Askin Tumor Research Articles

Askin's Tumor in the Chest Wall-a Rare Clinical Entity and Review of Literature.

Askin tumors are the rare malignancy of neuroectodermal origin of the thoracic wall. Its prevalence is more in younger age group who present with vague symptoms leading to delayed diagnosis. We hereby present a case report of complex management of large chest wall tumor in a young boy and review the literature of this entity.

Ewings Sarcoma Mimicking a Schwannoma: MRI Findings of a Rare Case

Ewing’s sarcoma is among the most frequent yet highly aggressive neoplasms of the bones presenting in adolescents and children under the age of ten with a slight male predilection. It is now broadly categorized into a set of tumors recognized as Ewing’s sarcoma family based on the same histology and genotype of these tumors. This group of tumors includes Ewing’s sarcoma of bones, the 2nd most frequent bone malignancy, Askin tumors, PNET, and those rarely occurring extraosseous Ewing sarcoma (peripheral neuroepithelioma). Extra-osseous Ewing’s is a rare presentation occurring in only 5% of patients. Here we present such a case of an extra-osseous Ewing’s sarcoma in a 13-year-old female presenting to our hospital with a large mass in the sacral region. The interesting imaging findings and his histopathology are discussed.

ASKIN’S TUMOR: A CASE REPORT AND LITERATURE REVIEW

Askin tumour is an uncommon form of malignant neoplasm that develops from the soft tissues of the thoracopulmonary wall and has a neuroectodermal origin and possess aggressive behavior. As it mimics other common pediatric disorders such as empyema, tuberculosis, lymphoma, rhabdomyosarcoma and neuroblastoma, it acts as a great diagnostic and therapeutic challenge to the treating clinicians. Its rarity, however, contributes greatly to the absence of standardized treatment protocols further facilitating detrimental prognosis. So, the major deciding factor of its survival rate is early and precise diagnosis followed by its treatment. The present study reports a case Askin’s tumor in a child which is locally aggressive and quite rare. While making the definitive diagnosis of an Askin’s tumour of the thorcopulmonary region the possibility of the occurrence of other primitive neuroectodermal tumors (PNET) was not ruled out.

A case of askin’s tumor presenting as acute paraplegia

Askin tumor is an uncommon malignant neoplasm of a neuroectodermic origin that arises from the soft tissues of the thoracopulmonary wall. Defined histologically by malignant small round cell tumor and immunohistochemistry positive for CD99. The lack of clinical guides that establish a standardized management contributes to its poor prognosis and short overall survival.

Askin’s Tumor in Children: Two Cases Report

Askin tumor is a rare malignant thoracopulmonary tumor that primarily affects children. In University Hospital Mohammed VI's Pediatric Oncology-Hematology department, two young patients with Askin's tumor were treated. Chest computed tomography imaging in both patients revealed an Askin's tumor, which was later confirmed histologically. Both patients got postoperative chemotherapy as well as a surgical excision of the tumor. One of them passed away as a result of a localized chest wall recurrence. In this article, we discuss our experiences with this serious pathology.

Ewing Sarcoma of Chest Wall (Askin\'s Tumor) in a 3-year-old Child: A Case Report

Fever and difficulty in breathing in a child are common symptoms encountered in routine practice. Often, the underlying cause will be infectious etiology due to bacterial, viral, or tuberculous. However, a malignancy of the paraspinal soft tissue region mimicking infectious disease clinically in a child is challenging to diagnose and treat. Here, we report the Askin's tumor of the chest wall diagnosed in a child presented with fever, worsening dyspnea, and unilateral lung whiteout on a chest radiograph to insist on the importance of extensive and earlier evaluation even for common clinical symptoms.

Askin's tumor – A dual case study of rare thoracic neoplasm

Askin's tumor – A dual case study of rare thoracic neoplasm

Penile Ewing's Sarcoma - A Rare Entity

Abstract Introduction/Objective Ewing sarcoma is a cancer involving the bones or the tissue around bones. It is the second most common malignant bone tumour comprising 10-15% of all primary bone tumours, most commonly affecting children and young adults. It can also be seen in adults also. Males are more commonly affected then females. Any bone can be affected but the lower extremity, the pelvis, upper extremity, axial skeleton and ribs, and face cases. Similar histology tumours when seen in soft tissue are peripheral primitive neuroectodermal tumour (pPNET), neuroepithelioma, and Askin tumour. It is always difficult to know the primary site when soft tissues are involved as it can be infiltration from bone or from soft tissue to bone. Penis is least common site involved with Ewing’s sarcoma in young male. Methods/Case Report This is a case report of a 22 year old male with penile lesion. He presented with the chief complaints of mass at the shaft of penis. The partial penectomy specimen was received along with the bilateral inguinal lymph node. On gross there was a ulcero-proliferative growth. Results (if a Case Study Enter NA) On histopathological examination showed small blue round cell tumour and further immunohistochemistry with CD99 and NKX2.2 revealed the diagnosis of Ewing’s sarcoma, however lymph nodes showed reactive lymphoid hyperplasia. Conclusion The Ewing sarcoma at the penile region is rarest entity. Only 7 cases are reported worldwide. The imaging modalities like CT or MRI can be used to know the extension. The diagnosis is primarily based on morphological features, immunohistochemical analysis and translocation analysis. However it is important to find out if any other soft tissue site is involved. The important prognostic markers are presence and absence of metastasis, size of tumour, necrosis and presence of fusion transcripts.

