Extraosseous Ewing's Sarcoma Research Articles

Extraosseus Ewing's Sarcoma in Pancreas: A Review.

Primitive neuroectodermal tumors (PNET, previously referred to as peripheral neuroepithelioma) are rare malignant tumors with various degrees of differentiation belonging to the Ewing’s family of sarcomas. They are classified as round cell tumors arising from soft tissues. In rare instances, PNETs may arise from solid organs containing neuroendocrine cells of kidney, bladder, heart, lungs, parotid glands and pancreas. Most cases occur in the second decade of life with a slight preponderance in males. PNET of the pancreas is an aggressive tumor with multiple recurrences and a relatively poor prognosis. These tumors should be considered in the differential diagnosis, especially in a diagnosed pancreatic tumor in individuals less than 35 years of age. Due to the nature of the tumor, surgery with subsequent chemoradiation are widely accepted modalities despite the poor prognosis. In this article, we review 25 cases of extraosseous Ewing’s sarcoma (ES) of the pancreas which to the best of our knowledge, enlists most cases reported in the literature thus far.

Multi-modality management of extraosseous Ewing sarcoma: 10-year experience from a tertiary care centre

AbstractAim:To analyse the presentation, diagnosis and patterns of care of extraosseous Ewing sarcoma treated at our institution between 2008 and 2018.Methods:Electronic medical records of extraosseous Ewing sarcoma patients treated at our institution between January 2008 and April 2018 were reviewed. Kaplan–Meier curves were plotted to assess the overall and disease-free survival with 95% confidence intervals. A univariate analysis was carried out to assess the impact of variables such as surgical excision, completeness of surgery, completeness of chemotherapy and addition of radiation therapy on the survivorship.Results:The records of 65 patients treated at our institution were available for review. The mean age was 26·4 years. The most frequent sites of extraosseous Ewing tumour were kidney—9/65 (13·8%) and brain—10/65 (15·4%). Sixteen (24·6%) patients presented with inoperable/metastatic disease at diagnosis. The other 49 (75·4%) had localised disease at presentation. The median overall survival of the 49 non-metastatic patients was 46 months, and the disease-free survival was 45 months.Conclusion:Extraosseous Ewing sarcoma is a rare and aggressive tumour diagnosed by molecular techniques. Multi-modality treatment including surgical resection with wide margins, adjuvant radiation when indicated and completion of systemic chemotherapy results in optimum outcomes.

Primitive neuroectodermal tumor (PNET)/Ewing sarcoma of kidney: A rare case report of three cases

Primitive neuroectodermal tumor (PNET) / Ewing sarcoma of kidney are rare aggressive tumor with poor prognosis. It occurs in young adults generally in 2nd to 3rd decade of life We report three cases of PNET/Ewing sarcoma of kidney in patients of age 25and 35 years with complain of hematuria and abdominal pain .CT scan revealed heterogeneously enhancing lesion in kidney and we received radical nephrectomy specimen with variegated appearance on gross. Microscopic examination showed tumor with malignant cells in sheets and pseudorosettes .The diagnosis was given as PNET/ES and was confirmed with immunohistochemistry. Renal PNET/ES is a member of Ewing sarcoma family. They are rare tumor originating from the neural crest and are more aggressive than PNET at any other site. The presence of Homer Wright rosettes is less common in extra-osseous Ewing sarcoma and their presence is a sure diagnosis of PNET/ES. The differential diagnosis for these small round cell tumor of kidney other than PNET include malignant lymphoma, renal neuroblastoma,,embryonalrhanbdomyosarcoma, wilms tumor. The immunohistochemical marker CD99 positivity is diagnostic. Other markers positive are S-100, vimentin and NSE (neuron specific enolase). Histopathology and immunohistochemistry remains gold standard for confirming the diagnosis.

Management and outcome of extraosseous ewing's sarcoma family tumors treated at a tertiary care center in North East India: A retrospective analysis

Introduction: Extraosseous Ewing's Sarcoma is rare, aggressive malignant soft-tissue tumors treated similar to Ewing's sarcoma of bone. This study evaluates the clinicopathological pattern, treatment and outcomes of localized extraosseous Ewing's sarcoma family tumors (ESFTs) treated with an uniform treatment regimen. Materials and Methods: This is a retrospective single institution study where we evaluated the hospital records of localized extraosseous ESFT treated between January 2011 and December 2018. Fifteen patients were found eligible for analysis. Patient demographics, management details, and outcomes were analyzed statistically. Time to event was measured from the date of diagnosis and survival curves were estimated by Kaplan–Meier method with Log-rank test for comparison. Results: The median follow-up of the cohort was 17 months (range: 3–81 months). The mean age of patients was 14.4 years and the average tumor size was 9.92 cm. Two-thirds of the patients received definitive radiotherapy as the local treatment with 93.3% patients receiving induction chemotherapy. The 5-year local control rate, progression-free survival, and overall survival (OS) were 80%, 53.3%, and 46.7%, respectively. On univariate analysis, tumor size

