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Soft Tissue Sarcomas
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Thomas F. DeLaney, David C. Harmon, Karol Sikora, Francis J. Hornicek
Alveolar rhabdomyosarcomas show a translocation at t(2;13)(q35;q14) or less often t(1;13)(p36;q14); the chimeric genes have been cloned and have been termed PAX3–FKHR and PAX7–FKHR, respectively. These translocations are associated with over-expression of the fusion product. PAX7–FKHR tumors more often present with extremity lesions, are more likely to be localized, and are less likely to metastasize widely than PAX3–FKHR tumors. A downstream target of PAX3–FKHR may be MET, which encodes a receptor involved in growth and motility signaling. Molecular determination of minimal residual disease in alveolar rhabdomyosarcoma is possible, and patients with positive peripheral blood after treatment show poorer survival than patients without micro-circulating disease.
Paediatric cancer
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
Microscopically, they are embryonal cells rich in glycogen and within which myofibrils can be demonstrated. Immunohistochemistry will be positive for desmin, myoglobin, myo-D1 and muscle-specific actin. The embryonal subtype is distinct from the alveolar rhabdomyosarcoma of adults, although this type can be seen in older children and adolescents.
Teratoma, rhabdomyosarcoma and other tumours
Published in Brice Antao, S Irish Michael, Anthony Lander, S Rothenberg MD Steven, Succeeding in Paediatric Surgery Examinations, 2017
From the above list, select the most appropriate answer for the following questions. The same item can be used more than once to answer any of the questions. An 11-year-old girl presents with a mass over the right leg. The mass was solid and an incisional biopsy was reported to be alveolar rhabdomyosarcoma. What is the overall prognosis for this child?A 6-month-old girl presents with vaginal bleeding and on examination has a fleshy mass protruding through the vaginal introitus. What is the most likely sarcoma?The histology report of a bladder/prostate rhabdomyosarcoma was ‘dense cellular sheets lacking intercellular stroma with no evidence of myogenesis’. What is the type of rhabdomyosarcoma?A child presents with a neck mass. What would be the first line of surgical management?A child presents with a large pelvic mass. What would be the surgical course in 74% of the patients?
Successful outcome following resection of metachronous pancreatic metastasis from a rhabdomyosarcoma
Published in Acta Chirurgica Belgica, 2021
M. Monge, H. Chiavelli, J. Pinson, E. Papet, L. Schwarz, J. J. Tuech
RMS constitute a unique group of soft tissue neoplasms that share propensity to undergo myogenesis, a well defined biologic process that primarily occurs during embryonal and fetal development [8]. RMS is the most common pediatric soft tissue sarcoma, comprising 50% of all soft tissue sarcomas and 10–12% of all malignant solid tumors in children but unusual in adults. If these tumors are highly invasive locally, explaining the high propensity for local recurrence, at presentation 35% of patients with alveolar rhabdomyosarcoma have metastatic disease [9]. The rhabdomyosarcoma metastases are usually located in the liver or bone marrow, but unusual sites have included breast, brain, and pancreas. Prognosis of RMS depends on the extent of disease, primary tumor site, histologic type and age [10].
Para-neoplastic optic neuritis presenting in a child with fusion positive localised para-testicular alveolar rhabdomyosarcoma
Published in Pediatric Hematology and Oncology, 2021
Katherine Gordon, Naveed Ahmad
Our patient recovered completely with a combination of steroids, immunoglobulins and standard chemotherapy for his underlying fusion positive alveolar rhabdomyosarcoma. The immunosuppressive regimen of steroids and immunoglobulins was based on our local guidelines in the department of pediatric neurology and ophthalmology. He went on to complete nine cycles of chemotherapy as per standard practice along with Proton radiotherapy to para-aortic lymph nodes and six cycles of maintenance chemotherapy. He remains on follow up more than three years from completing his treatment and there has been no concerns around his vision or optic atrophy. Our case is a useful addition to the existing knowledge on para-neoplastic syndromes in children, who was successfully treated with immunosuppressive therapy and standard chemotherapy.
A Review of the Role of Cytogenetics in the Diagnosis of Orbital Rhabdomyosarcoma
Published in Seminars in Ophthalmology, 2019
Paula Cortes Barrantes, Frederick A. Jakobiec, Thaddeus P. Dryja
Alveolar rhabdomyosarcoma (ARMS) develops most frequently in the 10- to 25-year-old age group. It is associated with a worse prognosis than ERMS, independent of the site of origin.2 Only 20% to 30% of RMS of the orbit are of the alveolar type. On microscopic examination, ARMS is composed of small round cells with scant cytoplasm. The cells are arranged around fine fibrous septae, giving a low power resemblance to the alveoli of the lung6,7 (Figure 2(b)). The degree of prominence of the fibrous stroma is variable; some tumors may completely lack this feature and have instead a sheet-like or solid pattern. Only one focus with alveolar histologic features is sufficient for designating the tumor as ARMS.6,9,10 The 5-year overall survival rate for childhood ARMS has been estimated to be 48%.2