Askin Tumor Research Articles

Primary Intra-abdominal Ewing's Sarcoma in Adults: A Multimodality Imaging Spectrum

Ewing sarcoma (ES) and peripheral primitive neuroectodermal tumor are part of a spectrum of neoplastic diseases known as the ES family of tumors, which also includes extraosseous ES, peripheral primitive neuroectodermal tumor, malignant small-cell tumor of the thoracopulmonary region (Askin's tumor), and atypical ES. Although imaging features of these tumors have been previously described, extensive discussion about intra-abdominal manifestations is scarce in the literature. We present an imaging spectrum of these tumors in the abdomen and pelvis with particular attention to multimodality approach for staging and restaging.

Comparison of clinical features and outcomes in patients with extraskeletal vs skeletal Ewing sarcoma: an SEER database analysis of 3,178 cases.

BackgroundThe clinicopathological characteristics, outcomes and prognostic factors of primary extraskeletal Ewing sarcoma (EES) remained insufficiently explored. We aimed to examine these aspects and compared the same with skeletal Ewing sarcoma (SES).Patients and methodsWe identified Ewing sarcoma, peripheral primitive neuroectodermal tumors or Askin tumor patients who were registered in the Surveillance, Epidemiology, and End Results database from 1973 to 2014. Clinicopathological features were assessed by using Fisher’s exact tests. Cancer-specific survival (CSS) and overall survival (OS) were estimated by using the Kaplan–Meier method and the Cox proportional hazards model. Prognostic factors were identified by multivariate Cox regression analysis.ResultsThe age of patients with EES was diagnosed to be higher and they were more likely to be female (46.1% vs 36.2%; P<0.001), have tumor <10 cm (49.8% vs 35.4%; P<0.001), have regional node involvement (5.4% vs 1.0%; P<0.001) and receive surgery (69.1% vs 53.8%; P<0.001) compared to patients with skeletal tumors. Metastatic status did not differ by origin. Kaplan–Meier analysis showed that the origin had significant difference in CSS and OS among patients aged 0–19 years and with metastatic stage at presentation, but not in patients aged 20–39, ≥40 years and with no-metastatic stage. A Cox multivariable model controlling for differences between groups confirmed inferior survival for patients with EES. Age, tumor size, tumor stage and surgery were the most important factors significantly influencing both CSS and OS in the EES and SES patients. Race, year of diagnosis and tumor site were associated with CSS and OS among patients with SES, but failed in EES.ConclusionThe clinicopathological characteristics, outcomes and prognostic factors differed among patients with EES compared to patients with SES. Extraskeletal origin was an unfavorable prognostic factor.

Recurrent Primitive Neuroectodermal Tumor (PNET) of the Pancreas

Ewing's family of tumors (EFT) originate from neuronal crest cells and share morphological, histological and cytogenic features. EFTs include typical (osseous) Ewing's sarcoma, atypical (non-osseous) Ewing's sarcoma, primitive neuroectodermal tumor (PNET), and Askin tumor. Only a few cases of ES/PNETs of the pancreas have been reported and even fewer cases of recurrent pancreatic PNETs are known. Herein we report a case of recurrent pancreatic PNET presenting as abdominal pain ten years following treatment of left scapular Ewing's sarcoma. Four years following partial resection, local radiation and CHOP chemotherapy for left scapular Ewing's sarcoma, a 54-year-old male presented with epigastric abdominal pain. CT scan of the abdomen demonstrated a mass at the head of the pancreas and biopsy confirmed a CD-99 positive round blue cell tumor. FISH showed 15 % ESWR1 locus rearrangement. Together these findings were consistent with pancreatic PNET. The patient was treated with local radiation and CHOP chemotherapy. Treatment response was monitored with serial PET scans. Unfortunately, after six years of sustained response the patient presented with epigastric abdominal pain. Laboratory studies were significant for elevated bilirubin (1.1 mg/dL). A mesenteric vein thrombosis was detected on CT scan as well as a 43 mm by 29 mm mass at the pancreatic head. PET scan confirmed a 19 mm by 22 mm FDG avid area at the head of the pancreas. Biopsy demonstrated a CD-99 positive round blue cell tumor. Overall the findings were consistent with recurrent pancreatic PNET.EFT/PNETs are round blue cell tumors with neuroendocrine differentiation (chromogranin A, synaptophysin or neuron specific enolase) and CD-99 expression. Although, CD-99 is nonspecific. Additionally, many EFTs demonstrate t(11; 12) (q24; q12) chromosomal translocation resulting in the fusion protein EWS-FLI1 which disrupts transcription and drives tumor growth. EFTs commonly arise from long bones and afflict young patients. Although rare, EFT/PNETs can arise from solid organs. Primary PNETs of the lung, urinary bladder, gall bladder, and uterus have been reported. Only 0.3 % of pancreatic masses are diagnosed as EFT/PNET. Our case highlights the importance of correlating clinical, radiographic, and pathological findings in the diagnosis of pancreatic PNET and emphasizes the importance of multimodality treatment of this aggressive disease.

