Relapsed chronic lymphocytic leukemia with a cardiac mass: A case report.

Chronic lymphocytic leukemia (CLL) is the most common hematological malignancy in the USA. Extra-medullary disease is very rare and is not well characterized. In practice, clinically significant cardiac or pericardial involvement by CLL is extremely rare with only a few case reports in literature. We report a 51-year-old male patient with a past medical history of CLL in remission, who presented with fatigue, dyspnea on exertion, night sweats and left supraclavicular lymphadenopathy. Laboratory investigations were notable for leukopenia and thrombocytopenia. Due to high suspicion of an underlying malignant process, a full body computerized tomography (CT) scan was obtained and showed an 8.8 cm soft tissue mass-like lesion occupying the majority of the right atrium and extending into the right ventricle, with probable pericardial involvement. Enlarged left supraclavicular and mediastinal lymph nodes were also present and had a mild mass effect on the traversing left internal thoracic artery and left pulmonary artery. A transesophageal echocardiogram and cardiac magnetic resonance imaging (MRI) were done to better characterize the cardiac mass. They confirmed a large infiltrating mass (measuring 10 × 7.4 cm) in the right atrium and ventricle, extending into the inferior vena cava inferiorly and coronary sinus posteriorly. A left supraclavicular excisional lymph node biopsy was performed and histopathology was consistent with Small Lymphocytic Lymphoma (SLL)/CLL. This case represents one of the few known cases of cardiac extramedullary-CLL presenting with an isolated cardiac mass. Further studies are needed to characterize the course of the disease, prognosis and optimum management along with the role of surgery.

Opportunities to integrate herders' indicators into formal rangeland monitoring: an example from Mongolia.

Despite increasing calls for knowledge integration around the world, traditional knowledge is rarely used in formal, Western-science-based monitoring and resource management. To better understand indicators herders use and their relationship to researcher-measured indicators, we conducted in-depth field interviews with 26 herders in three ecological zones of Mongolia. We asked each herder to (1) assess the overall condition of three different sites located along a livestock-use gradient from their winter camp using a numeric scale, (2) describe the indicators they used in their assessment, and (3) explain what caused their pastures to remain healthy or become degraded. At each site, we collected field data on vegetation variables and compared these with herders' ratings and indicators using linear regression. We used classification and ordination to understand how herders' assessment scores related to plant community composition, and determine how well multivariate analysis of factors determining plant community composition aligned with herders' observations of factors causing rangeland change. Across all ecological zones, herders use indicators similar to those used in formal monitoring. Herders' assessment scores correlated significantly and positively with measured total foliar cover in all three ecological zones, and with additional measured variables in the steppe and desert steppe. Ordination revealed that herder assessment scores were correlated with the primary ordination axis in each zone, and the main factors driving plant community composition in each zone were the same as those identified by herders as the primary causes of rangeland change in that zone. These results show promise for developing integrated indicators and monitoring protocols and highlight the importance of developing a common language of monitoring terminology shared by herders, government monitoring agencies, and researchers. We propose a new model for integrating herder knowledge and participation into formal monitoring in Mongolia, with implications for rangelands and pastoral people globally. We suggest practical ways of involving herders in formal monitoring that have potential broad application for promoting local and indigenous people's participation in implementing international agreements such as the UN Convention to Combat Desertification and the UN Convention on Biological Diversity, both of which call for involvement of local people and indigenous/traditional knowledges.

Sex-specific patterns of reproductive senescence in a long-lived reintroduced raptor.

For many species, there is evidence that breeding performance changes as an individual ages. In iteroparous species, breeding performance often increases through early life and is expected to level out or even decline (senesce) later in life. An individual's sex and conditions experienced in early life may also affect breeding performance and how this changes with age. Long-term monitoring of individuals from reintroduced populations can provide unique opportunities to explore age-related trends in breeding performance that might otherwise be logistically challenging. We used a unique dataset from a reintroduced population of white-tailed eagles Haliaeetus albicilla in Scotland, which has been intensively monitored since their initial reintroduction in 1975, to study age- and sex-specific trends in two measures of breeding performance. This monitoring provided data on the breeding performance of known individuals ranging in age from 3 to 26 years. We also explored changes in breeding performance in relation to early life experience (i.e., whether they were released or fledged in the wild). Breeding performance increased with age in early life in a similar manner for both sexes. We found stronger evidence for senescence in breeding performance in males than females. However, late-life female breeding success was associated with early life experience, while male senescent trends were not apparently impacted by conditions experienced during early life. Sexual differences in senescence mean that older males are less likely to breed successfully compared to older females, and this may influence females' mate changes later in life. This difference may suggest a linked sexual difference in survival rates or the possibility of proactive partner change by females in later life in this typically monogamous biparental species.

