Primary intramedullary extradural Ewing sarcoma 2024 Damer et al

[Andy]

Abstract

Ewing sarcoma is the second most frequent primary bone tumour of childhood and adolescence. The aim of this report is to describe the imaging, pathology, clinical findings, and treatment of a primary intradural extramedullary Ewing sarcoma with a unique intracranial metastatic component in a pediatric patient. A 14-year-old girl with a history of mood disorders presented to the emergency department with a 3-week history of neck torticollis, cervical pain, paresis, and paresthesia of the upper and lower extremities on the left side. Initially, non-organic causes such as somatization or conversion disorder were suspected. She returned 3 months later when her symptoms worsened.

MRI of the head and spine was performed, and demonstrated the presence of a suprasellar, retro-chiasmatic mass lesion. There was also diffuse leptomeningeal enhancement, another well-defined intradural extramedullary lesion the sacral region and several multifocal cauda equina soft tissue nodules.

The patient first underwent surgery. The patient was also treated with a combination of chemotherapy (vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide (VDC/IE)) and radiation as per the Children's Oncology Group AEWS1221 protocol. Most recent imaging conducted 22 months after the initial mass discovery revealed improvement of the suprasellar mass lesion with residual stable appearance of the prominence and enhancement of the pituitary stalk and tuber cinereum. There was interval improvement of the spinal lesions with no convincing residual.

Clinically, at almost three years since initial imaging findings, and 25 months since completing treatment, she is stable from an oncology perspective.

Open access, https://www.sciencedirect.com/science/article/pii/S1930043324001912

 

[Sean]

She returned 3 months later when her symptoms worsened.

A very critical 3 months.

 

[SNT Gatchaman]

As the article says, Ewing sarcoma is one of the more common paediatric malignancies. It's rare across the denominator of all children, though I probably see a new presentation every couple of months. Location here is unusual - hence the case report. Almost certainly the drop metastases were already present at the time of initial presentation to explain symptoms, but much smaller, some even dot-like. There is no question in my mind that the 3 month delay due to presumed conversion disorder negated any possibility of completely rescuing the cervical cord. Assuming chemo-radiation has fully treated the CNS malignancy, she may have lifelong significant disability.

Initially,the impression was that of a nonorganic cause such as somatization or conversion disorder.

Here is the relevant imaging obtained 3 months later. The arrowed small dark crescent on the left of the second image is the high cervical spinal cord, squashed to the right. The cord should be occupying the space taken up by the bright tumour. This required craniocervical decompression.

Clinically, at almost 3 years since initial imaging findings, and 25 months since completing treatment, she is stable from an oncology perspective. She continues to have limited mobility, but this is improving with intensive rehabilitation therapy.

As in the discussion —

back and neck pain as well as nonresolving pain complaints, especially in association with neurological complaints such as paresthesia and paresis should raise concern for more malicious pathogenesis, and although rare, intraspinal malignancies should be considered. In our case, the patient’s initial presentation of cervical pain and paresthesia was attributed to a nonorganic etiology. Only after worsening of the symptoms was imaging requested, but given the nonspecific findings associated with such detrimental disease processes, a clinician’s threshold for imaging, especially in the context of no identifiable organic cause, should be low.

We all make mistakes - and as Jo has commented here in the past they always stay with us and we learn - but this is an example of how a constantly reinforced systemic error of misattribution causes devastating harm multiple steps away from the literature that's promoting it. In-practice clinical thinking is so influenced by these ideas that it can seem perfectly correct and reasonable to attribute new-onset "paresis, and paresthesia of the upper and lower extremities on the left side" as psychosomatic. This young woman is now 17 with some unspecified degree of quadriplegia which I suspect will be significant and life-long judging from the degree of cord compromise and that could have been avoided.

Furthermore it's quite possible the story would have been different had she been a 14yo male hockey player. That might instead have seen imaging requested at initial presentation, with small enhancing nodules along the cord, perhaps a lumbar puncture able to diagnose on cytology (the brain lesion is not safely accessible and there was no marrow/extra-CNS disease), and I imagine the same successful VDC/IE chemo/neuraxis radiation but without needing the surgical decompression for advanced cord compromise.

 

[NelliePledge]

Genuine question based on not knowing anything. Has anyone in oncology or other areas done any work on common misattributions. It’s all very well doing TV ads as in uk to encourage people to go to their GP but systemic issues need addressing. My guess would be psychosomatic thinking is causing significant problems, I’d like to be proved wrong.

 

[Jonathan Edwards]

My guess would be psychosomatic thinking is causing significant problems, I’d like to be proved wrong.

Psychosomatic thinking is causing huge problems and large numbers of unnecessary deaths.
The whole concept of general practice needs changing. Someone with limb weakness or new paraesthesiae needs to go straight to a neurology clinic within days. Forget going to a GP who will 'watch and wait'. The whole system is a sham.