Askin Tumor Research Articles

Askin tumor: four case reports and a review of the literature.

Askin tumor is an uncommon malignant neoplasm in the thoracopulmonary region mainly occurring in children and adolescents. Four young patients with histologically proven Askin tumors were treated in our hospital. In all patients, chest computed tomography imaging demonstrated a chest wall mass with or without destruction of ribs. All patients underwent radical mass resection and postoperative chemotherapy. By the time this article was completed, two of the patients had died with local chest wall recurrences. Here we focus on the imaging features, differential diagnosis, pathology and prognosis of this rare disease.

Extraosseous Ewing’s sarcoma of the pancreas

The Ewing's family of tumors (EFT) comprises a molecularly defined group of "small round blue cell tumors", consisting of Ewing's sarcoma of bone (ESB), extraosseous Ewing's sarcoma (EES), peripheral primitive neuroectodermal tumor (pPNET), and Askin's tumor. Characteristic translocations that disrupt the EWSR1 gene located at 22q12 create novel fusion genes that are central to the pathogenesis. The EFT also shares certain clinical characteristics, such as a peak incidence during the teenage years, a tendency to spread rapidly, and responsiveness to the same chemotherapeutic regimens and radiation therapy. Nearly all patients have occult disseminated disease at diagnosis; hence, chemotherapy is routinely used. Improvements in multimodality treatment have had a dramatic impact on outcomes. EES/pPNET has been reported in a variety of sites, including the pancreas, though this is extremely rare. We describe a case of pancreatic EES/pPNET in a 35-year-old woman and provide a brief review of the relevant literature.

Oncogenic Fusion Protein EWS/FLI1 Down-regulates Gene Expression by Both Transcriptional and Posttranscriptional Mechanisms

Ewing family tumors are characterized by a translocation between the RNA binding protein EWS and one of five ETS transcription factors, most commonly FLI1. The fusion protein produced by the translocation has been thought to act as an aberrant transcription factor, leading to changes in gene expression and cellular transformation. In this study, we investigated the specific processes EWS/FLI1 utilizes to alter gene expression. Using both heterologous NIH 3T3 and human Ewing Family Tumor cell lines, we have demonstrated by quantitative pre-mRNA analysis that EWS/FLI1 repressed the expression of previously validated direct target genes at the level of transcript synthesis. ChIP experiments showed that EWS/FLI1 decreases the amount of Pol II at the promoter of down-regulated genes in both murine and human model systems. However, in down-regulated target genes, there was a significant disparity between the modulation of cognate mRNA and pre-mRNAs, suggesting that these genes could also be regulated at a posttranscriptional level. Confirming this, we found that EWS/FLI1 decreased the transcript half-life of insulin-like growth factor binding protein 3, a down-regulated direct target gene in human tumor-derived Ewing's sarcoma cell lines. Additionally, we have shown through reexpression experiments that full EWS/FLI1-mediated transcriptional repression requires intact EWS and ETS domains. Together these data demonstrate that EWS/FLI1 can dictate steady-state target gene expression by modulating both transcript synthesis and degradation.

Askin's Tumor: A Dual Case Study

Askin's tumor is a rare tumor arising from the chest wall. It is a subset of Ewing sarcoma characterized histologically by the presence of small round blue cells. It is a highly malignant tumor with guarded prognosis, which is dependent upon the extension of tumor at the time of diagnosis. A dual paper of Askin's tumors in young boys is being presented here.

Complete Clinical Response to Neoadjuvant Chemotherapy in a 54-year-old Male with Askin Tumor

Askin tumor is a tumor of the thoracopulmonary region that most commonly affects children and adolescents. These rare tumors are a form of primitive neuroectodermal tumor and typically carry a poor prognosis. Treatment is multimodal and consists of a combination of neoadjuvant chemotherapy, radical resection, and adjuvant chemo- and radiotherapy or all of the above. Surgery is advocated in most cases. We report a case of Askin tumor in a 54-year-old male who showed rapid and complete response to neoadjuvant chemotherapy. This allowed potentially radical surgery to be avoided. At one-year follow-up he remains disease-free.