Outpatient administration of BEAM conditioning prior to autologous stem cell transplantation for lymphoma is safe, feasible, and cost-effective.

High-dose BEAM chemotherapy (BCNU, etoposide, Ara-C, and melphalan) followed by autologous hematopoietic stem cell transplantation is frequently used as consolidative therapy for patients with recurrent or refractory Hodgkin or non-Hodgkin lymphoma. The BEAM regimen has traditionally been administered over 6 days in the hospital, with patients remaining hospitalized until hematologic recovery and clinical stability. In an effort to reduce the length of hospitalization for these patients, our institution has transitioned from inpatient (IP) to outpatient (OP) administration of BEAM conditioning. Here, we report the results of an analysis of the feasibility, cost, complications, and outcomes for the initial group of patients who received OP BEAM compared to a prior cohort of patients who received IP BEAM. Patient and disease characteristics were comparable for the two cohorts, as were engraftment kinetics. Length of hospital stay was reduced by 6 days for the OP cohort (P < 0.001), resulting in a cost savings of more than $17,000 per patient. Fewer complications occurred in the OP cohort, including severe enteritis (P = 0.01), organ toxicities (P = 0.01), and infections (P = 0.04). Overall survival rate up to 3 years posttransplant was better for the OP cohort (P = 0.02), likely due to differences in posttransplant therapies. We conclude that OP administration of BEAM conditioning is safe and may offer significant advantages, including decreased length of hospitalization, reduced costs, decreased risks for severe toxicities and infectious complications, and likely improvement in patient satisfaction and quality of life.

Assessing the impacts of livestock production on biodiversity in rangeland ecosystems.

Biodiversity in rangelands is decreasing, due to intense utilization for livestock production and conversion of rangeland into cropland; yet the outlook of rangeland biodiversity has not been considered in view of future global demand for food. Here we assess the impact of future livestock production on the global rangelands area and their biodiversity. First we formalized existing knowledge about livestock grazing impacts on biodiversity, expressed in mean species abundance (MSA) of the original rangeland native species assemblages, through metaanalysis of peer-reviewed literature. MSA values, ranging from 1 in natural rangelands to 0.3 in man-made grasslands, were entered in the IMAGE-GLOBIO model. This model was used to assess the impact of change in food demand and livestock production on future rangeland biodiversity. The model revealed remarkable regional variation in impact on rangeland area and MSA between two agricultural production scenarios. The area of used rangelands slightly increases globally between 2000 and 2050 in the baseline scenario and reduces under a scenario of enhanced uptake of resource-efficient production technologies increasing production [high levels of agricultural knowledge, science, and technology (high-AKST)], particularly in Africa. Both scenarios suggest a global decrease in MSA for rangelands until 2050. The contribution of livestock grazing to MSA loss is, however, expected to diminish after 2030, in particular in Africa under the high-AKST scenario. Policies fostering agricultural intensification can reduce the overall pressure on rangeland biodiversity, but additional measures, addressing factors such as climate change and infrastructural development, are necessary to totally halt biodiversity loss.

Aneurysmal bone cysts of the vertebrae.

PURPOSE: To review records of 14 patients with aneurysmal bone cysts (ABCs) of the spine. METHODS: Using the Scottish Bone Tumour Registry for the period of October 1952 to November 2005, records of 9 females and 5 males aged 8 to 63 (mean, 25.3) years who had ABCs of the spine and were followed up for a mean of 7.1 years were reviewed. RESULTS: The most commonly involved site was the lumbar vertebrae (n=6), followed by the thoracic (n=4), cervical (n=3), and sacral (n=1) vertebrae. The mean duration of symptoms at presentation was 8.8 (range, 0.3-24) months. The symptoms included gradually increasing pain in the back (n=14), a palpable spinal mass (n=4), spinal deformity (n=2), and neurological deficits (n=5). All the patients underwent surgery: intra-lesional excision (curettage) without bone grafting (n=3), excision (n=7, 2 of whom had adjuvant radiotherapy), and open excisional biopsy (n=4, 2 of whom had iliac crest bone grafting). One patient with a cervical ABC underwent preoperative angiographic embolisation. Another patient with a sacral ABC underwent percutaneous sclerotherapy. Two patients had recurrence. One had recurrence within 4 months and underwent adjuvant radiotherapy; another had recurrence 16.8 years later and underwent repeat curettage. No major complications were encountered. CONCLUSION: Most ABCs of the spine occurred in young females. Intra-lesional excision was an effective treatment.

