source: Journal of neuro-oncology

year: 2008

authors: Kenning T J, Kanwar V S, Qian J, Deshaies E M

BACKGROUND : Gardner’s Syndrome is a familial adenomatous polyposis (FAP) syndrome that presents with colorectal polyps, craniofacial osteomas, lipomas, epidermoid cysts, and ampullary cancers. These patients are at significant risk for developing desmoid tumors, which are locally infiltrating fibromatous lesions. These are most commonly found in the abdominal cavity, but may be extra-abdominal. Although FAP-associated desmoid tumors often occur at sites of surgical scarring, this is the first report following resection of an intracranial meningioma.

CASE DESCRIPTION : We present a patient with Gardner’s Syndrome who underwent resection of a foramen magnum meningioma and subsequently developed a de novo desmoid tumor at the surgical site. She had a complete surgical resection of the tumor but did not receive adjuvant therapy. At the time of her death 7 years after her desmoid tumor resection, she had no evidence of a recurrence at the skull base.

CONCLUSION : This case report emphasizes the need for careful follow-up in patients with Gardner’s Syndrome who undergo resection of a meningioma or other neurosurgical procedure. Adjuvant therapy may be of value in these patients.