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Joshua Seinfeld, Bette K. Kleinschmidt-Demasters, Shalini Tayal, and Kevin O. Lillehei

✓Desmoid-type fibromatosis involving the brachial plexus is a rare and challenging disease. Due to involvement of crucial neurovascular structures, wide local excision of the associated fibromas is rarely feasible and recurrence is common. The authors describe their experience in four surgically treated patients with desmoid-type fibromatosis involving the brachial plexus and review the relevant neurosurgical literature.

All tumors were assessed for c-KIT oncogene mutations in hopes of establishing a biological basis for using the tyrosine kinase inhibitor imatimib mesylate as an adjuvant therapy. Three patients experienced tumor recurrence requiring reoperation. Fractionated radiotherapy achieved local control in three patients, and the disease in one patient progressed beyond the treatment field. Single base pair changes at exon 10 of the c-KIT oncogene were identified in three tumors. One tumor with this mutation did not respond to treatment with imatimib mesylate. A review of the literature revealed 17 additional patients in two different case series. Analysis of these cases emphasizes the need for careful resection in patients with desmoid-type fibromatosis and supports the conclusion that without adjuvant radiotherapy a high local recurrence rate can be anticipated. For optimal local disease control, the authors recommend post-surgical radiation therapy regardless of the extent of resection achieved. The mutational status of the c-KIT oncogene remains an intriguing biological marker that in the future may predict which lesions will be responsive to imatimib mesylate; larger series will be necessary to test this hypothesis.

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Joshua Seinfeld, Bette K. Kleinschmidt-Demasters, Shalini Tayal, and Kevin O. Lillehei

Object

Desmoid-type fibromatoses are a locally invasive soft-tissue lesion that is most commonly encountered in abdominal sites. The tumor also affects head and neck areas, particularly the supraclavicular region, where it may encase and distort the brachial plexus and compromise neurovascular structures. Neurosurgeons may be called on to treat desmoid-type fibromatoses in these sites. The authors describe their experience in treating four patients with desmoid-type fibromatoses involving the brachial plexus and report the results of immunohistochemical analysis of the tumors.

Methods

Gross-total excision with nerve sparing was the first-line therapy of choice, although the surgery was challenging. Intraoperative identification of the site of tumor origin from musculoaponeurotic tissues by the neurosurgeon was necessary in two of the four cases to achieve a correct frozen section or final pathological diagnosis. Immunostaining for c-KIT (CD117) was undertaken in all cases in light of a previous report of positive CD117 immunoreactivity in abdominal desmoid-type fibromatoses.

All four tumors manifested weak focal immunostaining for c-KIT. One of the patients was given adjuvant imatinib mesylate therapy, with limited success. Subsequent polymerase chain reaction testing revealed that three of the four tumors manifested a single base pair change in exon 10 of the c-KIT gene (A to C in two cases and A to G in one case). There was local recurrence in three patients, despite gross-total excision. With the combination of surgery and radiation therapy, local disease control was achieved in three of the four patients.

Conclusions

This represents the first report of c-KIT sequencing in desmoid-type fibromatoses and suggests a possible biological basis for continuing to explore the use of adjuvant imatinib mesylate therapy.

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Christopher Roark, David Case, Mark Gritz, Patrick Hosokawa, David Kumpe, Joshua Seinfeld, Craig A. Williamson, and Anne M. Libby

OBJECTIVE

Aneurysmal subarachnoid hemorrhage (aSAH) has devastating consequences. The association between higher institutional volumes and improved outcomes for aSAH patients has been studied extensively. However, the literature exploring patterns of transfer in this context is sparse. Expansion of the endovascular workforce has raised concerns about the decentralization of care, reduced institutional volumes, and worsened patient outcomes. In this paper, the authors explored various patient and hospital factors associated with the transfer of aSAH patients by using a nationally representative database.

