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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

Peripheral primitive neuroectodermal tumor with Homer Wright rosettes.  The rosettes contain a circular wreath of oval nuclei that surround a pale eosinophilic, fibrillary core.
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fig2: Peripheral primitive neuroectodermal tumor with Homer Wright rosettes. The rosettes contain a circular wreath of oval nuclei that surround a pale eosinophilic, fibrillary core.

Mentions: One of the best descriptions of pPNET was written by Jaffe et al. [13], who described neuroectodermal tumor of bone with prominent Homer Wright rosettes (previously called “malignant neuroepithelioma” or “peripheral neuroepithelioma”). They proposed this entity to be a distinct neoplasm, although the Intergroup Ewing Sarcoma Study had included rosette-forming lesions as a form of ES [26]. Jaffe et al. noted that no attempt had been made in prior publications to distinguish Homer Wright rosettes from perivascular or apoptotic pseudorosettes, and they postulated that Homer Wright rosettes are a marker for pPNET. This observation was later confirmed by Llombart-Bosch and Schmidt [7, 27]. As per the original description of neuroblastoma by James Homer Wright, these rosettes should consist of a closely apposed circle of cells with a peripheral wreath of nuclei and a central core of neuropil (Figure 2). The number of rosettes found in pPNETs varies from isolated to numerous. If isolated, they generally contrast with the background of monotonous fields of small round blue cells, but at times the histology can be subtle. Lesions are termed pPNET independent of the degree of differentiation and the number of rosettes. Jaffe presciently proposed that ES may be the most undifferentiated form of pPNET. Subsequently, several retrospective studies indicated that by immunohistochemistry features of neural differentiation can also be revealed in ES, using antibodies against proteins such as neuron-specific enolase, Leu 7 (later termed CD57), and S100 [7, 28, 29]. In 1991 Schmidt et al. [27] proposed that pPNET could be defined as tumors having Homer-Wright rosettes or expressing two different neural immunomarkers. Their retrospective study, which covered several decades of treatment, indicated that pPNETs had a worse clinical outcome than other forms of ESFT. Using Schmidt's criteria, the reported incidence of pPNET varies from 12% to 23%, compared to other ESFTs (Table 2).


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)

Peripheral primitive neuroectodermal tumor with Homer Wright rosettes.  The rosettes contain a circular wreath of oval nuclei that surround a pale eosinophilic, fibrillary core.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

fig2: Peripheral primitive neuroectodermal tumor with Homer Wright rosettes. The rosettes contain a circular wreath of oval nuclei that surround a pale eosinophilic, fibrillary core.
Mentions: One of the best descriptions of pPNET was written by Jaffe et al. [13], who described neuroectodermal tumor of bone with prominent Homer Wright rosettes (previously called “malignant neuroepithelioma” or “peripheral neuroepithelioma”). They proposed this entity to be a distinct neoplasm, although the Intergroup Ewing Sarcoma Study had included rosette-forming lesions as a form of ES [26]. Jaffe et al. noted that no attempt had been made in prior publications to distinguish Homer Wright rosettes from perivascular or apoptotic pseudorosettes, and they postulated that Homer Wright rosettes are a marker for pPNET. This observation was later confirmed by Llombart-Bosch and Schmidt [7, 27]. As per the original description of neuroblastoma by James Homer Wright, these rosettes should consist of a closely apposed circle of cells with a peripheral wreath of nuclei and a central core of neuropil (Figure 2). The number of rosettes found in pPNETs varies from isolated to numerous. If isolated, they generally contrast with the background of monotonous fields of small round blue cells, but at times the histology can be subtle. Lesions are termed pPNET independent of the degree of differentiation and the number of rosettes. Jaffe presciently proposed that ES may be the most undifferentiated form of pPNET. Subsequently, several retrospective studies indicated that by immunohistochemistry features of neural differentiation can also be revealed in ES, using antibodies against proteins such as neuron-specific enolase, Leu 7 (later termed CD57), and S100 [7, 28, 29]. In 1991 Schmidt et al. [27] proposed that pPNET could be defined as tumors having Homer-Wright rosettes or expressing two different neural immunomarkers. Their retrospective study, which covered several decades of treatment, indicated that pPNETs had a worse clinical outcome than other forms of ESFT. Using Schmidt's criteria, the reported incidence of pPNET varies from 12% to 23%, compared to other ESFTs (Table 2).

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