Askin Tumour: A Rare Thoracopulmonary Tumour in Adults

Askin tumour, a primitive neuroectodermal tumour of the thoracopulmonary region, is a rare tumour presenting in childhood. Its presentation in adults is rare. We report a case of an Askin tumour in an adult patient who presented to us with worsening breathlessness and vague chest pain. Investigations including immunohistochemistry confirmed the diagnosis of Askin tumour.

Primary Ewing\u2019s sarcoma of the kidney: a rare masquerader of renal cell carcinoma on imaging

BackgroundThe Ewing sarcoma family of tumors comprises a group of high-grade small round cell tumors, including Ewing sarcoma of bone, extra-skeletal Ewing sarcoma, peripheral primitive neuro-ectodermal tumor (PNET), and Askin tumor (thoraco-pulmonary PNET). They are more prevalent in young children and adolescents. Ewing's Sarcoma is an aggressive tumor majorly described in bones. Primary renal Ewing's sarcoma is an extremely rare entity, characterized by a very aggressive course, with very few reported cases in the literature.Case presentationWe present an 18-year-old girl who presented with sudden onset left flank pain and hematuria. The patient had histopathology-proven primary renal Ewing's sarcoma, which was initially misdiagnosed as renal cell carcinoma on imaging.ConclusionsOwing to its non-specific radiological appearance, a high index of suspicion and a systematic approach is essential for detection of renal Ewing’s Sarcoma.

Intramedullary, periosteal, and extraskeletal Ewing sarcomas: retrospective study of a series of 126 cases in a reference center.

To evaluate the proportion of extraskeletal, periosteal, and intramedullary Ewing sarcomas among musculoskeletal Ewing sarcomas. Our single-center retrospective study included patients with musculoskeletal Ewing sarcoma diagnosed between 2005 and 2019 in our pathology center (cases from our adult bone tumor referral center and adult and pediatric cases referred for review). Recurrences, metastases, and visceral Ewing sarcomas were excluded. Intramedullary Ewing sarcomas were defined by involvement of the medullary cavity. Periosteal cases were defined by involvement of the subperiosteal area without extension to the medullary cavity. Extraskeletal cases were defined by the absence of involvement of the bone tissue and the subperiosteal area. Our series included 126 patients with musculoskeletal Ewing sarcoma, including 118 skeletal Ewing sarcomas (93.7%) and 8 extraskeletal Ewing sarcomas (6.3%). Of the 118 skeletal Ewing sarcomas 112 were intramedullary (88.9%) and 6 were periosteal (4.8%). Extraskeletal Ewing sarcomas were more common in women and in patients older than 40 (p < 0.05). The 6.3% proportion of extraskeletal Ewing sarcoma is lower than the median of 30% estimated from the literature. This difference could be explained by an overestimation of extraskeletal Ewing sarcomas of the chest wall (Askin tumors), an underestimation of periosteal cases confused with extraskeletal cases, and the presence of "Ewing-like" soft tissue sarcomas in previous series. Because of its prognostic and therapeutic impact, the distinction of morphologic subtypes requires the cooperation of experienced radiologists and pathologists.

Ewing Sarcoma in the Sciatic Nerve: Case Report

AbstractEwing sarcoma (ES) is a malignant neoplasm that affects bones and soft tissues, usually in young patients. Currently, ES is grouped with other tumors that share the same histological and genotypic characteristics, forming the Ewing Sarcoma Family of Tumors (ESFT), which includes ES of bone, extraosseous ES (peripheral neuroepithelioma), Askin tumor, and peripheral primitive neuroectodermal tumor (PNET). Its origin in peripheral nerves is extremely rare, making its diagnosis and treatment very challenging. We describe a case of a 27-year-old male with extraosseous ES originating in the sciatic nerve, which was surgically removed, and discuss the difficulties encountered in the management of this patient.

