Extraskeletal Ewing's Sarcoma Research Articles

Extraskeletal Ewing's Sarcoma Family of Tumours in Adults: Analysis of 57 Patients from a Single Institution

Aims Extraskeletal Ewing's sarcoma (EES) is a rare form of soft tissue sarcoma. The aim of the present study was to assess the outcome and the prognosis of adult patients presenting with EES treated with multi-modality therapy. Materials and methods All EES patients older than 15 years referred to our institution between January 1995 and December 2004 were reviewed. In total, 57 patients were identified. Their median age at diagnosis was 20 years (range 15–57). Results The median size of the primary tumour was 11 cm (range 4–30 cm). Eighteen patients (31%) had metastatic disease at initial presentation. Wide surgical resection with negative margins was achieved in 23 cases (40%). Chemotherapy consisting of vincristine, adriamycin, ifosfamide, actinomycin D was given in 50 patients (88%). Radiotherapy was delivered in 37 patients (65%). Forty-one patients (72%) achieved complete remission and 16 (28%) progressed on therapy. Twenty-one patients (51%) relapsed. Local recurrence was encountered in 15 patients (36%). At a median follow-up of 46 months (range 6–143 months), the 5-year event-free survival and overall survival rates were 35 and 47%, respectively. Metastases at presentation, tumour size and surgical resection margin associated significantly with overall survival and event-free survival. Conclusion EES is an aggressive type of tumour with a high incidence of local recurrence and distant metastasis. This series showed that the outcome of adult EES is not unlike that of skeletal Ewing's sarcoma in terms of response to multi-modality treatment and the prognostic factors influencing treatment outcome. Adequate surgical resection, aggressive chemotherapy and adjuvant local radiation therapy, when indicated, constitute the optimal treatment to achieve the best results in this rare type of disease.

Primary extraskeletal Ewing's sarcoma of the lung

Ewing's sarcoma is a neuroectodermal tumour characteristically presenting during the second decade of life and arising from bone. Extraskeletal Ewing's sarcoma is exceptionally rare. We present a case of a 44-year-old man who underwent pneumonectomy for a primary extraskeletal Ewing's sarcoma of the lung. This demonstrates a very rare pulmonary Ewing's sarcoma and highlights the importance of preoperative evaluation in aggressive tumours.

Soft tissue sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Soft tissue sarcomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Extraskeletal Ewing's sarcoma: a report of 18 cases andliterature review

Extraskeletal Ewing's sarcoma (EES) is a rare, rapidly growing, round-cell, malignant tumor that can develop in the soft tissues at any location. This study was to analyze the clinical features, diagnosis and treatment of EES. Clinical data of 18 patients with EES, treated at between Cancer Center of Sun Yat-sen University between 1995 and 2007, were analyzed. Of the 18 patients, 13 were male and 8 were female, aged from 8 months to 60 years. Twelve (66.7%) patients were between 5-25 years of age. Eight (44.4%) patients had tumors originated from low extremities.Sixteen patients had masses at their first visit. Sixteen patients were treated by the combined modality therapy, and 2 patients were treated by the single modality therapy. The 1-, 3- and 5- year actuarial survival rates were 82.4%, 64.2% and 32.1%, respectively. The presence of metastatic disease at the time of diagnosis and the mode of treatment were prognostic factors. EES is common in adolescent. It often manifests as a localized mass. The combined modality therapy is recommended for this disease. The presence of metastatic disease at the time of diagnosis and the mode of treatment are prognostic factors.

Primary cutaneous Ewing’s sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings

Extraskeletal Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are widely regarded as clinically and histologically identical tumors which consist of small blue round cells. Extraskeletal ESs/PNETs usually occur in the deep soft tissues of the paraspinal region, chest wall, or lower extremities. However, superficially located cases, so-called cutaneous ESs/PNETs, are exceedingly rare, and the vast majority of the reported cases present as a single small mass. We present magnetic resonance imaging (MRI) findings and clinical course of a unique case of primary cutaneous ES/PNET presenting as numerous huge masses with severe ulceration on them.