Ultrasound-guided continuous paravertebral block in management of Askin's tumor in an elderly patient

Ultrasound-guided continuous paravertebral block in management of Askin's tumor in an elderly patient

Askin's tumor in adult: A rare clinical entity

Askin's tumor in adult: A rare clinical entity

PUB010 Results after Interdisciplinary Management of 5 Patients with Askin Tumor

PUB010 Results after Interdisciplinary Management of 5 Patients with Askin Tumor

Caso Clínico: Tumor de Askin.

Introducción: El tumor neuroectodérmico primitivo (PNET) de la pared torácica también denominado tumor de Askin es una neoplasia rara y agresiva, que afecta mayoritariamente a niños y adolescente. Caso Clínico: Se reporta el caso de un joven de 17 años que presentó una gran tumoración subescapular derecha, acompañado de dolor y disnea a quien realizó previa quimioterapia neoadyuvante y dada buena repuesta, resección tumoral con márgenes oncológicos más reconstrucción plástica de hemitórax derecho. Constatándose como hallazgo intraoperatorio, no evidente en imágenes, metástasis pulmonar. Presenta evolución favorable posquirúrgica con posterior recurrencia local y metástasis a distancia, sin respuesta a la quimioterapia adyuvante con fallecimiento 7 meses luego de la cirugía. Conclusión: En el presente caso el TA tuvo una sobrevida corta a pesar el tratamiento neoadyuvante, resección quirúrgica y nuevo ciclo de tratamiento quimioterápico.

Atypical Presentation of Primary Non-metastatic Ewings Sarcoma of the Lumbosacral Spine-“Cauda Equina Syndrome” Clinically and a “Tuberculosis mimicking challenge” on MRI-A Case Report

Introduction: The Ewing family of tumors comprises Ewing's sarcoma (EWS), extraskeletal EWS, primitive neuroectodermal tumor (PNET) of bone and soft tissue, and chest wall tumor (Askin tumor). The translocation t(11; 22) (q24; q12) is identified in more than 90% of cases.EWS is the second most common primary bone malignancy in childhood. In contrast to long bone involvement, delays in spinal EWS diagnosis may occur because symptoms may not be present until neurological deficits occur. To date, although there have been reported cases of EWS in the lumbosacral region, the reported cases are very less and the presentations might drastically differ. Case Presentation: A 14 year old boy comes with a 12 weeks h/o urinary incontinence and constipation. Over the next 4 months, the patient had new-onset intermittent abdominal pain with worsening urinary symptoms.MRI confirmed a diagnosis of Koch's spine. However, the histopathology reported it as an “Ewings sarcoma/Primitive Neuroectodermal Tumor”. Discussion: Ewing’s tumor of sacrum is rare, but should be suspected in low backache in children. Cauda equina syndrome can be a valid presentation for EWS spine. MRI can identify cases early and enables early treatment though it is not specific. Histopathological diagnosis is a must before any definitive management.EWS spine can mimick Tuberculosis both clinico-radiologically, only to be confirmed with a tissue biopsy. Conclusion: Ewing’s sarcoma of the lumbosacral spine can have an atypical presentation and there should be a high degree of suspicion to diagnose it early.

