THE MULTIDISCIPLINARY APPROACH IN A CASE OF PEDIATRIC EWING’S SARCOMA

Ewing's Sarcoma is a rare, malignant osseous tumor arising from undifferentiated cells originating from the neural crest; however it may also develop in the soft tissue. The initial evaluation of a patient who is suspected to be suffering from Ewing's Sarcoma entails taking a thorough medical history along with performing a detailed physical examination. Our patient was diagnosed with Ewing's Sarcoma upon seeking medical attention due to the sudden appearance of pain in his left hip. The medical staff conducted a series of investigations including X-rays, MRI, CT and 3D-CT scans of the inferior limb and pelvis in order to yield the diagnosis of Ewing's Sarcoma; however, the definitive diagnosis was confirmed only by the biopsy performed before the surgery for modular endoprotesation. The patient received preoperative chemotherapy (in order to reduce the tumor dimension) according to the EuroEwing 2008 Protocol. After the seventh course of chemotherapy, surgery was performed, consisting of in-block resection of the proximal left femur and hip joint, followed by the insertion of a modular femoral endoprosthesis. Postoperative follow-up, however necessitated another six courses of chemotherapy.

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Localized primary intracranial Ewing's sarcoma of the orbital roof

Journal of Neurosurgery ◽

10.3171/jns.1979.50.6.0811 ◽

1979 ◽

Vol 50 (6) ◽

pp. 811-813 ◽

Cited By ~ 39

Author(s):

Antonio Alvarez-Berdecia ◽

Luis Schut ◽

Derek A. Bruce

Keyword(s):

Ewing’S Sarcoma ◽

Clinical Presentation ◽

Ewing's Sarcoma ◽

Intracranial Extension ◽

X Rays ◽

Content Type ◽

Orbital Roof ◽

The Right ◽

Tomography Scan

✓ A rare case of a primary Ewing's sarcoma arising from the intracranial portion of the right orbital roof is presented. Clinical presentation was that of a rapidly growing tender exostosis, associated with headaches. History indicated that the tumor may have been present for more than 1 year. Skull x-rays showed marked diastasis of the coronal suture and a “mottled” appearance on the right frontal bone suggestive of a sarcoma. Bone scan further confirmed the presence of a malignant bone tumor. Computerized tomography scan showed the intracranial extension of the tumor and a follow-up scan allowed us to see the response of the tumor to therapy. Only two other cases with details as to intracranial localization were found in the literature.

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Precise single column resection and reconstruction with femoral head plus THR for malignant pelvic tumors

10.29007/nvsk ◽

2018 ◽

Author(s):

Xiaohui Niu ◽

Yongkun Yang ◽

Qing Zhang ◽

Yuan Li ◽

Hairong Xu ◽

...

Keyword(s):

Femoral Head ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Posterior Column ◽

Surgical Margins ◽

Wide Resection ◽

Navigation Group ◽

Single Column ◽

And Function

Background Some malignant pelvic tumor may affect the anterior or posterior column at the acetabula area. The postoperative recurrenceand complication rate are high. Precise resection with safe surgical margin could cure the patients while saving healthier host bone for relative simple reconstruction.Questions/Purposes The purpose of this study is to evaluate the effect of precise single column resection and reconstruction with femoral head plus THR for malignant pelvic tumorswith respect to the (1) surgical safety, (2) oncological outcome and (3) prosthesis survivorship and function.Methods This is a clinical cases study. From 2007 to 2015, 19 patients with primary malignant tumors of the pelvis were enrolled in the study. The diagnosis included 16 cases of chondrosarcoma, 1 case of undifferentiated polymorphic sarcoma, 1 case of Ewing's sarcoma and 1 case of solitary plasmacytoma. All tumors were resected with safe surgical margins, which were proved by the postoperative specimen evaluation. Anterior column was involved in 17 cases and posterior column in 2 cases. Ten of 19 tumors were resected assisted by computer navigation. Femoral heads were used to reconstruct anterior or posterior column defects and fixed by screws; THR was used for the joint reconstruction. Oncologic outcome and function were evaluated by regular follow-up.Results The follow up time was more than 12 months in 14 cases with the average of 58.4 months (median 61, range 13-118) months. Surgical margins contained wide resection in 12 cases and marginal resection in 7 cases. The bony wide resection rate was 90% (9/10) in the navigation group and 77.8% (7/9) in free hand group respectively. One patient with Ewing's sarcoma died 14 months postoperative due to lung metastasis. There was only one case with chondrosarcoma was found recurrence in 61 months postoperatively, who was in the navigation group and having marginal margin resection. There was one prosthesis removed due to prosthesis infection (14 months postoperatively). There were another two patients with minor wound infection. The average MSTS function score was 24.8 (17-29).Conclusions The current treatment method is oncological safe and functional with less complications. The hardware is relatively cost effective and right on the shelf. However, this procedure is highly skill needed.