Unusual Myogenic and Melanocytic Differentiation of Soft Tissue pPNETs

All of the members of the peripheral primitive neuroectodermal tumor family (Ewing sarcomas, neuroectodermal tumors of bone, peripheral neuroepitheliomas, and Askin tumors) have similar morphologic and immunophenotypical features (ie, the proliferation of small and medium-sized round cells in a fibrous background showing strong and diffuse immunohistochemical positivity for CD99), and the common cytogenetic abnormality of a nonrandom translocation involving the EWS gene and one of several members of the erythroblastosis virus transforming sequence family of transcription factors. The combination of clinical information and morphologic/immunophenotypical characteristics is usually sufficient for a correct diagnosis, but there are rare cases in which an unusual predominant or multidirectional immunophenotypical differentiation makes diagnosis a challenge and requires the use of molecular cytogenetic or molecular techniques. We describe 3 such cases in which we employed fluorescence in-situ hybridization analysis to detect translocation involving the EWS gene and reverse transcription polymerase chain reaction followed by sequencing to detect the fusion transcript EWS-FLI1.

Tumeurs nerveuses du médiastin de l’adulte

In adults, mediastinal neurogenic tumours constitute the third group of mediastinal tumours, after thymomas and lymphomas. If the group of neurogenic tumour is frequent, each type of tumour is relatively unusual in everyday's clinic. Among them, nerve sheath tumours are the more frequent, followed by tumour of the autonomic system. Askin tumour remains uncommon. Treatment of this tumour requires complete preoperative work-up, including standard radiography, CT-scan, MRI, and sometimes nuclear imaging. In most cases, the treatment is based on surgical resection, and may be associated with radiotherapy or chemotherapy in case of malignant tumour or incomplete resection. Better understanding of these tumours, including their molecular abnormalities, may lead to new changes in their classifications, and to their management.

Primary Extraskeletal Ewing Sarcoma Originating from Chest Wall in a Child

We report the ultrasonography (US) and computed tomography (CT) findings of a rare case of extraskeletal Ewing sarcoma involving the left chest wall with a diameter of 4cm in a 5 years old girl. The lesion was heterogenously hypoechoic on sonography with a lobulated smooth contour. On CT, the lesion had central necrotic areas and was enhancing heterogeneously. With these US and CT findings surgical excision of the tumor was performed. Histopathologically the mass was evaluated as round cell malignant neoplasm (Ewing tumor/ “Askin tumor”/ primitive neuroectodermal tumor (PNET)). Chemotherapy and radiotherapy were planned to the patient.

Ewing’s sarcoma of larynx: Report of a rare case with review of literature

Primitive neuroectodermal tumors (PNETs) are a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissue and bone. Most peripheral primitive neuroectodermal tumors (pPNETs) manifest in the thoracopulmonary region (Askin tumor), pelvis, abdomen, and extremities and reveal a paucity of cases in the head and neck. The treatment of these is also not well defined. Here, we present a case report with aim of organ preservation.

Efficacy and survival of 92 cases of Ewing's sarcoma family of tumor initially treated with multidisciplinary therapy

Ewing's sarcoma family of tumor (ESFT) is aggressive. The optimal therapy modality for ESFT is still to be found. This study was to explore the clinical characteristics and therapy for ESFT. Ninety-two cases of ESFT were collected from January 1995 to April 2008 in Sun Yat-sen University Cancer Center and analyzed retrospectively. Of 92 cases, 23 were Ewing's sarcoma of bone, 21 extraosseous Ewing's sarcoma, 43 peripheral primitive neuroectodermal tumor, and 5 Askin tumor. Median follow-up time was 31.5 months (range, 10-137 months). Thirty-eight patients received multidisciplinary therapy and 19 single model therapy in non-metastasis group. Three-year overall survival (OS) and event-free survival (EFS) were significantly different between non-metastatic multidisciplinary therapy group and non-metastatic single model group (63% vs. 20%, 46% vs. 18%, respectively, P<0.001). The patients who received surgery plus chemotherapy and plus radiation or not had longer survival than those treated with chemotherapy plus radiation in non-metastatic multidisciplinary therapy group (Chi2=7.591, 9.212; P=0.006, 0.002). CAV/IE alternative regimen was superior to other regimens in event-free survival, but not in overall survival (Chi2=6.950, 3.530; P=0.008, 0.06). Cox regression analysis suggested therapy model and response to treatment were independent prognostic factors for ESFT. Our studying showed multidisciplinary therapy could significantly improve non-metastatic ESFT patients' survival. Chemotherapy plus surgery and plus radiation or not were superior to chemotherapy plus radiation in local control for the non-metastatic ESFT. Therapy model and response were independent prognostic factors.