Extraskeletal Ewing's Sarcoma of parapharyngeal space

Abstract Extraskeletal Ewing's Sarcoma (EES) of head and neck region, is a rare soft tissue tumour morphologically similar to the commoner Ewing's Sarcoma arising from bone. We report a case of EES in parapharyngeal space in a 6-year-old male; who presented with stridor. CT scan of neck showed soft tissue mass in right parapharyngeal space with intraorbital and intracranial extention. Histopathological examination showed round cell tumour and immunohistochemistry was positive for CD99 and Vimentin. The patient responded very well to Concurrent Chemoradiation. An extensive review of English literature, to the best of our knowledge, did not reveal any previous case of EES in parapharyngeal space in children; although one similar case in a 53-year male is reported.

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.

Fine needle aspiration cytology diagnosis of paravertebral extraosseus Ewing′s sarcoma

Extraskeletal Ewing’s sarcoma (EES) is a rare tumor. Paravertebral Ewing’s sarcoma requires more extensive therapy as compared to Ewing’s sarcoma of bone. Fine needle aspiration cytology (FNAC) plays an important role in the early diagnosis of these cases. We present a case where paravertebral extraosseous Ewing’s sarcoma was diagnosed on FNAC in a 19-year-old girl.

Extraskeletal Ewing's Sarcoma of the Breast, Mimicking Cyst

Extraskeletal Ewing’ sarcoma (EES) is a rare soft tissue tumor morphologically indistinguishable from the osseous Ewing’s sarcoma (ES). We report a case of recurrent EES of the breast that, to the best of our knowledge, has rarely been reported. It was initially confused for a cyst on ultrasound. In addition, MRI and breast-specific gamma imaging (BSGI) in our case have complementary value over conventional imaging.

Primary Extraosseous Ewing Sarcoma of the Kidney With Level III Inferior Vena Cava Thrombus

Ewing sarcoma typically presents as a skeletal-based tumor, with rare instances of peripheral primitive neuroectodermal tumor (PNET) arising in the soft tissues. Few examples of organ-based PNETs have been previously described in the literature. These tumors are exceedingly rare as a primary renal neoplasm. To our knowledge, this is the first report documenting a primary extraskeletal Ewing sarcoma of the kidney with thrombus extending into the intrahepatic inferior vena cava thrombus (level III).

Long-term results with combined treatment in adult localized extraskeletal Ewing's sarcoma (ES): A retrospective analysis of 51 patients (pts) from a single referral center

10548 Background: After the first description in 1975, only small series of adult extraskeletal ES have been reported. In general, adulthood is claimed to be an adverse prognostic factor, while the meaning of extraskeletal origin is less established. Methods: We retrospectively reviewed adult pts with extraskeletal, localized ES, undergoing medical therapy between 1988 and 2004, who were included in the dataset of the current Adult Sarcoma Medical Unit at Istituto Nazionale Tumori, Milano, Italy, which serves as a reference center for adult soft tissue sarcoma. Fifty-one pts were treated with a combined approach, including chemotherapy, surgery when appropriate, and radiation therapy. Overall, 27/51 pts were male, and age ranged from 16 to 63 years. Site of primary lesions was: thorax 14 (27%), limbs 18 (35%), paravertebral region 5 (10%), pelvis 4 (8%), head&neck 3 (6%), superficial trunk 6 (12%), other 1 (2%). Seven pts presented with locally advanced disease. Chemotherapy regimens included: vincristine (V), ifosfamide (I), epirubicin (E)/adriamycin (A), dacarbazine (D), actinomycinD (ActD), cisplatin (C), etoposide (Et). 35/51 pts (68%) underwent preoperative chemotherapy: 30 pts received IVE × 4–7 cycles; 5 pts received VAI alternating with IEt × 9 cycles. 42/51 pts underwent surgery; 38/51 pts received radiation therapy. Results: Response rate to induction chemotherapy including antracicline, ifosfamide + etoposide was 83%. Of these pts, 9 showed a complete response (CR), 17 a partial response (PR), 3 a minor response (MR), 3 stable disease (SD), and 3 progressive disease (PD). Overall, DFS at 10 years (y) was 55%. Pts with limb, head&neck, or superficial trunk lesions had a better outcome compared to those with other primary sites (DFS at 10y is 71% vs 33%). Conclusions: In this series of adult pts with extraskeletal ES, response rate and DFS were comparable to those of ES of bone in conventional series. The primary site may identify a subset of pts with worse outcome and prognosis, for whom more intensive treatments could be investigated. No significant financial relationships to disclose.

