Ecological, morpho-agronomical, and bromatological assessment of sorghum ecotypes in Northern Morocco.

Sorghum Bicolor is a cereal used for grains as feed and food, mainly cultivated in dry areas. To study the possibilities of increasing its cultivation for feed purposes, ecological, morpho-agronomical, and bromatological characterization of some local ecotypes was conducted as the first steps toward selecting better cultivars. Indeed, twenty-one ecotypes were collected from farms in Northern Morocco in 2018. The edapho-climatic parameters of the collection sites were evaluated. The ecotypes were cultivated in 2019 in an experimental field with a randomized complete block design with three replicates. At the maturity stage, plants were evaluated for agro-morphological parameters, and grains and straw (leaves and stems) were harvested and analyzed. The results indicated significant variations between ecotypes for almost all parameters and an interesting grain yield of 3.5 T/ha with a 176% yield variation. The nutritive value of grains was interesting compared to straw, especially for mean protein contents (10.5% DM) and organic matter digestibility (81.4%). The calculated genetic parameters emphasized the possibility of selecting highly productive and nutritive cultivars. Multivariate analysis clustered the ecotypes into five groups based on agro-morphological, bromatological, and antioxidant activity parameters; the third group was characterized by high grain-yielding ecotypes, and the fifth one by high nutritive ecotypes. The E21 ecotype, belonging to this last group, was a promising selection candidate as it combines both. No significant correlation link between agro-morphological and bromatological traits of grains and geographical distances was discerned. Sorghum bicolor could thus be improved only according to the researched agro-morphological and bromatological traits.

Ecological, morpho-agronomical, and nutritional characteristics of Sulla flexuosa (L.) Medik. ecotypes.

The present work was part of assessing wild genetic plant resources of forage interest in Northern Morocco and aimed to study the agro-morphology and nutritional value of Sulla flexuosa (L.) Medik. (Hedysarum flexuosum L.) ecotypes. The seeds of twenty-one wild S. flexuosa (L.) Medik. ecotypes were collected from 21 sites. The edaphic and climatic characteristics of the collection sites were studied and testified to the remarkable adaptability of S. flexuosa (L.) Medik. These 21 ecotypes were cultivated in three complete randomized blocks design for two consecutive years. Statistical analysis showed substantial variability between the collected ecotypes. Principal component analysis and heatmap analysis allowed to distinguish four groups of ecotypes mainly based on nutritional parameters (fiber content and digestibility), forage production (dry matter yield, number of leaves per plant, and total number of branches), and reproduction (number of inflorescences per plant and, weight of thousand seeds and seeds per plant). Furthermore, the present study pointed out the value of ecotype 1, which was dual purpose with its high productivity, nutritional value, and reproductive parameters. Ecotype 4 was also highlighted as having late flowering but intermediate productivity, which can be used mainly for haymaking as the drying period could coincide with the last rainfall in the region.

Recent advances in the diagnosis, classification and molecular pathogenesis of cutaneous mesenchymal neoplasms.

The diagnosis of cutaneous mesenchymal neoplasms remains challenging, due to a combination of overlapping histological features, the rarity of some diagnoses and often inadequate sampling in superficial biopsies. Here, we describe recent advances in cutaneous mesenchymal neoplasms. We discuss improvements in our understanding of the molecular pathogenesis of non-neural granular cell tumour, epithelioid fibrous histiocytoma, composite and retiform haemangioendothelioma and dermatofibrosarcoma protuberans. We also discuss recently described tumour types, including some discovered via molecular testing: EWSR1::SMAD3-rearranged fibroblastic tumour, clear cell neoplasm with MITF::CREM rearrangement and melanocytoma with CRCT1::TRIM11 rearrangement, and some discovered via traditional histopathology: superficial CD34-positive fibroblastic tumour, plexiform myofibroblastoma and clear cell neoplasm with melanocytic differentiation and ACTIN::MITF translocation.

Characterizing diversity in the tumor-immune microenvironment of distinct subclasses of gastroesophageal adenocarcinomas.

