Brain metastasis from extramammary Paget's disease

Area of Science:

• Oncology
• Neurology
• Pathology

Background:

• Extramammary Paget's disease (EMPD) is a rare condition, often resistant to treatment, with potential for distant metastasis.
• Brain metastasis from EMPD is exceptionally uncommon, posing significant diagnostic and therapeutic challenges.

Observation:

• A 76-year-old male with vulvar EMPD developed progressive disease despite chemotherapy.
• The patient presented with neurological symptoms (dyslexia) indicative of brain metastasis, confirmed by MRI.
• Pathological diagnosis confirmed brain metastasis from EMPD.

Findings:

• Surgical resection followed by stereotactic radiosurgery (SRT) led to a decrease in cerebellar lesion size.
• No recurrence of cerebral lesions was observed post-treatment with SRT.
• SRT was also applied to extracranial lymph node metastases.

Implications:

• Combined surgical debulking and SRT show promise as an effective treatment strategy for brain metastases from EMPD.
• This case highlights the importance of considering EMPD in the differential diagnosis of brain tumors, especially in patients with a history of the disease.
• Further research into optimal treatment protocols for metastatic EMPD is warranted.