Mesenteric fibromatosis mimicking a gastrointestinal stromal tumor.

Mesenteric fibromatosis is a locally aggressive tumor of the mesentery with a high propensity for bowel involvement. Mesenteric fibromatosis often mimics gastrointestinal stromal tumors in size, location and immunohistochemical features. We report the case of a 30-year-old male who underwent resection of a mesenteric tumor, initially diagnosed as gastrointestinal stromal tumor. The tumor was categorized as high-risk and the patient was treated with chemotherapy. Two years later the patient was found to have a mass in the mesentery and restarted on chemotherapy. The tumor did not respond to medical management. The patient underwent a second en bloc resection and pathology results were conclusive for mesenteric fibromatosis. This case highlights the significance of accurately differentiating mesenteric fibromatosis from gastrointestinal stromal tumor. Making a concrete diagnosis is often difficult because both gastrointestinal stromal tumors and mesenteric fibromatosis share a number of morphological and immunohistochemical features including CKIT expression.

Midterm follow-up of penetrating ulcer and intramural hematoma of the aorta.

OBJECTIVE: Most studies on variant forms of aortic dissection--penetrating ulcer and intramural hematoma--have focused on the initial presenting episode, with scant follow-up. This investigation provides midterm follow-up of penetrating ulcer and intramural hematoma to determine whether the aorta shows healing according to radiography, goes on to dilate, or tends to rupture during later follow-up. METHODS: Forty-five patients with penetrating ulcers (n = 26) or intramural hematomas (n = 19) were treated at our institution. Ten patients with penetrating ulcers were male and 16 were female, and their ages ranged from 54 to 87 years (mean 72 years). Eight patients with intramural hematomas were male and 11 were female, and their ages ranged from 54 to 88 years (mean 74 years). These patients all had symptoms of aortic disease. Patients with incidental imaging findings were not considered. RESULTS: In the group with penetrating ulcers, rupture occurred during the initial admission in 10 (38%) cases, 17 patients (65%) underwent surgery, and 22 patients (85%) survived to hospital discharge. Among those with intramural hematomas, rupture occurred during the initial admission in 5 cases (26%), 7 patients (37%) underwent surgery, and 16 patients (84%) survived to hospital discharge. Follow-up ranged from 1 month to 12.5 years (mean 3.4 years). No ischemic vascular complications occurred. Imaging follow-up was available for 26 of the 45 patients. Of these, 19% of lesions showed resolution, 23% had worsened, 39% had progressed to typical dissection, and 19% were unchanged. Six late deaths were known to be caused by rupture. In the group with penetrating ulcers, aortic diameter increased from 4.8 to 5.1 cm during the course of 14 months. In the group with intramural hematomas, aortic diameter increased from 5.3 to 5.9 cm during the course of 21 months. Overall survivals were 80% at 1 year, 73% at 3 years, and 66% at 5 years. CONCLUSIONS: Intramural hematoma and penetrating ulcer are lesions associated with advanced age. Women predominate. Penetrating ulcer and intramural hematoma rupture both early and late. Radiographically documented worsening, improvement, or frank dissection may occur with time. Aortic growth does occur (0.2 cm per year for penetrating ulcer and 0.4 cm per year for intramural hematoma). Vascular ischemic complications do not occur. Because of the high early rupture rate, the frequency of radiographic worsening, and the documented occurrence of late rupture, we now recommend surgical replacement of the aorta for these virulent vascular lesions as long as the patient's comorbidities do not preclude surgical intervention.

Alternate technique for implantation of left ventricular assist system: left thoracotomy for reoperative cases.

Reoperation for Novacor left ventricular assist device placement after prior cardiac surgery is fraught with multiple technical challenges. We have found that a thoracotomy approach obviates these dangers very favorably. The technique is performed off bypass except for apical coring and apical connection. Novacor outflow is to the descending aorta. This approach has been found safe, quick, and effective.

Yearly rupture or dissection rates for thoracic aortic aneurysms: simple prediction based on size.

BACKGROUND: Prior work has clarified the cumulative, lifetime risk of rupture or dissection based on the size of thoracic aneurysms. Ability to estimate simply the yearly rate of rupture or dissection would greatly enhance clinical decision making for specific patients. Calculation of such a rate requires robust data. METHODS: Data on 721 patients (446 male, 275 female; median age, 65.8 years; range, 8 to 95 years) with thoracic aortic disease was prospectively entered into a computerized database over 9 years. Three thousand one hundred fifteen imaging studies were available on these patients. Five hundred seventy met inclusion criteria in terms of length of follow-up and form the basis for the survival analysis. Three hundred four patients were dissection-free at presentation; their natural history was followed for rupture, dissection, and death. Patients were excluded from analysis once operation occurred. RESULTS: Five-year survival in patients not operated on was 54% at 5 years. Ninety-two hard end points were realized in serial follow-up, including 55 deaths, 13 ruptures, and 24 dissections. Aortic size was a very strong predictor of rupture, dissection, and mortality. For aneurysms greater than 6 cm in diameter, rupture occurred at 3.7% per year, rupture or dissection at 6.9% per year, death at 11.8%, and death, rupture, or dissection at 15.6% per year. At size greater than 6.0 cm, the odds ratio for rupture was increased 27-fold (p = 0.0023). The aorta grew at a mean of 0.10 cm per year. Elective, preemptive surgical repair restored life expectancy to normal. CONCLUSIONS: This study indicates that (1) thoracic aneurysm is a lethal disease; (2) aneurysm size has a profound impact on rupture, dissection, and death; (3) for counseling purposes, the patient with an aneurysm exceeding 6 cm can expect a yearly rate of rupture or dissection of at least 6.9% and a death rate of 11.8%; and (4) elective surgical repair restores survival to near normal. This analysis strongly supports careful radiologic follow-up and elective, preemptive surgical intervention for the otherwise lethal condition of large thoracic aortic aneurysm.