BACKGROUND: Gastroesophageal adenocarcinomas (GEAs) are heterogeneous cancers where immune checkpoint inhibitors have robust efficacy in heavily inflamed microsatellite instability (MSI) or Epstein-Barr virus (EBV)-positive subtypes. Immune checkpoint inhibitor responses are markedly lower in diffuse/genome stable (GS) and chromosomal instable (CIN) GEAs. In contrast to EBV and MSI subtypes, the tumor microenvironment of CIN and GS GEAs have not been fully characterized to date, which limits our ability to improve immunotherapeutic strategies. PATIENTS AND METHODS: Here we aimed to identify tumor-immune cell association across GEA subclasses using data from The Cancer Genome Atlas (N = 453 GEAs) and archival GEA resection specimen (N = 71). The Cancer Genome Atlas RNAseq data were used for computational inferences of immune cell subsets, which were correlated to tumor characteristics within and between subtypes. Archival tissues were used for more spatial immune characterization spanning immunohistochemistry and mRNA expression analyses. RESULTS: Our results confirmed substantial heterogeneity in the tumor microenvironment between distinct subtypes. While MSI-high and EBV+ GEAs harbored most intense T cell infiltrates, the GS group showed enrichment of CD4+ T cells, macrophages and B cells and, in ∼50% of cases, evidence for tertiary lymphoid structures. In contrast, CIN cancers possessed CD8+ T cells predominantly at the invasive margin while tumor-associated macrophages showed tumor infiltrating capacity. Relatively T cell-rich 'hot' CIN GEAs were often from Western patients, while immunological 'cold' CIN GEAs showed enrichment of MYC and cell cycle pathways, including amplification of CCNE1. CONCLUSIONS: These results reveal the diversity of immune phenotypes of GEA. Half of GS gastric cancers have tertiary lymphoid structures and are therefore promising candidates for immunotherapy. The majority of CIN GEAs, however, exhibit T cell exclusion and infiltrating macrophages. Associations of immune-poor CIN GEAs with MYC activity and CCNE1 amplification may enable new studies to determine precise mechanisms of immune evasion, ultimately inspiring new therapeutic modalities.

The effect of concentrate allocation on traffic and milk production of pasture-based cows milked by an automatic milking system.

Increased economic, societal and environmental challenges facing agriculture are leading to a greater focus on effective way to combine grazing and automatic milking systems (AMS). One of the fundamental aspects of robotic milking is cows' traffic to the AMS. Numerous studies have identified feed provided, either as fresh grass or concentrate supplement, as the main incentive for cows to return to the robot. The aim of this study was to determine the effect of concentrate allocation on voluntary cow traffic from pasture to the robot during the grazing period, to highlight the interactions between grazed pasture and concentrate allocation in terms of substitution rate and the subsequent effect on average milk yield and composition. Thus, 29 grazing cows, milked by a mobile robot, were monitored for the grazing period (4 months). They were assigned to two groups: a low concentrate (LC) group (15 cows) and a high concentrate (HC) group (14 cows) receiving 2 and 4 kg concentrate/cow per day, respectively; two allocations per day of fresh pasture were provided at 0700 and 1600 h. The cows had to go through the AMS to receive the fresh pasture allocation. The effect of concentrate level on robot visitation was calculated by summing milkings, refusals and failed milkings/cow per day. The impact on average daily milk yield and composition was also determined. The interaction between lactation number and month was used as an indicator of pasture availability. Concentrate allocation increased significantly robot visitations in HC (3.60±0.07 visitations/cow per day in HC and 3.10±0.07 visitations/cow per day in LC; P<0.001) while milkings/cow per day were similar in both groups (LC: 2.37±0.02/day and HC: 2.39±0.02/day; Ns). The average daily milk yield over the grazing period was enhanced in HC (22.39±0.22 kg/cow per day in HC and 21.33±0.22 kg/cow per day in LC; P<0.001). However the gain in milk due to higher concentrate supply was limited with regards to the amount of provided concentrates. Milking frequency in HC primiparous compared with LC was increased. In the context of this study, considering high concentrate levels as an incentive for robot visitation might be questioned, as it had no impact on milking frequency and limited impact on average milk yield and composition. By contrast, increased concentrate supply could be targeted specifically to primiparous cows.

Comparison of milk fatty acid profiles measured on Kouri cows near Lake Chad and on dairy cattle as reported by meta-analytical data.