Tumeur d’Askin : à propos d’un cas avec revue de la littérature

Askin tumor is a rare malignant tumor arising from soft tissues of the chest wall, rarely in the lung. It occurs predominantly in young adults. It still raises many questions about its individualisation and its links with Ewing's sarcoma. We report a case of Askin tumor in a 5-year-old child with reviewing the different data from the literature.

Ewing’s Sarcoma: Standard and Experimental Treatment Options

Ewing sarcoma family tumors (EWS), which include classic Ewing's sarcoma in addition to primitive neuroectodermal tumor and Askin tumor, are the second most common variety of primary bone cancer to afflict adolescents and young adults. Multi-disciplinary care incorporating advances in diagnosis, surgery, chemotherapy, and radiation has substantially improved the survival rate of patients with localized Ewing sarcoma to nearly 70%. Unfortunately, those advances have not significantly changed the long-term outcome for those with metastatic or recurrent disease; 5-year survival remains less than 25%. This apparent therapeutic plateau exists despite extensive effort during the last four decades to optimize the efficacy of cytotoxic chemotherapy through combination of chemotherapies of mechanistically diverse action, dose-dense scheduling (provided as frequently as every 2 weeks), increased adjuvant treatment duration, and higher dosage per cycle (facilitated with parallel strides in supportive care incorporating growth factors). As has already occurred for malignancies such as breast or colon cancer, the "-omics-based" revolution has enhanced our understanding of the molecular changes responsible for Ewing's tumor formation and identified a number of potential targets (such as IGF-1R or mTOR) amenable to biological therapy. It has also created both a challenge and an opportunity to develop predictive biomarkers capable of selecting patients most likely to benefit from targeted therapy. In this review, we discuss current standard-of-care for patients with Ewing's sarcoma and highlight the most promising experimental therapies in early-phase clinical trials.

Askin's Tumor in an Adult: Case Report and Findings on 18F-FDG PET/CT

Primitive neuroectodermal tumor (PNET) of the chest wall or Askin's tumor is a rare neoplasm of chest wall. It most often affects children and adolescents and is a very rare tumor in adults. In this case report, we present an Askin's tumor occurred in a 73-year-old male. The patient was admitted with a history of 3-month lower back pain and cough. In computed tomography, there was a lesion with dimensions of 70 × 40 × 65 mm in the superior segment of the lower lobe of the left lung. Positron emission tomography/computed tomography with 18F-flourodeoxyglucose revealed a pleural-based tumor in the left lung with a maximum standardized uptake value of 4.36. No distant or lymph node metastases were present. The patient had gone through surgery, and wedge resection of the superior segment of left lobe and partial resection of the ipsilateral ribs were performed. Pathology report with immunocytochemistry was consistent with PNET and the patient received chemotherapy after that.

Askin's Tumor: A Rare Chest Wall Neoplasm. Case Report and Short Review

Askin's tumor is a rare neoplasm of the chest wall with a dismalprognosis. We report the case of 16-year-old girl presenting with a painful mass on the right anterolateral chest wall. The patient underwent radical mass resection. Histopathology revealed an Askin's tumor infiltrating the 5th ipsilateral rib and pleura. The girl subsequently received postoperative chemo-radio therapy with a satisfactory response. This is the first reported case of Askin's tumor in Greece and we focus on the clinical, radiological,pathological and therapeutic aspects of this rare entity.

