systemic Involvement  ==
 
Wilms’ tumors often grow to a large size before any symptom manifestation. However, most Wilms’ tumors do not metastasize, despite their large size.<ref name="7">Goodman CC, Fuller KS: Pathology: implications for the physical therapist, ed 3, Philadelphia, 2009, WB Saunders.</ref> Hematuria occurs in approximately 30% of cases, and about 25% of children with the tumor will have hypertension.<ref name="7" /><br>As with all cancer treatment, there may be systemic side effects from chemotherapy and radiation. These may affect the heart, lungs, fertility, skin reactions, and may increase the risk for a second cancer.<ref name="3" /><br>
 
&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp; [[Image:Wilms Tumor Systemic Effects.jpg]]
 
== Medical Management (current best evidence)  ==
 
Management of Wilms’ tumor involves surgery, chemotherapy, and radiation therapy. Most children will receive more than one type of treatment. Treatment is based on both the stage and histology of the Wilms’ tumor.<ref name="6" />
 
'''Surgery''': The goal of surgery is to remove the tumor in one unit to prevent the spread of cancer cells into the abdomen.<ref name="3" /> There are several types of nephrectomy that can be performed:<br>
 
*Simple Nephrectomy: The entire kidney is removed, and the remaining kidney can increase its capacity and filter all of the blood.<ref name="2" />
*Partial Nephrectomy: This surgery includes resection of the tumor and a section of the kidney tissue surrounding it. Partial nephrectomy is common when the other kidney is damaged or removed.<ref name="2" />
*Radical Nephrectomy: The kidney and surrounding tissues of adrenal gland and ureter are removed.<ref name="2" />
 
'''Chemotherapy''': Most children diagnosed with a Wilms’ tumor will receive chemotherapy as one of their treatments. Powerful anti-cancer drugs are typically injected via vein, and the blood carries the drug throughout the entire system.8 Chemotherapy is typically used after the tumor has been surgically removed.<ref name="8">Children’s Cancer Research Fund:  Wilms Tumor/Nephroblastoma.  Available at http://www.childrenscancer.org/main/wilms_tumor_nephroblastoma/.  Accessed 15 March 2014.</ref><br>'''Radiation Therapy''': This treatment utilizes x-rays and other high-energy rays to destroy cancer cells and shrink the tumor size.<ref name="8" />
 
The type of treatment received is determined by the stage of the cancer, the type of cell, and the general health of the patient.<ref name="2" /><br>
 
*<u>Stage I or II</u>: The cancer is limited to the kidney or surrounding structures. Typically the kidney will be removed, as well as tissues and lymph nodes surrounding it. This will be followed with chemotherapy, and some stage II may need radiation.
*<u>Stage III or IV</u>: The cancer has spread outside of the kidney and cannot be removed with surgery. Radiation, along with surgery and chemotherapy will most likely take place.
*<u>Stage V</u>: Wilms’ tumors in bilateral kidneys. Chemotherapy is typically given first to shrink the tumor initially, followed by surgery and radiation.
 
&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp; [[Image:Wilms-tumor.jpg]]
 
== Physical Therapy Management (current best evidence)  ==
 
<br>There are several long-term side effects of chemotherapy. Childhood cancers tend to respond better to chemotherapy than adult cancers. <ref name="3" />Children’s bodies also tend to tolerate it better than adults do. &nbsp;Side effects of chemotherapy can include fatigue or extreme tiredness from having too few red blood cells. Musculoskeletal complications that can occur due to radiation therapy include growth abnormalities and hypoplasia.&nbsp;<ref name="13" />Mild scoliosis and mild asymmetry of all musculoskeletal structures in the radiotherapeutic can occur due to reduced growth of bone and paravertebral muscles.These effects are more pronounced the younger the patient is at the time of radiation therapy.&nbsp;<ref name="13" /><br>Physical therapists can work on increasing strength in postural muscles and also increasing endurance to promote and higher quality of life. Patients should avoid heavy lifting due to the decreased bone density and risk of osteoporosis.<ref name="13" /><br>
 
<br>
 
<br>
 
<br><br>
 
== Alternative/Holistic Management (current best evidence)  ==
 
Complimentary medicine is treatment used in combination with regular medical care. Alternative treatments are used instead of the traditional medical treatment.
 
