Rapidly Expanding Pediatric Post Radiation Brainstem Cavernoma Presenting with Singultus

Area of Science:

• Neuro-oncology
• Radiation Oncology
• Neuropathology

Background:

• Primitive neuroectodermal tumors (PNETs) are aggressive central nervous system neoplasms often treated with radiation therapy.
• Radiation therapy, while effective, can lead to secondary vascular malformations years after treatment.
• Cavernomas are a type of vascular malformation that can occur sporadically or secondary to other factors.

Observation:

• A pediatric patient, previously treated for PNET with cranial/spinal radiation, developed a medullary cavernoma seven years post-therapy.
• The cavernoma exhibited rapid, symptomatic growth over six weeks, presenting as intractable hiccups (singultus).
• Surgical resection of the symptomatic cavernoma led to complete resolution of the hiccups.

Findings:

• The case highlights the potential for delayed, rapid growth of radiation-induced cavernomas.
• Pathological review and mechanistic analysis suggest unique biological behavior in post-radiation cavernomas.
• The rapid growth and symptomatic presentation underscore the clinical significance of these secondary lesions.

Implications:

• Patients with a history of cranial/spinal radiation require vigilant surveillance for secondary vascular malformations.
• The clinical course and growth patterns of radiation-induced cavernomas may differ from sporadic cavernomas.
• This case emphasizes the importance of considering radiation history in the diagnosis and management of spinal vascular lesions.