Use of eltrombopag, a thrombopoietin receptor agonist, in post-transplantation thrombocytopenia.

Persistent thrombocytopenia after stem cell transplantation can lead to increased morbidity and mortality [1,2]. The underlying causes are often multifactorial in this patient population [3,4]. In autologous transplantation, thrombocytopenia is usually a result of poor engraftment or a sign of impending disease relapse. In allogeneic stem cell transplantation, the etiology is often more complex with engraftment deficits, medication effects, graft versus host disease (GVHD), and other immunologic processes potentially contributing. Eltrombopag is an orally available nonpeptide thrombopoietin (TPO) receptor agonist which interacts with the transmembrane domain of the receptor on bone marrow megakaryocytes and upstream progenitor/stem cells. It has been studied in patients with chronic idiopathic thrombocytopenic purpura [5] and in patients with thrombocytopenia secondary to hepatitis C infection [6]. Unlike the case with recombinant human TPO, its use has not been associated with anti-platelet antibody production [7]. We report two cases of post-transplantation thrombocytopenia, one allogeneic and one autologous, where eltrombopag was given to treat prolonged thrombocytopenia. The use of eltrombopag in these two cases was effective in elevating platelet counts to levels that eliminated the need for platelet transfusions.

Ewing's Sarcoma of the Zygomatic Arch Presenting in a 69-Year Old: An Unusual Case Report.

Objective. We report a rare case of Ewing's sarcoma of the zygomatic arch presenting in a 69-year-old patient. Method. Case report and a review of the world literature on Ewing's sarcoma incidence and management. Results. Ewing's sarcoma is a malignant round cell tumour of neuroectodermal origin that typically presents in the pelvis and long bones of children and adolescent boys. This report is the first to document the presentation of ewing's sarcoma of the zygomatic arch in a 69-year-old lady. Our patient underwent surgical excision and radiotherapy and at 4-year followup has no signs of recurrence or metastasis. Conclusion. To our knowledge this is the first case report to document Ewing's sarcoma of this location in a 69-year-old patient. This case report highlights the importance of diagnostic investigations in Ewing's sarcoma and discusses the management issues that this rare presentation raises.

Predictive factors for recurrence of simple bone cyst of the proximal humerus.

PURPOSE: To evaluate factors predictive of recurrence following curettage of simple bone cysts (SBCs) in the proximal humerus. METHODS: Records of 29 male and 3 female patients aged 3 to 22 (mean, 11) years who underwent curettage with or without bone grafting for a solitary SBC in the proximal humerus were reviewed. The appearance, size, location, activity level, and fracture pattern of each cyst were recorded. The cyst index indicated the risk of refracture. Recurrence was defined as a refracture or enlargement of the cyst. RESULTS: 31 patients presented with a pathological fracture. The main symptoms were pain (n=30), loss of function (n=22), and mass/swelling (n=15). 25 patients gave a history of trauma. The duration of symptoms was less than one month. 10 patients had recurrence after a mean of 10 (range, 4-27) months; 5 were refractures and another 5 were enlargement of the cysts. Six were treated conservatively and eventually healed, whereas 4 underwent further curettage. Factors predictive of recurrence were patient age 5 years or younger (p=0.014), right-sided cyst (p=0.01), larger cyst (p=0.039), multilocular cyst (p=0.004) and unimpacted fracture (p=0.04). Recurrence was not related to gender, cyst location, or cyst activity level. CONCLUSION: Most SBCs heal even if the fracture is treated expectantly. SBCs should be left alone unless symptomatic. If curettage is performed, grafts or bone substitutes should be used. More aggressive treatment might be necessary for unimpacted fractures to minimise the risk of recurrence.

Linking international agricultural research knowledge with action for sustainable development.

We applied an innovation framework to sustainable livestock development research projects in Africa and Asia. The focus of these projects ranged from pastoral systems to poverty and ecosystems services mapping to market access by the poor to fodder and natural resource management to livestock parasite drug resistance. We found that these projects closed gaps between knowledge and action by combining different kinds of knowledge, learning, and boundary spanning approaches; by providing all partners with the same opportunities; and by building the capacity of all partners to innovate and communicate.

