Ewing Sarcoma Family of Tumors

Maheshwari, Aditya V.; Cheng, Edward Y.

doi:10.5435/00124635-201002000-00004

Cited by 111 publications

(114 citation statements)

References 60 publications

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“…65% of the patients in the study were men. ESFT is more common in males than in females, with a reported ratio of 1.3 to 1.5:1 (Maheshwari et al, 2010), but no sexual predominance of EES/PNET was existence in published reports. The predominant location of EES in our series was the trunk.…”

Section: Discussionmentioning

confidence: 91%

“…EFST has a strong potential to metastasize. More than 10% of patients present with multiple bone metastases at initial diagnosis (Maheshwari et al, 2010). The most metastatic organs are the lung, bone, lymph node, or a combination, but metastases in the liver or brain are rare and malignant.…”

Section: Discussionmentioning

confidence: 99%

“…The Ewing sarcoma family of tumours (ESFT) consists of a group of tumours including classic Ewing sarcoma of bone, Askin tumours of the chest wall, Extraskeletal Ewing sarcoma (EES) and primitive neuroectodermal tumours of bone or soft tissues (Maheshwari et al, 2010). Although rare, such tumours constitute the second most frequent sarcoma of bone in child and adolescents (Herzog, 2005).…”

Section: Introductionmentioning

confidence: 99%

“…EES was first described by Tefft in 1969. He reported four patients who had paravertebral soft tissue tumours with a histologic appearance resembling Ewing sarcoma (Tefft et al, 1969), EES/PNET is usually seen in the second or third decades and can be developed from soft tissues in any location, the most common sites are extremities, trunk, and retroperitoneum (Raney et al, 1997;Maheshwari et al, 2010).…”

Section: Introductionmentioning

confidence: 99%

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Extraskeletal Ewing Sarcomas in Late Adolescence and Adults: A Study of 37 Patients

Tao

Hu²,

Wang³

et al. 2013

Asian Pacific Journal of Cancer Prevention

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Background: Extraskeletal Ewing sarcoma (EES)/primitive neuroectodermal tumours (PNET) are rare soft tissue sarcomas. Prognostic factors and optimal therapy are still unconfirmed. Materials and Methods: We performed a retrospective analysis on patients to explore the clinic characteristics and prognostic factors of this rare disease. A total of 37 patients older than 15 years referred to our institute from Jan., 2002 to Jan., 2012 were reviewed. The characteristics, treatment and outcome were collected and analyzed. Results: The median age was 28 years (range 15-65); the median size of primary tumours was 8.2 cm (range 2-19). Sixteen patients (43%) had metastatic disease at the initial presentation. Wide surgical margins were achieved in 14 cases (38%). Anthracycline or platinum-based chemotherapy was performed on 29 patients (74%). Radiotherapy was delivered in 13 (35%). At a median follow-up visit of 24 months (range 2-81), the media event-free survival (EFS) and overall survival (OS) were 15.8 and 30.2 months, respectively. The 3-year EFS and OS rates were 24% and 43%, respectively. Metastases at presentation and wide surgical margins were significantly associated with OS and EFS. Tumour size was significantly associated with OS but not EFS. There were no significant differences between anthracycline and platinum based chemotherapy regarding EFS and OS. Conclusions: EES/PNET is a malignant tumour with high recurrence and frequent distant metastasis. Multimodality therapy featuring wide surgical margins, aggressive chemotherapy and adjuvant local radiotherapy is necessary for this rare disease. Platinum-based chemotherapy can be used as an adjuvant therapy.

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Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Extraskeletal Ewing Sarcomas in Late Adolescence and Adults: A Study of 37 Patients

Tao

Hu²,

Wang³

et al. 2013

Asian Pacific Journal of Cancer Prevention

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“…Since the Ewing's sarcoma family of tumors includes tumors that display microscopic and immunohistochemical evidence of neural differentiation, peripheral primitive neuroectodermal tumors (PNET), we wondered whether other tumor types with neuroendocrine features or neuroectodermal origin may also express endosialin (13,14). Neuroblastoma, a malignancy of neural crest origin for which there is anecdotal evidence of endosialin expression was examined (1,15).…”

Section: Introductionmentioning

confidence: 99%

Endosialin: A novel malignant cell therapeutic target for neuroblastoma

Rouleau¹,

Smale²,

Sancho³

et al. 2011

Int J Oncol

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Abstract. Endosialin emerged recently as a potential therapeutic target for sarcoma. Since some sarcoma subtypes, such as Ewing's sarcoma, show characteristics of neuroendocrine differentiation, we wondered whether cancers with neuroendocrine properties and/or neuroectodermal origin, such as neuroblastoma, small cell lung cancer and melanoma, may express endosialin. Endosialin protein expression was surveyed in neuroblastoma, small cell lung cancer and melanoma in human clinical specimens by immunohistochemistry (IHC) and in human cell lines by flow cytometry. Side population cells were examined to determine whether cancer stem cells can express endosialin. Endosialin-expressing neuroblastoma cell lines were implanted in immunodeficient mice and allowed to grow. The xenograft tumors were resected and tested for endosialin expression by IHC. In human clinical specimens, vascular endosialin staining was observed in neuroblastoma, small cell lung cancer and melanoma. Malignant cell staining was strongest in neuroblastoma, weak in melanoma and rare in small cell lung cancer. In human cell lines, endosialin was detected in neuroblastoma cell lines, including cancer stem cell-like side population (SP) cells, but was absent in melanoma and was both rare and weak in small cell lung cancer. Human neuroblastoma xenograft tumors were found to be positive for endosialin. Our work suggests that endosialin may be a suitable therapeutic target for neuroblastoma.

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Picture of the Month—Diagnosis

2011

Arch Pediatr Adolesc Med

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Ewing Sarcoma Family of Tumors

Cited by 111 publications

References 60 publications

Extraskeletal Ewing Sarcomas in Late Adolescence and Adults: A Study of 37 Patients

Extraskeletal Ewing Sarcomas in Late Adolescence and Adults: A Study of 37 Patients

Endosialin: A novel malignant cell therapeutic target for neuroblastoma

Picture of the Month—Diagnosis

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