Rare case of extraskeletal Ewings sarcoma of the sinonasal tract

Yeshvanth, Sunil Kumar; Ninan, Kurian; Bhandary, Satheesh Kumar; Lakshinarayana, Kishan Prasad H; Shetty, Jyothi; Makannavar, J H

doi:10.4103/0973-1482.95197

Cited by 44 publications

(57 citation statements)

References 4 publications

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“…In the sinonasal tract, the differential diagnosis includes all tumours composed of small round cells, such as olfactory neuroblastoma and undifferentiated carcinoma (5). The diagnosis requires a histopathological examination, immunohistochemistry and cytogenetics.…”

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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“…3 These tumors can arise from bone (skeletal type) or occasionally from soft tissues (extra skeletal type). 2 The extra skeletal form has the same histological, immunohistochemical and molecular features of skeletal ES and affects soft tissue of lower limbs, paravertebral tissues, chest wall, retroperitoneum and rare in head and neck region (2%---7%). 2 The extra skeletal form has the same histological, immunohistochemical and molecular features of skeletal ES and affects soft tissue of lower limbs, paravertebral tissues, chest wall, retroperitoneum and rare in head and neck region (2%---7%).…”

Section: Discussionmentioning

confidence: 99%

“…3 The skeletal type is more frequent and occurs in long bones of the extremities. 2,3 Mandible and maxilla are the most common sites affected in the head and neck region and involvement of the paranasal sinuses is very rare. 2,3 Mandible and maxilla are the most common sites affected in the head and neck region and involvement of the paranasal sinuses is very rare.…”

Section: Discussionmentioning

confidence: 99%

“…2,3 Mandible and maxilla are the most common sites affected in the head and neck region and involvement of the paranasal sinuses is very rare. 2,3 Although the exact etiology remains unknown, majority has a specific gene sequence t(11:22)(q24:q12) i.e., fusion between the 5 end of the EWS gene from chromosome band 22q12 with the 3 portion of the 11q24 FLI1 gene, a member of the ETS family of transcription factors. 2 It usually affects individuals less than 20 years old, with a male preponderance.…”

Section: Discussionmentioning

confidence: 99%

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Ewing's Sarcoma: A Rarity in Sinonasal Region

Cruz

Azevedo

Trigueiros

et al. 2015

Acta Otorrinolaringologica (English Edition)

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“…The effective treatment for ES includes combined surgical excision and chemotherapy/ radiotherapy. 1 We report a case of extraskeletal ES of the sinonasal tract in an 18 yr old male who presented with nasal obstruction and epistaxis. The patient was treated with surgery followed by chemotherapy and radiotherapy.…”

Section: Introductionmentioning

confidence: 99%

A rare case of Ewing’s sarcoma in the sinonasal tract

Satish

Nandakumar

et al. 2017

Int J Otorhinolaryngol Head Neck Surg

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<p>Ewing's sarcoma (ES) first described by James Ewing in 1921, is a primary neoplasm of the skeletal system. Extraskeletal Ewing’s sarcoma (EES) is a rare, rapidly growing, round cell malignant tumour that can develop in the soft tissue at any location. Extraskeletal Ewings sarcoma of the head and neck is uncommon. Nearly 80% of patients are younger than 20 years with peak incidence in the second decade. Ewings sarcoma rarely affects the sinonasal tract. Diagnosis is after histopathological examination immunohistochemical studies and cytogenetic studies. Treatment will include a multidisciplinary approach with surgery as the first line followed by chemotherapy and radiotherapy. Ewings sarcoma in the head and neck region do not metastazise early hence carries a better prognosis. Evaluation of lesion using imaging, biopsy followed by histopathology and immunohistochemistry and cytogenetic analysis are necessary for early diagnosis and treatment. We present a case of an 18-year-old male patient with left sided epistaxis and left sided nasal obstruction, on examination a mass was seen the nasal cavity, endoscopic excision and biopsy of the mass was suggestive of an Ewings sarcoma. He received post-operative chemotherapy and radiotherapy. 2 years on regular follow up patient is fine without signs of metastasis or recurrence. We are presenting this case due to the rarity of its presentation in the sinonasal tract.</p>

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Rare case of extraskeletal Ewings sarcoma of the sinonasal tract

Cited by 44 publications

References 4 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 Sarcoma: A Rarity in Sinonasal Region

A rare case of Ewing’s sarcoma in the sinonasal tract

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