Kouri (Bos taurus) is a breed aboriginal from Lake Chad and threatened with extinction. This study aimed to compare milk fatty acid profiles measured on Kouri cows and on high-yielding dairy cattle in Europe and elsewhere as reported by meta-analytical data (22 experimentations). Milk samples were collected from 14 Kouri dairy cows in dry season (March to June) and fatty acids (FA) were determined by gas chromatography. Overall, 32 FA have been identified. Kouri showed lower values (P < 0.001) in the sum of saturated FA (SFA, -10.9 pts), cis-9, cis-12 18:2 (-1.00 pt) (P < 0.01, higher values (P < 0.001) in the sum of monounsaturated FA (MUFA, +15.3 pts), C18:0) (+3.5 pts), cis-9, trans-11 C18:2-CLA (+1.00 pts), trans-11 18:1 (+1.4 pts) and (P < 0.01) in cis-9, C18:1 (+3.00 pts) acids. The differences between the milk FA profile of the Kouri cows and that obtained from meta-analytical data could be the possible consequence of the use of particular lake pastures by Kouri cows.

S-100 protein expressing spindle cells in spindle cell lipoma: a diagnostic pitfall.

Spindle cell lipoma represents a distinct clinicopathological entity and is related to cellular angiofibroma and mammary-type myofibroblastoma. Spindle cell lipomas are composed of mature lipogenic cells and a variable number of CD34-positive spindle cells that show loss of retinoblastoma protein expression. Spindle cell lipomas occasionally express S-100 protein. We studied one case of purely dermal spindle cell lipoma and four cases of classical subcutaneous spindle cell lipoma arising in one female and four male patients (age ranged from 55 to 69 years). The neoplasms arose on the nose, the chin, the neck, the forehead and retroauricular, and all lesions had been marginally or incompletely excised. The studied cases showed classical histological and immunohistochemical features of spindle cell lipoma and, in addition, strong expression of S-100 protein by spindle-shaped tumour cells. S-100-expression in spindle cell lipoma may cause problems in the differential diagnosis with neural and melanocytic neoplasms and emphasizes the plasticity of the spindle cells in spindle cell lipoma.

Assessment of metastatic risk of gastric GIST based on treatment-naïve CT features.

OBJECTIVE: To study whether the CT features of treatment-naïve gastric GIST may be used to assess metastatic risk. METHODS: In this IRB approved retrospective study, with informed consent waived, contrast enhanced CT images of 143 patients with pathologically confirmed treatment-naïve gastric GIST (74 men, 69 women; mean age 61 years, SD ± 14) were reviewed in consensus by two oncoradiologists blinded to clinicopathologic features and clinical outcome and morphologic features were recorded. The metastatic spread was recorded using available imaging studies and electronic medical records (median follow up 40 months, interquartile range, IQR, 21-61). The association of maximum size in any plane (≤10 cm or >10 cm), outline (smooth or irregular/lobulated), cystic areas (≤50% or >50%), exophytic component (≤50% or >50%), and enhancing solid component (present or absent) with metastatic disease were analyzed using univariate (Fisher's exact test) and multivariate (logistic regression) analysis. RESULTS: Metastatic disease developed in 42 (29%) patients (28 at presentation, 14 during follow-up); 23 (16%) patients died. On multivariate analysis, tumor size >10 cm (p = 0.0001, OR 9.9), irregular/lobulated outline (p = 0.001, OR 5.6) and presence of a enhancing solid component (p < 0.0001, OR 9.1) were independent predictors of metastatic disease. On subgroup analysis, an irregular/lobulated outline and an enhancing solid component were more frequently associated with metastases in tumors ≤5 cm and >5-≤10 cm (p < 0.05). CONCLUSION: CT morphologic features can be used to assess the metastatic risk of treatment-naïve gastric GIST. Risk assessment based on pretreatment CT is especially useful for patients receiving neoadjuvant tyrosine kinase inhibitors and those with tumors <5 cm in size.

MDCT and clinicopathological features of small bowel gastrointestinal stromal tumours in 102 patients: a single institute experience.

