Pediatric Soft Tissue Sarcomas

Loeb, David M.; Thornton, Katherine A.; Shokek, Ori

doi:10.1016/j.suc.2008.03.008

Cited by 109 publications

(98 citation statements)

References 34 publications

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“…Soft tissue sarcomas account for 10% of all new pediatric malignancies, 50% of which are skeletal muscle-lineage rhabdomyosarcomas (RMS) (1,2). Children with high-risk RMS endure a 3-year event-free survival rate of only 20% (3), emphasizing the need to uncover the molecular underpinnings of RMS neoplasia.…”

Section: Introductionmentioning

confidence: 99%

Drosophila and mammalian models uncover a role for the myoblast fusion gene TANC1 in rhabdomyosarcoma

Avirneni-Vadlamudi¹,

Galindo²,

Endicott³

et al. 2012

J. Clin. Invest.

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Rhabdomyosarcoma (RMS) is a malignancy of muscle myoblasts, which fail to exit the cell cycle, resist terminal differentiation, and are blocked from fusing into syncytial skeletal muscle. In some patients, RMS is caused by a translocation that generates the fusion oncoprotein PAX-FOXO1, but the underlying RMS pathogenetic mechanisms that impede differentiation and promote neoplastic transformation remain unclear. Using a Drosophila model of PAX-FOXO1-mediated transformation, we show here that mutation in the myoblast fusion gene rolling pebbles (rols) dominantly suppresses PAX-FOXO1 lethality. Further analysis indicated that PAX-FOXO1 expression caused upregulation of rols, which suggests that Rols acts downstream of PAX-FOXO1. In mammalian myoblasts, gene silencing of Tanc1, an ortholog of rols, revealed that it is essential for myoblast fusion, but is dispensable for terminal differentiation. Misexpression of PAX-FOXO1 in myoblasts upregulated Tanc1 and blocked differentiation, whereas subsequent reduction of Tanc1 expression to native levels by RNAi restored both fusion and differentiation. Furthermore, decreasing human TANC1 gene expression caused RMS cancer cells to lose their neoplastic state, undergo fusion, and form differentiated syncytial muscle. Taken together, these findings identify misregulated myoblast fusion caused by ectopic TANC1 expression as a RMS neoplasia mechanism and suggest fusion molecules as candidates for targeted RMS therapy.

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Section: Introductionmentioning

confidence: 99%

Drosophila and mammalian models uncover a role for the myoblast fusion gene TANC1 in rhabdomyosarcoma

Avirneni-Vadlamudi¹,

Galindo²,

Endicott³

et al. 2012

J. Clin. Invest.

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“…5,6 Los trabajos publicados en la literatura sobre sarcomas de partes blandas en población pediátrica son escasos y, en general, analizan en forma conjunta estos dos subtipos. 2,8 En nuestra serie, presentamos 29 pacientes, todos con diagnósticos de tumores de partes blandas del tipo NRMS, tratados quirúrgicamente por un mismo equipo quirúrgico. Se describió una supervivencia del 72% a los 5 años, similar a los resultados antes reportados.…”

Section: Comentariounclassified

“…Con respecto a la quimioterapia, si bien representan un factor importante para los tumores del tipo RMS, los tumores NRMS se caracterizan por presentar mayor resistencia. 2,11 Veinticinco pacientes en nuestra serie recibieron quimioterapia como tratamiento adyuvante; sin embargo, no hemos encontrado diferencias en términos de supervivencia o recurrencia local entre los pacientes tratados con radioterapia, quimioterapia o con cirugía únicamente. El tamaño tumoral (> 5 cm), así como el margen quirúrgico positivo, han sido dos factores que fueron reportados como negativos sobre los resultados oncológicos de los pacientes.…”

Section: Comentariounclassified

“…1 Se dividen en dos grandes grupos: rabdomiosarcomas (RMS) y no rabdomiosarcomas (NRMS), y se caracterizan por tener un mejor pronóstico que los tumores de partes blandas del adulto. [2][3][4] En particular, los tumores NRMS representan un grupo heterogéneo de tumores malignos derivados de células mesenquimales y han sido escasamente reportados en la literatura como entidad única, debido a su baja frecuencia y heterogeneidad. 5,6 La localización central es un factor pronóstico negativo, que presenta un peor índice de supervivencia y una mayor tasa de recurrencia local en comparación con los tumores de las extremidades.…”

Section: Introductionunclassified

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Sarcomas de partes blandas en pacientes pediátricos. Análisis de una serie de casos del subtipo no rabdomiosarcoma

