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Ewing's Sarcoma

Ewing's Tumor

Ewing's sarcoma is a highly malignant tumor of bone, usually in the diaphyses of long bones, pelvis and ribs of children and adolescents.


Presentation

Localized pain is the most frequent symptom. Systemic symptoms like fever, weight loss, or anemia may occur. Fever is related to cytokines produced by the tumor cells. Other symptoms depend on the organs closest to the tumor.

Disorders caused by nerve compression or mechanical disorders are associated with pelvic tumors. Respiratory disorders or pleural effusion are associated with costal tumors. Radicular compression is associated with vertebral tumors.

The tumor initially develops most frequently in bone, particularly in the pelvis (30%), thorax (20%), femur (16%), tibia (9%), vertebrae (8%) and humerus (5%). The disease has a high potential for metastases.

Pain
  • Most common symptoms reported: Localised pain: bone pain; may come and go and vary in its intensity.[web.archive.org]
  • Patellar tumors occur infrequently and represent an uncommon etiology of anterior knee pain. We describe the rare case of a 41-year-old man who presented with a 3-4 month history of escalating right anterior knee pain and swelling.[ncbi.nlm.nih.gov]
  • We report the case of a 28-year-old female with primary extraosseous Ewing's sarcoma who presented initially with a low back pain and a right S1 radicular pain.[ncbi.nlm.nih.gov]
  • A 15-year-old girl presented with a 2-month history of non-specific right hip pain associated with pain in the back, right flank and foot. Her symptoms deteriorated, interfering with weight-bearing.[ncbi.nlm.nih.gov]
  • The patient was a young woman, and she presented with sudden right flank pain clinically. Ultrasonography revealed a large heterogeneous mass in the lower pole of her right kidney.[ncbi.nlm.nih.gov]
Swelling
  • Although the initial swelling subsided, the patient noticed a small 'lump', superior to the eyelid which persisted after the rest of the swelling disappeared.[ncbi.nlm.nih.gov]
  • Here, we present a case of primary Ewing's sarcoma of the skull with localised swelling in a young adult that involved the frontoparietal region of the skull and was very aggressive in nature.[ncbi.nlm.nih.gov]
  • A 40-year-old male was admitted with complaints of nonproductive cough, exertional dyspnea, and fatigue since 4 months with a history of abdominal wall swelling which was excised and proven to be Ewing's sarcoma/primitive neuroectodermal tumor (PNET).[ncbi.nlm.nih.gov]
  • A young patient presented with a progressive right knee pain and swelling that was worse on ambulation. Following appropriate investigations, he was confirmed having Ewing's sarcoma of the right patella.[ncbi.nlm.nih.gov]
  • Pain and swelling of the affected finger are the most frequent presenting features. We report two cases of Ewing's sarcoma located at ring finger and the thumb in two children aged 14 and 10 years.[ncbi.nlm.nih.gov]
Fever
  • Systemic symptoms like fever, weight loss, or anemia may occur. Fever is related to cytokines produced by the tumor cells. Other symptoms depend on the organs closest to the tumor.[symptoma.com]
  • A 17-year-old Japanese woman presented with left upper abdominal pain and high fever. Computed tomography and magnetic resonance imaging revealed a 15 10 cm tumor replacing the adrenal gland. Preoperative diagnosis was an adrenocortical carcinoma.[ncbi.nlm.nih.gov]
  • Slight fever. The tumour is composed of compact, uniform cells with large, round, or ovoid nuclei containing scattered chromatin. Miotic changes are common; small vascular channels may be present.[whonamedit.com]
  • Swelling, this can be seen if it is on a bone near the surface of the body but in other places, like on the pelvis, it may not be visible Less common symptoms, rare and very rare symptoms, fever (pyrexia), tiredness or feeling weary, (lethargy), pain[web.archive.org]
  • Toxicology studies in mini-pigs with doses comparable to the demonstrated in vivo efficacy dose resulted in transient fever, occasional limited hypertension at low- and high-dose assessment and transient liver enzyme elevation at high dose.[ncbi.nlm.nih.gov]
Weight Loss
  • loss and loss of appetite, breathlessness.[web.archive.org]
  • Systemic symptoms like fever, weight loss, or anemia may occur. Fever is related to cytokines produced by the tumor cells. Other symptoms depend on the organs closest to the tumor.[symptoma.com]
  • Pain around the site of the tumor Swelling and redness around the site of the tumor Fever Weight loss and decreased appetite Fatigue Paralysis and incontinence if the tumor is in the spinal region Symptoms related to nerve compression from a tumor such[ucsfbenioffchildrens.org]
  • Other symptoms include: A lump near skin that feels warm and soft to the touch Constant low fever Limping because your legs hurt Bone pain that gets worse when you exercise or during the night Broken bones without an obvious cause Weight loss Always being[webmd.com]
  • They can include: Pain around the site of the tumor that may come and go, but gets worse over time and with activity Swelling around the site of the tumor A lump (mass) A bone breaks for no known reason Fever Weight loss Feeling tired The symptoms of[urmc.rochester.edu]
Fatigue