OBJECTIVE: Small bowel (SB) is the second most common site of gastrointestinal stromal tumours (GISTs). We evaluated clinical presentation, pathology, imaging features and metastatic pattern of SB GIST. METHODS: Imaging and clinicopathological data of 102 patients with jejunal/ileal GIST treated at Dana-Farber Cancer Institute and Brigham and Women's Hospital (Boston, MA) between 2002 and 2013 were evaluated. Imaging of treatment-naive primary tumour (41 patients) and follow-up imaging in all patients was reviewed. RESULTS: 90/102 patients were symptomatic at presentation, abdominal pain and lower gastrointestinal blood loss being the most common symptoms. On pathology, 21 GISTs were low risk, 17 were intermediate and 64 were high risk. The mean tumour size was 8.5 cm. On baseline CT (n = 41), tumours were predominantly well circumscribed, exophytic and smooth/mildly lobulated in contour. Of 41 tumours, 16 (39%) were homogeneous, whereas 25 (61%) were heterogeneous. Of the 41 tumours, cystic/necrotic areas (Hounsfield units < 20) were seen in 16 (39%) and calcifications in 9 (22%). CT demonstrated complications in 13/41 (32%) patients in the form of tumour-bowel fistula (TBF) (7/41), bowel obstruction (4/41) and intraperitoneal rupture (2/41). Amongst 102 total patients, metastases developed in 51 (50%) patients (27 at presentation), predominantly involving peritoneum (40/102) and liver (32/102). 7/8 (87%) patients having intraperitoneal rupture at presentation developed metastases. Metastases elsewhere were always associated with hepatic/peritoneal metastases. At last follow-up, 28 patients were deceased (median survival, 65 months). CONCLUSION: SB GISTs were predominantly large, well-circumscribed, exophytic tumours with or without cystic/necrotic areas. Complications such as TBF, bowel obstruction and intraperitoneal perforation were visualized at presentation, with patients with perforation demonstrating a high risk of metastatic disease. Exophytic eccentric bowel wall involvement and lack of associated adenopathy are useful indicators to help differentiate GISTs from other SB neoplasms. ADVANCES IN KNOWLEDGE: SB GISTs are predominantly large, well-circumscribed, exophytic tumours, and may present with complications. They often are symptomatic at presentation, are high risk on pathology and metastasize to the peritoneum more commonly than the liver.

Is there a difference in post-transplant lymphoproliferative disorder in adults after solid organ and haematologic stem cell transplantation? Experience in 41 patients.

OBJECTIVE: To determine if there is a difference in post-transplant lymphoproliferative disorder (PTLD) in adults after solid organ transplantation (SOT) and haematologic stem cell transplantation (HST). METHODS: In this institutional review board-approved Health Insurance Portability and Accountability Act-compliant study, we reviewed clinical data and imaging at the time of diagnosis in 41 patients (26 SOT and 15 HST) (31 males and 10 females; mean age 51 years) with histopathology-confirmed PTLD seen at our institution from 2004 through 2013. Statistical analysis was performed to assess difference in distribution and survival between SOT and HST cohorts. RESULTS: SOT: 17 lung/cardiac, 8 renal and 1 liver transplant recipients. HST: 13 leukaemia/lymphoma and 2 patients with aplastic anaemia. Median time to diagnosis: SOT 3.0 years; HST 6 months (Fisher's exact test; p = 0.0011). There was no statistically significant difference in distribution of PTLD after SOT and HST with nodes (15/26; 8/15), lung (10/26; 5/15) and bowel (6/26; 4/15) being the most common sites. Hepatic (3/26) and neurologic (2/26) involvement occurred in only SOT cohort while splenic PTLD (5/15) occurred more often in HST cohort. Death occurred earlier in HST (9/15; 2 weeks) than SOT cohort (12/26; 11 months) (Wilcoxon test; p = 0.0188). CONCLUSION: PTLD did not differ significantly in distribution between SOT and HST cohorts. PTLD after HST occurred early and had shorter survival. ADVANCES IN KNOWLEDGE: The most common sites of PTLD were the nodes, lung and bowel. Distribution of PTLD does not differ significantly between patients with SOT and HST. PTLD after HST occurs early and has poor survival compared with PTLD after SOT.