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Ewing’s sarcoma of sinonasal tract: a rare case

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20200636 ◽

2020 ◽

Vol 6 (3) ◽

pp. 565

Author(s):

Ankur Gupta ◽

Ancy S. Sofia ◽

Kanwar Sen

Keyword(s):

Multidisciplinary Approach ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Histopathological Examination ◽

Malignant Tumour ◽

Rare Entity ◽

Round Cell ◽

First Line ◽

Cytogenetic Studies ◽

Immunohistochemical Studies

<p class="abstract">Extra skeletal Ewing’s sarcoma (EES) is a rare, rapidly growing, round cell malignant tumour that can develop in the soft tissue at any location. Involvement of the paranasal sinus is a very rare entity. Nearly 80% of patients are younger than 20 years. Diagnosis is made after histopathological examination, immunohistochemical studies and cytogenetic studies. Treatment includes a multidisciplinary approach with surgery as the first line followed by chemotherapy and radiotherapy.</p><p class="abstract"> </p>

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Long-term follow-up for patients with Ewing's sarcoma of bone treated with adjuvant and neoadjuvant chemotherapy

Oncology Reports ◽

10.3892/or.4.5.977 ◽

1997 ◽

Author(s):

G Bacci ◽

S Ferrari ◽

E Barbieri ◽

A Longhi ◽

C Forni ◽

...

Keyword(s):

Neoadjuvant Chemotherapy ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Long Term Follow Up

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Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the First Intergroup study.

Journal of Clinical Oncology ◽

10.1200/jco.1990.8.10.1664 ◽

1990 ◽

Vol 8 (10) ◽

pp. 1664-1674 ◽

Cited By ~ 376

Author(s):

M E Nesbit ◽

E A Gehan ◽

E O Burgert ◽

T J Vietti ◽

A Cangir ◽

...

Keyword(s):

Overall Survival ◽

Local Recurrence ◽

Multimodal Therapy ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Patient Characteristics ◽

Group I ◽

Long Term Follow Up

A total of 342 previously untreated eligible children were entered into the first Intergroup Ewing's Sarcoma Study (IESS) between May 1973 and November 1978. In group I institutions, patients were randomized between treatment 1 (radiotherapy to primary lesion plus cyclophosphamide, vincristine, dactinomycin, and Adriamycin [doxorubicin; Adria Laboratories, Columbus, OH] [VAC plus ADR]) or treatment 2 (same as treatment 1 without ADR), and group II institutions randomized patients between treatment 2 or treatment 3 (same as treatment 2 plus bilateral pulmonary radiotherapy [VAC plus BPR]). The percentages of patients relapse-free and surviving (RFS) at 5 years for treatments 1, 2, and 3 were 60%, 24%, and 44%, respectively. There was strong statistical evidence of a significant advantage in RFS for treatment 1 (VAC plus ADR) versus 2 (VAC alone) (P less than .001) and 3 (P less than .05) and also of treatment 3 versus 2 (P less than .001). Similar significant results were observed with respect to overall survival. Patients with disease at pelvic sites have significantly poorer survival at 5 years than those with disease at nonpelvic sites (34% v 57%; P less than .001). Among pelvic cases, there was no evidence of differing survival by treatment (P = .81), but among nonpelvic cases, there was strong evidence of differing survival by treatment (P less than .001). The overall percentage of patients developing metastatic disease was 44%; the percentages by treatments 1, 2, and 3 were 30%, 72%, and 42%, respectively. The overall incidence of local recurrence was 15%, and there was no evidence that local recurrence rate differed by treatment. Patient characteristics related to prognosis, both with respect to RFS and overall survival experience, were primary site (nonpelvic patients were most favorable) and patient age (younger patients were more favorable).

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The First Report of Extraosseous Ewing's Sarcoma in the Rectovaginal Septum

Tumori Journal ◽

10.1177/030089160208800419 ◽

2002 ◽

Vol 88 (4) ◽

pp. 345-346 ◽

Cited By ~ 24

Author(s):

Marija Petković ◽

Gordana Zamolo ◽

Damir Muhvić ◽

Miran Čoklo ◽

Sanja Štifter ◽

...

