Primary Ewing’s Sarcoma of the Temporal Bone: A Rare Case Report and Literature Review (original) (raw)

Case Report: Primary Ewing’s Sarcoma of the Temporal Bone in an Infant

2015

Introduction: Ewing's sarcoma is the second most common primary malignant tumor of bone found in children after Osteosarcoma. It accounts for 4-9% of primary malignant bone tumors and it affects bones of the skull or face in only 1-4% of cases. Hence it rarely affects the head and neck. Subject and Method: In this case report, we describe a case of primary Ewing's sarcoma occurring in the temporal bone. The tumor was surgically excised, and the patient underwent chemotherapy for ten months. Results: Neither recurrence nor distant metastasis was noted in these 10 months after surgery but about 18 months after surgery our patient was expired. Conclusion: Although the prognosis of Ewing's sarcoma is generally poor because of early metastasis to the lungs and to other bones, a review of the article suggested that Ewing's sarcoma occurring in the skull can often be successfully managed by intensive therapy with radical excision and chemotherapy. This result was supported by the case reported here.

Primary Ewing's Sarcoma of the temporal bone in an infant

International journal of hematology-oncology and stem cell research, 2015

Introduction : Ewing's sarcoma is the second most common primary malignant tumor of bone found in children after Osteosarcoma. It accounts for 4-9% of primary malignant bone tumors and it affects bones of the skull or face in only 1-4% of cases. Hence it rarely affects the head and neck. Subject and Method : In this case report, we describe a case of primary Ewing's sarcoma occurring in the temporal bone. The tumor was surgically excised, and the patient underwent chemotherapy for ten months. Results : Neither recurrence nor distant metastasis was noted in these 10 months after surgery but about 18 months after surgery our patient was expired. Conclusion : Although the prognosis of Ewing's sarcoma is generally poor because of early metastasis to the lungs and to other bones, a review of the article suggested that Ewing's sarcoma occurring in the skull can often be successfully managed by intensive therapy with radical excision and chemotherapy. This result was suppor...

Ewing`s Sarcoma of Temporal Bone: Case Report

The Egyptian Journal of Hospital Medicine, 2018

Primary Ewing`s Sarcoma (ES) is a small round tumor, more likely to present in long bones and rarely in skull bones. ES commonly present in the second decade of life, and it is usually treated by multi‐modality including surgery, chemotherapy, and radiotherapy. In this case, a 13 years old boy presented with left facial palsy and hearing loss for two weeks, treated with steroid but no improvement. MRI was done and showed left jugular foramen and cerebellopontine mass measuring 4.8*4.4* 3 cm. Debulking surgery of the tumor was done with multiple biopsies reported by histopathology as malignant small round cell tumor consistent with Ewing sarcoma.

Primary Ewing’s sarcoma of the temporal bone: a rare entity and review of the literature

BMJ Case Reports, 2019

Ewing’s sarcoma (ES) is the second most common malignant primary bone tumour in children and adolescents. It primarily affects the diaphysis of long bones and pelvis. ES arising from temporal bone is extremely rare. To date, 43 such cases have been described in the literature. Clinical and radiological features are non-specific. Diagnosis is based mainly on immunohistochemistry. The present article presents an extremely rare case of ES of the temporal bone in a 20-year young man, and he was successfully treated with multiagent chemotherapy and radiotherapy.

Cranial Ewing’s sarcoma in children

Neurological Sciences, 2011

Ewing's sarcoma is a highly malignant neoplasm of bones which accounts for the 10% of primary bone malignancies. Primary Ewing's sarcoma of skull vault is very rare and constitutes 1-6% of all Ewing's sarcomas. We present a case of a primary and a radiation-induced skull Ewing's sarcoma. The symptoms, neuroimaging findings and the treatment for these cases are reviewed. Both children were operated with favorable outcome.

