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First Case of Intradural Myopericytoma at the Cervicomedullary Junction Described

Key findings

  • A patient who was referred to Massachusetts General Hospital with progressively worsening headaches and radiating neck pain was eventually found to have a myopericytoma, the first ever observed at the cervicomedullary junction
  • Myopericytomas can mimic other perivascular tumors, and it is important to consider them in the differential diagnosis
  • Myopericytomas involving the central nervous system are exceedingly rare
  • Total resection of the tumor was curative

Myopericytoma, which was not described until 1998, is a type of perivascular tumor that most commonly develops in the soft tissues of the extremities. It can occur in children, but it usually affects adults between the ages of 30 and 70.

Intradural myopericytoma are exceedingly rare, with only 12 cases reported before now: eight intracranial and four located at the thoracic and lumbosacral spinal cord. John H. Shin, MD, director of Metastatic Spine Oncology and Spinal Deformity Surgery at Massachusetts General Hospital, and colleagues recently described the first case of an intradural myopericytoma at the cervicomedullary junction. It is also the first known myopericytoma to occur near a major cranial blood vessel, in this case the vertebral artery.

Dr. Shin and his colleagues report in Neurosurgery that a 64-year-old woman was referred to them because of progressively worsening headaches and neck pain, which radiated to her left suboccipital and retroauricular regions. MRI demonstrated an enhancing lesion at the left craniovertebral junction at the level of C1–C2.

Just 30 days later, repeat imaging demonstrated substantial enlargement of the mass along the course of the intradural vertebral artery, from 7-by-3 mm, to 1.2-by-1.2 mm. Angiography showed no filling from the vertebral artery. The patient described a dermatomal area of pain reflective of left C2 nerve root involvement.

The patient agreed to surgery, and a far lateral craniotomy with removal of the C1 posterior arch was performed. A soft reddish lesion was observed that appeared to be either a neoplasm or vascular lesion. The surgeons were able to separate it from the accessory and C2 nerve roots, permitting total resection.

Dr. Shin and his colleagues explain that perivascular tumors (such as glomus tumors, myofibromas, hemangiopericytomas and myopericytomas) all have a "staghorn" branching vascular pattern on histopathology. Myopericytomas can be distinguished by concentric proliferation of ovoid and spindled cells around the vessels, and the pathologist, in this case, noted such a pattern.

Postoperatively, the patient's headaches resolved completely. Because total resection of myopericytomas is usually curative, no radiation therapy was pursued, and the patient has no evidence of recurrence three years after surgery.

The authors emphasize that it is crucially important to consider myopericytoma when encountering a perivascular tumor in the central nervous system. Each type of perivascular tumor has its own distinct clinical behaviors and management. Myopericytomas typically have a benign course, but they may adopt angioinvasive behavior or grow intravascularly.

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