Extraosseous Ewing's Sarcoma Research Articles

Primary pediatric intraspinal sarcomas

Sarcomas that arise from within the spinal canal are rare, particularly within the pediatric population. In general, these primary intraspinal sarcomas are highly aggressive, posing unique treatment challenges with respect to surgery and choice of adjuvant therapy. The goal must be to obtain the most complete resection possible to minimize the risk of recurrence and metastasis, while preventing potential neurological deficits that may result from aggressive surgery. Among these primary intraspinal sarcomas are malignant peripheral nerve sheath tumors and members of the Ewing sarcoma family of tumors. The authors present 3 cases of unique spinal sarcomas in children-2 malignant peripheral nerve sheath tumors in patients without neurofibromatosis and an intradural extraosseous Ewing sarcoma arising from the sensory component of a lumbar spinal nerve-and discuss their management and outcome with a review of the current literature.

Extraosseous Ewing sarcoma of the thyroid gland

Ewing sarcoma of the bone is a highly malignant round-cell tumor that typically presents between 10 to 20 years of age and is more common in boys. It can have an extraosseous origin, although it is rare. We report a case of extraosseous Ewing sarcoma in the thyroid gland in a 9-year-old girl.

Primary Vaginal Extraosseous Ewing Sarcoma/Primitive Neuroectodermal Tumor with Cranial Metastasis

Extraosseous Ewing sarcoma is now regarded as a member of the Ewing sarcoma/primitive neuroectodermal tumor (PNET) family. It typically involves the soft tissues of the chest wall, pelvis, paravertebral region, abdominal wall, retroperitoneal region and extremities of children, adolescents and young adults, but it seldom occurs in the female genital tract. We report an extremely rare case of retrospective diagnosis of vaginal extraosseous Ewing sarcoma/PNET which metastasized to the right frontoparietal scalp, skull, and dura. Surgical resection, followed by adjuvant radiotherapy and chemotherapy resulted in a favourable clinical outcome. Both the vaginal and head tumors had similar light microscopic features supporting the diagnosis.

Extra-Osseous Ewing’s Sarcoma of the Thumb

The hand is an uncommon location for Ewing's tumour of the bone. This paper details the presentation, pathology and management of an extra-osseous Ewing's sarcoma of the thumb in an adult woman. The management entailed a combination of neo-adjuvant chemotherapy and surgical excision, followed by reconstruction with an interposition bone graft from the subcutaneous surface of the ulna.

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.

Soft-tissue sarcomas of the extremities in patients of pediatric age

The extremity site is a peculiar location for soft-tissue sarcomas (STS) of children and adolescents. Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006. The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma. Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy. For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure. The outcome was particularly poor for patients with large invasive tumors, hand/foot involvement and/or nodal or distant metastases, and for patients who were not given radiotherapy. For the extraosseous Ewing sarcoma cases, 5-year EFS was 74%. For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors. While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results. The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.

Primary extraosseous Ewing sarcoma of the cervical epidural space

J. Neurosurg: Spine / Volume 6 / February, 2007 Extraosseous Ewing sarcomas (EESs) are rare malignant neoplasms of the soft tissue that do not arise from bone and have histological characteristics similar to osseous ESs and peripheral primitive neuroectodermal tumors.1,2 Extraosseous ESs are usually found in the lower extremities and paravertebral regions in patients in their 20s and 30s.2,4 Most primary EESs extend into the anterior epidural space through foraminal widening and bone destruction;4 involvement of only the cervical epidural space is highly unusual, although EESs with both intradural5 and extradural3 settling have been reported. This 28-year-old man presented with progressively increasing neck pain and gait disturbance. Imaging of the cervical spine revealed a well-circumscribed, posterior epidural mass compressing the spinal cord and extending from C-3 to C-5 without foraminal widening (Fig. 1A and B). The patient underwent a C3–4 laminoplasty and gross-total resection of the lesion (Fig. 1C). Immunohistochemical studies revealed a malignant round-cell tumor consistent with osseous ES (Fig. 2), which had no primary focus. The patient’s symptoms resolved immediately after surgery. The patient underwent a chemotherapy regimen that included ifosfamide (2500 mg/ m2 for 3 days), mesna (2500 mg/m2 for 3 days), and doxorubicin (60 mg/m2 for 1 day). This was repeated six times at 21-day intervals. Radiotherapy targeted the spine between C-2 and C-5 with a fractionated total dose of 4500 cGy (180 cGy/day for 25 days). No evidence of recurrence or metastasis was found during 18 months of follow up. Due to its similarity to other lesions, establishing the diagnosis of EES is difficult1 but should nonetheless be considered in the differential diagnosis of spinal epidural tumors. Because all lesion types cannot be identified on the basis of imaging studies alone, a histopathological examination must be conducted to establish a definitive diagnosis.1,2,4 Early, aggressive resection combined with chemoand radiotherapy can produce good results in patients with EES.1,2

