Factors predictive of mortality in pediatric extremity rhabdomyosarcoma

LaQuaglia, Michael P.; Ghavimi, Fereshteh; Penenberg, Darryl; Mandell, Lynda R.; Healey, John H.; Hadju, Steven I.; Exelby, Phillip R.

doi:10.1016/0022-3468(90)90409-3

Cited by 39 publications

(17 citation statements)

References 14 publications

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“…The clinical-and probably biological-peculiarity of soft part tumors arising in the limbs is especially evident for RMS, where the extremities are involved in around 15-20% of cases [13][14][15][16][17]. Table 3 compares ours with a large series of RMS of all sites, showing that RMS of the extremities are more likely to occur in adolescents, to be of alveolar subtype, to be large in size and locally invasive, and to spread to the lymph nodes.…”

Section: Discussionmentioning

confidence: 76%

“…All these clinical variables are known to have a negative influence on the final outcome. Various previous reports have confirmed that the results of treatment for extremity RMS are suboptimal, to say the least, by comparison with RMS cases at other sites [13][14][15][16][17]. This may be for various reasons, particularly the strict inter-relationships between the site variable and other prognostic factors (histology, tumor size, node involvement, and patient's age).…”

Section: Discussionmentioning

confidence: 89%

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Soft-tissue sarcomas of the extremities in patients of pediatric age

Casanova

Meazza

Gronchi

et al. 2007

Journal of Children's Orthopaedics

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Purpose The extremity site is a peculiar location for softtissue sarcomas (STS) of children and adolescents. Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006. Methods The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma. Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy. Results For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure. The outcome was particularly poor for patients with large invasive tumors, hand/foot involvement and/or nodal or distant metastases, and for patients who were not given radiotherapy. For the extraosseous Ewing sarcoma cases, 5-year EFS was 74%. For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors. Discussion While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results. The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.

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Section: Discussionmentioning

confidence: 76%

Section: Discussionmentioning

confidence: 89%

Soft-tissue sarcomas of the extremities in patients of pediatric age

Casanova

Meazza

Gronchi

et al. 2007

Journal of Children's Orthopaedics

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“…3,5 The overall prognosis for patients with rhabdomyosarcoma has improved since the introduction of multidisciplinary treatment. 2,14 Using combination chemotherapy and appropriate radiotherapy, patients with tumors confined to the site or organ of origin had a favorable prognosis regardless of surgical resectability. 7 On the contrary, a previous report indicated that patients who were treated with surgery alone did better than those who were treated with surgery and chemotherapy after 2 years, but not after 5 years.…”

Section: Discussionmentioning

confidence: 99%

Rhabdomyosarcoma Presenting as an Anterior Cervical Mass in an Adult: Report of a Case

Tsuchiya¹,

Furukawa²,

Endo³

et al. 2000

Surgery Today

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We report herein the case of a 61-year-old man who was referred to our hospital for treatment of a rapidly growing tumor in the right anterior cervical region. A 13 x 14 x 15 cm mass was palpated on physical examination. Computed tomography (CT) findings indicated a thyroid tumor, but cytology results suggested rhabdomyosarcoma. A tumor, measuring 9.3 x 7.2 x 5.2 cm and weighing 220 g, was resected and histopathological examination confirmed a diagnosis of rhabdomyosarcoma. Cervical rhabdomyosarcoma is rarely found in adults.

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“…For example, RMS of the extremities is more likely to occur in adolescents, usually alveolar subtype histology, large in size, and locally invasive with lymph node involvement and distant metastasis [8,[17][18][19][20]. It is not well understood why alveolar RMS tends to metastasize relatively early to bone marrow and lymph nodes compared with embryonal RMS; however, several target genes expressed in alveolar RMS have been identified that intervene in metastatic behavior: PAX3-FKHR, MET and the chemokine receptor CXCR4 with the stromal derived factor-1 (SDF-1) [21].…”

Section: Discussionmentioning

confidence: 99%