Head and neck soft-tissue sarcoma in adults

Galy-Bernadoy, C.; Garrel, R.

doi:10.1016/j.anorl.2015.09.003

Cited by 46 publications

(43 citation statements)

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“…A recent study found an estimated 5‐years disease‐specific survival for the study group was 72% and the estimated overall survival was 61%, which is comparable to other studies showing 5‐years disease‐specific survival of 72–83% . Overall 5‐years survival from studies between 1993 and 2016 varies from 44 to 80% . Margin status is also an important factor that can affect prognosis.…”

Section: Prognosis and Survival From Head And Neck Stsmentioning

confidence: 55%

“…There are no specific radiological diagnostic criteria for STS, but worrying features include a progressively enlarging mass (Fig. ), tumour size >5 cm with sub‐aponeurotic irregular contours, necrotic areas (although these are also found in metastatic squamous cell carcinoma) and irregular septa within the tumour . Increased heterogeneity may be seen on both T1‐ and T2‐weighted images .…”

Section: Radiologymentioning

confidence: 99%

“…Overall survival is poorer in head and neck STS than for other locations. Some authors such as Galy Bernadoy & Garrel suggest this may be due to the distribution of histotypes with more aggressive tumours such as angiosarcoma and fibrosarcoma arising in the head and neck. However, overall generalisations of prognosis can also be difficult due to the heterogeneity of the histotypes .…”

Section: Prognosis and Survival From Head And Neck Stsmentioning

confidence: 99%

“…Metastatic neck nodes are associated with disease‐specific mortality (<0.001), and in 86% of 183 patients studied, the primary tumours were <5 cm . Therefore, in contrast to metastatic squamous cell carcinoma most authors therefore do not advocate elective neck dissection unless there is either clinical or radiological evidence of nodal involvement .…”

Section: Neck Metastasesmentioning

confidence: 99%

“…More than 50 histological subtypes have been described with the majority (80%) arising from soft tissues . The most frequent histological subtypes seen are malignant fibrous histiocytoma (MFH), fibrosarcoma, angiosarcoma, malignant peripheral nerve sheath tumour and non‐classified/non‐differentiated sarcoma . Immunopositivity for vimentin, α1‐antichymotrypsin and Ki‐67 help to differentiate MFH from other STS .…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Current update on the diagnosis and management of head and neck soft tissue sarcomas

Tudor-Green

Fonseca

Gomez

et al. 2017

J Oral Pathology Medicine

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Head and neck soft tissue sarcomas are a group of rare heterogeneous tumours arising from embryonic mesoderm. They comprise <1% of all head and neck malignancies and 5-15% of all sarcomas with most head and neck sarcomas arising from soft tissues. Although rare, they are associated with both high recurrence and mortality rates. We review the current management of head and neck soft tissue sarcomas.

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Section: Prognosis and Survival From Head And Neck Stsmentioning

confidence: 55%

Section: Radiologymentioning

confidence: 99%

Section: Prognosis and Survival From Head And Neck Stsmentioning

confidence: 99%

Section: Neck Metastasesmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Current update on the diagnosis and management of head and neck soft tissue sarcomas

Tudor-Green

Fonseca

Gomez

et al. 2017

J Oral Pathology Medicine

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Intensity‐Modulated Proton and Carbon‐Ion Radiation Therapy in the Management of Head and Neck Sarcomas