Metastasis in dedifferentiated liposarcoma: Predictors and outcome in 148 patients.

OBJECTIVE: To describe the pattern of dedifferentiated liposarcoma (DDLPS) metastases and to analyze their predictors and outcome. MATERIALS AND METHODS: In this retrospective study, we reviewed the imaging and clinical records of all consenting patients with histopathology-confirmed DDLPS seen from 2000 through 2012. The predictive value of clinical and histopathologic parameters for metastasis later in the disease course was analyzed using univariate and multivariate analyses. Survival of patients with and without metastasis was compared using Log-rank test. RESULTS: Records of 148 patients (57 women, 91 men; mean age 59 years, range 30-87 years) were reviewed. Distant metastases were observed in 44/148 patients (29.7%), 9/44 (20.5%) at presentation and 35/44 (79.5%) developing them later at a median interval of 8 months (IQR = 0.80-26 months). Median duration of follow-up was 38 months (IQR = 18-74 months) with 77/148 patients (31 with metastases) deceased at the time of analysis. Median survival was 28 months (IQR = 10-56 months) for patients with metastases and 38 months (IQR, 17-65 months) for patients without metastases (p = 0.0123, Log-Rank test; Hazard ratio 1.79 [95% confidence interval 1.11-2.84]). Lung was the most common site of metastases (33 patients, 22.3%). On univariate analysis, grade and local recurrence were associated with subsequent risk of metastasis where as age, tumor size, site, de novo dedifferentiation, number of previous surgical resections, margin positivity and chemoradiation were not. On multivariate analysis, high tumor grade (p-value = 0.0005, OR 5.05; 95% CI 2.01-13.48) and local recurrence (p-value = 0.0025, OR 4.46; 95% CI 1.67-13.40) predicted metastasis. CONCLUSION: Lung was most frequent site of DDLPS metastases. Risk of developing metastatic disease was statistically associated with tumor grade and local recurrence. Metastatic disease was associated with decreased survival.

Imaging features of primary and metastatic extremity synovial sarcoma: a single institute experience of 78 patients.

OBJECTIVE: To study the appearance of primary and metastatic extremity synovial sarcoma (SS) on cross-sectional imaging. METHODS: In this institutional review board-approved, Health Insurance Portability and Accountability Act-compliant retrospective study, the imaging features of 78 patients (42 males and 36 females; mean age, 40 years) with primary and metastatic extremity SS on MRI and multidetector CT were reviewed, with baseline MRI of the primary available in 31 patients. RESULTS: Primary SSs were predominantly well-circumscribed (27/31) and heterogeneously enhancing solid (18/31) or solid-cystic (13/31) tumours. Imaging features visualized included the presence of perilesional oedema (14/31), interfascial (15/31) and intercompartmental extension (7/31), triple sign (11/31), intratumoral haemorrhage (10/31), calcification (6/31), bowl of grapes appearance (5/31) and bone involvement (3/31). Smaller T1 stage tumours (8/31) appeared as heterogeneously enhancing lesions, with some lesions demonstrating interfascial and intercompartmental extension and perilesional oedema. Recurrent/metastatic disease developed in 49/78 (63%) patients. Of these, 20/78 (26%) had metastasis at presentation, while the remaining developed metastatic disease at a median interval of 27 months (range, 3-161 months). Pleuropulmonary metastases (46/78) were the most common sites, with most of the metastases being pleural based. On univariate analysis, larger tumour size, the presence of perilesional oedema, intercompartmental extension, the presence of intralesional haemorrhage and bowl of grapes appearance on MRI were associated with a significantly higher incidence of metastatic disease. CONCLUSION: Certain imaging features of primary SS predict the risk of development of metastatic disease. Imaging features of T1 stage tumours included heterogeneous enhancement, interfascial extension and perilesional oedema. Pleural-based metastases are commonly seen in SSs. ADVANCES IN KNOWLEDGE: Imaging features of primary SS correlate with metastatic disease. Pleural-based metastases are often present in SSs.

Effect of bovine colostrum intake on growth, reproductive parameters and survival in red kids.

