Pediatric soft tissue sarcomas

Wexler, Leonard H.; Helman, Lee J.

doi:10.3322/canjclin.44.4.211

Cited by 36 publications

(18 citation statements)

References 104 publications

(118 reference statements)

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“…Previous studies have shown that approximately one-third carry activating mutations of either K-ras, H-ras or N-ras (Stratton et al, 1989;Wilke et al, 1993;Wexler and Helman, 1994;Yoo and Robinson, 1999;Garcia et al, 2000). Constitutive Ras activation leads to deregulated cell proliferation and blocks myogenic differentiation by downregulating myogenic factors such as MyoD and myogenin (Lassar et al, 1989).…”

Section: Introductionmentioning

confidence: 99%

Cooperation of oncogenic K-ras and p53 deficiency in pleomorphic rhabdomyosarcoma development in adult mice

et al. 2006

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Human rhabdomyosarcomas (RMSs) frequently demonstrate genetic alterations in ras and p53. To investigate their possible involvement in the tumorigenesis, we generated a knock-in mouse line with oncogenic K-ras, conditionally expressed by Cre/LoxP system on a background of p53 alteration. Electroporation of Cre expression vector in skeletal muscle tissues resulted in the generation of tumor in adults with tumor incidences of 100% at 10 weeks and 40% at 15 weeks, in p53 À/À and p53 À/ þ backgrounds, respectively. The tumor histology was pleomorphic RMS with characteristic bizarre giant cells, positive for desmin and a-sarcomeric actin and exhibiting remarkable increase in total and phosphorylated extracellular signal-regulated protein kinase (ERK)1 and ERK2. Loss of the wild-type p53 was detected in K-rasG12V-expressed tumors of p53 À/ þ mice. Early lesions 3 weeks after electroporation consisted of proliferating populations of myogenic progenitors, including stem cells positive for ScaI antigen, immature cells positive for desmin and neural cell adhesion molecule-positive myotubes. Thus, cooperation of oncogenic K-ras and p53 deficiency resulted in the development of pleomorphic RMS in adult mice, providing a useful mouse model for further detailed studies.

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Section: Introductionmentioning

confidence: 99%

Cooperation of oncogenic K-ras and p53 deficiency in pleomorphic rhabdomyosarcoma development in adult mice

et al. 2006

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“…MRI features are nonspecific and similar to congenital fibrosarcoma [24,25], therefore the differentiation has to be made at a histologic level. Malignant rhabdomyoblasts [38] and immunohistochemical markers (SMA and desmin) [19] are present in rhabdomyosarcoma but not in congenital fibrosarcoma.…”

Section: Discussion and Treatmentmentioning

confidence: 99%

Orthopaedic Case of the Month: Rapidly Progressive Shoulder Soft Tissue Mass in an 8-week-old Girl

Behnke

Patel

Davidson

et al. 2011

Clinical Orthopaedics &Amp; Related Research

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“…It is originate from embryonic mesenchymal cells, with potential to differentiate into skeletal muscle cells, and is characterized by a tendency to exhibit histologic and molecular features of skeletal myogenesis. These tumors may arise anywhere in the body, even in sites where skeletal muscle is not normally found (7), and at diagnosis, the more frequent location of the tumor was: head and neck, genitourinary and limbs (8). Table 5.…”

Section: Definition E Incidencementioning

confidence: 99%

“…Most rhabdomyosarcomas occur sporadically without predisposing factors, and only one third of patients have recognizable genetic anomalies (7,9). The cause of the rhabdomyosarcomas remains unknown; but now we know, that certain genetic alterations are associated with the development of this tumor.…”

Section: Etiology Pathogenesis and Cytogeneticsmentioning

confidence: 99%

“…Alveolar RMS has a characteristic translocation between the long arm of chromosome 2 and the long arm of chromosome 13. This translocation has been cloned molecularly, and shown to involve the juxtaposition of the PAX3 gene, which thought to regulate transcription during early neuromuscular development (7). The embryonal rhabdomyosarcoma, has loss of heterozygosity (LOH) at the 11p15 locus (10).…”

Section: Etiology Pathogenesis and Cytogeneticsmentioning

confidence: 99%

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