BACKGROUND Angiosarcoma of the breast is a rare entity. The objectives of this study were to evaluate prognostic factors and determine outcomes in a large contemporary series of patients. METHODS Clinical and pathologic factors were analyzed in all patients with angiosarcoma of the breast treated between 1990 and 2003. Overall survival (OS) and disease‐free survival (DFS) were estimated using the methods of Kaplan and Meier. Multivariate analyses were performed to evaluate prognostic factors. RESULTS Fifty‐five women with angiosarcoma of the breast were identified. The median age was 49.1 years, and the median follow‐up time was 3.7 years. The median OS and DFS were 2.96 years and 2.26 years, respectively. For the 32 patients with primary angiosarcoma of the breast 5‐year OS was 59%. Twenty‐three (42%) patients had received prior radiation therapy for the treatment of breast cancer. These patients with radiation therapy‐associated angiosarcoma were on average 30 years older and less likely to present with distant metastatic disease than patients presenting with radiation‐naive angiosarcoma of the breast. Although radiation‐naive patients appeared to have had better early DFS and OS, the Kaplan–Meier curves were not statistically different between patients with radiation therapy‐associated disease and radiation therapy‐naive patients. On multivariate analysis, tumor recurrence (P = 0.006) was the only significant adverse prognostic factor noted for OS. CONCLUSIONS In this series of 55 patients with angiosarcoma of the breast, radiation therapy‐naive angiosarcomas occurred in younger patients, but they behaved similarly to radiation therapy‐associated angiosarcomas. Cancer 2005. © 2005 American Cancer Society.

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Muenke syndrome caused by the FGFR3 P250R mutation is an autosomal dominant disorder mostly identified with coronal suture synostosis, but it also presents with other craniofacial phenotypes that include mild to moderate midface hypoplasia. The Muenke syndrome mutation is thought to dysregulate intramembranous ossification at the cranial suture without disturbing endochondral bone formation in the skull. We show in this study that knock-in mice harboring the mutation responsible for the Muenke syndrome (FgfR3 P244R) display postnatal shortening of the cranial base along with synchondrosis growth plate dysfunction characterized by loss of resting, proliferating and hypertrophic chondrocyte zones and decreased Ihh expression. Furthermore, premature conversion of resting chondrocytes along the perichondrium into prehypertrophic chondrocytes leads to perichondrial bony bridge formation, effectively terminating the postnatal growth of the cranial base. Thus, we conclude that the Muenke syndrome mutation disturbs endochondral and perichondrial ossification in the cranial base, explaining the midface hypoplasia in patients. Developmental Dynamics 240:2584-2596,

Background: Scalp erosion in patients with deep brain stimulation (DBS) hardware is an uncommon complication that lacks a clearly defined management strategy. Previous studies have described various therapies including conservative treatment with antibiotics and surgical debridement with or without hardware removal. Objectives: The aim of this study was to review the efficacy of a hardware-sparing management strategy for the treatment of scalp erosion. Methods: Five patients with previous DBS implantation presented with scalp erosion and visible hardware exposure at the calvarial burr hole site, and underwent tension-free, vascularized, rotational scalp flap, with preservation of the leads under the pericranium. Two of the procedures were performed after an unsuccessful attempt at primary closure and 3 as a primary procedure. Each patient was followed clinically for at least 14 months postoperatively to evaluate for wound-healing and adverse effects. Results: The median duration from initial DBS hardware implantation to erosion and revision surgery was 12 months (range 1.5-62 months). Three patients were documented to have positive intraoperative cultures in spite of the absence of purulence. At the last follow-up, all patients were noted to have complete wound-healing and no evidence of infection or erosion. Conclusions: DBS scalp erosion can be managed by rotational scalp flap without hardware removal, even in cases where infection is identified.

Risk, IV.