Askin Tumor: A Case Report of a Rare Tumor

Askin tumor is a rare malignant neoplasm of neuro-ectodermal origin that emerges from the soft tissue of the thoraco-pulmonary wall and has aggressive activity. This paper presents a study on a 10-year-old boy having breathing difficulty and mass on the anterior portion of the left thoracic wall. On examination, there was a mass on the left anterior chest wall measuring 13x13 cm2, which was hard, tender, and fixed to the chest wall. There was associated pleural effusion and infiltration into the left lung. Histopathology confirmed primitive neuro-ectodermal tumor (Askin tumor) with IHC marker CD99 positive. Chemotherapy was used to treat the patient. Early detection of this tumor would necessitate a high index of suspicion, allowing for a better prognosis and result. The overall prognosis is poor due to the higher rate of local recurrence.

A case of Ewing Sarcoma of the rib with metastasis to chest wall

A 13-year-old girl presented to the Respiratory Medicine Department with complaints of left sided chest pain, progressive dyspnoea &amp; fever of 1 month's duration. Clinical examination revealed diffuse swelling on antero-lateral aspect of left chest with moderate pleural effusion on the left side. FNAC from the swelling was suggestive of Askin tumor &amp; pleural fluid was hemorrhagic with repeat cytology positive for malignant cells. Histopathology showed neoplastic cells with scanty cytoplasm, hyperchromatic nuclei and rosette formation suggestive of Ewing sarcoma, and on follow-up she developed metastasis in the upper part of chest wall. Because of this rare presentation we are reporting this case.

A CASE OF PRIMITIVE NEUROECTODERMAL TUMOR (PNET) PRESENTING AS ANTERIOR MEDIASTINAL MASS

Peripheral primitive neuroectodermal tumors (pPNET) are a group of highly malignant tumors composed of small round cells of neuroetodermal origin that affect soft tissue and bone ,most commonly present in the thoracopulmonary region (Askin tumor), abdomen , pelvis and rarely in the head and neck. They usually present in the second decade of life, with slight male preponderance and account for 4-17% of all soft tissue tumors. Clinical symptoms depend on the site of presentation but invariably include pain and swelling of surrounding structure due to mass effect. Current recommendations advocate complete surgical resection whenever possible, adjuvant or neoadjuvant chemotherapy and radiotherapy. Here we present a 20 year old male patient who presented with chest pain and breathlessness and on investigation found to have an anterior mediastinal mass which further evaluation found to be a primitive neuroectodermal tumor

Interdisciplinary Radical "En-Bloc" Resection of Ewing Sarcoma of the Chest Wall and Simultaneous Chest Wall Repair Achieves Excellent Long-Term Survival in Children and Adolescents.

Introduction: Ewing sarcomas of the chest wall, historically known as “Askin tumors” represent highly aggressive pediatric malignancies with a reported 5-year survival ranging only between 40 and 60% in most studies. Multimodal oncological treatment according to specific Ewing sarcoma protocols and radical “en-bloc” resection with simultaneous chest wall repair are key factors for long-term survival. However, the surgical complexity depends on tumor location and volume and potential infiltrations into lung, pericardium, diaphragm, esophagus, spine and major vessels. Thus, the question arises, which surgical specialties should join their comprehensive skills when approaching a child with Ewing sarcoma of the chest wall.Patients and Methods: All pediatric patients with Ewing sarcomas of the chest wall treated between 1990 and 2020 were analyzed focusing on complete resection, chest wall reconstruction, surgical complications according to Clavien-Dindo (CD) and survival. Patients received neo-adjuvant chemotherapy according to the respective Ewing sarcoma protocols. Depending on tumor location and organ infiltration, a multi-disciplinary surgical team was orchestrated to perform radical en-bloc resection and simultaneous chest wall repair.Results: Thirteen consecutive patients (seven boys and six girls) were included. Median age at presentation was 10.9 years (range 2.2–21 years). Neo-adjuvant chemotherapy (n = 13) and irradiation (n = 3) achieved significant reduction of the median tumor volume (305.6 vs. 44 ml, p < 0.05). En-bloc resection and simultaneous chest wall reconstruction was achieved without major complications despite multi-organ involvement. Postoperatively, one patient with infiltration of the costovertebral joint and laminectomy required surgical re-intervention (CD IIIb). 11/13 patients were treated with clear resections margins (R1 resection in one patient with infiltration of the costovertebral joint and marginal resection <1 mm in one child with multiple pulmonary metastases). All patients underwent postoperative chemotherapy; irradiation was performed in four children. Two deaths occurred 18 months and 7.5 years after diagnosis, respectively. Median follow-up for the remaining patients was 8.8 years (range: 0.9–30.7 years). The 5-year survival rate was 89% and the overall survival 85%.Conclusion: EWING specific oncological treatment and multi-disciplinary surgery performing radical en-bloc resections and simultaneous chest wall repair contribute to an improved survival of children with Ewing sarcoma of the chest wall.

