N 510 Module 7: Discussion Question
N 510 Module 7: Discussion Question
N 510 Module 7: Discussion Question:
Choose one type of childhood cancer and discuss pathophysiology and prognosis of the disease.
Your initial posting should be 200 to 300 words in length and utilize at least one scholarly source other than the textbook. Please reply to at least two classmates. Replies to classmates should be between 100 and 200 words in length. To properly “thread” your discussion posting, please click on REPLY.
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N 510 Module 7: Discussion Question
N 510 Module 7: Discussion Question – Childhood Cancer- Wilms Tumor
Children’s cancer’s pathology is multifactorial but more based in oncogenes and tumor suppressors than environmental elements. Although children’s cancer differs from adult cancer in that there are much fewer genetic mutations, some gene mutations continue to hold responsibility for childhood cancers. Beckwith-Wiedemann syndrome (BWS) is a hereditary syndrome that is caused by gene mutation, resulting in an imprinting alteration. This alteration may reflect only one gene code being copied (normally there are 2 copies). There is also DNA demethylation in BWS which can result in an increased expression of one gene and decreased expression of another gene. This syndrome has clinical manifestations at birth of macrosomia (large body size), macroglossia (large tongue), omphalocele (an abdominal wall defect that causes the wall to protrude through the navel) and unusual ear creases. Children born with this syndrome are at increased risk for tumors (Wilms tumor and hepatoblastoma), kidney disease and hypoglycemia. (Beckwith-Wiedemann Syndrome, 2016), (Pathophysiology, 2014)
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Wilms tumor is a triphasic renal tumor that is derived from nephrotic blastema cells. This pathologically contains the components of metanephric blastema, stromal and epithelial derivatives. There is the presence of abortive tubules, glomeruli and spindle stroma cells within the tumor. The spindle stroma may include fat tissue, striated muscle, cartilage, bone and fibrous tissue. This is a malignant tumor with typical metastasis to the lungs in later stages. There may be spreading to the perirenal soft tissue. There is also risk of traumatic rupture. Most epithelial predominant tumors are stage 1 and most blastema predominant tumors are stage 3 and 4 (Mandolin , 2018). 43% of these tumors are diagnosed in stage 1 prior to 6 years of age (Mandolin , 2018). Overall prognosis for this cancer is good. According to the US National Cancer Institute 80% to 90% of cases are cured with only a small percentage of the cases developing second neoplasms. (Mandolin , 2018)
N 510 Module 7: Discussion Question References
Beckwith-Wiedemann Syndrome. (2016, May). Retrieved from ASCO.org : https://www.cancer.net/cancer-types/beckwith-wiedemann-syndrome
Mandolin , Z. (2018, July 16). Kidney Tumor Childhood Neoplasms . Retrieved from Pathology Outlines.com: http://www.pathologyoutlines.com/topic/kidneytumorwilmkids.html
Pathophysiology. (2014). In K. L. McCance, S. E. Huether, V. L. Brashers, & N. S. Rote, The Biological Basis for Disease in Adults and Children. St. Louis: Elsevier Mosby.
N 510 Module 7: Discussion Question –
Retinoblastoma
Cancer begins when cells grow out of control. Cells can become cancer in any part of the body. Retinoblastoma is a type of cancer that begins in the retina. The retina is the back portion of the eye and is the most common type of cancer in children (DeJong, DeGaaf, Noij, Maeder, Galluzzi, Briss, Moll, & Castelijns, 2014). Retinoblastoma is rare and complex. It almost always begins with a mutation in the retinoblastoma (RB1) gene (DeJong et al., 2014). Normal RB1 gene will assist in keeping cells from growing out of control, however, the change within the gene will stop working as it should. When a change in the RB1 gene happens, two different type of retinoblastoma can occur. In general, retinoblastomas are diagnosed and treated before it has spread outside of the eyeball (DeJong et al., 2014). Retinoblastoma occasionally spreads and can grow along the optic nerve and into the brain (DeJong et al., 2014). It can also spread into the socket of the eye, eyelids, lymph nodes, liver, bones and bone marrow (DeJong et al., 2014). Treated depends on the stage and type and prognosis will be determined on staging and treatment.
Another type of cancer is intraocular medulloepithelioma. This type of cancer is a tumor that begins in the eye and it not a retinoblastoma, however, it occurs most often in young children and is very rare (DeJong et al., 2014). It begins in the ciliary body, and the majority of tumors are malignant, rarely spread outside of the eye, cause pain and loss of vision (DeJong et al., 2014). Diagnosis is initially made with an eye exam. If a tumor is suspected, a biopsy is completed to confirm the diagnosis (DeJong et al., 2014). Treatment is most likely removal of the eye. Removal of the affected eye typically removes all of cancer.
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