Primary Ewing's sarcoma of the jejunum presenting as sepsis

Extraosseous Ewing's sarcoma (ES), also referred to as extraskeletal ES, is a rare tumor of primitive cells that occurs in children, adolescents, and young adults. This tumor mainly involves the soft tissue of the extremities and thorax. We report a case of extraskeletal ES of the jejunum without associated skeletal location. A 26-year-old woman with no medical history presented to the emergency department with a 3-day history of a fever along with nausea, vomiting, and abdominal pain. An abdominal contrast computed tomography scan revealed a homogeneous bowel mass size 8.5 cm × 4.3 cm × 6.1 cm in the lower abdomen with invasion of the ileum is noted. Extraluminal free air and abscess formation size 10.6 cm × 5.2 cm × 8.3 cm is noted, suggestive of bowel perforation. After the surgery, the specimen was sent to the pathology department for examination. We received one segmental jejunum (25 cm in length and 6 cm in diameter of the lumen) that weighed 450 g in total. Sections revealed an ill-ulcerative mass measuring 6 cm × 4 cm × 0.5 cm with gray-to-brown coloration and extensive necrosis with perforation; the mass was located 15 and 7 cm in length from the bilateral cut ends. In addition, focal vascular proliferation, and infiltrate deeply to the serosa with mesenteric enlarged lymph nodes were observed. Furthermore, the mesenteric regional lymph nodes show metastatic sarcoma. The definitive diagnosis was based on the findings of histomorphology, immunohistochemistry, and molecular pathology.

Primary Intradural Extramedullary Extra-OsseousEwing’s Sarcoma of Cervical Spine (C5-C7)

Ewing's sarcoma is a rare aggressive tumor common in bone or skeletal component. Primary Intradural Extra-medullary Extra-Osseous Ewing sarcoma of cervical spine is extremely rare malignant neoplasm and only few cases have been reported in the literature. There is a paucity of data regarding the clinical features, natural course, pre-operative diagnosis, treatment modalities and post-operative management. Herein, we report a case of a 22-year-old man who presented with inability to walk and numbness in both lower limbs. MRI findings suggested a possibility of nerve sheath tumor but was diagnosed as Intradural Extra-skeletal Ewing sarcoma on histopathological analysis. The literature with regard to primary spinal intradural extra-skeletal Ewing sarcoma was reviewed.

A rare case of primary extra-osseous Ewing’s sarcoma presenting in the posterior neck

Ewing sarcoma is classified as Ewing’s sarcoma of the bone tissue, extraskeletal Ewing’s sarcoma, peripheral primitive neuroendocrine tumor, malignant small cell tumor of the thoracopulmonary region (Askin) and non-typical Ewing’s sarcoma. Extraosseous Ewing’s sarcoma may be seen throughout the body, however only reported at 4% in the neck. In our case patient has a painless mass growing on his posterior neck for 3 months.On the magnetic resonance imaging, a well-defined, encapsulated, well-demarcated mass lesion between the perivertebral muscles and the subcutaneus fat tissue was detected. Tumor was surgically resected and diagnosis was pathologically Ewing’s sarcoma. 22q11 translocation was detected in the molecular examination. Extraosseous Ewing sarcomas are rarely seen soft tissue masses. Although imaging features are not sufficient to make a specific diagnosis, it is important take the biopsy from the appropriate place and staging the tumor. In addition, complete surgical resection has been shown to associated with better survival rates compared to other Ewing sarcoma family. For this reason, imaging has an important role in the guidance of surgery and resectability of the tumor. Young patients with a fast growing, palpable mass should be evaluated carefully. Although the tumor is thought to be morphologically benign in the first, ekstraosseous Ewing’s sarcoma must be considered in the differential diagnosis of head and neck soft tissue masses.