METHODS

The 2013 and 2014 years of the National Inpatient Sample (NIS) were used to define an observational cohort of patients with ruptured brain aneurysms. The initial search identified patients with SAH (ICD-9-CM 430). Those with concomitant codes suggesting trauma or other intracranial vascular abnormalities were excluded. Finally, the patients who had not undergone a subsequent procedure to repair an intracranial aneurysm were excluded. These criteria yielded a cohort of 4373 patients, 1379 of whom had undergone microsurgical clip ligation and 2994 of whom had undergone endovascular repair. The outcome of interest was transfer status, and the NIS data element TRAN_IN was used to define this state. Multiple explanatory variables were identified, including age, sex, primary payer, median household income by zip code, race, hospital size, hospital control, hospital teaching status, and hospital location. These variables were evaluated using descriptive statistics, bivariate correlation analysis, and multivariable logistic regression modeling to determine their relationship with transfer status.

RESULTS

Patients with aSAH who were treated in an urban teaching hospital had higher odds of being a transfer (OR 2.15, 95% CI 1.71–2.72) than the patients in urban nonteaching hospitals. White patients were more likely to be transfer patients than were any of the other racial groups (p < 0.0001). Moreover, patients who lived in the highest-income zip codes were less likely to be transferred than the patients in the lowest income quartile (OR 0.78, 95% CI 0.64–0.95). Repair type (clip vs coil) and primary payer were not associated with transfer status.

CONCLUSIONS

A relatively high percentage of patients with aSAH are transferred between acute care hospitals. Race and income were associated with transfer status. White patients are more likely to be transferred than other races. Patients from zip codes with the highest income transferred at lower rates than those from the lowest income quartile. Transfer patients were preferentially sent to urban teaching hospitals. The modality of aneurysm treatment was not associated with transfer status.

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David A. Kumpe, Jeffrey L. Bennett, Joshua Seinfeld, Victoria S. Pelak, Ashish Chawla, and Mary Tierney

Object

The use of unilateral dural sinus stent placement in patients with idiopathic intracranial hypertension (IIH) has been described by multiple investigators. To date there is a paucity of information on the angiographic and hemodynamic outcome of these procedures. The object of this study was to define the clinical, angiographic, and hemodynamic outcome of placement of unilateral dural sinus stents to treat intracranial venous hypertension in a subgroup of patients meeting the diagnostic criteria for IIH.

Methods

Eighteen consecutive patients with a clinical diagnosis of IIH were treated with unilateral stent placement in the transverse-sigmoid junction region. All patients had papilledema. All 12 female patients had headaches; 1 of 6 males had headaches previously that disappeared after weight loss. Seventeen patients had elevated opening pressures at lumbar puncture. Twelve patients had opening pressures of 33–55 cm H2O. All patients underwent diagnostic cerebral arteriography that showed venous outflow compromise by filling defects in the transverse-sigmoid junction region. All patients underwent intracranial selective venous pressure measurements across the filling defects. Follow-up arteriography was performed in 16 patients and follow-up venography/venous pressure measurements were performed in 15 patients.

Results

Initial pressure gradients across the filling defects ranged from 10.5 to 39 mm Hg. Nineteen stent procedures were performed in 18 patients. One patient underwent repeat stent placement for hemodynamic failure. Pressure gradients were reduced in every instance and ranged from 0 to 7 mm Hg after stenting. Fifteen of 16 patients in whom ophthalmological follow-up was performed experienced disappearance of papilledema. Follow-up arteriography in 16 patients at 5–99 months (mean 25.3 months, median 18.5 months) showed patency of all stents without in-stent restenosis. Two patients had filling defects immediately above the stent. Four other patients developed transverse sinus narrowing above the stent without filling defects. One of these patients underwent repeat stent placement because of hemodynamic deterioration. Two of the other 3 patients had hemodynamic deterioration with recurrent pressure gradients of 10.5 and 18 mm Hg.

Conclusions

All stents remained patent without restenosis. Stent placement is durable and successfully eliminates papilledema in appropriately selected patients. Continuing hemodynamic success in this series was 80%, and was 87% with repeat stent placement in 1 patient.

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François Bertucci, Anthony Gonçalves, Patrice Viens, Genevieve Monges, and Patrice Dubreuil

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Rebekah M. Ahmed, Geoffrey D. Parker, and G. Michael Halmagyi