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.
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.
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.
[Show abstract][Hide abstract] ABSTRACT: Although there has been great development in the anatomical understanding and operative techniques for skull base tumors, controversy still exists regarding the optimal surgical strategies for the FMMs. We report clinical and radiologic features as well as the surgical findings and outcome for patients with FMM treated at our institution over the last 15 years.
We reviewed 114 consecutive cases of FMM operated between May 1993 and June 2008 in the neurosurgery department at Beijing Tiantan Hospital.
There were 68 female and 46 male patients (mean age, 52.3 years; range, 28-76 years). Foramen magnum meningiomas were classified as anterior (80 cases), anterolateral (24 cases), and posterolateral (10 cases). Mean duration of symptoms was 11.7 months (ranging from 1.5 to 240 months). Cervico-occipital pain (80.7%) and headache and dizziness (42.1%) were the most common presenting symptoms. The preoperative KPS was 72.5 +/- 8.3. Mean maximum diameter of the tumors on MRI was 3.35 cm (range, 1.5-4.7 cm). Posterior midline approach was performed in 10 cases, far-lateral retrocondylar approach in 97 cases, and extended far-lateral approach in 7 cases. Gross total resection was achieved in 86.0% of patients and subtotal resection in 14.0%. Surgical mortality was 1.8%. Follow-up data were available for 93 patients, with a mean follow-up of 90.3 months (range, 1-180 months), of which 59 (63.4%) lived a normal life (KPS, 80-100).
Our experience suggests that most anterior and anterolateral FMMs can be completely resected by a far-lateral retrocondylar approach without resection of the occipital condyle. Complete resection of the tumor should be attempted at the first operation. Postoperative management of FMM is important for the prognosis.
[Show abstract][Hide abstract] ABSTRACT: Desmoid tumours (DT) are myofibroblastic proliferations found in 10-25% of familial adenomatous polyposis (FAP) patients, either intra-abdominally (IA), in the abdominal wall (AW) or elsewhere (extra-abdominal (EA)). Most DT occur following prophylactic colectomy but occasionally patients present with pre-operative DT. Mutations 3' to codon 1444, predispose to DT and attenuated phenotype, leading to a potential strategy of delaying surgery in patients at high risk of DT. Here we assess if the existence of a pre-operative AW-DT or EA-DT predisposes to IA-DT following laparotomy. Data were collected on FAP patients undergoing primary surgery at St Mark's Hospital. Patients were stratified into those having no DT, EA-DT or AW-DT pre-operatively. Relative-risks were calculated and proportions compared using Fisher's exact-test. 587 FAP patients were identified; nine discovered with IA-DT intra-operatively were excluded. 5(0.9%) and 6(1%) of the remainder had a pre-operative EA-DT and AW-DT, respectively; one (0.2%) had both. Six of these 12 developed a post-operative IA-DT. 566(98%) had no pre-operative DT, 50(9%) of these developed IA-DT post-operatively; median time to tumour was 2 (IQR, 1-3.5) years. A pre-operative AW-DT predisposed to IA-DT post-operatively (RR = 7.6, 95% CI 4.0-14.1, P = 0.0009) whilst EA-DT did not (RR = 2.3, 0.4-13.3, P = 0.38). A 3' mutation was significantly associated with post-operative IA-DT in the 'no pre-operative DT' group (P = 0.002). The presence of a DT external to the abdominal cavity pre-operatively, poses a clinical challenge with regards to surgical decision-making. Patients with an AW-DT pre-operatively may warrant a conservative surgical approach, to minimize post-operative IA-DT risk.
Familial Cancer 09/2010; 9(3):407-11. DOI:10.1007/s10689-010-9342-9 · 1.98 Impact Factor
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