Keyword(s):

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Malignant Neoplasm ◽

External Beam Radiation ◽

X Rays ◽

Rectovaginal Septum ◽

Beam Radiation ◽

Palpable Mass ◽

Residual Mass ◽

Extraosseous Ewing’S Sarcoma

Aims and Background To report an extremely rare case of Ewing's sarcoma located in the rectovaginal septum. Ewing's sarcoma is a highly malignant neoplasm of bone, which usually occurs during childhood. Common extraosseous localizations of Ewing's sarcoma include the trunk, extremities, uterus, cervix and vagina. Methods A 45-year-old woman presented to us with a six-month history of pain in the lower abdomen during intercourse. Pelvic examination was performed and a palpable mass was found. The mass had a size of 9 × 6 cm, a soft tissue consistency, was partially movable and the patient felt the pain during palpation. Examination of the inguinal lymph nodes revealed no signs of inguinal adenopathy. The results of laboratory tests, rectoscopy, chest X-rays, barium enema and bone scan were normal. Computed tomography (CT) showed an inhomogeneous expansive mass in the rectovaginal septum measuring 8.7 × 6.1 cm, without any signs of rectum or bladder invasion. The vascular structures of the pelvis were normal. At laparotomy the process was judged inoperable and only biopsy of the tumor mass was carried out. Histology showed a neoplasm with small, round to oval cells with scarce cytoplasm. Immunohistology with the monoclonal antibody CD99 (MIC-2 gene product, Ewing's sarcoma marker, clone 12E7, DAKO A/S, Glostrup, Denmark) revealed an extraosseous Ewing's sarcoma. The patient was treated with chemotherapy followed by whole-pelvis external beam radiation and intracavitary brachytherapy. Results A residual mass measuring 3.5 × 2.5 cm was visible on a control CT scan 18 months after treatment; however, the patient was feeling well and refused surgery to remove the residual mass. Conclusions To our knowledge this is the first reported case of extraosseous Ewing's sarcoma in the rectovaginal septum.

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Ewing's Sarcoma: An Approach to Radiological Diagnosis

Tumori Journal ◽

10.1177/030089167906500316 ◽

1979 ◽

Vol 65 (3) ◽

pp. 389-399 ◽

Cited By ~ 7

Author(s):

Fabrizio Lombardi ◽

Marco Gasparini ◽

Cristina Gianni ◽

Raffaele Petrillo ◽

John David Tesoro-Tess ◽

...

Keyword(s):

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Primary Site ◽

Long Bones ◽

Age Group ◽

Pediatric Age Group ◽

Localized Disease ◽

The Mean ◽

Small Bones

All the pertinent radiographs of 83 patients with histologically proven Ewing's sarcoma were reviewed. Forty-nine patients were in the pediatric age group, and 34 were adults. The mean age, the symptoms and time from symptoms to diagnosis were evaluated in the 2 groups. The site of primary involvement was in 54 % the long bones, 35 % the flat bones, 8 % the small bones and 3 % extraosseous. For the primary site we considered the diagnostic results of the standard radiographic investigations and in some cases the usefulness of angiography, xeroradiography and telethermography. At presentation we also evaluated the possible diffusion of the disease with standard radiographic surveys (chest and skeletal, including limbs) and with foot lymphography in selected cases. In this way, 57 patients (69 %) were considered to have localized disease. In this group, we also considered the value of the periodic radiographic follow-up, which enabled us to disclose the appearance of metastases (chest 64 %, bone 54 %, lymph nodes 11 %) in 28 cases (49 %). Finally, we made a comparison of the different radiologic and epidemiologic findings between children and adults.

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Ewing’s Sarcoma of the Hand

Journal of Hand Surgery (European Volume) ◽

10.1177/1753193408094922 ◽

2008 ◽

Vol 34 (1) ◽

pp. 35-39 ◽

Cited By ~ 6

Author(s):

O. A. ANAKWENZE ◽

W. L. PARKER ◽

L. E. WOLD ◽

K. K AMRAMI ◽

P. C. AMADIO

Keyword(s):

Radiation Therapy ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Case Series ◽

Surgical Excision ◽

Case Review ◽

Retrospective Case ◽

Postoperative Radiation Therapy ◽

Definitive Surgery

A retrospective case review was carried out to report the outcomes in a contemporary case series of Ewing’s sarcoma originating in the hand. We identified five patients treated since 1995. All five had wide surgical excision, one by ray amputation. All were treated with chemotherapy. Four patients also received radiation therapy, two to treat metastases and two as an adjunct to local excision. There were no local recurrences. Two patients developed metastases. Both died of their disease. Neither of these two patients had received local postoperative radiation therapy; one did not receive chemotherapy before definitive surgery. The other three patients were alive and free of disease at last follow-up, 4 to 12 years after initial presentation.

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