Terminal presentation and postmortem diagnosis of a mesenchymal chondrosarcoma with extensive intracranial involvement

This is the authors' abstract. We don't add key points for this paper.

To the Editor: Mesenchymal chondrosarcoma is a rare type of sarcoma that is molecularly defined by the presence of HEY1::NCOA2 fusion.1 Mesenchymal chondrosarcoma can affect all ages but most frequently arises in the second and third decades of life.2 Although this tumor can occur anywhere in the bone and soft tissues of the body, 12%-45% of mesenchymal chondrosarcomas arise in the head and neck.1 In contrast to other types of chondrosarcoma, mesenchymal chondrosarcoma can be relatively fast-growing and tends to present with symptoms related to mass effect.3 In this report, we describe a case of mesenchymal chondrosarcoma with extensive intracranial involvement resulting in mass effect and herniation, which was discovered as a terminal event and diagnosed at the time of autopsy. The patient was a 29-year-old woman with a past medical history significant only for chronic headaches. She presented to the emergency department of a local medical center for a headache that was significantly worse than her typical headaches and was not responsive to over-the-counter medications. She was administered medication for symptom relief and advised to follow up with her primary care provider; no imaging was obtained. She was subsequently lost to follow-up. Approximately 2 months later, the patient was brought to the emergency department by a family member after being found unresponsive. She was noted to have an approximately 2-week history of ongoing headaches, nausea, and sleeping much more than usual. On arrival, computed tomography (CT) imaging of the head showed a large intranasal and intracranial mass with destruction of the cribriform plate and ethmoid air cells, mass effect on the right orbit and globe, and mass effect on the frontal lobes with associated cerebral edema (Figure 1A and B). On physical examination, she was noted to have fixed and dilated pupils and marked proptosis of the right eye. While in the emergency department, she went into pulseless electrical activity and was resuscitated twice. She was transported to a tertiary care center for a higher level of care and evaluation for potential neurosurgical intervention. Neurosurgical intervention was not pursued due to the poor prognosis, and brain death was pronounced.