Primary Ewings Sarcoma/Primitive Neuroectodermal Tumor (PNET) of the Breast: a Rare Tumor

Extra osseous Ewing’s sarcoma (EES) is regarded as a member of Ewing’s sarcoma family of tumors (ESFT). A 16-year-old lady presented to the outpatient department of our institute with awareness of progressively increasing lump in the right breast of 5 months duration. Biopsy from the lump done elsewhere was suggestive of poorly differentiated malignancy. On examination, there was a large mass occupying almost the entire right breast. Computed tomography scan (chest) showed a large well-defined minimally enhancing heterogeneous soft tissue lesion of size 17.3 × 14.6 × 14.4 cm in the right breast. Both lungs were normal. Metastatic work up was within normal limits. Review of the biopsy at our center showed fragmented tissue exhibiting sheets of monotonous population of round cells with dark staining nuclei with mitotic figures and minimal cytoplasm. On immunohistochemical (IHC) analysis, the neoplastic cells exhibited CD99 and Friend leukemia integration 1 (FLI1) positivity whereas they were negative for leucocyte common antigen (LCA), desmin, and myogenin, which was consistent with Ewing’s sarcoma/PNET family of tumor. She received 2 cycles of neoadjuvant chemotherapy with VAC (vincristine, doxorubicin, cyclophosphamide) + IE (ifosfamide, etoposide) alternately following which simple mastectomy with latissimus dorsi myocutaneous flap reconstruction was done. Less than 4 cases of EES of the breast have been documented in Indian literature so far. In this report, we discuss the clinicopathological features of this rare and fascinating tumor with a brief review of literature.

Atypical Presentation of Ewing’s Sarcoma

Abstract Background: Ewing’s sarcoma (ES) is an osseous malignancy of small round blue cells which may manifest even in soft tissue. It is the second most common primary tumor of bone in childhood. The common areas of occurrence are the diaphyses of femur followed by tibia, humerus, pelvis, and clavicle. However, there are many unusual presentations of ES on the basis of the site, e.g., craniofacial bones, paravertebral mass, and visceral organs. Chemotherapy with radiation therapy and cytoreductive surgery/limb salvage surgery is the mainstay treatment along with a strict patient compliance and counseling, critically being important for long-term survival. This study depicts presentations of ES who either presented with some unusuality at the time of diagnosis or developed peculiar features uncharacteristic of ES during their course of treatment. Context: This study provides an insight into the patterns of unusual presentation of ES and prognosis of such patients with current line of management. Multiple sites of metastases have a very poor dismal outlook. Aims: This study purports the importance of considering ES as a systemic disease rather an osseous malignancy and reviews the pattern of unusual sites of presentation. Setting and Design: This observational study was carried out in the Department of Radiotherapy, Gandhi Medical College, Bhopal. Only patients with unusual patterns of metastases were included in the study cohort. Materials and Methods: We systematically reviewed patients with confirmed and immunohistochemistry-proven ES from July 2014 to July to 2017. A total of 69 patients were registered within the time frame. Of the 69 patients, 36 (52.2%) were males and 33 (47.8%) were females. Of the 11 (16%) unusual presentations, 8 were males and 3 were females. The mean age of presentation was 22.5 (3–52 years). All patients received chemotherapy as per treatment guidelines. Each patient was individually followed up and metastatic workup was performed in a systematic manner. Statistical Analysis: Since the pattern of metastasis was observed, no significant statistical analysis was required for this study. Results: Of the 11 patients, six succumbed during the course of treatment, one patient was lost to follow-up, while four patients are on regular follow-up. Of the six patients who succumbed, four had multiple metastases at the time of presentation to our outpatient department, while the other two patients had paraspinal ES with paraplegia. Two patients, one male and one female, had associated comorbidities with spherocytosis and bronchial asthma. The youngest patient was a 3-year-old child with paraspinal ES, while the oldest was a 52-year-old with extraosseous ES of the right thigh who presented with lung, liver, and supraclavicular metastases. Conclusion: In our study, pattern of metastases determined the patients’ survival. Those patients who presented with multiple metastases at the time of presentation had a very poor prognosis, with death occurring within 2–3 months. The chemotherapy protocols were neither modified nor changed. They were evaluated after every cycle. Those patients who had developed multiple metastases at the time of admission to our outpatient clinic fared the worst. Those who had metastases to the brain and lung also succumbed to the malignancy. It is time we consider ES as an osseo-systemic malignancy and incorporate newer techniques such as circulating tumor cells in the investigation and evaluation portfolio for better and aggressive management. A multidisciplinary tumor board is absolutely essential and weekly meetings mandatory to individualize the treatment scenario. In our study, uncustomary as it may seem, their metastatic sites demand a certain degree of limelight in the Annals of Oncology.

