Ewing Tumors of the Head and Neck

Whaley, J.T.; Indelicato, Daniel J.; Morris, Christopher G.; Hinerman, Russell W.; Amdur, Robert J.; Mendenhall, William M.; Keole, Sameer R.; Marcus, Robert B.

doi:10.1097/coc.0b013e3181aaca71

Cited by 61 publications

(60 citation statements)

References 40 publications

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“…When treating ES, a multidisciplinary approach consisting of surgical resection and radiotherapy plus chemotherapy has increased the 5-year survival rate from 20 to 58% (7,8). The most effective chemotherapy agents against ES are vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide.…”

Section: Discussionmentioning

confidence: 99%

Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report

Negru

Sponghini

Rondonotti

et al. 2015

Molecular and Clinical Oncology

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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 (6x7 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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Section: Discussionmentioning

confidence: 99%

Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report

Negru

Sponghini

Rondonotti

et al. 2015

Molecular and Clinical Oncology

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“…Of these 29 cases, 23 (79%) had no evidence of disease (NED) at last follow-up. Of these, 2 patients [37,40]. Four patients (14%) were dead of disease (DOD) at last follow-up with 2 having local failure, 1 distant failure (bone) and 1 showing local and distant failure (breast) [36,40].…”

Section: Discussionmentioning

confidence: 99%

“…Of these, 2 patients [37,40]. Four patients (14%) were dead of disease (DOD) at last follow-up with 2 having local failure, 1 distant failure (bone) and 1 showing local and distant failure (breast) [36,40]. Two (7%) additional patients had metastases (lung and chest wall) and were subsequently lost to follow-up [15].…”

Section: Discussionmentioning

confidence: 99%

Ewing’s Family of Tumors of the Sinonasal Tract and Maxillary Bone

Hafezi

Seethala

Stelow

et al. 2010

Head and Neck Pathol

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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.

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“…EES arising in head and neck is extremely rare and only some cases have been reported in the orbit, scalp, face, nasal cavity, paranasal sinus, nasopharynx, parapharyngeal space, larynx, hard palate, submandibular gland, parotid gland, thyroid gland, and soft tissue of the neck [4][5][6][7][8][9][10][11][12][13][14][15][16][17]. However, no case originating from the pterygomandibular space has been reported to date.…”

Section: Discussionmentioning

confidence: 96%

“…Although it has been reported in the orbit [4], scalp [5], face [6], nasal cavity [7], paranasal sinus [8], nasopharynx [9], parapharyngeal space [10,11], larynx [12], hard palate [13], submandibular gland [14], parotid gland [15], thyroid gland [16] and soft tissue of the neck [17], to our knowledge, there has been no documented case regarding involvement of the pterygomandibular space by EES. This article reports a rare case of EES originating from the pterygomandibular space with the submandibular space, mouth floor extension and describes the clinical, radiological, histopathological and immunohistochemical features.…”

Section: Introductionmentioning

confidence: 86%

Extraosseous Ewing's sarcoma arising from the pterygomandibular space

Li¹,

Huang²,

Chen³

et al. 2011

OJST

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Extraosseous Ewing's sarcoma (EES) arising in head and neck is extremely rare. We report on a rare case of EES originating from the pterygomandibular space. A 15-year-old boy was seen with a rapidly growing mass in the right upper neck, difficulty in opening mouth, dysphagia, numbness in the right lower lip and buccal skin. Clinical and radiological examinations indicated that a soft tissue mass originated from the pterygomandibular space with the submandibular space and mouth floor extension. Histological and immunohistochemical evaluations of the biopsy specimen revealed poorly differentiated small blue round cells with positive stains for CD99 and neuron-specific enolase. According to the clinical manifestation, CT and MRI findings, histological pattern and the results of the immunohistochemical studies, the final diagnosis was EES. Our patient was treated with chemotherapy and radiotherapy. The lesion recurred locally after 10 months and he died of multiple distant metastases 22 months later. Early and confident diagnosis coupled with combined surgical excision and modern chemotherapy/radiotherapy appears to be the most effective treatment plan.

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Ewing Tumors of the Head and Neck

Abstract: In the context of limited data on head and neck Ewing tumors, combined modality therapy provides excellent local control with reasonable acute and late toxicity. Large tumors were associated with poor disease control.

Cited by 61 publications

References 40 publications

Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report

Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report

Ewing’s Family of Tumors of the Sinonasal Tract and Maxillary Bone

Extraosseous Ewing's sarcoma arising from the pterygomandibular space

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