Although there&nbsp;are not any alternative medicine instead of surgery, radiation or chemotherapy, there are complementary treatments including art therapy or play therapy to reduce stress, peppermint tea to relieve nausea, and acupuncture to help relieve pain.&nbsp;<ref name="3" /><br>
 
Under&nbsp;resources there is a link the healthy living after treatment for childhood cancer <ref name="11">Children's Oncology: Healthy living after treatment for childhood cancer. Group.www.survivorshipguidelines.org/pdf/EducationalIssues.pdf.(accessed March 24 2014)</ref>
 
== Differential Diagnosis<br>  ==
 
Differential diagnosis of Wilms tumor includes:&nbsp;<ref name="9" />
 
- Clear cell sarcoma &nbsp;
 
- Malgnant rhaboid tumor &nbsp;
 
- Autosomal recessive polycystic kidney disease (ARPKD)
 
- Autosomal dominant polycystic kidney disease (ADPKD)
 
- Hydronephrosis
 
- Renal carbuncles
 
- Hemorrhage
 
- Neuroblastoma, an embryonal malignancy of the adrenal gland, usually affect the same age group and commonly arise in the same general region of the abdomen&nbsp;<ref name="9" /><br>
 
[[Image:Wilms_tumor.png]]<ref name="13">Pediatric Oncology Education Materials. Available at: http://www.pedsoncologyeducation.com/wilmstumor_differential.asp.Accessed March 24 2014.</ref>


== Case Reports/ Case Studies  ==
== Case Reports/ Case Studies  ==


add links to case studies here (case studies should be added on new pages using the [[Template:Case Study|case study template]])<br>
{{pdf|WT1 mutation as a cause of 46 XY DSD and Wilm's tumour a case report and literature review.pdf|WT1 mutation as a cause of 46 XY DSD and Wilm's tumour a case report and literature review}}<br>{{pdf|Wilms' Tumor Metastatic to Bilateral Testes at Presentation Case and Review of the Literature.pdf|Wilms' Tumor Metastatic to Bilateral Testes at Presentation Case and Review of the Literature}}{{pdf|Extrarenal Wilms' Tumor of the Ovary- A Case Report and Short Review of the Literature.pdf|Extrarenal Wilms' Tumor of the Ovary- A Case Report and Short Review of the Literature}}<br>
 
{{pdf|WT1 mutation as a cause of 46 XY DSD and Wilm's tumour a case report and literature review.pdf|WT1 mutation as a cause of 46 XY DSD and Wilm's tumour a case report and literature review}}  
 
<br> <br> {{pdf|Resection_of_Metastases_in_Wilms'_Tumor-_A_Report_of_3_Cases_Cured_of_Pulmonary_and_Hepatic_Metastases.pdf‎|A Report of Three Cases Cured of Pumonary and Hepatic Metastases}} <br>
 
{{pdf|Wilms' Tumor Metastatic to Bilateral Testes at Presentation Case and Review of the Literature.pdf|Wilms' Tumor Metastatic to Bilateral Testes at Presentation Case and Review of the Literature}} <br>
 
{{pdf|Extrarenal Wilms' Tumor of the Ovary- A Case Report and Short Review of the Literature.pdf|Extrarenal Wilms' Tumor of the Ovary- A Case Report and Short Review of the Literature}} <br>
 
<br>
 
<br>
 
<br>
 
<br>
 
== &nbsp;Resources <br>  ==
 
{{pdf|American Cancer Society- Wilms Tumor.pdf|American Cancer Society: Wilms Tumor}} <br>
 
[http://www.cancer.gov/cancertopics/pdq/treatment/wilms/Patient National Cancer Institute: General Information about Wilms' Tumor and Other Childhood Kidney Tumors]<br>
 
[http://kidshealth.org/parent/medical/kidney/wilms.html Wilms Tumor: Parent Resource]<br>
 
[[Www.survivorshipguidelines.org/pdf/EducationalIssues.pdf|Healthy living after treatment for childhood cancer]]
 
== Recent Related Research&nbsp;  ==
<div class="researchbox"><rss>http://www.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=10IsjDYWWF_-cMKP9TdTTWMt_NO7MjdN6xO7NxcJrS_f4HKsAg|charset=UTF-8|short|max=10</rss></div>  
== References  ==
== References  ==


<references />&nbsp;
<references />&nbsp;
[[Category:Oncology]]

Latest revision as of 01:58, 28 January 2023

Original Editors - Students from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Rachel Flaherty and Allison Hafele 

Introduction[edit | edit source]

Wilms tumour is a malignant paediatric renal tumour. Also known as nephroblastoma.[1]

Epidemiology[edit | edit source]

Wilms tumours are the most common paediatric renal cancer (reportedly 85% of cases) and 7% of all childhood cancers are Wilms tumours. They are most common in children ages 3 to 4 and becomes much less common after the age of 5.[2] Wilms’ tumor affects boys and girls equally, and can also be found in adults, though it is very rare.[3]

Etiology[edit | edit source]

The cause of Wilms tumor is not precisely known, but genetic alterations that deal with the normal embryological development of the genitourinary tract are thought to be causative. Wilms patients with a relative with the disease (typically not a parent) number less thyan 1%.[4]

Watch this 1 minute video on Wilms tumour.                                  