Farfalli¹,

Iriberry²,

Albergo³

et al. 2014

Arch Argent Pediat

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RESUMENSe evaluó una serie de 29 pacientes con diagnóstico de sarcomas de partes blandas variante no rabdomiosarcomas tratados quirúrgicamente entre 2000 y 2010, y se analizó la supervivencia global y los factores que influyen en el pronóstico. La edad media fue de 11,6 años (rango de 3 meses-17 años); 16 pacientes eran de sexo masculino y el tiempo promedio de seguimiento fue de 56 meses (de 8 a 132 meses). Se documentaron 8 variedades histológicas diferentes de tumores malignos, y el sarcoma sinovial resultó ser el más frecuente (14 pacientes). La cirugía de conservación de miembro fue posible en 28 pacientes y se asoció tratamiento adyuvante en 26. La supervivencia global de la serie estudiada fue de 72% a los 5 años y, en 9 pacientes, se diagnosticó una recurrencia local. La presencia de metástasis (p < 0,0001) y la recurrencia local (p < 0,007) resultaron ser factores de pronóstico negativo para la supervivencia global. Palabras clave: sarcomas de partes blandas no rabdomiosarcomas, supervivencia, recurrencia local. ABSTRACTWe evaluated 29 patients with non rhabdomyosarcoma soft tissue sarcomas treated with surgery between 2000 and 2010; we analyzed overall survival and which factors affect the prognosis. The mean age was 11.6 years (range 3 months-17 years); 16 patients were males and the median follow-up was 56 months (8 to 132 months). Eight different histological malignant tumors were identified, being synovial sarcoma the most prevalent one (14 patients). Twenty-eight patients were treated with limb salvage surgery and in 26 cases, adjuvant therapy was used. Five years overall survival was 72%. Nine of the 29 patients presented a local recurrence. The presence of metastases (p <0.0001) and local recurrence (p <0.007) were negative prognostic factors for overall survival.

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“…7 Rhabdomyosarocma is the commonest paediatric soft tissue sarcoma seen but was also the commonest type in a study in Niger delta region of Nigeria in both the adult and paediatric age group. 8,9 A review of soft tissue sarcoma over a 20 year period showed the peak incidence of age occurred in the third and sixth decades of life, while another study showed the mean age of presentation of soft tissue sarcoma to be 37.4±12.6 years and the age range from 18 to 85 years among adult population but with inclusion of paediatric age group in another study, the youngest patient seen was 3 years and the oldest was 73 in another study. 8 There is a slight gender predilection seen in soft tissue sarcoma in males compared to females.…”

Section: Introductionmentioning

confidence: 98%

Clinicopathological pattern of soft tissue sarcoma in a tertiary health institution in North Western Nigeria

et al. 2018

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Background: Soft tissue sarcomas are a rare group of heterogeneous tumours that consist of several neoplasia which differentiate into different cell lines. They could arise from the mesodermal or ectodermal embryonic germ layers. Rhabdomyosarcoma is the most common soft tissue tumour in the paediatric age group in most studies, while in adults, malignant fibrous histocytoma, liposarcoma and fibrosarcoma are seen to be more predominant. The study aims to highlight the histological patterns, clinical presentation, associated risk factors and the patients’ performance status at presentation.Methods: This is a 5-year retrospective between the period of 2010 to 2015 of all cancer patients diagnosed with soft tissue sarcoma, seen in the department of radiotherapy and oncology, a tertiary health centre in Sokoto Nigeria. Data collected included socio-demographic, histological type, clinical features, and performance status.Results: A total of 123 patients were reviewed during the study period of 2010 to 2015: males were 81 (65.9%) and females were 42 (34.1%), with a male to female ratio of 1.93:1. The commonest histological type in both the adult and paediatric age group was rhabdomyosarcoma. Most patients presented with a swelling as seen in 62 (50.4%) cases. The extremities were the commonest site of involvement, with the lower limbs consisting 41 (33%) of the total patients reviewed.Conclusions: Rhabdomyosarcoma was the most predominant histological type of soft tissue sarcoma seen over the review period and was seen in both the paediatric and adult age groups as the commonest type.

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Pediatric Soft Tissue Sarcomas

Cited by 109 publications

References 34 publications

Drosophila and mammalian models uncover a role for the myoblast fusion gene TANC1 in rhabdomyosarcoma

Drosophila and mammalian models uncover a role for the myoblast fusion gene TANC1 in rhabdomyosarcoma

Sarcomas de partes blandas en pacientes pediátricos. Análisis de una serie de casos del subtipo no rabdomiosarcoma

Clinicopathological pattern of soft tissue sarcoma in a tertiary health institution in North Western Nigeria

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