Sarcoma de Ewing extraóseo en la columna dorsal con compresión medular al momento del diagnóstico. Reporte de un caso

Extraosseous primitive neuroectodermal tumors are very rare neoplasms. Only a few cases have been published in the literature. This report is about a 9-year-old female patient whose clinical manifestations showed spinal cord compression, so different diagnosis should be considered, including oncology disease. The patient showed a mass of the dorsal spine with spinal cord compression. She was diagnosed with Ewing Sarcoma after surgical intervention and pathology study. It is necessary to highlight the importance of the present case due to the low incidence of the extraosseous Ewing Sarcoma, and its infrequent location at the dorsal spine and the spinal cord compression as initial presentation of pediatric cancer, as well as the poor information related to the best therapeutic strategy to treat this disease at this location.

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.

Rhabdomyosarcoma and Extraosseous Ewing Sarcoma.

Rhabdomyosarcoma (RMS) is a malignant tumor that represents the most common form of pediatric soft tissue sarcoma. It arises from mesenchymal origin and forms part of the group of small round blue cell tumors of childhood. It has a constant annual incidence of 4.5 cases per 1,000,000 children. The known histological diagnosis of the two major subtypes (embryonal and alveolar) has been recently enhanced by tumor biological markers and molecular differentiation diagnostic tools that have improved not only the updated classification based on risk stratification, but also the treatment approach based on the clinical group. Ewing sarcoma (ES) is a round cell tumor, highly malignant and poorly differentiated that is currently the second most common malignant bone tumor in children. In rare instances, it develops from an extraskeletal origin, classified as extraosseous Ewing sarcoma (EES). We provide an updated, evidence-based and comprehensive review of the molecular diagnosis, clinical and diagnostic approach and a multidisciplinary medical and surgical management according to the latest standard of care for the treatment of pediatric RMS and EES.

Augmented reality in computer‐assisted interventions based on patient‐specific 3D printed reference

Augmented reality (AR) can be an interesting technology for clinical scenarios as an alternative to conventional surgical navigation. However, the registration between augmented data and real-world spaces is a limiting factor. In this study, the authors propose a method based on desktop three-dimensional (3D) printing to create patient-specific tools containing a visual pattern that enables automatic registration. This specific tool fits on the patient only in the location it was designed for, avoiding placement errors. This solution has been developed as a software application running on Microsoft HoloLens. The workflow was validated on a 3D printed phantom replicating the anatomy of a patient presenting an extraosseous Ewing's sarcoma, and then tested during the actual surgical intervention. The application allowed physicians to visualise the skin, bone and tumour location overlaid on the phantom and patient. This workflow could be extended to many clinical applications in the surgical field and also for training and simulation, in cases where hard body structures are involved. Although the authors have tested their workflow on AR head mounted display, they believe that a similar approach can be applied to other devices such as tablets or smartphones.

Ewing Sarcoma Presenting as a Congenital Scalp Mass.

Ewing sarcoma is a locally aggressive, highly malignant tumor most commonly seen in the skeletal system. The "Ewing family of tumors" also includes other tissue types that are not common, such as soft tissue origin classified as extraosseous Ewing sarcoma (EES) or primitive neuroendocrine origin. Age of onset most often occurs within the first 2 decades of life. Congenital presentation of EES is exceedingly rare. We report the first described case to our knowledge of congenital EES originating from the scalp.

Case report and literature review of Ewing's sarcoma in the gastrointestinal tract

We present a case of a young female patient with extraosseous Ewing's sarcoma (EES) arising in the lesser sac, as confirmed by membranous staining for CD99 and an ESWR1 gene translocation on fluorescence in situ hybridization. We also provide comprehensive review of the English literature of Ewing's sarcoma (ES) occurring in the gastrointestinal tract (GIT). A systematic review of the PubMed database was carried out with the following MeSH terms: Ewing's AND sarcoma AND (oesophagus, stomach, small bowel OR intestine, large bowel OR intestine, colon, sigmoid, rectum, pancreas, peritoneum, lesser sac, greater sac, liver and gallbladder). Fifty‐seven cases were collated from 46 articles. The most common overall symptom was pain (60.71 per cent), which occurred in patients as young as 2 years and as old as 72 years. The median age was 31 years, and the mean age was 29 years. No bimodal distribution was demonstrated. More females appeared to be affected than males. EES of the GIT is rare. It differs from ES with respect to patient age and sex. No standard therapy for EES has been widely adopted.

