Rhabdomyosarcoma alveolar

E. S. Massuda, E. J. Dunphy, R. A. Redman, J. J. Schreiber, L. E. Nauta, F. G. Barr, I. H. Maxwell, and T. P. Cripe, Regulated expression of the diphtheria toxin A chain by a tumor-specific chimeric transcription factor results in selective toxicity for alveolar rhabdomyosarcoma cells, Proc. Natl. Acad. Sci. USA 94 14701 (1997). 

Khan J, Simon R, Bittner M, et al. Gene expression profiling of alveolar rhabdomyosarcoma with cDNA microarrays. Cancer Res 1998 58 5009-13. 

FIGURE 4.10 The solid form of primitive embryonal or alveolar rhabdomyosarcoma represents a prototypical small round cell malignancy for which several adjunctive studies are necessary. 

Montone KT, LiVolsi V. Alveolar rhabdomyosarcomas of the sinonasal tract. Head Neck, in press. 

Mercado GE, Barr FG. Fusions involving PAX and FOX genes in the molecular pathogenesis of alveolar rhabdomyosarcoma recent advances. Curr Mol Med. 2007 7(1) 47-61. 

FIGURE 17.5 Alveolar rhabdomyosarcoma displays fibrovascular septa lined by small, round tumor cells surrounding spaces with dispersed individual tumor cells (hematoxylin-eosin, x200). 

FIGURE 17.8 Desmin displays strong cytoplasmic reactivity in alveolar rhabdomyosarcoma (immunoperoxidase, x400). 

FIGURE 17.11 Myogenin decorates nuclei and highlights the FICURE17.12 Myogenin stains variably in proportions of nuclei in architecture of alveolar rhabdomyosarcoma (immunoperoxidase, embryonal rhabdomyosarcoma (immunoperoxidase, x400). x400). 

Davicioni E, Einckenstein EG, Shahbazian V, et al. Identification of a PAX-EKHR gene expression signature that defines molecu-lat classes and determines the prognosis of alveolar rhabdomyosarcomas. Cancer Res. 2006 66 6936-6946. 

Sorensen PH, Lynch JG, Qualman SJ, et al. PAX3-EKHR and PAX7-EKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma a report from the children s oncology group. J Clin Oncol. 2002 20 2672-2679. 

Barr EG, Qualman SJ, Macris MH, et al. Genetic heterogeneity in the alveolar rhabdomyosarcoma subset without typical gene fusions. Cancer Res. 2002 62 4704-4710. 

Young RH, Scully RE. Alveolar rhabdomyosarcoma metastatic to the ovary. A report of two cases and a discussion of the differential diagnosis of small cell malignant trrmors of the ovary. Cancer. 1989 64 899-904. 

Both embryonal and alveolar rhabdomyosarcoma are associated with different cytogenetic abnormalities, which indicate the different molecular etiology of both tumor identities and explain the different clinical behavior. 

Alveolar rhabdomyosarcoma is a heterogeneous tumor and about 90% of this tumor are associated with the t(2 13)(q35 ql4) translocation, whereas the other 10% of this tumor are associated with another analogous genetic abnormality namely the t (1 13)(p36 ql4) translocation generating a homologous fusion transcript. 

B. Detection by Specific Genetic Abnormalities (Alveolar Rhabdomyosarcoma)... 

Anderson, J., Renshaw, J., McManus, A. etal. (1997) Amplification ofthe t(2 13) and t(l 13) translocations of alveolar rhabdomyosarcoma in small formalin-fixed biopsies using a modified reverse-transcriptase polymerase chain reaction. Am J Pathol, 150, 477- 2. 

Ramirez-Peinado S, Alcazar-Limones F, Lagares-Tena L, Mjiyad NE, Cato-Maldonado A, Tirado OM, Munoz-Pinedo C. 2-Deoxyglucose induces noxa-dependent apoptosis in alveolar rhabdomyosarcoma. Cancer Res. 2011 71 6796-806 doi 10.1158/0008-5472. CAN-11-0759. 

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