A Rare Neck Mass: Extraskeletal Ewing's Sarcoma

Malaysian Journal of Medicine and Health Sciences, 2022

Ewing's sarcoma is the second most malignant bone tumour and commonly occurs in paediatric patients. Extraskeletal Ewing's sarcoma can arise from the head and neck as the primary site of origin of the tumour despite being rare. This soft tissue tumour morphologically mimics Ewing's sarcoma of bone. We report a case of extraskeletal Ewing's sarcoma of the neck region in a 53-year-old lady who presented to the emergency department with a rapidly growing mass over three weeks associated with pain over the posterolateral neck region. It is challenging to reach the diagnosis at the first encounter as this lesion is found at an uncommon location and presented with a sebaceous cyst-like physical appearance.

Sao Paulo Medical Journal, 2000

CONTEXT: Ewing's sarcoma is a rare neoplasm, which usually arises in long bones of the limbs and in flat bones of the pelvis, with the involvement of head and neck bones being very unusual. CASE REPORT: a case of Ewing's sarcoma occurring in the mandible of a 35-year-old female. Pain and swelling of the tumor were the main complaints. The early hypothesis was an undifferentiated malignant neoplasm, possibly a sarcoma. The CT scan depicted an expansive lesion, encapsulated, with septa and characteristics of soft tissue, involving the left side of the mandible and extending to the surrounding tissues. The patient underwent surgical excision of the lesion, the definitive diagnosis of Ewing's sarcoma was established, and the patient commenced on radiotherapy.

Cancer, 1987

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.

Iranian Journal of Otorhinolaryngology, 2019

Extraosseous Ewing's sarcoma (EES) of the head and neck region is a rare occurrence, and Ewing's sarcoma of the parapharyngeal space is even rarer. To the best of our knowledge, only three cases of EES of the parapharyngeal space have been reported in the literature. We report a rare case of EES of the parapharyngeal space in an 8-year-old girl. She presented with complaints of earache, difficulty in breathing and swallowing and bleeding from the mouth. Investigations revealed a large parapharyngeal mass causing narrowing of the nasopharyngeal and oropharyngeal airway with skeletal and lung metastasis. Biopsy from the parapharyngeal mass was suggestive of malignant small round cell tumor. The patient was treated with chemotherapy and radiotherapy, but developed brain metastasis and succumbed to disease approximately 1 year after diagnosis. Herein, we describe the characteristic clinicopathological features and treatment with a comprehensive review of the literature. EES in t...

International Surgery Journal, 2021

Primary extra skeletal Ewing’s sarcoma is a very rare clinical entity. Patients are normally females in the second decade of their life. Clinically it normally presents as a fleshy mass which is mobile, not attached to the underlying structures. Diagnosis is difficult, final diagnosis being made by histopatholoical, immunohistochemistry and cytoskeletal studies combined. Ewing’s sarcoma is a tumour which normally affects the bones, and to a much lesser extent (10%) the soft tissues. Primary extra skeletal Ewing’s sarcoma affecting the back (as in the case we are discussing) is an extremely rare clinical entity with lesser than 50 cases reported worldwide. The entity being so rare doesn’t have any much literature for review.

Puerto Rico health sciences journal, 2018

The case of a 27-year-old Hispanic female who presented with an occipito-parietal tumor after suffering trauma to the area. A physical examination revealed no tenderness to palpation and with evidence of healing ulcerations. The biopsy was consistent with a synovial sarcoma. A wide excision of the mass (15cm x 14cm x 6cm) followed by a pericranial flap was performed. A follow-up CT showed recurrence involving the parietal sagittal sinus. After a second biopsy the mass was determined to be a small-cell sarcoma, consistent with Ewing's sarcoma. Chemotherapy included 8 cycles of doxorubicin, vincristine, and cyclophosphamide, with alternating cycles of etoposide and ifosfamide. A year later, a second wide excision of the mass was performed, followed by bilaminate skin substitute and skin graft placement for reconstruction of the soft-tissue defect. After chemotherapy, a follow-up PET scan showed no signs of re-uptake in any soft tissue or skeletal structures. After 2 years, the pat...

Balkan Journal of Medical Genetics, 2022

Ewing sarcoma (ES), described as a diffuse endothelioma of the bone, is divided into two categories: osseous and extraosseous, which mainly affects adolescents. Extraosseous Ewing Sarcomas (EES) are rare tumors originating from soft tissues. Their clinical presentation depends mainly on the primary location of the tumor and are highly chemosensitive and radiosensitive. The purpose of this study was to describe the clinical characteristics and outcomes of 3 children with EES and uncommon presentation treated in our Unit. The diagnosis of EES was confirmed by biopsy and cytogenetic analysis with fluorescence in situ hybridization (FISH). Surgical excision was planned as primary treatment, followed by adjuvant chemotherapy according to EURO-E.W.I.N.G protocol. To date, all patients are alive, 1, 3 and 4 years after completion of treatment, with no signs of recurrence or metastasis.

Head & Neck, 1994

This is the first reported case of congenital extraskeletal Ewing's sarcoma (EES) in the head and neck region. The tumor arose from the medial aspect of the right lower eyelid and rapidly increased in size despite surgery and chemotherapy. Accurate histologic diagnosis is emphasized, and the differential diagnosis, pathology, and treatment are discussed.

Medical Journal of Dr. D.Y. Patil Vidyapeeth, 2021

Extraskeletal Ewing's sarcoma (ES) is an aggressive, malignant small round-cell tumor usually occurring in children and adolescents. It needs to be differentiated from other malignant small round-cell tumors. Immunohistochemistry plays a pivotal role in establishing the diagnosis. ES/peripheral primitive neuroectodermal tumor is presumed to be neuroectodermal in children and young adults.