Cjc 112 exam iv study guide

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Cjc 112 exam iv study guide

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Abstract Rhabdomyosarcoma RMSa malignant tumor of mesenchymal origin, is the third most common extracranial malignant solid tumor in children and adolescents.

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The embryonal and alveolar histologic variants are more commonly seen in pediatric patients, while the pleomorphic variant is rare in children and seen more often in adults. Advances in the research of the embryonal and alveolar variants have improved our understanding of certain genes and biologic pathways that are involved in RMS, but much less is known for the other variants.

Multimodality therapy that includes surgery and chemotherapy with or without radiation therapy is the mainstay of treatment for RMS. However, for adult patients, in great part due to rarity of the disease and the lack of consensus on optimal treatment, clinical outcome is still poor.

Many factors have been implicated for the differing outcomes between pediatric RMS versus adult RMS, such as the lack of standardized treatment protocols for adult RMS patients and the increased prevalence of advanced presentations.

Now that there are increased numbers of survivors, we can appreciate the sequelae from therapy in these patients, such as bone growth abnormalities, endocrinopathies, and infertility. Improvements in risk stratification have led to clinical trials using lower doses of chemotherapy or radiation therapy with the intention of decreasing the incidence of side effects without compromising survival outcome.

It is the third most common extracranial solid tumor in children after neuroblastoma and Wilms tumor. The ERMS mainly occurs in the head and neck and genitourinary regions, and ERMS demonstrates a bimodal age of distribution, with a larger peak between 0—5 years and a smaller peak in adolescence.

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More than one-half of ERMS cases occur before the age of 5 years. For example, adolescent patients are more likely to have the ARMS compared to younger patients. Also, adult RMS patients tend to have an increased likelihood of primary tumors occurring at unfavorable anatomic sites.

Associated genetic syndromes Most cases of RMS are currently considered sporadic in origin, but there have been associations of RMS with a number of genetic syndromes. Li— Fraumeni syndrome; neurofibromatosis type 1; Rubinstein—Taybi syndrome; Beckwith—Wiedemann syndrome overgrowth syndrome with macrosomia, organomegaly, hemihypertrophy, and omphalocele ; Costello syndrome mental retardation, neonatal feeding problems, coarse facies, low birth weight, hirsutism, loose soft skin ; Noonan syndrome; hereditary retinoblastoma; and Gorlin basal cell carcinoma nevus syndrome.

Li—Fraumeni syndrome has been linked to germline mutations de novo or inherited of the tumor suppressor gene for p Family members of afflicted children have an increased frequency of other types of cancers, such as breast cancer, acute leukemia, soft tissue and bone sarcomas, adrenocortical carcinoma, and brain tumors.

The cell of origin for RMS remains a subject of debate. It has been suggested that committed muscle stem cells and also multipotent mesenchymal stem cells can give rise to RMS.

Although they share a commonality of being mesenchymal-derived sarcomas, each subtype likely represents a distinct entity with its own unique biology for formation and possibly with different cells of origin.

Studies derived from these models have shown chemical and physical heavy metals, ionizing radiation, polycyclic aromatic hydrocarbonsbut also biological viral proteins, p53 pathway alterations, rat sarcoma [RAS] or hepatocyte growth factor alterations triggers of RMS.

Cjc 112 exam iv study guide

This was mainly observed in aged dystrophic mice, suggesting that cumulative deoxyribonucleic acid damage — over time, together with paracrine signals — could direct the muscle cells and their progenitors toward tumor formation.

Under light microscopy, ERMS tumor resembles immature skeletal muscle. It typically arises in the deep soft tissues of the extremities of patients older than 45 years. It is an aggressive neoplasm that is probably closer, both biologically and clinically, to adult, high-grade soft-tissue sarcomas than to pediatric RMS.

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One animal study was able to demonstrate the development of the pleomorphic RMS tumors in pdeficient mice when KRAS was overexpressed. For example, patients with parameningeal tumors may initially present with cranial nerve palsies, while patients with intra-abdominal tumors may present with bowel obstruction.

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Pain is not a consistent presenting symptom and usually arises due to mass effect and compression upon adjacent neural structures. For active adolescents and young adults, swelling in certain areas, such as the extremities and the paratesticular site, is often mistaken as a sequelae of perceived musculoskeletal injury, and because there is often no associated pain, the area of question may not be brought to medical attention for some time.

Imaging studies should include computed tomography CT scan or magnetic resonance imaging of the primary tumor to determine the size and possible involvement of vital organ structures; these are parameters to be considered when surgical resection is planned.

For tumors arising from the head, imaging studies should include full views of the neck to evaluate the cervical lymph node chains.This General Chemistry II Fall Study Guide 7 pages was uploaded by Jazmen Thompson, an elite notetaker at USC on Sep 16 and has been viewed 84 times. Browse this and other USC study guides, notes and flashcards at StudySoup.

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