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Radiation treatment in a rare case of sinonasal ewing's sarcoma: A case report
... If surgically, negative margins are not attainable as it is difficult in sinonasal areas; local regional control with radiotherapy is preferred treatment modality. 13,14 Studies show combined treatment management has increased the overall 5-year survival rate 20 to 70%. 13,14 In our case, the patient was surgically treated and referred to cancer center for chemotherapy and radiotherapy as per oncologist prospective. ...
... 13,14 Studies show combined treatment management has increased the overall 5-year survival rate 20 to 70%. 13,14 In our case, the patient was surgically treated and referred to cancer center for chemotherapy and radiotherapy as per oncologist prospective. Patient was lost to follow up subsequently. ...
Sinonasal and pharyngeal mass lesion of Extraskeletal Ewing sarcomas (EEWSs) are extremely rare. Herein we present a case of EEWSs of ethmoid sinus extending into nasal and pharyngeal spaces mimicking as angiofibroma clinically. Histopathological examination in conjunction with immunohistochemistry puzzled out the diagnostic dilemma.
Ewings sarcoma (ES) and primitive neuroectodermal tumor are closely related family of small round cell tumors seen in childhood and adolescence. The incidence of these tumors occurring in the head and neck region is just 2-7%. Mandible and maxilla are the most common sites, whereas involvement of the sinonasal tract is very rare. We report a case of extraskeletal ES of the sinonasal tract in a 29-year-old female who presented with nasal obstruction and epistaxis. The patient was treated with 14 cycles of chemotherapy, combined with surgery and radiotherapy with complete recovery. We present this case due to its rarity, to analyze the clinical, histopathological and immunohistochemical findings, so as to differentiate from other small round cell tumors of the sinonasal tract for appropriate treatment.
The aim of this study was to compare various dosimetric parameters of dynamic mlc intensity modulated radiotherapy (IMRT) plans with volumetric modulated arc therapy (VMAT) plans for sino-nasal cancers, which are rare and complex tumors to treat with radiotherapy. IMRT using five fields, coplanar in the sagittal plane and VMAT employing two coplanar arc plans were created for five patients. The plans were assessed by comparing Conformity Index and Sigma Index (dose homogeneity) in the Planning Target Volume (PTV) and through comparison of dose-volume characteristics to the following organs at risk (OARs): Spinal cord, brainstem, eye, ipsilateral and contralateral optic nerve and the volume of brain receiving 10% of the prescribed dose (V(10%)). The total monitor units required to deliver the plan were also compared. Conformity Index was found to be superior in VMAT plans for three patients and in IMRT plans for two patients. Dose homogeneity within the PTV was better with VMAT plans for all five cases. The mean difference in Sigma Index was 0.68%. There was no significant difference in dose between IMRT and VMAT plans for any of the OARs assessed in these patients. The monitor units were significantly reduced in the VMAT plan in comparison to the IMRT plan for four out of five patients, with mean reduction of 66%. It was found in this study that for the treatment of sino-nasal cancer, VMAT produced minimal, and statistically insignificant improvement in dose homogeneity within the PTV when compared with IMRT. VMAT plans were delivered using significantly fewer monitor units. We conclude in this study that VMAT does not offer significant improvement of treatment for sino-nasal cancer over the existing IMRT techniques, but the findings may change with a larger sample of patients in this rare condition.
Introduction and purpose. Primary Ewing's sarcoma arising from the
bones of the head and neck region is extremely rare representing only 1– 4% of all Ewing's
sarcoma cases. Previous reports suggest a better prognosis for that particular anatomic site.
The purpose of this study was to analyze the clinico-epidemiologic characteristics of that rare clinical presentation, as
well as its patterns of failure and prognosis following treatment.
Ewing sarcoma is a highly malignant neoplasm, which very rarely involves the head and neck region. Only a few cases have been reported in the maxila or palate. This case is being reported for its rarity. A patient presented to the Department of Oral Pathology, Faculty of Dental Sciences, CSM Medical University, Lucknow, India, with a lesion expanding the nasal cavity and posteriorly reaching up to the anterior border of nasopharynx and inferiorly causing the destruction of the hard palate extending into the oral cavity. The biopsy specimen histopathologically revealed undifferentiated malignant neoplasm, and the definitive diagnosis of Ewing sarcoma was made with immunohistochemistry. The patient underwent surgical excision of the lesion followed by radiotherapy and chemotherapy.
Tumors in the Ewing family (EFTs) are the second most common bone tumors in children and adolescents. Despite aggressive chemotherapy, one-third of patients with localized tumor still may develop recurrences. This implies that not all tumor cells are eradicated and that the patients may have a level of residual disease. EFTs are characterized by specific chromosomal translocations that result in chimeric transcripts that can be detected with reverse transcriptase-polymerase chain reaction (RT-PCR) analysis.