Extra-osseous Ewing sarcoma of the thyroid gland mimicking a hemorrhagic cyst of Hashimoto thyroiditis

Ewing’s family of tumors, including Ewing sarcoma, Askin tumor, and peripheral primitive neuroectodermal tumors, were first identified by James Ewing in 1921. Ewing sarcoma is more common in bones. However, extraosseous Ewing sarcoma (EoES) is a very rare separate entity and was first described by Tefft and al. in 1969. It is a highly malignant round-cell tumor and contains a reciprocal translocation between chromosomes 11 and 22, t(11;22). In this report, we present the case of a 34 years old female patient who was unfortunately diagnosed with Ewing sarcoma of the thyroid after being operated for a hemorrhagic cyst in a context of Hashimoto thyroiditis.The patient was followed at the endocrinology department for an auto-immune thyroiditis for two years. She complained of neck pain and a suspicious hemorrhagic cyst was discovered on thyroid ultrasound. She underwent total thyroidectomy; three weeks later, she suffered from right sided firm swelling in her neck. The pathology was in favor of EoES of the thyroid. To date and to the best of our knowledge, this is the first case of Ewing sarcoma of the thyroid in auto-immune thyroiditis. EoES of the thyroid is a very rare entity and should be considered in the differential diagnosis of a solid tumor in the thyroid. Diagnosis is based on ultrastructural examination, immunostaining and evidence of abnormal translocation t(11;22). It is usually an aggressive disease.Early diagnosis with adequate surgical management and chemotherapy are the mainstay of the treatment and may change the outcome.

Askin Tumor in Pregnant Women

Askin Tumor in Pregnant Women

Askin tumour following traumatic rib injury in a young adult

Askin tumour is a form of primitive neuroectodermal tumour arising from the thoracopulmonary region. The patient being reported is a young adult who initially presented in dyspnoea and was found to have hemothorax following chest wall injury. About one year later, he again presented in dyspnoea. But this time he was found to have a mass arising from the previous traumatic site, which was diagnosed as Askin tumour. Such a scenario of Askin tumour arising from a traumatic site is uncommon. Moreover, Askin tumour itself is a rare presentation in adults.Asian Journal of Medical Sciences Vol.8(3) 2017 84-86

胸壁原発悪性小細胞腫瘍(いわゆるAskin腫瘍)の1例

胸壁原発悪性小細胞腫瘍(いわゆるAskin腫瘍)の1例

Askin Tumor: A Case Report of Primitive Neuroectodermal Tumors of the Chest Wall – Computed Tomography Findings

AbstractAskin tumor is a rare malignant neoplasm in the thoracopulmonary region. Its diagnostic approach is complex and requires a multidisciplinary team. Given the rarity of this entity, we report a case of Askin's tumor.

Kidney Cancers in Adolescents &amp; Young Adults

Kidney Cancers in Adolescents &amp; Young Adults

Imaging in Askin tumors

Objective This study aimed to identify the patient demographics and describe the imaging patterns of Askin tumors.

