ABSTRACT. We describe a pediatric patient with histiocytic sarcoma involving the T6 and L4 vertebral bodies and the lungs. His tumor progressed during chemotherapy designed for Langerhans' cell histiocytosis and sarcoma. High-dose radiation, on the other hand, was effective. Pediatrics 2005;116:e322-e325. URL: www. pediatrics.org/cgi/doi/10.1542/peds.2005-0026; sarcoma, histiocytes, Langerhans' cell histiocytosis, histiocytic sarcoma.ABBREVIATIONS. LCH, Langerhans' cell histiocytosis; CT, computed tomography; 2CdA, 2-chlorodeoxyadenosine.H istiocytic and dendritic neoplasms are rare, especially in children. These tumors arise from antigen-processing phagocytes (histiocytes) and antigen-presenting dendritic cells. The cell types are derived from hematopoietic or mesenchymal stem cells. 1 Currently, the World Health Organization includes the following 6 entities under this designation: Langerhans' cell histiocytosis (LCH), Langerhans' cell sarcoma, follicular dendritic cell sarcoma, interdigitating dendritic cell sarcoma, dendritic cell sarcoma not otherwise specified, and histiocytic sarcoma. 2 The latter malignancy is a proliferation of histiocytes (tissue macrophages), which are characterized by positive expression of the macrophage-associated antigen CD68, negative expression of the T-cell-associated antigen CD1a, negative expression of the dendritic cell-associated antigens CD21/CD35, and lack of Birbeck granules on electron microscopy. 3 The rarity of histiocytic sarcoma continues to make its management challenging, and we are unaware of any recommended therapy for young children. We describe a child with histiocytic sarcoma to highlight his poor response to LCH-and sarcoma-based chemotherapy. By contrast, high-dose radiation proved to be effective. CASE REPORTThis previously healthy 3-year-old boy experienced intermittent low back pain radiating to the right inguinal region for ϳ2 months. His symptoms initially responded to ibuprofen. The pain intensity increased over a 2-week period, and he refused to walk. Review of systems was significant for pain with urination. With the exception of being unable to stand, his physical examination was unremarkable. The laboratory tests showed normal blood counts and normal liver and renal function. An MRI showed collapse of the T6 and L4 vertebral bodies and a soft tissue mass in the anterior epidural space at the level of L4 (Fig 1 A and B). The chest and abdominal computed tomography (CT) scans were normal. Bone marrow aspiration revealed no malignant infiltration. A technetium bone scan showed increased uptake limited to the T6 and L4 regions. CT-scan-guided needle biopsy of the L4 mass revealed infiltrative proliferation of the bone and soft tissue by sheets and clusters of large ovoid cells with abundant eosinophilic cytoplasm (Fig 2A). The nuclei were round/oval, eccentric, and pleomorphic with occasional grooving. Distinct, small nucleoli were present. Multinucleated giant cells were also present ( Fig 2B). Immunohistochemistry showed expression of CD68 (macroph...

Background-Carotid intraplaque hemorrhage has been associated with symptomatic stroke and can be accurately detected with magnetization-prepared rapid acquisition with gradient-echo (MPRAGE). Currently, there are no studies analyzing carotid MPRAGE signal and territorial ischemic events defined by diffusion restriction in the acute setting. Our aim was to determine the association of carotid MPRAGE signal with acute territorial ischemic events using carotid MPRAGE and brain diffusion tensor imaging. Methods and Results-After the addition of the MPRAGE sequence to the neck MR angiographic protocol, 159 patients with suspected acute stroke were evaluated with both brain diffusion tensor imaging and carotid MPRAGE sequences over 2 years, providing 318 carotid artery and paired brain images for analysis. Forty-eight arteries were excluded due to extracarotid sources of brain ischemia and 4 were excluded due to carotid occlusion. Two hundred sixty-six arteries were eligible for data analysis. Carotid MPRAGE-positive signal was associated with an acute cerebral territorial ischemic event with a relative risk of 6.4 (PϽ0.001). The relative risk of a diffusion tensor imaging-positive territorial ischemic event with carotid MPRAGE-positive signal was increased in mild, moderate, and severe stenosis categories (10.3, PϽ0.001; 2.9, Pϭ0.01; and 2.2, Pϭ0.01, respectively). Conclusions-In the workup of acute stroke, carotid MPRAGE-positive signal was associated with an increased risk of territorial cerebral ischemic events as detected objectively by brain diffusion tensor imaging. The relative risk of stroke was increased in all carotid stenosis categories but was most elevated in the mild stenosis category. (Circ Cardiovasc Imaging. 2012;5:376-382.)

Specific genetic loci responsible for CHARGE association are currently unknown. Herein, we describe a neonate with clinical manifestations consistent with CHARGE association who has a de novo interstitial deletion involving bands 8q11.2 to 8q13. Genetic mapping and genomic microarray technology have been used to more accurately define the breakpoints of this deletion. Within the deleted region, there are approximately 150 expressed genes, one or more of which may contribute to the manifestations of CHARGE association. © 2005 Wiley‐Liss, Inc.