Osteoclast-like giant cell tumor arising in the soft tissue of the breast: report of a case.

Extraosseous manifestations of osteoclast-like giant cell tumors (OGCTs) in soft tissue are unusual, especially in the breast. However, multinucleated osteoclast-like giant cells have been described in association with epithelial malignancy, as a variant of breast carcinoma. We report a case of OGCT of the soft tissue of the breast, not associated with epithelial elements. To the best of our knowledge, this is only the second such case reported.

Fumigation of wheat using liquid ethyl formate plus methyl isothiocyanate in 50-tonne farm bins.

Australian Standard White wheat, Triticum aestivum L. (a marketing grade with mixed grain hardness),with a moisture content of 12.5% was fumigated with a new ethyl formate formulation (95% ethyl formate plus 5% methyl isothiocyanate) identified and developed by Commonwealth Scientific and Industrial Research Organization Entomology, Canberra, Australia. Wheat was fumigated with the formulation at a calculated application rate of 80 g/m3 in two 50-tonne sealed metal vertical silos located at Fisherman Islands, Queensland, Australia. Access was gained through the top of the silo where the application of the formulation was completed within a few minutes by pouring it onto the top of the wheat. After 2 h of recirculation, using a 0.5-kW fan, the in-bin concentrations of ethyl formate achieved equilibrium with a concentration variation < 7%. The ethyl formate concentration, in both silos 1 and 2, during the first day's exposure period remained above 10 g/m3. The concentration of ethyl formate by time product achieved was 790 and 650 g h/m3 in silos 1 and 2, respectively. In silo 1, the formulation was sufficient to kill all life stages of mixed age cultures of Sitophilus oryzae (L.), Rhyzopertha dominica (F.), and Tribolium castaneum (Herbst). In silo 2, control was 100% for R. dominica and T. castaneum and 99.4% for S. oryzae. After 5 d fumigation, the silo top-hatch was opened but no forced aeration was initiated. The in-bin concentration of ethyl formate was lower than the Australian experimental threshold limit value of 100 ppm. The ethyl formate and methyl isothiocyanate residues in the grain had declined to below the Australian experimental maximum residue limit of 0.2 and 0.1 mg/kg, respectively. The workspace and environmental levels of ethyl formate and methyl isothiocyanate were less than the detection limit of 0.1 ppm. The treatment with ethyl formate formulation had no affect on the wheat germination and seed color compared with untreated controls.

Imatinib in the management of multiple gastrointestinal stromal tumors associated with a germline KIT K642E mutation.

Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gut and are distinguished by expression of CD117 (c-Kit). Oncogenic mutations in the KIT or PDGFRA gene are detected in approximately 85% of sporadic GISTs. In recent years, examples of familial GIST have been reported in which germline mutations of KIT or PDGFRA result in multiple GISTs, skin disorders, and other abnormalities. The most common germline mutations are in KIT exon 11, mutations in exons 8 and 17 have also been described, and there are 2 families with germline PDGFRA mutations. We present a case in which a germline KIT exon 13 mutation (K642E) was discovered in a patient with multiple GISTs of rectum, small intestine, and esophagus, as well as diffuse hyperplasia of the interstitial cells of Cajal. To our knowledge, this is only the second germline example of this particular mutation. The patient's esophageal tumors were stabilized with imatinib.

Land use change around protected areas: management to balance human needs and ecological function.

Protected areas throughout the world are key for conserving biodiversity, and land use is key for providing food, fiber, and other ecosystem services essential for human sustenance. As land use change isolates protected areas from their surrounding landscapes, the challenge is to identify management opportunities that maintain ecological function while minimizing restrictions on human land use. Building on the case studies in this Invited Feature and on ecological principles, we identify opportunities for regional land management that maintain both ecological function in protected areas and human land use options, including preserving crucial habitats and migration corridors, and reducing dependence of local human populations on protected area resources. Identification of appropriate and effective management opportunities depends on clear definitions of: (1) the biodiversity attributes of concern; (2) landscape connections to delineate particular locations with strong ecological interactions between the protected area and its surrounding landscape; and (3) socioeconomic dynamics that determine current and future use of land resources in and around the protected area.

Tumours of the hand presenting as pathological fractures.