Intraparenchymal central nervous system Ewing's sarcoma. A case treated according to the modified Euro-EWING protocol

Ewing's sarcoma (ES) is the second most common malignant bone tumor in children and young adults. ES may also occur as a primary soft tissue neoplasm without involvement of the bone and is then referred to as extraosseous ES (EES). The paediatric central nervous system extraosseous Ewing's sarcoma (CNS-EES) is an extremely rare entity since less than 20 cases have been reported in literature. Moreover, only less than 10 cases correspond to brain parenchyma primary tumors. These cases have been often misdiagnosed with other varieties of primary brain tumors, particularly those which were called PNET (Primitive Neuro-Ectodermal Tumor). We present a case of true intraparenchymal CNS-EES and a comparison of epidemiology, behaviour, treatment, and prognosis of CNS-EES and intracranial PNET cases.

Extra skeletal Ewing's sarcoma: extra skeletal Ewing’s sarcoma of mesentery masquerading dysgerminoma ovary

Extraosseous Ewing's sarcoma is a rare entity that involves the lower extremities, paravertebral regions of the spine, retroperitoneum, pelvis, and the chest wall. Involvement of mesentery as this case has been only reported five times in English literature. An eleven-year-old girl was planned for gynaecological surgery for suspected ovarian dysgerminoma. Intraoperatively she was referred to the surgical team since tumor arising from mesentery of the terminal ileum. Complete resection of the tumor with a right hemicolectomy was done. Histopathological assessment of the tumor revealed features of small blue round cell tumor which after an expert immunohistochemical staining reported as extraosseous Ewing sarcoma. She underwent a course of adjuvant chemotherapy and currently well during follow up. Due to the rarity of this tumor, a proper consensus on management has not been outlined. However, complete resection and adjuvant chemotherapy remain a standard therapy which showed an excellent post-operative outcome.

PICTORIAL REVIEW OF EXTRAOSSEOUS EWING’S TUMOUR: A SINGLE CENTER EXPERIENCE

Purpose: Ewing’s family tumour is an extremely rare tumour, with annual incidence rates amongst Caucasian children <21 years being in the range of 2–3 cases per million in the U.S. There are mainly three subtypes including Ewing’s sarcoma (ES) of bone, extraosseous (EO) Ewing’s tumour and Peripheral primitive neuroectodermal tumour. Although extremely rare, this study represents a review of various types of cases and the significance of imaging including its baseline and post-treatment response radiological characteristics. There are a very few cases of EO ES in the current literature with variable spectrum of tumour site and their imaging characteristics.Materials and Methods: Electronic records were retrospectively reviewed from 1 May 2011 to 1 May 2016 with patients who were diagnosed as histologically proven ES. A number of patients, gender and base line computed tomography (CT)/magnetic resonance imaging findings for staging were reviewed.Results: A total of 568 patients with diagnosed ES were analysed, of which 15 patients had EO type of ES. Of these only 8 patients had baseline imaging available which included tumours arising from the occipital region, orbit, anterior mediastinum, anterior abdominal wall, mesentery, kidney, prostate gland and presacral region.Conclusion: EO ES is a rare entity and can involve a wide array of soft tissue organs. A cross-sectional imaging with CT and MR has a key role in pre- and post-treatment assessment.Key words: Computed tomography, Ewing’s sarcoma, extraosseous Ewing’s, magnetic resonance imaging, peripheral primitive neuroectodermal tumour

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.

Surgical treatment of retrosternal extraosseous Ewing Sarcoma in a 6-years old female: A clamshell approach with hemysternectomy and application of a non-crosslinked extracellular matrix

Abstract Background Ewing Sarcoma (ES) and Neuroblastoma (NB) belong to a family of tumours of primitive neuroectodermal origin (PNET) that occurs in both bone and soft tissue. Notwithstanding ES and NB are two distinct malignant tumours, sometimes there could be a link between them. Case report We describe a case of an extraosseous ES localized in the retrosternal region and the upper lobe of the right lung, which had been previously treated for NB in a 6 years old female. We treated this case with a clamshell approach which allows, in a one-step surgery, a complete excision of the mass reconstructing the hemysternectomy with a non-crosslinked matrix. Conclusion the clamshell approach is therefore useful to achieve the retrosternal space and the lung with a single surgical access. According to our experience, we consider appropriate to use a non-crosslinked matrix for sternal reconstruction.