Paraneoplastic Syndromes and Meningioma
Paraneoplastic syndromes can occur with meningioma, but this is exceedingly rare and limited to specific histologic subtypes, particularly chordoid meningioma. 1
Specific Association: Chordoid Meningioma
The only well-documented paraneoplastic syndrome associated with meningioma occurs with the chordoid subtype:
- Chordoid meningioma can present with systemic manifestations of Castleman disease, a lymphoproliferative disorder characterized by angiofollicular lymphoid hyperplasia of soft tissue 1
- This association may include dysgammaglobulinemia and other paraneoplastic syndromes 1
- Chordoid meningioma represents a rare histologic variant, making this paraneoplastic presentation exceptionally uncommon 1
Context: Meningiomas Are Not Typical Paraneoplastic Tumors
It is critical to understand that meningiomas themselves are not among the cancers typically associated with paraneoplastic syndromes:
- The most common neoplasms causing paraneoplastic syndromes include small cell lung cancer, thymic neoplasm, breast cancer, ovarian teratoma or carcinoma, testicular teratoma or seminoma, neuroblastoma, and lymphoma 1
- Meningiomas are notably absent from this list 1, 2, 3
- Paraneoplastic syndromes affect less than 1/10,000 cancer patients overall, with an estimated 10-15% of all cancer patients experiencing some form of paraneoplastic syndrome 3, 4
Clinical Implications
When evaluating a patient with meningioma and unusual systemic symptoms:
- Consider alternative diagnoses first, including direct tumor effects, metastases (which can occur with anaplastic grade III meningiomas to lung, liver, lymph nodes, or bone), or unrelated conditions 1
- If the meningioma is chordoid subtype on pathology, specifically evaluate for Castleman disease features including lymphadenopathy, constitutional symptoms, and immunoglobulin abnormalities 1
- Do not routinely screen for paraneoplastic antibodies in meningioma patients, as these are associated with other malignancies (anti-Hu with small cell lung cancer, anti-Yo with ovarian/breast cancer) 2, 3