Tumours represent one of the important differential diagnoses that need to be considered while investigating fractures of the small bones of the hand, as this can sometimes be the sole or the first presenting complaint. We conducted a retrospective study of the Scottish Bone Tumour Registry, analysing the records of patients with hand tumours which primarily presented as pathological fractures. The registry held records of 233 patients with tumours involving the bones of the hand, of which 53 (22.7%) had pathological fractures as the first presenting complaint. The average age at presentation was 36.9 yrs. The proximal phalanx was the most common bone involved (50.9%). The distal phalanx rarely developed pathological fractures due to tumours (5.3%). Most of the lesions affected the fifth ray (43.9%) Chondroma was the most common tumour seen (43 patients). Malignant lesions were an infrequent cause of pathological fractures (7 chondrosarcomas and 1 Ewing's sarcoma). Tumours are less commonly seen as a cause of fractures in the hand with most such fractures initially treated as minor injuries with buddy strapping and early mobilisation. A carefully obtained history and study of radiographs is essential to diagnose these lesions.

Pigmented villonodular synovitis of the foot and ankle: Forty years of experience from the Scottish bone tumor registry.

Fourteen cases of pigmented villonodular synovitis (PVNS) of the foot and ankle accrued from the Scottish Bone Tumor Registry are presented with an average follow-up of 4.6 years. This study analyzed the clinical, radiological, and histopathological features and investigated their clinical behavior and the factors influencing recurrence. The mean age of the patients was 26.4 years (range, 8-52 years). There were 8 women and 6 men. The mean delay in presentation was 10.3 months. The anatomical sites were phalanges (n = 2), tarso-metatarsal area (n = 3), and hindfoot (n = 9) (6 extraarticular soft tissue swellings around the ankle, 2 ankle, 1 subtalar joint). Eight (57.1%) cases presented with a painless lump, 5 (35.7%) patients had painful masses, and 1 case had a lump associated with toe deformity. Peri-articular tissue invasion and cortical infiltration were found in one third on plain films. Magnetic resonance imaging findings were suggestive of synovial sarcoma in 2 cases because of extensive low-signal soft tissue hypertrophy and bone erosion. Excision of the lump was performed in 4 cases with a complete recovery. Phalangeal lesions were treated with toe amputation through the metatarsophalangeal joint, and no cases had recurrence. There were 2 recurrences affecting the ankle and the subtalar joint. There was a 14.3% recurrence rate, while complete recovery was achieved in 85.7% cases (12/14). A high index of suspicion for PVNS should be observed for cases presenting with a painless or painful mass in the foot and ankle region. Complete recovery can be achieved in the majority by complete excision. Toe amputation may be considered for foot phalangeal PVNS.

Clinicopathologic and molecular genetic characterization of low-grade fibromyxoid sarcoma, and cloning of a novel FUS/CREB3L1 fusion gene.

Low-grade fibromyxoid sarcoma (LGFMS) is an indolent, late-metastasizing malignant soft-tissue tumor that is often mistaken for either more benign or more malignant tumor types. Cytogenetic analyses have identified a recurrent balanced translocation t(7;16) (q32-34;p11), later shown by molecular genetic approaches to result in a FUS/CREB3L2 fusion gene. Whereas preliminary studies suggest that this gene rearrangement is specific for LGFMS, its incidence in this tumor type and the possible existence of variant fusion genes have not yet been addressed. For this purpose, a series of potential LGFMS were obtained from nine different soft-tissue tumor centres and subjected to molecular analysis as well as careful histopathologic review. Reverse transcriptase-polymerase chain reaction analysis disclosed a FUS/CREB3L2 fusion transcript in 22 of the 23 (96%) cases that remained classified as LGFMS after the histologic re-evaluation and from which RNA of sufficient quality could be extracted, whereas none of the cases that were classified as other tumor types was fusion-positive. In one of the tumors with typical LGFMS appearance, we found that FUS was fused to the CREB3L1 gene instead of CREB3L2. The proteins encoded by these genes both belong to the same basic leucine-zipper family of transcription factors, and display extensive sequence homology in their DNA-binding domains. Thus, it is expected that the novel FUS/CREB3L1 chimera will have a similar impact at the cellular level as the much more common FUS/CREB3L2 fusion protein. Taken together, the results indicate that virtually all LGFMS are characterized by a chimeric FUS/CREB3L2 gene, and that rare cases may display a variant FUS/CREB3L1 fusion.