  • A 40-year-old male was admitted with complaints of nonproductive cough, exertional dyspnea, and fatigue since 4 months with a history of abdominal wall swelling which was excised and proven to be Ewing's sarcoma/primitive neuroectodermal tumor (PNET).[ncbi.nlm.nih.gov]
  • Pain around the site of the tumor Swelling and redness around the site of the tumor Fever Weight loss and decreased appetite Fatigue Paralysis and incontinence if the tumor is in the spinal region Symptoms related to nerve compression from a tumor such[ucsfbenioffchildrens.org]
  • Ewing Sarcoma The symptoms of depend on the bone or soft tissue site in which the cancer develops, but they usually include: Pain at the site of the mass, often with swelling Patients may have general symptoms such as loss of appetite, fever, malaise, fatigue[curesearch.org]
  • Shortly after his diagnosis, Mar began chemotherapy treatments, which caused side effects including pain, nausea, loss of appetite, weight loss, fatigue, and drowsiness.[cancer.org]
  • The most common side effects include fatigue, nausea, dizziness, vomiting, increased liver enzymes, cough, constipation, and diarrhea.[cancer.net]
Abdominal Pain
  • A 17-year-old Japanese woman presented with left upper abdominal pain and high fever. Computed tomography and magnetic resonance imaging revealed a 15 10 cm tumor replacing the adrenal gland. Preoperative diagnosis was an adrenocortical carcinoma.[ncbi.nlm.nih.gov]
  • This report describes an EWS/PNET in a 25-year-old woman who presented with abdominal pain lasting 3 days. Radiologic evaluation revealed a 9 cm 6 cm homogeneous mass in the lower abdomen with homogeneous enhancement and invasion of the ileum.[ncbi.nlm.nih.gov]
  • A 38-year-old male presented to the emergency department with abdominal pain and bulge. He had a history of irritable bowel syndrome for 1 year with complaint of dyspepsia.[ncbi.nlm.nih.gov]
  • In the second case, a child was admitted due to abdominal pain and a hydroureter in the right kidney, as determined by ultrasonography. A tumor was found in the right ureter at the level of iliac vessels.[ncbi.nlm.nih.gov]
  • Patients mostly present with nonspecific symptoms such as abdominal pain and gross hematuria. Since EWS/PNET has a rapid clinical progression with early metastasis and death, it is essential to make an accurate and early diagnosis.[ncbi.nlm.nih.gov]
Upper Abdominal Pain
  • A 17-year-old Japanese woman presented with left upper abdominal pain and high fever. Computed tomography and magnetic resonance imaging revealed a 15 10 cm tumor replacing the adrenal gland. Preoperative diagnosis was an adrenocortical carcinoma.[ncbi.nlm.nih.gov]
Right Flank Pain
  • The patient was a young woman, and she presented with sudden right flank pain clinically. Ultrasonography revealed a large heterogeneous mass in the lower pole of her right kidney.[ncbi.nlm.nih.gov]
Dyspepsia
  • He had a history of irritable bowel syndrome for 1 year with complaint of dyspepsia. Physical examination revealed a distended abdomen with a huge palpable mass located in the paraumblical region.[ncbi.nlm.nih.gov]
Ulcer
  • This lump subsequently progressed over a span of 3 months and then caused ulceration of the overlying skin. Further testing revealed the lump to be an Ewing's sarcoma arising from the frontal bone.[ncbi.nlm.nih.gov]
  • […] used in bone cancer chemotherapy include: Carboplatin Cisplatin Cyclophophamide Doxorubicin Epirubicin Etoposide Ifosphamide Methotrexate with Leucovorin Common side effects include: nausea vomiting diarrhea loss of appetite loss of hair weakness mouth ulcers[news-medical.net]
Bone Pain
  • Clinical presentation is usually dominated by local bone pain and a mass. Imaging reveals a technetium pyrophosphate avid lesion that, on plain radiograph, is destructive, diaphyseal and classically causes layered periosteal calcification.[ncbi.nlm.nih.gov]
  • Most common symptoms reported: Localised pain: bone pain; may come and go and vary in its intensity.[web.archive.org]
  • There may be multiple diagnostic tests such as: Bone scans — A nuclear imaging method to detect bone diseases and tumors and determine the cause of bone pain or inflammation.[ucsfbenioffchildrens.org]
Knee Pain
  • Patellar tumors occur infrequently and represent an uncommon etiology of anterior knee pain. We describe the rare case of a 41-year-old man who presented with a 3-4 month history of escalating right anterior knee pain and swelling.[ncbi.nlm.nih.gov]
  • A young patient presented with a progressive right knee pain and swelling that was worse on ambulation. Following appropriate investigations, he was confirmed having Ewing's sarcoma of the right patella.[ncbi.nlm.nih.gov]
Myalgia
  • […] or pelvic sites. [10] Age younger than 15 years also seems to be a more favorable prognosis. [10, 11] Treatment complications Complications of chemotherapy can include the following: Vincristine - Primarily causes neuropathy, including constipation, myalgias[emedicine.com]
Anterior Knee Pain
  • Patellar tumors occur infrequently and represent an uncommon etiology of anterior knee pain. We describe the rare case of a 41-year-old man who presented with a 3-4 month history of escalating right anterior knee pain and swelling.[ncbi.nlm.nih.gov]
Papilledema
  • He had UMN facial paresis and papilledema. Imaging revealed a heterogenous space occupying lesion in the middle cranial fossa base with mass effect and midline shift and non homogenous contrast enhancement with erosion of the petrous bone.[ncbi.nlm.nih.gov]