Interaction between ERK and GSK3β Mediates Basic Fibroblast Growth Factor-induced Apoptosis in SK-N-MC Neuroblastoma Cells

The Ewing's sarcoma family of tumors (ESFT) includes Ewing's sarcoma (ES), Askin's tumor of the chest wall, and peripheral primitive neuroectodermal tumor. Basic fibroblast growth factor (FGF2) suppresses the growth of ESFT cells and causes their apoptosis. The underlying mechanism is unclear. Using a human peripheral primitive neuroectodermal tumor cell line, SK-N-MC, we demonstrated FGF2 stimulated phosphorylation of ERK1 and ERK2 (pERK1/2) and GSK3beta (pGSK3beta(Tyr-216)), all of which were primarily retained in the cytoplasm. FGF2 promoted the association between ERK and pGSK3beta(Tyr-216). Inhibitors for GSK3beta (TDZD and LiCl) and ERK (PD98059) protected cells from FGF2-induced apoptosis. On the other hand, inhibitors of GSK3beta, but not PD98059 decreased ERK/pGSK3beta(Tyr-216) association and caused a nuclear translocation of pERK1/2. Similarly, expression of a kinase-deficient (K85R) GSK3beta or GSK3beta-small interfering RNA inhibited FGF2-regulated ERK/pGSK3beta(Tyr-216) association and translocated pERK to the nucleus. Both K85R GSK3beta and small interfering RNA offered protection against FGF2-induced cell death. In contrast, overexpression of wild-type GSK3beta sensitized cells to FGF2 cytotoxicity. Hydrogen peroxide and ethanol enhanced FGF2-stimulated pGSK3beta(Tyr-216), ERK/pGSK3beta(Tyr-216) association, and cytoplasmic retention of pERK1/2. As a result, they potentiated FGF2-induced cell death. Taken together, our results suggested that FGF2-induced accumulation of pERK1/2 in the cytoplasm is toxic for SK-N-MC cells. The formation of an ERK.GSK3beta complex retained pERK1/2 in the cytoplasm. In contrast, disruption of the ERK.GSK3beta complex resulted in nuclear translocation of pERK1/2 and offered protection.

Ewing's Sarcoma Family of Tumors: An Overview From Diagnosis to Survivorship

The Ewing's sarcoma family of tumors (ESFT) is a malignant primary bone tumor often involving soft tissue that affects not only children but also young adults. Since 1992, with the addition of ifosfamide and etoposide to standard chemotherapy for primary tumors, much improvement has been made in the treatment of ESFT, with a primary focus on children. Though often recognized as a childhood cancer, it can affect individuals into the middle years of their lives, but little is known about the outcomes of adults with ESFT. ESFT, which includes Ewing's sarcoma, extraosseous Ewing's sarcoma, Askin tumor, and primitive neuroectodermal tumor, is the second most common primary malignant bone tumor in children and adolescents. It accounts for 10% of primary malignant bone tumors in children and 3% of all childhood malignancies. The most common presenting symptoms of ESFT are pain or swelling. Treatment for ESFT consists of a multimodal approach, including chemotherapy, radiation therapy, and surgery. Children and young adults with Ewing's sarcoma face many physical challenges from their illness and the complications of their treatments. Nurses play an instrumental role in assessment techniques, which lead to prompt evaluation and intervention. Nurses are vital in the education and reinforcement of supportive care needs for this patient population.

Emerging Chemotherapeutic Strategies and the Role of Treatment Stratification in Ewing Sarcoma

The Ewing sarcoma family of tumors (ESFT) is one of the most common groups of malignancies arising in children, adolescents, and young adults up to approximately 25 years of age. It comprises Ewing sarcoma arising from bone and extraosseous Ewing sarcoma arising from soft tissues (which includes peripheral neuroectodermal tumors and Askin tumor arising from the chest wall). Ewing sarcoma is treated successfully in many cases by a combination of chemotherapy, surgery, and radiotherapy. A number of prognostic factors have been identified that can be used to stratify patients according to the risk of relapse, allowing optimization of treatment. These can be categorized as tumor-related factors (presence of metastases, tumor site, volume, lactic dehydrogenase level, chromosomal translocation type, presence of fusion transcripts in blood and bone marrow), treatment-related factors (local therapy, histologic response to chemotherapy, radiologic response to chemotherapy, chemotherapy regimen), and patient-related factors (gender, age). Newer chemotherapeutic agents are currently being investigated, and there is now increasing interest in the identification of molecular targets in ESFT that could be exploited therapeutically, which include the mammalian target of rapamycin (mTOR) and insulin growth factor-1 (IGF-1) receptor pathways.

