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Pathobiologic markers of the ewing sarcoma family of tumors: state of the art and prediction of behaviour.

Pinto A, Dickman P, Parham D - Sarcoma (2010)

Bottom Line: Drug-resistant disease at diagnosis or at relapse remains a major cause of mortality among patients diagnosed with ESFT.An improved understanding of the cell of origin and the molecular pathways that regulate tumorigenicity in ESFT should aid us in the search for novel therapies for ESFT.The purpose of this paper is thus to outline current concepts of sarcomagenesis in ESFT and to discuss ESFT patterns of differentiation and molecular markers that might affect prognosis or direct future therapeutic development.

View Article: PubMed Central - PubMed

Affiliation: Calgary Laboratory Services, University of Calgary, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada T3B 6A8.

ABSTRACT
Over the past three decades, the outcome of Ewing sarcoma family tumor (ESFT) patients who are nonmetastatic at presentation has improved considerably. The prognosis of patients with metastatic disease at the time of diagnosis and recurrence after therapy remains dismal. Drug-resistant disease at diagnosis or at relapse remains a major cause of mortality among patients diagnosed with ESFT. In order to improve the outcome for patients with potential relapse, there is an urgent need to find reliable markers that either predict tumor behaviour at diagnosis or identify therapeutic molecular targets at the time of recurrence. An improved understanding of the cell of origin and the molecular pathways that regulate tumorigenicity in ESFT should aid us in the search for novel therapies for ESFT. The purpose of this paper is thus to outline current concepts of sarcomagenesis in ESFT and to discuss ESFT patterns of differentiation and molecular markers that might affect prognosis or direct future therapeutic development.

No MeSH data available.


Related in: MedlinePlus

Classical Ewing's sarcoma, microscopic image of typical histomorphology.  The lesion comprises patternless sheets of small blue cells with round, regular nuclei, even nuclear margins, minimal cytoplasm, and indistinct boundaries.
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fig1: Classical Ewing's sarcoma, microscopic image of typical histomorphology. The lesion comprises patternless sheets of small blue cells with round, regular nuclei, even nuclear margins, minimal cytoplasm, and indistinct boundaries.

Mentions: Microscopically, classical (typical) ES is the prototypic undifferentiated small round cell tumor, composed of sheets of monotonous round or oval cells with primitive-appearing nuclei and moderate amount of clear to amphophilic cytoplasm (Figure 1). The nuclear characteristics of the prototypic ES are those of a primitive uncommitted cell, that is an oval configuration with smooth contours, single inconspicuous nucleoli, and finely dispersed chromatin. The nuclei are generally centrally located and surrounded by a thin rim of clear to vacuolated cytoplasm. The lesions are highly cellular with little intervening intercellular space. Paradoxically, typical cases contain few mitoses. Cytologically, these tumors comprise a mixture of lightly staining “primary” cells and darkly staining “secondary” cells. Some feel that the “secondary” cells are effete versions of the “primary cells” that result from degenerative changes that lead to nuclear hyperchromasia or basophilia, but some ultrastructural studies show no signs of degenerative changes [25]. Sixty to seventy-five per cent of ESFTs [23, 24] are reported to have this classical histology (Table 1).


Pathobiologic markers of the ewing sarcoma family of tumors: state of the art and prediction of behaviour.

Pinto A, Dickman P, Parham D - Sarcoma (2010)

Classical Ewing's sarcoma, microscopic image of typical histomorphology.  The lesion comprises patternless sheets of small blue cells with round, regular nuclei, even nuclear margins, minimal cytoplasm, and indistinct boundaries.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2957858&req=5

fig1: Classical Ewing's sarcoma, microscopic image of typical histomorphology. The lesion comprises patternless sheets of small blue cells with round, regular nuclei, even nuclear margins, minimal cytoplasm, and indistinct boundaries.
Mentions: Microscopically, classical (typical) ES is the prototypic undifferentiated small round cell tumor, composed of sheets of monotonous round or oval cells with primitive-appearing nuclei and moderate amount of clear to amphophilic cytoplasm (Figure 1). The nuclear characteristics of the prototypic ES are those of a primitive uncommitted cell, that is an oval configuration with smooth contours, single inconspicuous nucleoli, and finely dispersed chromatin. The nuclei are generally centrally located and surrounded by a thin rim of clear to vacuolated cytoplasm. The lesions are highly cellular with little intervening intercellular space. Paradoxically, typical cases contain few mitoses. Cytologically, these tumors comprise a mixture of lightly staining “primary” cells and darkly staining “secondary” cells. Some feel that the “secondary” cells are effete versions of the “primary cells” that result from degenerative changes that lead to nuclear hyperchromasia or basophilia, but some ultrastructural studies show no signs of degenerative changes [25]. Sixty to seventy-five per cent of ESFTs [23, 24] are reported to have this classical histology (Table 1).

Bottom Line: Drug-resistant disease at diagnosis or at relapse remains a major cause of mortality among patients diagnosed with ESFT.An improved understanding of the cell of origin and the molecular pathways that regulate tumorigenicity in ESFT should aid us in the search for novel therapies for ESFT.The purpose of this paper is thus to outline current concepts of sarcomagenesis in ESFT and to discuss ESFT patterns of differentiation and molecular markers that might affect prognosis or direct future therapeutic development.

View Article: PubMed Central - PubMed

Affiliation: Calgary Laboratory Services, University of Calgary, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada T3B 6A8.

ABSTRACT
Over the past three decades, the outcome of Ewing sarcoma family tumor (ESFT) patients who are nonmetastatic at presentation has improved considerably. The prognosis of patients with metastatic disease at the time of diagnosis and recurrence after therapy remains dismal. Drug-resistant disease at diagnosis or at relapse remains a major cause of mortality among patients diagnosed with ESFT. In order to improve the outcome for patients with potential relapse, there is an urgent need to find reliable markers that either predict tumor behaviour at diagnosis or identify therapeutic molecular targets at the time of recurrence. An improved understanding of the cell of origin and the molecular pathways that regulate tumorigenicity in ESFT should aid us in the search for novel therapies for ESFT. The purpose of this paper is thus to outline current concepts of sarcomagenesis in ESFT and to discuss ESFT patterns of differentiation and molecular markers that might affect prognosis or direct future therapeutic development.

No MeSH data available.


Related in: MedlinePlus