Soft tissue sarcomas: ESMO Clinical Recommendations for diagnosis, treatment and follow-up

The following recommendations apply to adult-type soft tissue sarcomas arising from limbs and superficial trunk. Recommendations on retroperitoneal sarcomas, desmoidtype fibromatosis, uterine sarcomas head and neck sarcomas and breast sarcomas are provided separately at the end of the chapter with regard to those main aspects by which they differ from more frequent soft tissue sarcomas. In general, the main principles of diagnosis and treatment may well apply to all soft tissue sarcomas, including the rarest presentations [e.g. visceral sarcomas other than gastrointestinal stromal tumours (GISTs)], which therefore are not specifically covered. Specific histological types, however, may deserve specific approaches, not necessarily covered hereafter, given the scope of these Recommendations. Extraskeletal Ewing sarcoma as well as embryonal and alveolar rhabdomyosarcoma are covered by other ESMO Clinical Practice Guidelines, inasmuch as they need completely different approaches. The same applies to GIST. Kaposi’s sarcoma is excluded from this chapter.

Ewing's sarcoma of the bone: ESMO Clinical Recommendations for diagnosis, treatment and follow-up

Ewing's sarcoma of the bone: ESMO Clinical Recommendations for diagnosis, treatment and follow-up

Small Cell Extraskeletal Osteosarcoma

Extraskeletal osteosarcoma is a rare malignant mesenchymal neoplasm that accounts for <4% of all osteosarcomas and approximately 1.2% of all soft tissue sarcomas. Among the extraskeletal osteosarcomas, the small cell type is extremely rare. This article describes a 31-year-old man who had small cell extraskeletal osteosarcoma arising from the semimembranosus muscle. An incisional biopsy was performed and the histopathological findings showed many small cells and osteoid formation. The results were reported as a malignant small round cell tumor, consistent with an extraskeletal Ewing's sarcoma or primitive neuroectodermal tumor. Immunohistochemically, the tumor showed reactivity with antibodies against CD99 and neuron-specific enolase, but not with antibodies against S100 protein, CD138, alpha smooth muscle actin, chromogranin, Ki-67, leukocyte common antigen, epithelial membrane antigen, CD30, or desmin. The patient refused neoadjuvant chemotherapy. One week after an open biopsy, a wide marginal resection was performed. The final diagnosis was small cell extraskeletal osteosarcoma. Adjuvant chemotherapy was performed using doxorubicin, ifosfamide, and cisplatin together with a total of 60 Gy of radiation therapy. At 2-year follow-up, the functional Enneking score of the operated lower extremity was 28 points. We performed chest computed tomography, magnetic resonance imaging, and positron emission tomography-computed tomography. There were no regional recurrence and distant metastasis. Presently the patient is disease free.

Manifestaciones radiológicas infrecuentes del sarcoma de Ewing en la infancia

ObjectivesTo review the cases of Ewing's sarcoma (ES) diagnosed in children at our hospital from 1995 to 2005 and to analyze uncommon imaging signs at diagnosis or during the course of the disease. Material and methodsWe reviewed the cases of 21 patients with ES (12 boys and 9 girls) diagnosed between the ages of 2 and 14 years. We analyzed the types of presentation, the imaging findings (plain-films, CT, and MRI), and the patients’ evolution. All cases were confirmed at histological study. ResultsThe following uncommon manifestations were found in 11 patients: 2 vertebral, one cervical, and one dorsal ES that initially manifested as spinal cord compression and as a mediastinal mass, respectively; 2 sacral ES with ascending epidural masses; 1 mandibular ES; 1 costal ES with hemorrhagic cerebral metastases; 3 extraskeletal (thigh, buttocks, and pelvis minor) ES with bone metastases at diagnosis: the first two had spinal cord and nerve compression due to vertebral metastases; 2 ES had benign-appearing radiological findings (cortical lesion of the radius with osseous remodeling of the ulna and an expanding multilocular lesion of the fibula). ConclusionsAlthough ES is the second most common malignant bone tumor in children, its unusual imaging findings are less well known. More than half (52.4 %) of our last 21 patients presented some of these manifestations at diagnosis or during follow-up, so radiologists should be aware of them and include ES in the prebiopsy diagnostic possibilities.

