Parents of a 2-year-old female presented to pediatric OPD when they noticed progressive swelling of patient’s head since 1month. They also noticed visual disturbance, difficulty in walking since 20 days, fever and episodes of vomiting in last 2 days. No history of trauma/ seizures/ loss of consciousness.
CECT Brain showed fairly well defined lobulated heterogeneous soft tissue density lesion (35-45 HU) measuring 5.1x9.4x.4.9 cm (AP X TR X CC) epicentered in occipital bone on left side showing internal chunky calcification (Figure 1) causing extensive destruction of inner and outer tables with sunburst pattern of periosteal reaction(Lodwick Type III) with intracranial (involving extra-axial space) and extracranial components (Figure 2).
On contrast study, the lesion showed heterogeneous enhancement with few areas of hypodense necrosis.
Anteriorly, there was displacement of left cerebellar hemisphere and mass effect on brain stem with displacement of basal cisterns and ascending transtentorial herniation. It was causing upstream dilatation of third and bilateral lateral ventricles with periventricular CSF seepage - obstructive hydrocephalus.
There was no evidence of infiltration into brain parenchyma (Figure 3).
It was displacing left transverse sinus and straight sinus.
Medially, the lesion was crossing midline and causing displacement of right cerebellar hemisphere.
Bilateral optic nerves appeared tortuous with flattening of posterior globe- papilledema.
Ewing sarcoma (ES) is a malignant small, round cell tumour arising from bone and soft tissue in children and young adults. It can occur at osseous and extra osseous sites [1].
There is male predilection in the ratio of 1.8 : 1. Approximately 90% of cases occur in the first two decades of life, peak incidence being between 5 and 13 years [2].
The mesenchymal stem cell is proposed as the cell of origin, but there could be other cell types that may give rise to this tumour based on its ability to occur at multiple sites [3]. Its usual locations are diaphysis of long bones followed by pelvis, ribs, vertebrae, and rarely skull (1).
Intracranial involvement is rare, with a reported incidence of 1-6% of all cases. Calvarial involvement is found in <1% patients [4]. Temporal bone is commonly involved followed by parietal and occipital bone. Sphenoid and ethmoid bones involvement is less seen [2].
Ewing’s sarcoma typically grows extradurally; usually, large mass is seen before dural invasion or clinical detection or both. The duration of symptoms ranges from 2 weeks to 2 years. Symptoms develop as a result of dural invasion or raised intracranial pressure. Headache and scalp swelling are the most common symptoms, papilloedema is the most common sign. However, these rarely present with hydrocephalus [2].
Histologically, characteristic feature include sheets of small round blue cells with an increased nucleus-to-cytoplasm ratio. Pseudorosettes may be present. Mitoses are common. Bony spicules may be present, and CD99 and vimentin may be expressed [2].
The chromosomal translocation t(11;22)(q24;q12) is pathognomonic [5].
The CT scan usually shows an isodense mass that may extend intra- or extracranially. The enhancement is usually strong and heterogeneous. There may be bony destruction and/or new bone formation, with a spiculated pattern distinctive of Ewing’s sarcoma. The MRI imaging shows heterogeneous signal intensity, along with scattered areas of haemorrhage and necrosis [2].
Anaemia, leukocytosis, elevated levels of ESR, ALP and C-reactive protein seen. An elevated LDH level is associated with a poor prognosis [6].
Age <15years, female gender, absence of metastases at diagnosis, no regional lymph node involvement, peripheral location of the tumour, tumour <8 cm, and initial LDH< 170 IU/L favours good outcome [6].
Surgery remains the best treatment modality. Adjuvant therapy after resection including radiotherapy and chemotherapy is essential [2,7].
Cranial Ewing’s sarcoma rarely shows early metastasis and has a better but still severe prognosis compared to many counterparts [8].
Ewing’s sarcoma
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The patient is a 2-year-old female, presenting with progressive cranial swelling, fever over the past two days, and recurrent vomiting. The main CT (plain and contrast) findings are as follows:
Based on the patient's age, clinical symptoms, and imaging features, the following possible diagnoses or differential diagnoses are considered:
Commonly seen in children and adolescents, classified as a small round cell malignant tumor. It often affects long bones or flat bones, including the skull. CT imaging may show osteolytic destruction alongside “onion-skin” type periosteal reaction. Clinically, patients can present with local swelling, pain, and raised intracranial pressure.
Neuroblastoma frequently occurs in young children and may metastasize to the skull or epidural space. However, such metastases typically appear after an evident primary lesion in the abdomen or chest is identified.
In children, skull involvement can occur in lymphoma or leukemia. Generally, other systemic symptoms are also noted, such as generalized lymphadenopathy or abnormal hematologic findings. Imaging often reveals multiple areas of bone destruction.
LCH may involve the skull, often presenting as “punched-out” osteolytic lesions. However, the overall aggressiveness is usually less pronounced than that of Ewing’s sarcoma, and associated scalp lesions or skin/ear discharge may be more common.
Considering the patient’s age (2 years), clinical presentation (head swelling, symptoms of raised intracranial pressure, possible visual disturbances, gait instability), and the above-mentioned CT findings and literature review, Ewing’s Sarcoma aligns most closely with the overall presentation. A definitive diagnosis typically relies on pathological examination (small round cells, immunohistochemical staining positive for CD99 and vimentin, or the characteristic chromosomal translocation t(11;22)).
Ewing’s sarcoma generally requires a comprehensive treatment strategy. The following approach is recommended:
Rehabilitation should be individually tailored and progressed gradually at each stage of treatment. For this young patient, professional guidance from a rehabilitation therapist or pediatric specialist is advised:
Throughout the rehabilitation process, closely observe the child’s temperature, wound healing, and overall tolerance. Adhere to the FITT-VP principle (Frequency, Intensity, Time, Type, Progression, and Individualization) to maximize rehabilitation benefits while maintaining safety.
Disclaimer: The above analysis report is for reference only and cannot substitute for in-person clinical diagnosis or professional medical advice. For any specific treatment plan, please consult a specialist or visit the hospital.
Ewing’s sarcoma