The aim of this study is to evaluate the efficacy of frozen Azawak colostrum supplementation on body weight (BW), average daily gain (ADG), reproductive parameters (mean age at first parturition, fertility, fecundity, prolificacy) and mortality rate among red kids. The study was conducted at the goat farm secondary centre of Maradi in Niger from September 2010 to September 2011. The control animals (n = 20) were left with their mother, while the treatment animals (n = 20) received in addition 50 ml/animal/day of bovine colostrum at birth and 15 ml/animal/day from d2 to d15. Weight was measured weekly from birth to d365. Mortalities were also recorded over the same period. For reproductive parameters, observations began at weaning (d197). Growth rate was higher (p < 0.001) in supplemented animal, and the treatment effects on ADG were observed up to 150 day after the end of supplementation. A similar long-lasting trend was also observed in relation to the mortality rate (25% for ColG vs. 55% for ConG; p = 0.05). The age at first kidding tended to be lower in the treated group (13.8 ± 0.7 vs. 14.1 ± 0.8 month; p < 0.1). In conclusion, mild bovine colostrum supplementation induces a long-lasting positive impact on growth rate and to a lower extent on reproduction parameters and survival rate.

MDCT features of succinate dehydrogenase (SDH)-deficient gastrointestinal stromal tumours.

OBJECTIVE: To describe the multidetector CT (MDCT) features and metastatic pattern of succinate dehydrogenase (SDH)-deficient gastrointestinal stromal tumours (GISTs). METHODS: In this institutional review board-approved, Health Insurance Portability and Accountability Act-compliant study, we retrospectively identified 34 patients (20 females; mean age, 34 years; range, 12-59 years) with histopathology-confirmed SDH-deficient GIST, who were seen at our institution from 1999 through 2012. MDCT of primary tumour in 8 patients and follow-up imaging in all 34 patients over median follow-up of 106 months [interquartile range (IQR), 52-175 months] were reviewed by two radiologists in consensus. Clinical information was extracted from electronic medical records. RESULTS: Primary tumour in all 34 patients was located in the stomach. Mean tumour size (n = 8) was 9.6 cm (range, 8-14 cm). Primary tumours were lobulated, variable in growth pattern, hypo- (1/8) to isodense (7/8) and similar in enhancement to the skeletal muscle. Two were multifocal, four of eight had necrosis and one of eight had haemorrhage. Tumour rupture with haemoperitoneum and tumour-bowel fistula was noted in one patient each. During follow-up, 12/34 patients developed tumour in surgical bed, and 28/34 patients developed metastases. Most common sites of metastases were the liver (24/34), peritoneum (20/34) and lymph nodes (18/34). Carney triad and Carney-Stratakis syndrome were noted in 5/34 and 1/34 patients, respectively. At the time of writing, six patients had deceased at a median interval of 109 months (IQR, 54-126 months). CONCLUSION: SDH-deficient GISTs occur in young patients, commonly arise in stomach, can be multifocal and may be associated with Carney triad or Carney-Stratakis syndrome. They frequently metastasize to lymph nodes in addition to the liver and peritoneum and are associated with indolent course despite metastatic spread. ADVANCES IN KNOWLEDGE: The presence of features unusual for conventional GIST on imaging should alert the radiologist for the possibility of SDH-deficient GIST, especially, because SDH-deficient GISTs are resistant to imatinib. Young age at diagnosis, prolonged survival, association with Carney triad and Carney-Stratakis syndrome and occurrence of concurrent renal cell carcinoma and thyroid malignancies necessitates long-term follow-up of patients with SDH-deficient GISTs.

Exploring the association of succinate dehydrogenase complex mutations with lymphoid malignancies.

The succinate dehydrogenase (SDH) complex exerts a fundamental role in mitochondrial cellular respiration and mutations in its encoding genes (SDHA, SDHB, SDHC, SDHD, collectively referred to as SDHx) lead to a number of inherited endocrine cancer predisposition syndromes, including familial paraganglioma/pheochromocytoma. Recent studies suggest a possible role for the SDH complex and other mitochondrial enzymes in the pathogenesis of hematological malignancy. Our aim was to search and identify pedigrees of patients affected by germline SHDx mutations treated at our institution for endocrine and other tumors, and seek to identify cases of hematological malignancy. We also analyzed cancer genome databases for reported cases of SDHx mutations outside of endocrine neoplasms. We report of two unrelated pedigrees carrying SDHx mutations with members affected by lymphomas. Sequencing data revealed one case of chronic lymphocytic leukemia with a SDHB mutation. This novel set of observations demonstrates the need for collaborative databases of patients with endocrine cancers with SDHx mutations, and the investigation of their role in hematological (lymphoid) malignancy.

Multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma: experience in 26 patients.

OBJECTIVE: To describe the multimodality imaging features, metastatic pattern and clinical outcome in adult extraskeletal Ewing sarcoma (EES). METHODS: In this institutional review board-approved, health insurance portability and accountability act-compliant retrospective study, we included 26 patients (17 females and 9 males; mean age, 36 years; range, 18-85 years) with pathologically confirmed EES seen at our institute between 1999 and 2011, who had imaging of primary tumour. Imaging of primary tumour in all 26 patients and follow-up imaging in 23 was reviewed by two radiologists in consensus. Clinical data were extracted from electronic medical records. RESULTS: The most common primary sites were the torso (n = 13), extremities (n = 10) and head and neck (HN) region (n = 3). The mean tumour size was 9 cm (range, 3-22 cm); tumours of the torso were larger than those of other areas (p > 0.05). Compared with the skeletal muscle, tumours were isodense on CT (21/21), hypointense (n = 5) to isointense (n = 14) on T1 weighted image, hyperintense on T2 weighted image (19/19) and were fluorine-18 fludeoxyglucose ((18)F-FDG)-avid [10/10; mean maximum standardized uptake value of 7 (range, 3-11)]. Necrosis (15/26), haemorrhage (5/26) and adjacent organ invasion (14/26) were present without calcification. Median follow-up was 16 months. 5 patients had local recurrence (torso, 3; extremity, 1; and HN, 1). Metastases developed in 11 patients (torso, 7; extremities, 3; and HN, 1; p > 0.05); 8 at presentation, most commonly to lung (9/11), peritoneum (4/11), muscles (4/11) and lymph nodes (4/11). Nine patients (torso, 7; extremity, 1; and HN, 1) died (10 months median survival) (p > 0.05). CONCLUSION: Adult EESs are large tumours, which frequently invade adjacent organs and metastasize to the lung. EESs of the torso are larger, have more frequent metastases and poorer outcomes. ADVANCES IN KNOWLEDGE: Adult EESs of the torso have poor outcomes compared with other EESs.

Imaging features of primary and metastatic alveolar soft part sarcoma: single institute experience in 25 patients.

OBJECTIVE: To describe imaging features of primary and metastatic alveolar soft part sarcoma (ASPS). METHODS: In this institutional review board-approved and Health Insurance Portability and Accountability Act-compliant retrospective study, 25 patients (14 males; mean age, 25 years; range, 18-40 years) with pathologically proven ASPS seen at our institute between 1995 and 2013 were included. Imaging of primary tumours in 5 patients and follow-up imaging in 25 patients were reviewed by 2 radiologists in consensus. Clinical information was obtained from electronic medical records. RESULTS: The most common sites for the primary tumour were extremities (17/25, 68%) and torso (6/25, 24%). Primary tumours (n = 5) were well circumscribed, compared with skeletal muscle, were isodense on CT, hyperintense on T1 and T2 weighted images with intense post-contrast enhancement, prominent feeders on CT and flow voids on MRI. Metastases developed in 23/25 (92%) patients, 18 at presentation. The most common sites of metastases were the lungs (100%), lymph nodes (74%), bones (57%) and brain (43%). Visceral and nodal metastases were hypervascular. At the time of reporting the results, 15 patients have died, 6 are alive and 4 were lost to follow-up. Median survival was 74 months for those without brain metastases (n = 8) and 60 months for those with brain metastases (n = 7). Median survival was shorter for patients with metastases at presentation. CONCLUSION: ASPS most commonly involves the lower extremities of young adults, is hypervascular on imaging, often metastasizes at presentation, frequently to lung, nodes, bones and brain, and has an indolent course despite metastases. Brain metastases and high tumour burden (number of metastatic sites) at presentation decreased survival in our study. ADVANCES IN KNOWLEDGE: ASPS has an unusual pattern of metastases to the brain and nodes in addition to lung and bones. It has an indolent course despite metastases.