Yang¹,

Gao²,

Qiu³

et al. 2019

Cancer Medicine

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Purpose We report our experience of intensity‐modulated proton and carbon‐ion radiotherapy (IMPT/IMCT) for head and neck sarcomas (HNS). Methods and Materials An analysis of the ongoing prospective data registry from the Shanghai Proton and Heavy Ion Center (SPHIC) for patients with HNS was conducted. The 12‐ and 24‐month rates of local recurrence‐free, overall, distant metastasis‐free, progression‐free survival (LRFS, OS, DMFS, and PFS), and acute/late toxicities were calculated. The prognostic factors for the effectiveness of the treatment were also analyzed. Results Between 7/2014 and 5/2018, 51 consecutive patients with HNS received definitive doses of IMCT (41 cases), IMPT (two cases), or their combination (eight cases). One patient had R0 resection and another treated on the Chinese Food and Drug Administration registration trial received IMPT only. Twenty‐seven patients were treated according to various dose escalation trials or institutional protocols using IMCT or IMPT + IMCT boost. Twenty‐two patients with locoregional recurrence (10 and four patients failed surgery or surgery followed by radiotherapy, respectively) or radiation‐induced second primary sarcomas (eight patients) received salvage particle radiotherapy. With a median follow‐up time of 15.7 months, four patients with second primary sarcoma died. The 1‐ and 2‐year OS, PFS, LRFS, and DMFS rates for the entire cohort were 92.9% vs 90%, 73.6% vs 57.4%, 88.4% vs 78.9%, and 84.6% vs 76.5%, respectively. Those rates for patients without prior radiotherapy were 100% vs 100%, 82.1% vs 65.8%, 93.6% vs 85.3%, and 88.4% vs 79.5%, respectively. Multivariate analyses revealed that re‐irradiation was an independent prognostic factor for both LRFS and PFS ( P = 0.015 and 0.037, respectively). In addition, gross tumor volume (GTV) was an independent prognostic factor for PFS ( P = 0.048). One patient experienced Grade 3 acute toxicity (oral mucositis); another experienced Grade 4 acute event (hemorrhage) which required embolization. He lately died from hemorrhage (Grade 5) at 3.4 months after the completion of treatment. No patient experienced radiation‐induced acute/late toxicity of ≥ Grade 2 otherwise. Conclusion With few observed acute/late toxicities, IMPT/IMCT provided effective short‐term tumor control in our patients with HNS. Further investigations, preferably in a prospective fashion, will be required to confirm the efficacy and toxicities of IMPT/IMCT in this group of patients.

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RREB1‐MKL2 fusion in a spindle cell sinonasal sarcoma: biphenotypic sinonasal sarcoma or ectomesenchymal chondromyxoid tumor in an unusual site?

Mechtersheimer

Andrulis

Delank

et al. 2021

Genes Chromosomes & Cancer

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Biphenotypic sinonasal sarcoma (BSNS) is a rare, low grade spindle cell sarcoma, recently recognized in the WHO classification of head and neck tumors, which is characterized by a dual myogenic and neural differentiation and recurrent gene fusions, often involving PAX3‐MAML3, and less commonly PAX3 fusions with other partners such as NCOA1, NCOA2, or WWTR1. Yet, in about 4% of tumors no gene rearrangements are identified. Herein, we describe a RREB1‐MKL2 fusion in a BSNS lesion occurring in a 73‐year‐old female patient with a right maxillo‐ethmoidal angle lesion. The polypoid, moderately cellular tumor with infiltrative submucosal growth was composed of fascicles of relatively bland spindle cells embedded in a loose collagenous matrix. The tumor cells showed moderate amounts of eosinophilic cytoplasm with indistinct borders and uniform, pale, ovoid to slender nuclei. The slowly proliferating neoplastic cells co‐expressed smooth muscle actin and S100, and showed focal nuclear positivity for ß‐catenin, while lacking staining for cytokeratins, desmin, myogenin, caldesmon, glial fibrillary acid protein, and SOX‐10. Molecular analysis by targeted RNA‐based next‐generation sequencing identified an in‐frame fusion between exon 8 of RREB1 and exon 11 of MKL2, a genetic event that was reported to be a molecular hallmark of ectomesenchymal chondromyxoid tumor. Gene rearrangements in both genes were independently verified by fluorescence in situ hybridization (FISH). To evaluate its recurrent potential an additional group of 15 fusion negative BSNS were tested for abnormalities in RREB1 and MKL2 genes by FISH, but no additional positive cases were identified.

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Head and neck soft-tissue sarcoma in adults

Cited by 46 publications

References 50 publications

Current update on the diagnosis and management of head and neck soft tissue sarcomas

Current update on the diagnosis and management of head and neck soft tissue sarcomas

Intensity‐Modulated Proton and Carbon‐Ion Radiation Therapy in the Management of Head and Neck Sarcomas

RREB1‐MKL2 fusion in a spindle cell sinonasal sarcoma: biphenotypic sinonasal sarcoma or ectomesenchymal chondromyxoid tumor in an unusual site?

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