Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report.
| Title: | Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report. |
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| Authors: | Negru ME; Medical Oncology Unit, School of Medicine, University Hospital Maggiore della Carità, I-28100 Novara, Italy.; Sponghini AP; Medical Oncology Unit, School of Medicine, University Hospital Maggiore della Carità, I-28100 Novara, Italy.; Rondonotti D; Medical Oncology Unit, School of Medicine, University Hospital Maggiore della Carità, I-28100 Novara, Italy.; Platini F; Medical Oncology Unit, School of Medicine, University Hospital Maggiore della Carità, I-28100 Novara, Italy.; Giavarra M; Medical Oncology Unit, School of Medicine, University Hospital Maggiore della Carità, I-28100 Novara, Italy.; Forti L; Medical Oncology Unit, School of Medicine, University Hospital Maggiore della Carità, I-28100 Novara, Italy.; Lombardi M; Radiodiagnostic and Interventional Radiology Institute, School of Medicine, University Hospital Maggiore della Carità, I-28100 Novara, Italy.; Masini L; Department of Radiotherapy, School of Medicine, University Hospital Maggiore della Carità, I-28100 Novara, Italy.; Boldorini R; Department of Health Sciences, School of Medicine, University Hospital Maggiore della Carità, I-28100 Novara, Italy.; Galetto A; Medical Oncology Unit, School of Medicine, University Hospital Maggiore della Carità, I-28100 Novara, Italy. |
| Source: | Molecular and clinical oncology [Mol Clin Oncol] 2015 Jul; Vol. 3 (4), pp. 807-810. Date of Electronic Publication: 2015 Apr 24. |
| Publication Type: | Journal Article |
| Language: | English |
| Journal Info: | Publisher: Spandidos Publications UK Ltd Country of Publication: England NLM ID: 101613422 Publication Model: Print-Electronic Cited Medium: Print ISSN: 2049-9450 (Print) Linking ISSN: 20499450 NLM ISO Abbreviation: Mol Clin Oncol Subsets: PubMed not MEDLINE |
| Imprint Name(s): | Original Publication: London, UK : Spandidos Publications UK Ltd |
| Abstract: | Ewing's sarcoma (ES) is an aggressive tumour that may present with skeletal and extraskeletal forms. The extraskeletal form is rarely encountered in the head and neck region and is extremely rare in the sinonasal tract. This is the case report of a ES of the ethmoid sinus with intracranial and orbital extension in a 33-year-old male patient who presented with anosmia, epistaxis, reduction of visual acuity in the left eye and headache. On otorhinolaryngological clinical examination and biopsy via flexible endoscope, the lesion was misdiagnosed as ethmoid sinus carcinoma. The subsequent magnetic resonance imaging (MRI) of the brain revealed a large mass (6×7 cm) eroding the ethmoid and sphenoid sinuses, extending beyond the orbits and occupying the anterior cranial fossa with a maximum extension of ~5 cm. The patient underwent surgical resection and the microscopic examination of the specimen established the diagnosis of ES (immunohistochemically positive for CD99, neuron-specific enolase, CD56, synaptophysin, pancytokeratin, low-molecular weight cytokeratins and vimentin. The periodic acid Schiff stain exhibited strong intracytoplasmic block positivity and fluorescence in situ hybridization revealed a t(22;11) translocation. First-line chemotherapy was administered for 3 cycles; however, on restaging MRI, local disease progression was diagnosed. The patient received radiotherapy and second-line chemotherapy for 6 cycles. At 15 months after the diagnosis, the patient remains recurrence-free and maintains a good functional status and quality of life. |
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| Contributed Indexing: | Keywords: Ewing's sarcoma; chemotherapy; intracranial extension; magnetic resonance imaging; sinonasal tract |
| Entry Date(s): | Date Created: 20150715 Latest Revision: 20201001 |
| Update Code: | 20260130 |
| PubMed Central ID: | PMC4487037 |
| DOI: | 10.3892/mco.2015.548 |
| PMID: | 26171185 |
| Database: | MEDLINE |
Journal Article