The authors report the prognostic potential of the positive chimeric transcript (EWS/FLI1) in bone marrow (BM) and/or peripheral blood (PBL) in 26 patients with EFT during a long follow-up period (median, 61 months).
At diagnosis, 43% of patients had positive RT-PCR BM results, with no correlation to tumor progression (P = 0.3). During follow-up, 58% of patients had positive RT-PCR results in their last sample analyzed (BM and/or PBL). A highly significant correlation between the presence of the chimeric transcript and disease progression was detected (P = 0.0028). In a multivariate analysis, the percentage of tumor necrosis (P = 0.007) and RT-PCR results during follow-up (P = 0.02) remained significant prognostic markers. In 10 of 11 patients who developed disease progression, BM and/or PBL samples were positive for the chimeric transcript before evidence of overt clinical recurrence.
Occult tumor cells in BM and/or PBL samples during long follow-up are strong predictors of recurrent disease in patients with nonmetastatic EFTs.
The extra-skeletal form is an unusual type of Ewing sarcoma (ES) arising from soft tissue and in the literature there are reports of less than 50 patients describing the tumor in the paranasal sinuses and skull base. The histological diagnosis is crucial to plan the correct treatment and the molecular confirmation is mandatory in equivocal patients. A multimodality treatment with chemotherapy, surgery and radiotherapy improved the outcomes of these diseases during the last decades and a free-margin resection with the endoscopic transnasal technique is one of the most recent ways to manage these pathologies in selected patients, reducing the morbidities of the external approaches and preserving the quality of life of the patient.Here, the authors present the first patient of primary sinonasal ES free from disease after 5 years of follow-up and treated with an endoscopic endonasal approach and a second patient of sinonasal metastases of ES treated with and endoscopic transnasal approach.
Ewing's sarcoma is a rare tumor of the bone. In the Intergroup Ewing's Sarcoma Study (IESS) approximately 4% of the primary bone tumors arose in the bones of the head and neck. The mean age of patients was 10.9 years; boys slightly dominated the group. Signs and symptoms were local in distribution, with a mass or swelling most frequent. As in other sites, we categorized the dominant histologic pattern as diffuse or filigree, the latter carrying a more unfavorable prognosis. The radiographic appearance may be atypical as compared to Ewing's sarcoma at other sites. Prognosis of head and neck Ewing's is significantly better than Ewing's sarcoma overall. The gnathic bones were commonly affected, yet there was no associated mortality. No patient with primary disease in the bones of the head and neck who survived for 5 years on any of the IESS protocols has subsequently died.
Primary malignant bone sarcomas (MBS) are rare and there are few studies examining their incidence and outcome. Here, the incidence and survival of all subtypes of MBS registered in England between 1979 and 2007 were analysed from patient registry data held by the National Cancer Intelligence Network (NCIN). Over 11,002 new cases of MBS were registered, an average of 379 per year. There was no change in incidence demonstrated over the study period (p = 0.08). Although a peak incidence is observed in adolescence, approximately half of MBS are diagnosed in patients over 50 years. An improvement in outcome of MBS was observed between those patients registered from 1979 to 1983 and 1983 to 1987 (p < 0.0001), but there has been no improvement since. In the most recent period studied (patients diagnosed 1998-2002) 5-year survival was 55% in Ewing sarcoma, 70% in chondrosarcoma, 56% in chordoma and 43% in osteosarcoma. Patients diagnosed with osteosarcoma over the age of 40 years or with a non-extremity tumour have a significantly inferior outcome; 22% 5-year survival >40 years compared with 53% <40 years (p < 0.0001) and 16% non-extremity tumour compared to 48% extremity tumour (p < 0.0001). This population-based study has allowed us to confidently define the English incidence and survival rates of both the commoner bone tumours such as osteosarcoma, and rarer entities such as chordoma as well as groups with inferior outcome. The lack of significant improvement over recent decades for these diseases is cause for concern and further research.
To review our experience and critically evaluate treatment strategy and results in children with head and neck rhabdomyosarcoma and Ewing's sarcoma.
Retrospective charts review of children affected by non-orbital rhabdomyosarcoma or Ewing's sarcoma of the head and neck who were treated at our institution from January 1996 to August 2009.
Seven consecutive children with head and neck rhabdomyosarcoma or Ewing's sarcoma were identified. Four children had rhabdomyosarcoma, 3 children had Ewing's sarcoma. Regions involved were: cheek, ethmoid and maxillary sinuses, nasopharynx, middle ear/mastoid and frontal bone. In one case, surgery was performed as primary treatment modality; the other children were treated firstly with chemotherapy. Three patients underwent surgical resection after chemotherapy, while 4 patients received radiotherapy. Five children are disease free after a median of 7.7 years from initial diagnosis. Two patients relapsed after 10 and 29 months from initial diagnosis respectively; despite the administration of additional therapy both children died of disease.