Primary Ewing’s sarcoma of cranial bones: analysis of ten patients

Acta Neurochirurgica, 2011

Objective Ewing's sarcomas are the second most common bone tumors in children and primary involvement of the cranium is uncommon. We analyzed retrospectively the data of ten patients with this rare subset of disease, who had been treated at our institute since 2005. Our aim was to assess the outcomes, recurrence rates and the selection of appropriate treatment methods. Methods The patients were reviewed with respect to their clinical presentations, treatment, and outcomes. Computed tomographic scanning of the brain was performed for all patients. Skeletal surveys with routine radiographs and technetium-99 bone scans to detect extracranial Ewing's sarcomas were performed for all patients. For all ten patients, radical tumor excision was achieved surgically. Chromosomal translocation studies were carried out on paraffin blocks for nine patients, using fluorescence in situ hybridization (FISH) and polymerase chain reaction (PCR). All patients were then subjected to adjuvant multidrug chemotherapy and radiotherapy. The follow-up periods ranged from 2 months to 5 years (mean, 17.6 months). Results The predominant presenting features were headaches, increased intracranial pressure, scalp swelling and trigeminal nerve involvement. The erosion of dura and intradural extension was noted in eight patients in our series. All nine patients, in whom FSIH and reverse transcriptase PCR (RT-PCR) was done, tested positive for EWS-FLI1(t22:12) translocation. All patients underwent radical excision within safe limits, followed by chemoradiation. Three patients had local recurrences, which were detected within 12 months after surgery. All three of them died within weeks of presentation with recurrence. One patient experienced a recurrence after 30 months. This recurrent tumor was completely excised, and additional chemotherapy was administered. There was a local recurrence again after 18 months that was treated with surgery and chemoradiation, and the patient is still surviving 5 years after the primary surgery. One patient had metastasis at presentation and died within 2 months of surgery. The remaining five seem to have good outcomes, though the follow-ups were not very long. Conclusion The treatment of primary Ewing's sarcoma of the cranium still remains to be radical surgery, aggressive

Ewing's sarcoma of the facial zygomatic area bones in a young child: A case presentation

American Journal of Otolaryngology, 2000

We present the case of a young patient with Ewing's sarcoma of the facial zygomatic area bones. This type of tumor in a very young child is a rare event and poses significant diagnostic and therapeutic challenges for the attending physician. In this case, the diagnosis was made by a computed tomography scan with subsequent histological confirmation. The differential diagnoses and therapeutic options are discussed, (Am J Otolaryngol 2000;21:213-215.

Primary Ewing's sarcoma of the skull

BMJ case reports, 2013

Ewing's sarcoma, a highly malignant bone tumour, typically affects the pelvis and the long bones of the lower extremities in children and young adults and primary involvement of the skull is rare. Here, we present a case of primary Ewing's sarcoma of the skull with localised swelling in a young adult that involved the frontoparietal region of the skull and was very aggressive in nature. Even with aggressive surgery, the patient had multiple recurrences within 1 month of surgery and ultimately the patient died.

AN UNUSUAL BONE TUMOUR - EWING’S SARCOMA AT ELEVEN MONTHS OF AGE

Ewing’s sarcoma below the age of 5 is extremely rare and radiological features of young Ewing’s is not established and can be ambiguous. We present the radiological features of a 11 m onths old infant that is pathologically proven as Ewing’s sarcoma. Ewing’s sarcoma can be included in the differential diagnosis list in the presence of sunburst appearance in X - ray and hair on end low signal striations in MRI .

Primary Ewing's Sarcoma of the Occipital Bone. Case Report

Neurologia medico-chirurgica, 2001

A 17-year-old female presented with a very rare case of primary Ewing's sarcoma of the skull involving the occipitotemporal region. Systemic examination found no evidence of metastasis. The tumor was surgically removed, and the patient underwent radiotherapy and chemotherapy. Fourteen months after surgery there has been no recurrence of the tumor. Cranial primary Ewing's tumor has a good prognosis after radical surgery and adjuvant therapy.