Second children born after autotransplantation of cryopreserved ovarian tissue in a young patient previously treated with chemotherapy for Askin's diseasethe successful of fertility preservation program

Oncological treatment was associated with a reduced ovarian reserve, the chance of a successful pregnancy is currently around 1/3 for those with ovarian-tissue autotransplantationOvarian tissue cryopreservation is an effective option,gaining ground as a valid method for fertility preservation especially for pre-pubertal patients and patients who have a short time between diagnosis of a disease and gonadotoxic treatment.More than 62 children worldwide have now been born following this procedure. Case report and a retrospectively analyse, of patients whose underwent ovarian tissue cryopreservation at IFER, Argentina between 1999-2015.One of these women, had orthotopic transplantation, after chemotherapy premature ovarian failure (POF) has now conceived again following natural conception. She gave birth to a healthy girl on 7/2015 and is therefore the second woman in the world to have had two children, from separate pregnancies, born as a result of transplanting frozen/thawed ovarian-tissue. 68 patients referred from private oncological units underwent cryopreservation of ovarian tissue before gonadotoxic therapy for malignant disease. 7 patients (10,3%) died during follow-up due to recurrence of disease. 24 post-pubertal patients without the need for grafts of cryopreserved tissue. 4 patients had undergone ovarian tissue transplantation in order to restore their fertility after remission of the disease and one of these women became pregnant twice and has given birth two healthy babies. In 2005 a 28 year old patient was referred for fertility preservation before undergoing aggressive chemotherapy for Askin tumor. Several strips of ovarian cortical tissue were obtained by laparoscopy and cryopreserved . The patient underwent surgical treatment, and chemotherapy (CVEA), ovarian failure was established. In 2009 orthotopic transplantation of frozen-thawed ovarian tissue was carried out by laparoscopy in the right ovary,.Three months after transplantations she resumed regular menses. Four IVF cycles were performed with no pregnancy.9/2012 POF was established. In 2/2013 another laparoscopy was performed with replacement of ovarian tissue this time in the left ovary. In 5/2013 spontaneous menses began. An Intrauterine Insemination resulted in pregnancy. In 2/2014, and after an uneventful pregnancy, a healthy baby boy was born.The patient was breastfeeding her first baby and returned to our fertility clinic in 9/2014 a pregnancy test revealed that she had already conceived naturally,after an uneventful pregnancy a normal healthy girl was born in 7/2015. The present result supports cryopreservation of ovarian tissue as a valid method of fertility preservation to be used in women facing gonadotoxic treatment. However alternative procedures such as oocyte or embryo cryopreservation should be considered as first options especially for older patients.

CT findings of Askin tumor of chest wall

Objective To analyze CT manifestations of chest wall Askin tumor and to illustrate its CT diagnostic and differentially diagnostic key points. Methods Eleven cases of chest wall Askin tumors were pathologically confirmed in our hospital between May 2006 and November 2014. Of them, 10 cases were children and adolescents, and 9 cases had chest pain as the first symptom. All patients received plain CT scan, while 7 cases received contrast enhanced scan. CT signs of this group were retrospectively analyzed, including the location, quantity, shape, size, density, adjacent tissue invasion and distant metastasis of the tumors. Results CT examination showed a single oval mass on the chest wall in all 11 cases. The tumor was located completely within the thoracic cavity in 10 cases, while a tumor's main part was within thoracic cavity. Nine tumors had long diameters of more than 9 cm. All the tumors were heterogeneous without calcification, wherein 10 cases showed necrosis and cystic degeneration. On contrast enhanced scan, 7 cases showed heterogeneously mild to moderate enhancement. All tumors invaded adjacent tissues, including 7 cases of bone damage of a single rib, and 7 cases of pleural involvement, wherein 6 cases had pleural effusion. No remote metastases were found in this group. Conclusions CT manifestations of Askin tumors of chest wall have some characteristics, and familiarity with these manifestations is conducive to diagnosis and differential diagnosis of this disease. Key words: Askin tumor; Thoracic wall; Tomography, X-ray computed

Askin Tumor in Egyptian Patients; 5 Years Experience at the National Cancer Institute, Cairo University