Workup

No specific blood tests are used in the diagnosis of Ewings sarcoma. However, the following tests and procedures may be used to diagnose or stage the malignancy: physical exam, magnetic resonance imaging, X-ray, fluorodeoxyglucose positron emission tomography (FDG-PET), laboratory tests (blood cultures, C-reactive protein levels, erythrocyte sedimentation rate and complete blood count). A biopsy may be done to definitively diagnose Ewings sarcoma. [3]

There is no standard staging system. Ewing sarcoma is described based on whether the cancer is localized or metastatic.

Mediastinal Mass
  • We conclude that, although this is an unusual location, EES should be contemplated in the differential diagnosis of mediastinal masses.[ncbi.nlm.nih.gov]
  • In a study done by Ahmad et al. 6 including 24 patients with EES, posterior mediastinal mass was only identified in one.[archbronconeumol.org]

Treatment

Treatment starts systematically with chemotherapy with alternating courses of 2 regimens: vincristine, doxorubicin, and cyclophosphamide; and ifosfamide and etoposide. Chemotherapy is administered to reduce the size of the primary tumor and to eradicate or prevent metastases [4].

For more severe forms of the disease (limited response to initial chemotherapy or metastases already have been diagnosed), high dose chemotherapy with stem cell transplantation is suggested as a last resort treatment.

Only patients who have a good initial response to standard chemotherapy are considered for stem cell transplantation. Surgery and radiotherapy are recommended whenever the location of the tumor allows. Local radiotherapy sometimes is necessary, with surgery or alone for inoperable tumors [5] [6].

Prognosis

The main prognostic factors of Ewing’s sarcoma are: age, general health of the patient, location and size of the tumor, the stage of the disease, surgical outcome and response to treatment.
The survival of patients with Ewings sarcoma depends highly on the initial manifestation of the disease.

Approximately 80% of patients present with localized disease, whereas 20% present with clinically detectable metastatic disease, most often to the lungs, bone, or bone marrow. Less common sites include the central nervous system and lymph nodes.

Five-year survival for localized Ewings sarcoma is 70% to 80% when treated with chemotherapy [7].

