Clinicopathologic analysis of patients with adult rhabdomyosarcoma

Hawkins, William G.; Hoos, Axel; Antonescu, Cristina R.; Urist, Marshall J.; Leung, Denis H. Y.; Gold, Jason S.; Woodruff, James M.; Lewis, Jonathan J.; Brennan, Murray F.

doi:10.1002/1097-0142(20010215)91:4<794::aid-cncr1066>3.0.co;2-q

Cited by 186 publications

(152 citation statements)

References 31 publications

(30 reference statements)

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“…In adults, embryonal histology has not been found to have a better prognosis like in children. 2,9 The survival of patients with RMS in our series was only 35% at 5 and 10 years and is comparable to the Memorial Sloan-Kettering and Harvard series. Hawkins et al reported the Memorial experience of 84 adult RMS patients and found a similar 5-year survival of 35%.…”

Section: Discussionsupporting

confidence: 75%

“…The Memorial experience also demonstrated the preponderance of pleomorphic histology particularly in adults > 40 years of age. 2 Some have questioned the existence of the pleomorphic histology in adults, although recent data confirm its status as a diagnostic entity. [6][7][8] More commonly in the past, there has been some confusion with the diagnosis of pleomorphic RMS and malignant fibrous histiocytoma (MFH).…”

Section: Discussionmentioning

confidence: 99%

“…In a study from Memorial Sloan-Kettering Cancer Center, RMS comprised 2% of all soft tissue sarcoma in patients 16 years of age. 2 As a result, there is paucity of information regarding the behavior of this disease in this population. Few studies exist, with limited numbers of patients.…”

Section: Introductionmentioning

confidence: 99%

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Presentation, prognostic factors and patterns of failure in adult rhabdomyosarcoma

Simon

Paulino

Ritchie

et al. 2001

International Journal of Radiation Oncology*Biology*Physics

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Section: Discussionsupporting

confidence: 75%

Section: Discussionmentioning

confidence: 99%

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Presentation, prognostic factors and patterns of failure in adult rhabdomyosarcoma

Simon

Paulino

Ritchie

et al. 2001

International Journal of Radiation Oncology*Biology*Physics

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“…Rhabdomyosarcomas (RMSs) are histologically classified into three types: embryonal and alveolar in childhood, and pleomorphic mostly in adults over 40 years of age (Hawkins et al, 2001). Pediatric RMSs are the most common forms of soft tissue sarcomas in the head and neck and the genitourinary tract, resemble developing skeletal muscle and have a relatively good prognosis.…”

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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“…1 Despite a higher prevalence among younger individuals, rhabdomyosarcoma also affects adults. 2,3 Based on morphology and molecular biology, rhabdomyosarcoma tumors are currently classified using the modified International Classification of Rhabdomyosarcoma into three main categories: alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, and pleomorphic rhabdomyosarcoma. 4 Although both alveolar and embryonal rhabdomyosarcoma tumors exhibit features indicative of rhabdomyoblastic differentiation, the two groups diverge in several aspects that include morphology, molecular genetics, and clinical outcome.…”

mentioning

confidence: 99%

Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcoma

et al. 2006

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Both epidermal growth factor receptor (EGFR) and ErbB-2 play an important role in cancer biology and constitute promising molecular targets of therapy. EGFR and ErbB-2 expression has been observed in rhabdomyosarcoma cell lines but not analyzed systematically in rhabdomyosarcoma tumors. Tissue microarray sections representing 66 rhabdomyosarcoma tumors (34 embryonal rhabdomyosarcoma, 32 alveolar rhabdomyosarcoma) were surveyed by immunohistochemistry using antibodies specific for EGFR and ErbB-2. Immunostains were assessed for intensity (0: no immunostaining; 1: weak; 2: moderate; 3: strong) and percentage of at least 500 neoplastic cells exhibiting membranous or membranous and cytoplasmic immunostaining. EGFR and ErbB-2 expression was considered positive if the product of intensity and percentage was greater than 10. Patients had a median age of 5.7 years (range 8 months-19.1 years), and of 65/ 66 patients, 38 were males and 27 were females. Expression of ErbB-2 was identified in 22/66 (33%) cases and tended to be more frequent in the alveolar subtype (13/32, 41%, vs 9/34, 26%, P ¼ 0.30). Expression of EGFR was identified in 31/66 (47%) cases and correlated with the embryonal subtype (26/34, 76%, vs 5/32, 16%, Po0.0001) independent of stage, age, and gender. Coexpression of EGFR and ErbB-2 was identified in eight tumors, of which six were embryonal rhabdomyosarcoma. None of the cases exhibited EGFR or ErbB-2 gene amplification, as assessed using fluorescence in situ hybridization. Furthermore, analysis of 11 additional rhabdomyosarcoma tumors (six alveolar; five embryonal) revealed no evidence of mutations in EGFR exons 18, 19, 20, and 21. In summary, expression of EGFR and/or ErbB-2 is detected in a sizeable subset of rhabdomyosarcoma tumors without evidence of EGFR or ErbB-2 amplification or mutations in the EGFR tyrosine kinase domain. Notably, expression of EGFR correlates with the embryonal subtype, which is also more likely to coexpress EGFR and ErbB-2.

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Clinicopathologic analysis of patients with adult rhabdomyosarcoma

Cited by 186 publications

References 31 publications

Presentation, prognostic factors and patterns of failure in adult rhabdomyosarcoma

Presentation, prognostic factors and patterns of failure in adult rhabdomyosarcoma

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

Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcoma

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