Refined diagnostic criteria and classification of mast cell leukemia (MCL) and myelomastocytic leukemia (MML): a consensus proposal.

Mast cell leukemia (MCL), the leukemic manifestation of systemic mastocytosis (SM), is characterized by leukemic expansion of immature mast cells (MCs) in the bone marrow (BM) and other internal organs; and a poor prognosis. In a subset of patients, circulating MCs are detectable. A major differential diagnosis to MCL is myelomastocytic leukemia (MML). Although criteria for both MCL and MML have been published, several questions remain concerning terminologies and subvariants. To discuss open issues, the EU/US-consensus group and the European Competence Network on Mastocytosis (ECNM) launched a series of meetings and workshops in 2011-2013. Resulting discussions and outcomes are provided in this article. The group recommends that MML be recognized as a distinct condition defined by mastocytic differentiation in advanced myeloid neoplasms without evidence of SM. The group also proposes that MCL be divided into acute MCL and chronic MCL, based on the presence or absence of C-Findings. In addition, a primary (de novo) form of MCL should be separated from secondary MCL that typically develops in the presence of a known antecedent MC neoplasm, usually aggressive SM (ASM) or MC sarcoma. For MCL, an imminent prephase is also proposed. This prephase represents ASM with rapid progression and 5%-19% MCs in BM smears, which is generally accepted to be of prognostic significance. We recommend that this condition be termed ASM in transformation to MCL (ASM-t). The refined classification of MCL fits within and extends the current WHO classification; and should improve prognostication and patient selection in practice as well as in clinical trials.

MDCT of primary, locally recurrent, and metastatic duodenal gastrointestinal stromal tumours (GISTs): a single institution study of 25 patients with review of literature.

AIM: To describe the multidetector computed tomography (MDCT) features of primary, locally recurrent, and metastatic duodenal gastrointestinal stromal tumours (GISTs). MATERIALS AND METHODS: In this institutional review board-approved, Health Insurance Portability and Accountability Act of 1996 (HIPAA)-compliant, retrospective study, 25 patients [13 men, 12 women; mean age 56 years (34-74 years)] with histopathologically confirmed duodenal GISTs seen at Dana Farber Cancer Institute and Brigham and Women's Hospital from December 1999 to October 2009 were identified. The MDCT of primary tumours in six patients and follow-up imaging in all the 25 patients was reviewed by two radiologists in consensus. Electronic medical records were reviewed to document the clinical characteristics and management. RESULTS: The mean size of the primary tumour was 3.7 cm (range 2.5-5.6 cm). Three of six primary tumours were in the second and third portions of the duodenum, one in the third portion, one in the third and fourth portions, and one in the fourth portion. Three of six of the tumours were exophytic, two were both exophytic and intraluminal, and one was intramural. The tumours were well-circumscribed, round or oval masses, with few lobulations, and were either homogeneously hyper-enhancing or heterogeneously isodense at MDCT. None of the tumours had necrosis, haemorrhage, calcification, or loco regional lymphadenopathy on imaging. Sixteen of 25 (64%) patients developed metastatic disease, the most common sites being liver (14/16; 87.5%) and peritoneum (5/16; 31%). CONCLUSION: Duodenal GISTs are well-circumscribed, round or oval masses, and occur in the second through fourth portions of the duodenum, without lymphadenopathy or duodenal obstruction. Duodenal GISTS metastasize frequently to the liver and peritoneum.

Resistance to treatment in gastrointestinal stromal tumours: what radiologists should know.

Gastrointestinal stromal tumour resistance to treatment with imatinib occurs due to pre-existing or acquired mutations. Computed tomography and positron-emission tomography play an essential role in prompt recognition of resistance to treatment. Primary resistance to treatment, which is encountered in the first 6 months of treatment, is associated with specific mutations. Imaging of these tumours shows no anatomical or metabolic response to treatment. Secondary resistance to treatment, which develops after an initial response, is associated with a variety of mutations acquired after the start of treatment. Imaging findings of secondary resistance are of disease progression.