Pediatric Undifferentiated Sarcoma of the Soft Tissues: A Clinicopathologic Study

Somers, Gino R.; Gupta, Abha; Doria, A.; Ho, Michael; Pereira, Carlos Augusto; Shago, Mary; Thorner, Paul; Zieleńska, Maria

doi:10.2350/08-05-0098.1

Cited by 45 publications

(67 citation statements)

References 37 publications

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“…Details of antibodies for immunohistochemistry, TUNEL procedures, and Factor VIII scoring are provided in Supplemental Methods. Scoring for ILK and phosphorylated JNK (Thr183/Tyr185) was completed using a modification of a previously described system (45). The immunohistochemical score was reported as the product of the stain intensity and distribution, both with a maximum of 3, to yield a maximum product of 9.…”

Section: Methodsmentioning

confidence: 99%

JNK1 determines the oncogenic or tumor-suppressive activity of the integrin-linked kinase in human rhabdomyosarcoma

et al. 2009

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Introduction Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood. Despite advances in multimodality therapy, the failure-free survival of patients diagnosed with metastatic RMS remains low, at 25% (1). RMS tumors are believed to arise from committed mesenchymal or myogenic progenitor cells that accumulate genetic damage and fail to terminally differentiate (2). RMS tumors largely belong to 2 different histologic subgroupings: 63% are of embryonal RMS (ERMS) histology, while nearly 20% are of alveolar RMS (ARMS) histology (2). In addition to the morphologic and ultrastructural differences between these 2 tumor histologies, they display distinct clinical profiles. ARMS tumors are more likely to be found in the extremities of older children, while ERMS tumors are more commonly found in the head and neck, trunk, and genitourinary systems of younger children (2). ARMS tumors are further associated with a poorer clinical course, exemplified by a higher incidence of bone marrow and lung metastases and higher mortality rate (2).The molecular differences between ARMS and ERMS tumors have been intensively studied (2). Of ARMS tumors, 70% display chromosomal translocations, fusing the neuromuscular development-associated genes paired box 3 (PAX3) and PAX7 to the PKB/

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Section: Methodsmentioning

confidence: 99%

JNK1 determines the oncogenic or tumor-suppressive activity of the integrin-linked kinase in human rhabdomyosarcoma

et al. 2009

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“…The sections were scored as per previously published criteria. 12 Briefly, scores for intensity were 1 for low, 2 for moderate and 3 for high, with the positive control used as the standard for high intensity. The distribution of staining was scored as 1 for o10% of cells positive, 2 for 11-50% of cells positive and 3 for 450% of cells positive.…”

Section: Immunohistochemistry For Id2 Mcm7 and Nestinmentioning

confidence: 99%

“…The histological differential diagnoses include other childhood sarcomas, namely malignant peripheral nerve sheath tumor, Ewing sarcoma/primitive neuroectodermal tumor and undifferentiated sarcoma. 12,13 Malignant peripheral nerve sheath tumor can be particularly difficult to distinguish from synovial sarcoma, as they share several morphological, immunohistochemical and ultrastructural features. [13][14][15] Some authors have even reported the rare occurrence of a t(X;18) translocation in malignant peripheral nerve sheath tumor, 16 adding further difficulty in differentiating these two tumors.…”

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confidence: 99%

Expression of MYCN in pediatric synovial sarcoma

et al. 2007

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Synovial sarcoma accounts for between 6 and 10% of childhood sarcomas and histological diagnosis can be challenging, even for experienced pathologists. Several other tumors enter the differential diagnosis, including malignant peripheral nerve sheath tumor, Ewing sarcoma/primitive neuroectodermal tumor and undifferentiated sarcoma. Several recent reports utilizing expression array techniques have documented expression of the MYCN oncogene in synovial sarcoma. In order to more fully investigate this finding, a series of 12 synovial sarcomas and 29 other sarcomas (four malignant peripheral nerve sheath tumors, 15 Ewing sarcoma/primitive neuroectodermal tumors, 10 undifferentiated sarcomas) were examined for MYCN expression and gene amplification. By RT-PCR, nine of 12 synovial sarcomas (75%) expressed MYCN. Five synovial sarcomas (42%) expressed MYCN at high levels. Of the other sarcomas, one malignant peripheral nerve sheath tumor (25%) and five Ewing sarcoma/primitive neuroectodermal tumors (33%) expressed MYCN at low levels, and all other cases were negative for MYCN. None of the synovial sarcomas had genomic amplification, suggesting that high MYCN expression levels resulted from epigenetic phenomena. Examination of selected downstream targets of MYCN in synovial sarcoma revealed expression of MCM7 (minichromosome maintenance protein 7) in all synovial sarcomas, and expression of nestin (n ¼ 10; 83%), ID2 (inhibitor of DNA binding protein 2) (n ¼ 6; 50%) and MRP1 (multidrug resistance protein 1) (n ¼ 1; 8%) in a subset of synovial sarcomas. Expression of downstream targets did not correlate with expression of MYCN. Neither MYCN nor expression of downstream targets significantly correlated with metastases at presentation, progression-free survival or overall survival in this small series. In summary, high levels of MYCN expression was useful for distinguishing synovial sarcoma from other childhood-spindled cell sarcomas with specificity and sensitivity of 100 and 42%, respectively, in this series. The clinical and biological significance of this finding deserves further study.

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“…In parallel with their diminishing numbers and refinements in the identification of aggressive sarcomas that were previously included among the UNDS, the prognosis of the remaining UNDS has improved [7][8][9][10].…”

Section: Introductionmentioning

confidence: 99%

“…These neoplasms are distinct from rhabdomyosarcoma, Ewing sarcoma, synovial sarcoma, and high-grade pleomorphic undifferentiated sarcoma (or malignant fibrous histiocytoma) [7].…”

Section: Introductionmentioning

confidence: 99%

Undifferentiated sarcoma: does it exist? A clinicopathologic study of 7 pediatric cases and review of literature

et al. 2009

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Pediatric Undifferentiated Sarcoma of the Soft Tissues: A Clinicopathologic Study

Cited by 45 publications

References 37 publications

JNK1 determines the oncogenic or tumor-suppressive activity of the integrin-linked kinase in human rhabdomyosarcoma

JNK1 determines the oncogenic or tumor-suppressive activity of the integrin-linked kinase in human rhabdomyosarcoma

Expression of MYCN in pediatric synovial sarcoma

Undifferentiated sarcoma: does it exist? A clinicopathologic study of 7 pediatric cases and review of literature

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