Diagnostic imaging of 8 cases of Askin tumor

Objective To study the imaging features of askin tumor and to improve the knowledge and differential diagnosis ability for askin tumor.Methods All cases were pathologically proved and the imaging appearances of askin tumors in 8 patients were reviewed retrospectively.All eight patients had plain chest films,7 patients had chest CT scans,1 patient had chest MR imaging and 4 patients had bone scintigraphy.Results Chest wall soft tissue masses were demonstrated in all 8 cases.4 cases were on the left side and 4 cases on the right.6 large masses were heterogeneous on CT scan and 2 small masses were homogeneous on CT scan or MR imaging.There were cystic and necrotic areas in 4 cases on CT scan.Rib destructions were demonstrated in 6 cases.Four cases were demonstrated on CT scan,1 case on MR imaging and 3 cases were demonstrated increased activities on bone scintigraphy among of them.Pleural effusions were demonstrated in 6 cases.The interfaces between lung and tumor were irregular in 7 cases and regular in 1 case.There was no calcification in all 8 cases.Conclusion The imaging feature of askin tumor is chest wall soft tissue mass,however it is non-specific.Askin tumor should be taken into account in the differential diagnosis when a chest wall mass is identified in children and young adults.

Diagnosis and Treatment of Ewing's Sarcoma

Ewing's sarcoma is a small round-cell tumor typically arising in the bones, rarely in soft tissues, of children and adolescents. Ewing's sarcoma has retained the most unfavorable prognosis of all primary musculoskeletal tumors. Prior to the use of multi-drug chemotherapy, long-term survival was less than 10%. The development of multi-disciplinary therapy with chemotherapy, irradiation, and surgery has increased current long-term survival rates in most clinical centers to greater than 50%. In addition, the preferred method of tumor resection has changed; limb salvage has nearly replaced amputation of the affected limb. Limb salvage procedures can be performed in place of amputation without compromising patient survival rates. Recent studies have revealed that the pathognomonic translocations involving the EWS gene on chromosome 22 and an ETS-type gene, which is most commonly the Fli1 gene on chromosome 11, are implicated in more than 95% of Ewing's sarcomas, primitive neuroectodermal tumors and Askin's tumors. Therefore, these lesions have become regarded as a single entity, dubbed the Ewing's family of tumors. RT-PCR to detect EWS-ETS gene arrangements is widely used to confirm the diagnosis of Ewing's family of tumors. Experimental results suggest that inhibition of the signaling pathway downstream of the EWS-ETS gene may lead to the development of molecularly targeted therapy in the future.

Askin tumor with metastasis to the scalp: a histochemical, immunohistochemical and ultrastructural study

A 29-year-old woman presented with facial edema, and imaging disclosed a tumor extending from the anterior chest wall to the anterosuperior aspect of the mediastinum. Transbronchial cytology of the primary tumor and biopsy of the metastatic scalp lesion were performed. Histologically, the tumor consisted of closely packed small round cells. The neoplastic cells generally had round nuclei, finely dispersed chromatin, and small to prominent nucleoli. Histochemically, the cytoplasm of the neoplastic cells contained abundant glycogen and stained with Grimelius silver. Immunohistochemically, the neoplastic cell membranes reacted with CD99 (MIC2) and the neoplastic nuclei reacted with Fli-1, but various other markers, including lymphocyte and skeletal muscle markers, were not detected. No neoplastic cells were also reactive for chromogranin A, synaptophysin, and neurofilament. Ultrastructurally, some neoplastic cells had delicate cytoplasmic processes and contained membrane-bound dense core granules in the cytoplasm. Even if results are immunohistochemically negative for neuroendocrine markers, the combination of immunohistochemistry of CD99 (MIC2) and Fil-1 may be useful in diagnosing Askin tumor or its metastatic lesion.