Retrospective Analysis of Ploidy in Primary Osseous and Extraosseous Ewing Family Tumors in Children

Perotti, Daniela; Corletto, Valentina; Giardini, Roberto; Parafioriti, Antonina; Fossati-Bellani, Franca; Luksch, Roberto

doi:10.1177/030089169808400411

Cited by 7 publications

(5 citation statements)

References 26 publications

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“…EES and IES have similar overall survival [99,109]. These findings are corroborated by other smaller case series published previously [117,118]. However, when considering patients who present with localized disease, EES may have a slightly better prognosis as compared to IES, as reported by Applebaum et al (10-year OS = 65.2% 95% CI [59.3, 70.5%] for localized EES vs. 55.3% 95% CI [51.5, 58.8] for localized IES) [99].…”

Section: Prognosis Of Retroperitoneal Extraosseous Ewing Sarcomasupporting

confidence: 89%

Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma

et al. 2023

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With the exception of well-differentiated liposarcoma, dedifferentiated liposarcoma, leiomyosarcoma, solitary fibrous tumour, malignant peripheral nerve sheath tumour, and undifferentiated pleomorphic sarcoma, the majority of the ≈70 histologic subtypes of retroperitoneal sarcoma are defined as ‘ultra-rare’ sarcomas, with an incidence of ≤1–5/1,000,000 persons/year. For most of these ultra-rare RPS subtypes, diagnosis and treatment follows international guidelines for the management of more common RPS histologies, with en bloc surgical resection as the mainstay of curative treatment, and enrolment in clinical trials where possible. Because the treatment of RPS is heavily driven by histology, the surgeon must be familiar with specific issues related to the diagnosis and management of ultra-rare sarcoma subtypes. Expert radiological and surgeon reviews are required to differentiate similarly presenting tumours where surgery can be avoided (e.g., angiomyolipoma), or where upfront systemic therapy is indicated (e.g., extraosseous Ewing’s sarcoma). Thus, the management of all retroperitoneal sarcomas should occur at a sarcoma referral centre, with a multidisciplinary team of experts dedicated to the surgical and medical management of these rare tumours. In this focused review, we highlight how diagnosis and management of the ultra-rare primary RPS histologies of malignant perivascular epithelioid cell tumour (PEComa), extraosseous Ewing sarcoma (EES), extraosseous osteosarcoma (EOS), and rhabdomyosarcoma (RMS) critically diverge from the management of more common RPS subtypes.

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Section: Prognosis Of Retroperitoneal Extraosseous Ewing Sarcomasupporting

confidence: 89%

Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma

et al. 2023

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“…The tumor's location is another issue of importance in this case. However, though there is controversy, the outcomes associated with extraosseous and osseous ES appear to be similar [10][11][12]. Altogether, our patient had criteria favoring both a good and a poor prognosis.…”

Section: Discussionmentioning

confidence: 54%

Primary Spinal Epidural Extraosseous Ewing's Sarcoma Mimicking a Spinal Abscess

Erkutlu

Büyükhatipoğlu

Alptekin

et al. 2007

Pediatric Hematology and Oncology

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Ewing's sarcoma is one of the most common malignant tumors of the skeletal system in children and young adults. It most frequently stems from the long bones of the extremities. However, though uncommon, extraosseous localization can be seen. Epidural extraosseous presentations are extremely rare. In this case, the authors report on the long-term follow-up of a patient with this well-demonstrated, but uncommon localization, and the initial unique presentation of the patient, which clinically and radiologically mimicked a cervical abscess.

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“…In cases without cytogenetic information, image cytometry (ICM) and flow cytometry (FCM) are two alternative techniques to determine the DNA content of fresh and paraffinembedded tumor tissues. Two retrospective studies evaluated the reliability of these techniques on ET samples and found 82% agreement between ICM and FCM [60,61]. Most of the cases were diploid in both studies, but aneuploidy was associated with an unfavorable prognosis.…”

Section: Prognostic Implication Of Genetic Markers In Etmentioning

confidence: 99%

Molecular Cytogenetics in Ewing Tumors: Diagnostic and Prognostic Information

2000

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Ewing tumors (ETs) are characterized by rearrangements of the EWS gene located in 22q12 and a high MIC2 expression. In 85% of ETs, the t(11;22)(q24;q12) generates chimeric fusion transcripts between the EWS and the FLI1 gene, whereas in the remaining cases the EWS gene is rearranged with different partners of the ETS oncogene family. Besides classical cytogenetic analysis, fluorescence in situ hybridization (FISH) and RT-PCR can be used to demonstrate these 22q12 rearrangements which are pathognomonic for ETs. To visualize 22q12 rearrangements in individual cells, DNA probes flanking the EWS-R1 breakpoint region on chromosome 22 can be hybridized in double-target FISH experiments on tumor cell preparations. Intact chromosomes 22 are indicated by juxtaposition of the DNA probes, whereas rearrangements of the EWS gene separate the hybridization signals. In addition to 22q12 rearrangements, numerical aberrations of chromosomes 8 and 12 can be observed in about 50% of ETs, deletions at the short arm of chromosome 1 and der (16)t(1;16)(q12;q11.2) chromosomes in about 20% of the cases. Numerical aberrations, deletions at 1p36.3, and the t(1;16) were detected by using double-target FISH on touch, cytospin, and chromosome preparations, on frozen and paraffin sections and isolated deparaffinized nuclei. So far, numerical aberrations of chromosomes 8 and 12 did not show prognostic impact. However, deletions at 1p36.3 and imbalances between the long and short arms of chromosome 1 were associated with adverse clinical outcome in a group of patients with localized disease.

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Retrospective Analysis of Ploidy in Primary Osseous and Extraosseous Ewing Family Tumors in Children

Cited by 7 publications

References 26 publications

Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma

Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma

Primary Spinal Epidural Extraosseous Ewing's Sarcoma Mimicking a Spinal Abscess

Molecular Cytogenetics in Ewing Tumors: Diagnostic and Prognostic Information

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