Laparoscopic Removal of Extraosseous Ewing's Sarcoma of the Kidney in a Pediatric Patient

In the pediatric population, to the best of our knowledge, only 2 cases of renal extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (EES/PNET) have been published. We report the initial case of renal EES/PNET occurring in a 10-year-old girl treated by a laparoscopic radical nephrectomy. The regimen used is the first documented use of neoadjuvant chemotherapy prior to laparoscopic radical nephrectomy for PNET. This approach obviated the need for a large incision and a prolonged postsurgical recovery. The minimally invasive nature of the laparoscopic procedure allowed for a rapid convalescence and resumption of her chemotherapy regimen within 14 days of the surgery.

Ewing's sarcoma of the vulva-a case report

Extraosseous Ewing's sarcoma of the vulva is extremely rare with only three such cases described. A 26-year-old woman presented with Ewing's sarcoma of the vulva. She received chemotherapy to shrink the lesion. However, the response was suboptimal and she subsequently received radiotherapy. Although resection was planned, she developed chest metastases within a short period of time and subsequently died.

Localized unresectable non‐rhabdo soft tissue sarcomas of the extremities in pediatric age

Treatment of initially unresectable nonrhabdo soft tissue sarcomas (NRSTS) in pediatric age is debated, due to their different chemosensitivity. The authors objective was to evaluate clinical features and treatment results observed in a series of Italian patients over a 24-year period. Fifty-two patients age 8-18 years (median 8 years) were observed (1979-2002). Primary sites were on the lower limbs in 41 and on the upper limbs in 11 cases. Clinical TNM and surgical Intergroup Rhabdomyosarcoma Staging systems were adopted. Therapeutic guidelines recommended an initial biopsy plus neoadjuvant chemotherapy. Aggressive delayed surgery of residual disease, including compartmental resections, was recommended. Radiotherapy was suggested only for patients age > 3 years when surgery was incomplete or not feasible. The evaluation was performed dividing the patients into two categories according to their chemosensitivity. Chemosensitive (CTs) sarcomas, 21: synovial sarcoma, 11; extraosseous Ewing sarcomas, 5; primitive peripheral neuroectodermic tumors, 5. Nonchemosensitive (CTns) sarcomas, 31: fibrosarcoma, 11; malignant peripheral nerve sheet tumors, 10; liposarcoma, 2; hemangiopericitoma adult type, 2; epithelioid sarcoma, 2; and alveolar soft part sarcoma, leiomyosarcoma, clear cell sarcoma, and sarcoma NOS, each 1. Nineteen of 21 patients with CTs-NRSTS were alive without disease: the 5-year overall survival (OS) and progression-free survival (PFS) were 94.4% and 79.3%, respectively; 23 of 31 patients with CTns-NRSTS were alive without disease: 5-year OS and PFS were 75.3% and 68.3%, respectively. Response to neoadjuvant chemotherapy was complete or partial in 10 of 20 evaluable CTs and in 8 of 26 evaluable CTns tumors. The achievement of complete delayed resection was particularly important for CTns-NRSTS. Tumor size < 5 cm, distal site, and tumor grading for CTns sarcomas were often linked to a favorable outcome; no conclusive results were detected concerning age of the patients or T status of the tumor. Multidisciplinary treatment without mutilating procedures allowed the cure of most patients with CTs and CTns-NRSTS. Relapses were cured in several cases of CTs tumors, whereas almost all patients with relapsed CTns tumors died due to the high rate of metastatic spread.

Extraosseous Ewings Sarcoma of the Tongue Initially Mistaken for Lymphoblastic Lymphoma: Keypoints in Diagnosis

Background. Ewings sarcoma typically presents as a bone tumor and less frequently at extraosseous sites. When presenting at unusual extraosseous sites, these peripheral primitive neuroectodermal tumors may be mistaken for other small round cell tumors.

Protocol for the examination of specimens from pediatric and adult patients with osseous and extraosseous ewing sarcoma family of tumors, including peripheral primitive neuroectodermal tumor and ewing sarcoma.