COMPUTER TOMOGRAPHY AND X-RAY DIAGNOSTICS OF PERIPHERAL PRIMITIVE NEUROEECTODERMAL TUMORS (PNET) IN CHILDREN

Relevance: Peripheral primitive neuroectodermal tumor (PNET) belongs to the group of malignant tumors that develop from migrating embryonic neural crest cells. PNET includes a number of nosological forms: Askin's tumor, esthesioneuroblastoma, the very peripheral primitive neuroectodermal tumor, Ewing's sarcoma . PNET accounts for 3–9% of all soft tissue tumors in children and 19% of all soft tissue sarcomas. In European countries and the United States, the frequency of their occurrence is 3.4 cases per year per 1 million children under 15 years of age, in Kazakhstan - 0.6-1.2 cases per 1 million child population. Rapid tumor growth, malignancy and early metastasis to other organs and systems predetermines its special role in oncology. Objective of the study: to improve the quality and availability of early diagnosis of sarcomas in children in medical institutions of the general medical network. Results of the study: 35 cases of peripheral PNET in children were analyzed. The age of the patients is from 1.5 to 17 years. The average age is 9.3 years. There were 1.3 times more boys among patients than girls. Extra-skeletal localization was found in one patient (3.6%). Radiographically, it is often almost impossible to make a differential diagnosis between the manifestations of Ewing's sarcoma and primary chronic or "healed" (antibiotic) forms of acute hematogenous osteomyelitis in the initial phase of the process, before the formation of the extraosseous soft tissue component. The process of bone damage is more often localized in its diaphysis and subsequently spreads to the metaphyses of the bone.

Ewing Sarcoma/Peripheral Neuroectodermal Tumor of Bone and Soft Tissue in Infants: A Report from Children Cancer Hospital of Egypt

Introduction: Ewing sarcoma is an aggressive malignancy of bone and soft tissue. It is rare in children under age 5years. Ewing's sarcoma family tumors include classic Ewing’s sarcoma, primitive neuroectodermal tumour and Askin tumor ES is the second most common variety of primary bone cancer in adolescents and young adults. The treatment strategy for ES is characterized by multi-disciplinary collaboration between pediatric oncologists, medical oncologists, radiation oncologists, and orthopedic surgeons. Although the survival rate of ES patients has improved, their prognosis remains unsatisfactory, and the treatment of ES is still challenging specially in age group below 3 years. Patients and methods: A Retrospective study of children below 3 years with a diagnosis Ewing Sarcoma/PNET treated at our CCHE from July 2009 till December 2018. Results: A review of 46 patients with age ranged from 3 months to 3 years, Mean of age was 1.8. Primary site was Osseous and extraosseus in 23 infant patients for each. Main site was mainly axial in 28 cases (60.8%). Cases were treated according to COG protocol. Infantile Ewing sarcoma OS and EFS at 5-years OS were 75.5% and 66.7%. Conclusion: Patients below 3 years represent an unusually young age group. Conclusion: ES/PNET in age below 3 years is a unique age group with a different management and outcome.

Ewing sarcoma of the clavicle (case report)

Introduction: The American pathologist James Stephen Ewing (1866 -1943) first described this tumor in 1920. The sarcoma originates in the beginnings of nerve tissue. It contains small, round, immature cells, and it is closely related to soft tissue tumors, such as pPNET, Askin's tumor and neuroepithelioma, which together form a family - Ewing's tumors (ESFT). The tumor affects more often Caucasian men, is extremely rare in African Americans, and is typical of children and adolescents. In 95% of cases, the patients are between 4 and 25 years old. The clavicular localization of the tumor is extremely rare, almost casuistic, especially in male individuals over 35 years of age. A few similar cases have been described in the available literature. Аim: The aim of this article is to focus on the diagnostic challenges, the difficulties associated with surgical removal, as well as the early metastases of Ewing's sarcoma with clavicular localization. Materials and Methods: The presented patient is a 35-year-old man with late-diagnosed Ewing's sarcoma of the left clavicle. Results: Despite the en-bloc excision of the tumor mass, chemo- and radiotherapy, the patient survived for only three years. Conclusion: Early diagnosis of a sarcoma located in the clavicle is very important due to the anatomical features of the area, which makes soft tissue radicalization of the tumor extremely difficult at an advanced stage.

Rosettes-Diagnostic Feature of Askin Tumor on Cytology.

Askin tumor is a rare malignant small round cell neoplasm of primitive neuroectodermal origin in the thoracopulmonary region, predominantly seen in childhood and adolescence. It belongs to the spectrum of Ewing's sarcoma family of tumors (ESFTs). Cytologically, ESFTs show small round cells with pseudorosettes. We report a case of an Askin tumor in an adult patient, diagnosed on fine-needle aspiration cytology. Clinico-radiological findings, immunochemistry, and cytogenetics help in confirming the diagnosis.