April 2018

35-year-old male with facial palsy - Tumor

History:

35-year-old male presents with sudden onset of left-side facial palsy.

Exam

Awake, alert, able to name normally, normal memory.
Cranial nerves II-XII are intact except for left peripheral VII palsy.
Strength- 5/5 upper extremities.
No sensory loss.
DTR within normal limits. Cerebellar function is normal.
Gait normal.

 

Fig 1

Fig 2

Fig 3

Fig 4

Pathology

 
1. What is your next step?
2. Pathology shows the pathology shown in the introduction. What is the diagnosis?
3. An additional pathology test that would distinguish the diagnosis is:
4. Which of the following describes you?
5. I practice in one of the following locations.
6. Comments
7. After surgery what is the next step?

Case Explanation

The patient presents with a clival chordoma with brainstem compression.
Question 1. The correct answer is trans-nasal biopsy. This is a low risk procedure and will establish a diagnosis that can inform further treatment decisions. Once the diagnosis of chordoma is established, then surgery for definitive resection may be discussed. Transcranial procedures or a trans-oral approach would be associated with more potential morbidity. 
Question 2. The correct answer is chordoma. The pathology shows the classic physaliphorous cells. Chordoma has uniform cells with small, oval nuclei and numerous vacuoles. They will be positive for cytokeratin and EMA. Chondrosarcoma will have cartilaginous components and pleomorphic chondrocytes. Metastatic carcinoma will have large number of cells and frequent mitoses. Esthesioneuroblastoma will have sheets of small uniform cells and prominent reticular background that will be positive for neuronal markers like synaptophysin. 
Question 3. The correct answer is brachyury. This marker is positive in chordoma and negative in chondrosarcomas. GFAP is found in glial tumors. INI-1 is lost in ATRT tumors. IDH-1 is a marker of low grade glioma or secondary GBM. 
Question 4. The correct answer is fractionated radiotherapy. Radiotherapy has been shown to decrease recurrence rates and improve overall survival for chordomas. Fractionated radiation to the tumor bed and surrounding structures is most commonly given. Radiosurgery is limited to treating small residual and recurrent tumors. Proton beam radiotherapy usually preferable if available. 

 

References

  • Di Maio et al. Current comprehensive management of cranial base chordomas: 10 year meta-analysis of observational studies. J Neurosurg 2011. 
  • McDonald et al. Influence of residual tumor volume and radiation dose coverage in outcomes for clival chordoma. Int J Radiat Oncol Biol Phys 2016.

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