Protocol for the examination of specimens from pediatric and adult patients with osseous and extraosseous ewing sarcoma family of tumors, including peripheral primitive neuroectodermal tumor and ewing sarcoma.

Protocol for the Examination of Specimens From Pediatric and Adult Patients With Osseous and Extraosseous Ewing Sarcoma Family of Tumors, Including Peripheral Primitive Neuroectodermal Tumor and Ewing Sarcoma

Protocol for the Examination of Specimens From Pediatric and Adult Patients With Osseous and Extraosseous Ewing Sarcoma Family of Tumors, Including Peripheral Primitive Neuroectodermal Tumor and Ewing Sarcoma

Radiologic Findings of Extraosseous Ewing's Sarcoma

Purpose: The purpose of this study is to evaluate the radiologic findings of the extraosseous Ewing's sarcoma. Materials and Methods: Six patients with pathologically confirmed extraosseous Ewing's sarcoma were retrospectively reviewed. Patients included two men and four women with an average age of 21.5 years (age range 9-48 years). Plain radiographs (six patients), magnetic resonance (MR) images (five patients), computed tomographic (CT) scans (three patients) and whole body scintigraphy (two patients) were reviewed and analyzed. Images were evaluated with regard to lesion location, size, margin, muscle or bone involvement and intrinsic imaging characteristics on CT and MRI. Results: The tumors were located in the thigh (three patients), back (two patients) and upper arm (one patient). The tumors ranged in size from 2.3 cm to 7.5 cm (mean, 5.2 cm), were mainly well circumscribed and showed no evidence of calcification prior to treatment. Margins were well defined in four out of the six patients. Four patients had subcutaneous lesions and the other two patients had intramuscular lesions. Muscle (two patients) and bone invasion (one patient) were present. The masses were heterogenous low signal intensity on T1 weighted images and heterogeneous high signal intensity on T2 weighted images compared with muscle. Heterogeneous enhancement within the lesion was observed in all patients on CT and MRI. Whole body scintigraphy using Tc-99 m MIBI showed increased uptake in the masses of two patients. Conclusion: Extraosseous Ewing's sarcomas were frequently seen as a well-circumscribed ovoid mass with nonspecific findings on CT and MRI. Despite being a relative rare tumor, it should be included in the differential diagnosis of a non-calcified soft-tissue mass, especially in subcutaneous tissue.

Primary Cutaneous Ewing’s Sarcoma

A 57‐year‐old woman presented with a three‐week history of an erythematous nodule on her palm. The clinical diagnosis was pyogenic granuloma. Histology revealed a circumscribed dermal nodule of uniform, primitive round cells with numerous mitotic figures and apoptopic cells. The tumor cells showed strong membranous immunoreactivity for CD99 and nuclear immunoreactivity for Fli‐1. The tumor cells were negative for S‐100 protein, cytokeratin AE1/3, cytokeratin 20, chromogranin, synaptophysin, and actin. The diagnosis of Ewing’s sarcoma was made. CT scans showed no evidence of an osseous or deep soft tissue primary tumor or metastatic disease. The tumor was excised with 1 cm margins, and the patient received six courses of adjuvant chemotherapy. One year after diagnosis, the patient is alive without evidence of disease. Extraosseous Ewing’s sarcoma (EES) rarely presents as a primary skin tumor and should be considered in the differential diagnosis of small round cell tumors involving the skin. It is important to distinguish primary cutaneous EES from secondary involvement of the skin by ES, as primary cutaneous EES has a more indolent course compared to classic EES or osseous ES. Immunohistochemical stains for CD99 and Fli‐1 are useful markers to confirm the diagnosis of cutaneous ES.

Intracranial Ewing sarcoma

The occurrence of primary extraosseous Ewing sarcoma (EES) of the central nervous system (CNS) has only rarely been reported in the literature. It is important to distinguish this entity from the more common central primitive neuroectodermal tumor (PNET) of brain, since the management of these tumors is different from that of EES. We present the clinical, radiologic, and pathologic features of two cases of EES occurring in the brain. The diagnosis was further confirmed by detection of a rearrangement of the FLI1 and/or EWS gene loci in tumors from both patients using fluorescent in situ hybridization (FISH). Although rare, the possibility of EES should be considered particularly when tumors that arise near the meningeal surface of the brain and have the pathologic appearance of a PNET. Demonstration of t(11;22)(q24;q12) by molecular analysis essentially confirms the diagnosis and enables the oncologist to choose appropriate therapy.