Etiology

Most cases of Ewings sarcoma occur due to a translocation between chromosomes 11 and 22. This translocation joins the Ewing’s sarcoma gene (EWS) on chromosome 22 to a gene of the ETS family, friend leukemia insertion (FLI1), on chromosome 11.

Epidemiology

Ewings sarcoma is the second most common bone cancer in children, but it's also relatively rare uncommon. It accounts for only 1% of all childhood cancers. The incidence is 0.6 per million with a rate of 0.3 cases per million in children under 3 years of age, and as high as 4.6 cases per million in adolescents aged 15–19 years. The incidence of these tumors in males is higher than in females. It very rarely occurs in adults over the age of 30.

Sex distribution
Age distribution

Pathophysiology

Classic Ewings sarcoma is a poorly differentiated, homogeneous population of small round-cells with high nuclear-to-cytoplasmic ratio, characterized by strong expression of glycoprotein MIC2 and CD99.

Prevention

There are no guidelines for prevention of Ewings sarcoma.

Summary

Ewings sarcoma is a malignant small round cell bone tumor with strong metastatic potential. It is a primary bone cancer that affects mainly children and adolescents. The most common areas in which it occurs are the pelvis, the femur, the humerus, the ribs and clavicle. Ewings sarcoma also may be found in the soft tissues of the trunk, arms, legs, abdominal cavity, head and neck.

Other names for Ewings sarcoma include primitive neuroectodermal tumor, Askin tumor, extraosseous Ewing sarcoma and peripheral neuroepithelioma. All these types are called Ewing sarcoma family of tumors [1] [2].

Patient Information

Ewings sarcoma is a type of tumor that forms from a certain kind of cell in bone or soft tissue. It may be found in the bones of the skull, arms, hands , legs, feet, chest, pelvis, and spine. Ewings sarcoma also may be found in the soft tissue of various areas. They most commonly occur in teenagers and young adults.

Signs and symptoms

Examinations

The following tests may be used to diagnose the disorder: physical exam, magnetic resonance imaging (MRI), computed tomography (CT) scan, positron emission tomography (PET) scan, bone marrow aspiration and biopsy, X-ray, laboratory studies (complete blood count, blood chemistry studies).

Staging is the process that used to find out if cancer has spread in the body. The disease is grouped to localized or metastatic. Localized Ewings sarcoma refers to a tumor which is found where it began. Metastatic Ewings sarcoma means that the cancer has spread from the bone or soft tissue in which it began to other parts of the body.

Treatment

Three types of standard treatment are used: Chemotherapy is part of the treatment for all patients with Ewings tumors. Chemotherapy may also be given to shrink the tumor prior to surgery or radiation therapy. Radiation therapy is used when the tumor cannot be removed surgically. It is also used to make the tumor smaller, and decrease the amount of tissue that required to be removed during surgery. Surgery is usually done to remove cancer that is left after chemotherapy or radiation therapy.

References

Article

  1. Iwamoto Y., Diagnosis and treatment of Ewing's sarcoma, Jpn. J. Clin. Oncol. 37 (2) 2007: 79–89.
  2. Bernstein M, Kovar H, Paulussen M, Randall RL, Schuck A, Teot LA, Juergens H. Ewing's sarcoma family of tumors: current management. Oncologist. 2006; 11(5): 503-19.
  3. Paulussen M, Craft AW. Ewing Tumours: Management and Prognosis in, Education Book, International Society of Paediatric Oncology, 37th Congress of the International Society of Paediatric Oncology Vancouver, British Columbia, Canada - September 20-24, 2005.
  4. Dai X, Ma W, He X, Jha RK., Review of therapeutic strategies for osteosarcoma, chondrosarcoma, and Ewing’s sarcoma. MED Sci Monit. 2011; 17 (8): RA177–RA190.
  5. Spira A. and Ettinger D., The Use of Chemotherapy in Soft-Tissue Sarcomas. The Oncologist. 2002 vol. 7 no. 4 348-359.
  6. Grier HE, Krailo MD, Tarbell NJ, et al. Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. Feb 20 2003;348(8):694-701.
  7. Lahl M, Fisher VL, Laschinger K. Ewing sarcoma family of tumours: an overview from diagnosis to survivorship, Clin J Oncol Nurs 12 2008 (1): 89–97.

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Last updated: 2019-07-11 22:04