[5] 

Characteristics/Clinical Presentation[edit | edit source]

Wilms’ tumor is often hard to find early because the tumor can grow large without any signs or symptoms. These children may look and act normally.[6] The most common first clinical sign is swelling or a hard mass in the abdomen. It is often firm and large enough to be palpated on both sides of the abdomen, and is usually not painful.[6] Haematuria occurs in ~20% of cases. Hypertension, due to excessive renin production, is found in up to 1/4 of patients. Acquired von Willebrand disease occurs in 8% of cases. [1]

Diagnosis[edit | edit source]

Kidney cancer stage

Diagnosis includes:

Management[edit | edit source]

Management of Wilms’ tumor involves surgery, chemotherapy, and radiation therapy. Most children will receive more than one type of treatment. Treatment is based on both the stage and histology of the Wilms’ tumor.[7]

  • Unilateral Wilms tumours are, usually, treated by a combination of nephrectomy and chemotherapy.
  • Radiotherapy has a small role, but may be used if peritoneal spread or incomplete resection occurs.

Cure today is possible ~90% of the time. Recurrence can occurs within the tumour area, or distally within the lungs or liver.[1]  

Physical Therapy Management[edit | edit source]

There are several long-term side effects of chemotherapy. Childhood cancers tend to respond better to chemotherapy than adult cancers. [6]Children’s bodies also tend to tolerate it better than adults do.  Side effects of chemotherapy can include fatigue or extreme tiredness from having too few red blood cells.

Musculoskeletal complications that can occur due to radiation therapy include growth abnormalities and hypoplasia. [6]Mild scoliosis and mild asymmetry of all musculoskeletal structures can occur due to reduced growth of bone and paravertebral muscles. These effects are more pronounced the younger the patient is at the time of radiation therapy. [6]

Physical therapy interventions may include strengthening postural muscles and also increasing endurance to promote a higher quality of life. Patients should avoid heavy lifting due to the decreased bone density and risk of osteoporosis.[6]

Alternative/Holistic Management[edit | edit source]

Complementary medicine is treatment used in combination with regular medical care. Alternative treatments are used instead of the traditional medical treatment.[6] Although there are not any alternative medicines to surgery, radiation or chemotherapy, there are complementary treatments including art therapy or play therapy to reduce stress, peppermint tea to relieve nausea, and acupuncture to help relieve pain. [6]

Differential Diagnosis[edit | edit source]

  • - Clear cell sarcoma  
  • - Malignant rhaboid tumor  
  • - Autosomal recessive polycystic kidney disease (ARPKD)
  • - Autosomal dominant polycystic kidney disease (ADPKD)
  • - Hydronephrosis
  • - Renal carbuncles
  • - Hemorrhage
  • - Neuroblastoma, an embryonal malignancy of the adrenal gland, usually affect the same age group and commonly arise in the same general region of the abdomen [8]

[6]

Case Reports/ Case Studies[edit | edit source]

WT1 mutation as a cause of 46 XY DSD and Wilm's tumour a case report and literature review
Wilms' Tumor Metastatic to Bilateral Testes at Presentation Case and Review of the LiteratureExtrarenal Wilms' Tumor of the Ovary- A Case Report and Short Review of the Literature

References[edit | edit source]

  1. ↑ 1.0 1.1 1.2 Radiopedia Wilms tumour Available:https://radiopaedia.org/articles/wilms-tumour?lang=gb (accessed 27.1.2023)
  2. ↑ 2.0 2.1 Mayo Clinic: Wilms Tumor. Available from: http://www.mayoclinic.org/diseases-conditions/wilms-tumor/basics/definition/con-20043492. Accessed 29 October 2022.
  3. ↑ St. Jude’s Children Research Hospital: Wilms Tumor. Available from:http://www.stjude.org/stjude/v/index.jsp?vgnextoid=5ceb061585f70110VgnVCM1000001e0215acRCRD. Accessed 29 October 2022.
  4. ↑ Leslie SW, Sajjad H, Murphy PB. Wilms tumor.Available:https://www.ncbi.nlm.nih.gov/books/NBK442004/ (accessed 27.1.2023)
  5. ↑ UCSFPediatricSurgery. What is Wilms Tumor? Available from: https://www.youtube.com/watch?v=5Jfh3AxUHz0
  6. ↑ 6.0 6.1 6.2 6.3 6.4 6.5 6.6 6.7 6.8 6.9 American Cancer Society: Wilms Tumor. Available from: http://www.cancer.org/cancer/wilmstumor/index. Accessed 29 October 2022.
  7. ↑ Dome JS, Huff V. Wilms Tumor Overview. 2003 Dec 19 [Updated 2022]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1294/. (accessed 29 October 2022).
  8. ↑ Huszno J, Starzyczny-Slota D, Jaworska M, Nowara E. Adult Wilms’ tumor-diagnsis and current therapy. Cent Eur J Urol. 2013:39-44 Available from:http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3921847/pdf/CEJU-66-00262.pdf. (accessed 29 October 2022)

 

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