Background: Askin tumor is a primitive neuroectodermal tumor of the Ewing sarcoma family arising from soft tissues of the chest wall. It is the commonest chest wall malignancy in children and adolescents. Its diagnosis is complex and management requires a multidisciplinary work including chemotherapy for systemic disease and radiation therapy to assist local control which is achieved through surgery with or without reconstruction. Objective: To analyze report and understand the clinicopathological features, results and outcome of this tumor with assessment of early and late postoperative complications following resection and chest wall reconstruction. Materials and Methods: This is a retrospective analysis of 30 cases with chest wall ES/PNET presented to the National Cancer Institute; Cairo University between January 2011 and December 2015. All patients’ records were revised for age, sex, clinical presentation, imaging, pathology, operative notes, different treatment modalities given, early and late postoperative complications following surgical resection or reconstruction, and outcome including overall survival (OS) and disease free survival (DFS). Results: Our cohort included 30 patients with chest wall ES. The median follow up period (n = 30) was 33.7 months (ranging from 3.7 to 69.3 months). The median OS was 54.2 months with cumulative OS at 60 months which was 45.6%. The median DFS was 27.9 months with cumulative DFS at 60 months which was 40.6%. All cases were below 18 years with a male predominance (n = 19). The commonest affected sites were ribs (n = 22, 73.3%), the scapula (n = 5, 16.6%), the clavicle (n = 2, 6.66%) and the sternum (n = 1, 3.33%). 23 patients (76.6%) were presented with localized disease, and 7 patients (23.4%) were metastatic from the start. All patients received neoadjuvant chemotherapy (4 cycles of VAC/IE) followed by local control: either surgery (26 cases, 86.6%) or radical radiotherapy (3 cases, 10%). A single case of a rib ES with initial bilateral lung deposited where no local control was done. After chest wall resection, closure of the defect was done by 1 ry closure with no reconstruction or double layer prolene mesh and bone cement that was covered by pedicled flap (latissimus dorsi, serratus anterior or pectoralis major muscle flap). Postoperative radiation therapy was given to 9 patients: 4 (13.3%) had postoperative poor chemotherapy effect (<90% tumor necrosis), 3 (10%) had +ve microscopic safety margin and 2 (6.6%) had pleural based nodules with malignant pleural effusion at initial presentation. Conclusion: Askin’s tumor shows a dramatic response to polychemotherapy. Treatment of such tumor should include multidisciplinary working groups for optimum results and better survival.

Askin tumor: A rare neoplasm of thoracopulmonary region.

Askin tumor is a rare neoplasm of thoracopulmonary region. But it mimics other common pediatric disorders, such as empyema, lymphoma, and tuberculosis, posing a great diagnostic and therapeutic challenge to the treating clinicians. So it is of utmost importance to make an early diagnosis and proper referral/treatment in such cases. We highlighted diagnostic challenge, treatment, and favorable outcome of a case that presented to us.

Askin's tumor: 11 cases and a review of the literature

Askin's tumor is a peripheral primitive neruoectodermal tumor within the thoracopulmonary region, which primarily occurs in children and young adults. In addition, Askin's tumor is commonly misdiagnosed, as it is rare and easily mistaken for other small round-cell tumors. The present study aimed to investigate the clinical characteristics, prognostic factors and treatment outcomes of patients diagnosed with Askin's tumor. Computed tomography (CT) scans, histopathology and immunohistochemical analysis were used for diagnosis. Patients were treated with combined (surgery-chemotherapy-radiotherapy) or mono-therapy (chemotherapy or radiotherapy) methods. A total of 11 consecutive patients with Askin's tumor (aged 8-22 years) were treated at the First Affiliated Hospital of Zhengzhou University between April 2010 and June 2013; nine patients underwent combined therapy and two patients were treated using mono-therapy. Chest lumps, swelling and pain were the most common presenting symptoms. Patients were followed up for ≤24 months post surgery and the results revealed that the median survival time of the combined and mono-therapy treatment groups were 15 and 7 months, respectively. Primary tumor size, metastasis, lactate dehydrogenase indicators and tumor stages were found to be important prognostic factors affecting patient outcome. In conclusion, the results of the present study demonstrated that the combination of surgery, chemotherapy and radiotherapy resulted in the optimal outcome for Askin's tumor patients.