Extraskeletal neuroectodermal tumour of the vagina: a single case report and review

A case report and review of an extraskeletal neuroectodermal tumour of the vagina. Ewing's sarcoma (ES) and primitive neuroectodermal tumours (PNETs) account for approximately 6-10% of primary malignant bone tumours and, following osteosarcoma, are the second most common group of bone sarcomas in children. Ewing's sarcoma rarely affects the genitourinary tract. We report a rare case of extraskeletal Ewing's sarcoma diagnosed in a 47-year-old Indian woman presenting with a simple cystic lesion in the posterior wall of the vagina. The histopathology revealed a rare presentation of a primitive malignant extraskeletal neuroectodermal tumour. As our PubMed review found only six previously reported cases of vaginal extraskeletal Ewing's sarcoma. Presentation as vaginal masses helped in early disease detection.

Multimodal Treatment of Primary Extraskeletal Ewing's Sarcoma of the Chest Wall: Report of 2 Cases

Extraskeletal Ewing's sarcoma (EES) is a type of Ewing's sarcoma that arises in soft tissue and is now regarded as a member of a family of small round cell neoplasms of bone and soft tissue, including primitive neuroectodermal tumors (PNETs). EES occurs predominantly in adolescents and young adults between the ages of 10 and 30 years. The disease follows an aggressive course with a high recurrence rate. The presence of a distant metastasis is also common. EES arises in the soft tissue of either the trunk or extremities. We recently experienced two cases of EES that occurred in the chest wall. The two patients underwent wide resection and combined radiochemotherapy. There was no evidence of disease 30 and 22 months, respectively, after surgery. Although extremely rare, EES should be considered in the differential diagnosis of chest wall tumors. We report two cases of EES with a brief review of the literature.

Primary Intradural Extraosseous Ewing's Sarcoma

Ewing's sarcoma usually arises from skeletal bone, but rarely may have an extraskeletal origin. However, Ewing's sarcoma that originates around the spinal column, especially, the intradural extramedullary type is extremely rare. We report a rare case of primary intraspinal extraskeletal Ewing's sarcoma.

Primary Adult Renal Ewing's Sarcoma: A Rare Entity

Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patients with primary renal Ewing's sarcoma were identified. All four patients with nonmetastatic disease had radical nephrectomy and received adjuvant chemotherapy +/− radiotherapy. Two developed metastatic disease while on adjuvant chemotherapy, and one patient relapsed after 55 months. The three patients with metastatic disease at presentation did not have nephrectomy and were treated with chemotherapy. All three patients had disease progression with a dismal outcome. Only one patient in the whole group is alive and disease free. The median overall survival was 62.8 months, and the median disease-free survival in patients with nonmetastatic disease after combined modality treatment was 30.3 months. Conclusion. Primary adult renal Ewing's sarcoma is an aggressive tumor with a propensity for early metastasis. Radical nephrectomy with adjuvant combination chemotherapy produced the best results but the outlook remained poor with only one patient experiencing long disease-free survival.

Soft tissue sarcomas in Mosul: a pathologic evaluation

Objective: Histochemical and immunohistochemical examination of soft tissue sarcomas(STS) in Mosul to assess the value of these techniques in verifying the primary diagnosis. Methods: Paraffin embedded tissue blocks of 35 cases of soft tissue sarcomas collected over a period of 20 months in Mosul, were utilized. H&E, reticulin ,and Van Gieson's staining techniques and immunohistochemical marker for S-100 protein were applied to all the cases. Results: Soft tissue sarcoma accounts for 16.4%of the total of 213 cases of benign and malignant soft tissue neoplasms,with a mean age of 30.5 years ,and a male preponderance( Male: Female ratio of1.7:1). The most common histologic subtypes are extraskeletal Ewing’s sarcoma(ES), dermatofibrosarcoma protuberans(DFSP) ,and spindle cell sarcoma, not other wise specified (NOS). The extremities are affected in 51.4% of all cases, particularly the lower limbs. The majority of soft tissue sarcomas belong to the high grade category by applying French Federation of Cancers Centers Sarcoma Group ( FFCCSG) and American Joint Cancer Committee (AJCC) grading schemes .Both reticulin and Van Gieson's stains have been successfully depicting the growth patterns of different subtypes. Immunoreactivity for S-100 protein was positive in two cases. Conclusions: High quality H&E stained sections remain the best method for establishing the diagnosis of sarcomas .Reticulin stain proved extremely helpful in subclassifying sarcomas and S-100 protein was instrumental in changing the diagnosis of sarcoma in one case.