Treatment for rhabdomyosarcoma and Ewing's sarcoma consists in a multimodal therapy involving chemotherapy, radiotherapy and surgery. The optimum use, timing and intensity of these three treatments are still matters of international debate. Chemotherapy in association with radiotherapy has proven capable to obtain local and distant control of disease. But when surgery is unfeasible or fails in radicality, local control is difficult without radiotherapy. Despite additional therapeutic efforts, prognosis of relapsing disease remains poor.
The Ewing's family of tumors (EFT) are malignant neoplasms affecting children and young adults. Most cases arise in the long bones or the pelvis. Primary EFT of head and neck is uncommon and primary sinonasal EFT is even rarer. Previous studies have not focused on the sinonasal region specifically, and the published literature on sinonasal EFT consists of sporadic case reports. Fourteen cases of sinonasal EFT were available and had H&Es for review and immunohistochemical stains for CD99, S100, keratins, synaptophysin and desmin. FISH or RT-PCR was performed for EWSR1 abnormalities on 8 cases. The 14 identified patients included 5 males and 9 females, ranging from 7-70 years of age (mean 32.4 years). Tumors involved nasal cavity (5), sinuses (5) or both (4). Five patients had dural, orbital or brain involvement. The majority involved bone radiologically and/or microscopically. All cases were composed of small cells with variable cytoplasmic clearing. Focal or prominent nesting was noted in most cases. All cases were positive for CD99. Keratins (AE1/3 and/or CAM5.2), S100 and synaptophysin were positive in 4, 3 and 5 cases, respectively. All cases were negative for desmin. The 8 cases tested by FISH or RT-PCR were positive for EWSR1 abnormalities. Follow-up in 8 patients ranged from 1-168 months (average 11.3 m) showing 1 death due to metastatic disease, 1 death due to local disease, 1 patient alive with metastases and 5 patients disease-free at last follow-up. Interestingly, however, an analysis of the literature suggests a better prognosis for sinonasal EFT than EFT overall.
Ewing's sarcoma/PNET are small round cell tumors showing a varying degree of neuroectodermal differentiation. They are one of the commonest tumors of childhood and occur in bone and within soft tissues. Traditionally, light microscopy with the aid of immunohistochemical stains was suitable for diagnosis. But now translocation analyses are being used not only for the diagnosis and classification of small round cell tumors, but to ascertain their prognostic significance, detect micrometastasis, and monitor minimal residual disease, with potential for targeted therapy. This article analyzes the pathology, biology, and molecular aspects of Ewing's sarcoma/PNET and discusses their clinical and therapeutic implications.
Primary bone sarcomas arising in osseous structures of the head and neck are rare. These tumors are often incompletely resected and treated with radiotherapy for local control.
We report a case of a 9-year-old girl with a maxillary Ewing's sarcoma. This patient was successfully treated with neoadjuvant chemotherapy followed by surgical resection and prosthetic reconstruction of the primary site. The surgical approach that was used consisted of a subtotal maxillectomy by means of a facial degloving approach, sparing the orbital contents and the inferior orbital rim and orbital floor.
This approach produced excellent cosmetic, functional, and oncologic outcome. The patient remains without evidence of disease recurrence more than 4 years after surgery.
This case illustrates a novel surgical approach to the resection of a maxillary Ewing's sarcoma and highlights the need for a multidisciplinary team approach to the management of head and neck sarcomas in children.
Ewing's sarcoma is a small round-cell tumor typically arising in the bones, rarely in soft tissues, of children and adolescents. Ewing's sarcoma has retained the most unfavorable prognosis of all primary musculoskeletal tumors. Prior to the use of multi-drug chemotherapy, long-term survival was less than 10%. The development of multi-disciplinary therapy with chemotherapy, irradiation, and surgery has increased current long-term survival rates in most clinical centers to greater than 50%. In addition, the preferred method of tumor resection has changed; limb salvage has nearly replaced amputation of the affected limb. Limb salvage procedures can be performed in place of amputation without compromising patient survival rates. Recent studies have revealed that the pathognomonic translocations involving the EWS gene on chromosome 22 and an ETS-type gene, which is most commonly the Fli1 gene on chromosome 11, are implicated in more than 95% of Ewing's sarcomas, primitive neuroectodermal tumors and Askin's tumors. Therefore, these lesions have become regarded as a single entity, dubbed the Ewing's family of tumors. RT-PCR to detect EWS-ETS gene arrangements is widely used to confirm the diagnosis of Ewing's family of tumors. Experimental results suggest that inhibition of the signaling pathway downstream of the EWS-ETS gene may lead to the development of molecularly targeted therapy in the future.
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Ewing's family of tumours of the sinonasal tract and maxillary bone
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