Rhabdomyosarcoma embryonal

Therapeutic modalities in cancer treatment may involve surgery, radiation, and/or chemotherapy. The objectives of cancer chemotherapy include (1) cure, (2) reduction in tumor size, and (3) prolongation of life. At the present time, approximately 50 percent of patients with cancer can be cured, with drug treatment estimated to contribute in 17 percent of cases. Cancer chemotherapy can be curative in testicular cancer, diffuse large cell lymphoma, Hodgkin s disease, choriocarcinoma, certain childhood tumors (acute lymphoblastic leukemia, Burkitt s lymphoma, Wilms tumor, and embryonal rhabdomyosarcoma). Certain cancers are more resistant to chemotherapy than others (e.g., lung and colon). 

O Connell, M. Hoskin, P. Mayles, W. McElwain, T. Barrett, A. Intravaginal iridium-192 in the management of embryonal rhabdomyosarcoma. Clin. Oncology 1991, 3, 236-239. 

Tsokos M, Howard R, Costa J. Immunohistochemical study of alveolar and embryonal rhabdomyosarcoma. Lab Invest. 1983 48 148-155. 

FIGURE 17.4 Embryonal rhabdomyosarcoma consists of small, round, polygonal, and elongated tumor cells with variable amounts of eosinophilic cytoplasm and hyperchromatic nuclei (hematoxylin-eosin, x200). 

FIGURE 17.7 Desmin displays cytoplasmic reactivity in embryonal rhabdomyosarcoma and highlights elongated straplike cells (immu-noperoxidase, x200). 

FIGURE 17.9 Muscle-specific actin displays cytoplasmic reactivity and highlights spindled and rhabdomyoblastic cells in embryonal rhabdomyosarcoma (immunoperoxidase, x400). 

Paler RJ, Felix JC. Desmin, myoglobin, and muscle-specific actin immunohistochemical staining in a case of embryonal rhabdomyosarcoma of the ovary. Appl Immunohistochem Mol Morphol. 1999 7 237-241. 

Malignant effusions caused by non-epithelial neoplasms are more frequently encountered in children than in adults. The most common causes of malignant effusions in children are lymphoma and leukemia, followed by non-epithelial neoplasms including Wilms tumor, neuroblastoma, Ewing s sarcoma, and embryonal rhabdomyosarcoma. 

Wilm s, Ewing sarcoma, embryonal rhabdomyosarcoma, methotrexate-resistant gestational choriocarcinoma, testicular tumors... 

Embryonal rhabdomyosarcoma (both spindle cell and botryoid types) ... 

Embryonal rhabdomyosarcoma is responsible for only 1% of rhabdomyosarcoma (RMS) in children. There is no difference between genders and it occurs typically in children less than 5 years of age. 

On CT, embryonal rhabdomyosarcoma will be seen as a hypodense lesion with heterogeneous attenuation localized in the hilum, but sometimes maybe seen in one lobe. After contrast injection the tumor will have a very variable enhancement. Contrast CT is very helpful in the evaluation of vascular involvement, and after surgery it helps to identify post-surgical abscess or hematomas (Gazelle et al. 1998) (Fig. 4.11a). 

Fig. 4.11a-e. Embryonal rhabdomyosarcoma of the biliary tree, a CT reveals a large mainly cystic mass. b,c Pre- and post-contrast MR images show irregular yet florid enhancement. d,e Thrombus is shown in the hepatic veins secondary to the obstructing lesion... 

Sarcoglycan gene sarcolemmal ay-sarcoglycan protein deficiencies (loss-or-function point mutations familial, vertical somatic, acquired). For mice with dystrophin and sarcoglycan deficiencies Sgca developing embryonal rhabdomyosarcoma with excess of murine double minute MDM-2 protein and missense-mutated p53, chck Fernandez K et al Am J Pathol 2010 176 416-434. 

Walter D, Satheesa S, Albrecht P, Bornhauser BC, D Alessandro V, Oesh SM, Rehrauer H, Leuschner J, Koscielniak E, Gengler C, Moch H, Bernasconi M, Niggli FK, Schafer BW. CWS, Study Group. CD133 positive embryonal rhabdomyosarcoma stem-like cell population is enriched in rhabdosphere. PLoS One. 2011 65 el9506. doi 10.1371/j ournal.pone.0019506. 

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