Weichteilmalignome bei Kindern und Jugendlichen - Pathologische Anatomie und ihre klinische Relevanz

Soft tissue malignancies in childhood and adolescence encompass a wide variety of histologically and genetically different tumor entities. In the files of the Kiel Pediatric Tumor Registry, 4,272 soft tissue malignancies were collected since 1977. Rhabdomyosarcomas are by far the most frequent sarcomas (44.6 % of the cases), followed in decreasing order of frequency by the family of Ewing tumors (peripheral primitive neuroectodermal tumors and extraosseous Ewing's sarcomas; altogether 22.3 %), malignant peripheral nerve sheath tumors (8.1 %), synovial sarcomas (5.0 %), leiomyosarcomas (3.2 %), fibrosarcomas (2.4 %), extrarenal malignant rhabdoid tumors (2.0 %), and alveolar soft tissue sarcomas (1.1 %). A further group (11.3 %) includes rare tumors, intermediate fibrohistiocytic tumors, and unclassified sarcomas. Embryonal rhabdomyosarcomas are 2.5 times more frequent than the alveolar rhabdomyosarcomas, which are prognostically unfavorable and located predominantly in the extremities and the trunk. With regard to clinical findings, histology, molecular biology and prognosis, embryonal and alveolar rhabdomyosarcomas have to be considered as two different tumor types. The family of Ewing tumors includes extraosseous Ewing's sarcoma and peripheral primitive neuroectodermal tumors (synonym: malignant peripheral neuroectodermal tumors), the former tumors without and the latter with neural differentiation. Many cases of infantile malignant peripheral nerve sheath tumors and infantile fibrosarcomas are low-grade malignancies and prognostically more favorable than their "adult" counterparts.

Das freie mikrovaskuläre Fibulatransplantat zur Skelettrekonstruktion nach Tumorresektionen am Unterarm - Erfahrungen aus fünf Fällen

Plastic-reconstructive surgery for upper extremity tumors not only follows the fundamental principles of oncologic surgery, but also requires attention to functional and aesthetic aspects. For soft tissue reconstruction, a variety of surgical options are available, whereas for the reconstruction of extensive bony defects only the free microvascular fibular transplant appears to be a reasonable method. Its advantages are its slim shape and variable length, the possibility of achieving a stable osteosynthesis suitable for early physiotherapy, and low donor site morbidity, even if the fibula is harvested as an osteoseptocutaneous flap. Between 1994 and 2000, five patients underwent a free fibula transplantation for radius reconstruction. In all cases, a two-stage reconstructive approach with initial tumor biopsy was chosen. The resected tumors were two osteosarcomas, one extraosseous Ewing sarcoma, one aneurysmatic bone cyst and one desmoplastic fibroma. Surgical and pathohistological tumor-free margins were achieved in all cases (R(0)). Two patients received an osteocutaneous graft. All grafts were revascularized to the radial artery and subcutaneous veins. The transplanted fibula grafts were between 10 cm and 17 cm in length. All proximal osteosyntheses were performed fibuloradially, the distal stabilizations were fibulo-scapho-lunate (2 x) and fibulo-radial (3 x), depending on the extent of resection. All microvascular transplants survived completely and showed a stable osseous integration, both clinically and radiologically. Depending on the postoperative course, an adapted intensive physiotherapy regimen yielded good or satisfactory function. In the follow-up of up to seven years in one case a tumor recurrence (osteosarcoma, 35 months post-operatively) required forearm amputation. The reconstruction of tumor-associated wide bony defects in the upper extremity with a free microvascular fibula graft allows for a successful therapy with respect to oncologic, functional and aesthetic considerations.

Spinal cord Ewing’s sarcoma metastasis: presentation of one case

Cervical spinal Ewing's sarcomas are rare and cause problems in diagnosis. We present an unusual case of a primary extraosseous Ewing's sarcoma arising from the spinal cord. An 18-year-old woman with fever, headache and back pain lasting one month was admitted to the hospital. Whole body bone scintigraphy was performed with 1110 MBq technetium-99m methylenediphosphonate. Scintigraphy clearly showed abnormal technetium-99m methylenediphosphonate accumulation in the level of the 5th and 6th cervical vertebrae. Magnetic resonance imaging could also confirm this examination finding. After the scintigraphic study, the patient underwent surgery. Pathological diagnosis of the operation specimen was Ewing's sarcoma.