Clinical and imaging features in adult patients with bone marrow haemophagocytosis with and without haemophagocytic lymphohistiocytosis: a single-institution experience.

AIM: To evaluate clinical, laboratory, imaging findings, and outcomes of adult patients with bone marrow haemophagocytosis (BMH) who meet the diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH) with those who do not meet the criteria. MATERIALS AND METHODS: A pathology database search was performed from 2009 to 2019 to identify adult patients with BMH. Electronic medical records of 41 patients were reviewed to distinguish those who fulfil the HLH-2004 diagnostic guidelines, which identified 22 patients (11 men; mean age, 53.5 years) who met the criteria (HLH+) and 19 patients (13 men; mean age, 54.7 years) who did not meet the criteria (HLH-). Multi-modality imaging was reviewed to record imaging features. Clinical, laboratory, imaging findings, and outcomes were compared between the two groups using Fisher's exact test and Wilcoxon test. RESULTS: Malignancy (non-Hodgkin's lymphoma) was the major trigger for both groups. 86% of HLH+ and 31% of HLH- patients presented with fever. Compared to the HLH- group, the HLH+ group exhibited higher serum ferritin, triglycerides, and lower fibrinogen levels (p<0.05). Alveolar opacities and hepatosplenomegaly were the most common imaging findings identified in both groups. Median overall survival of HLH+ and HLH- were 123.5 (interquartile range [IQR]: 40.7-681.7 days) and 189 days (IQR: 52-1680 days), respectively. Distribution of imaging features and overall survival did not differ between the groups. CONCLUSION: Malignancy is the major trigger for BMH in both HLH+ and HLH- groups. HLH+ and HLH- groups have similar imaging manifestations or clinical outcomes. Therefore, presence of BMH alone is correlated with high morbidity and mortality.

Head-to-head comparison of prostate MRI using an endorectal coil versus a non-endorectal coil: meta-analysis of diagnostic performance in staging T3 prostate cancer.

AIM: To compare the diagnostic performance of prostate magnetic resonance imaging (MRI) with an endorectal coil (ERC) to performance without an ERC using either body-array (BAC) or pelvic phased-array coil (PAC) in staging T3 prostate cancer. MATERIALS AND METHODS: An electronic search of the PUBMED and EMBASE databases was performed until 10 October 2018 to identify studies performing a head-to-head comparison of prostate MRI using a 1.5 or 3 T magnet with an ERC and with a BAC/PAC for staging T3 prostate cancer. Pooled sensitivity and specificity of all studies were plotted in a hierarchical summary receiver operating characteristic plot. The diagnostic performance of the two techniques in staging T3 disease was evaluated using bivariate random-effects meta-analysis. RESULTS: Eight studies comparing head-to-head prostate MRI with an ERC and with a BAC/PAC were identified of which six studies compared the diagnostic performance. The pooled sensitivity and specificity of MRI with an ERC for detecting T3a, T3b and T3a+b was 53% and 95%; 52% and 92%; 72% and 65% respectively. For MRI with a BAC/PAC these were 34%, and 95%; 45% and 94%; 70% and 66%. There was no statistical difference between an ERC and a BAC/PAC in terms of sensitivity (p=0.41) and specificity (p=0.63) for T3a. The area under the receiver operating characteristic (AUROC) curve for T3a, T3b and T3a+b was 0.830, 0.901, 0.741 for an ERC and 0.790, 0.645, 0.711 for BAC, respectively. CONCLUSION: There is no significant difference in the diagnostic performance of MRI of prostate with an ERC and with a BAC/PAC in staging T3 prostate cancer.

Imaging surveillance of gastrointestinal stromal tumour: current recommendation by National Comprehensive Cancer Network and European Society of Medical Oncology-European Reference Network for rare adult solid cancers.

Imaging plays an active role in the surveillance of gastrointestinal stromal tumours (GISTs). Risk stratification schemes, based on size, mitotic count, and anatomical site of origin of the GIST, help in planning preoperative and postoperative imaging strategies especially in determining the frequency and duration of surveillance; however, there is no clear consensus on the optimal imaging strategies in patients with GISTs who are completely cured by surgery and patients who are at risk of recurrence. In addition, current surveillance protocols depend on the resectability of the primary tumour and presence of metastatic disease. The objective of this article is to provide a comprehensive review of the role of the different imaging methods for surveillance of GISTs, focusing on the guidelines recommended by National Comprehensive Cancer Network and European Society of Medical Oncology - European Network for Rare adult solid Cancers, and to propose practical guidelines for surveillance of GISTs for various risk categories.

Imaging of hepatic toxicity of systemic therapy in a tertiary cancer centre: chemotherapy, haematopoietic stem cell transplantation, molecular targeted therapies, and immune checkpoint inhibitors.

The purpose of this review is to familiarise radiologists with the spectrum of hepatic toxicity seen in the oncology setting, in view of the different systemic therapies used in cancer patients. Drug-induced liver injury can manifest in various forms, and anti-neoplastic agents are associated with different types of hepatotoxicity. Although chemotherapy-induced liver injury can present as hepatitis, steatosis, sinusoidal obstruction syndrome, and chronic parenchymal damages, molecular targeted therapy-associated liver toxicity ranges from mild liver function test elevation to fulminant life-threatening acute liver failure. The recent arrival of immune checkpoint inhibitors in oncology has introduced a new range of immune-related adverse events, with differing mechanisms of liver toxicity and varied imaging presentation of liver injury. High-dose chemotherapy regimens for haematopoietic stem cell transplantation are associated with sinusoidal obstruction syndrome. Management of hepatic toxicity depends on the clinical scenario, the drug in use, and the severity of the findings. In this article, we will (1) present the most common types of oncological drugs associated with hepatic toxicity and associated liver injuries; (2) illustrate imaging findings of hepatic toxicities and the possible differential diagnosis; and (3) provide a guide for management of these conditions.

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.

Imaging yield from 133 consecutive patients with prostate cancer and low trigger PSA from a single institution.

AIM: To investigate the yield of imaging in patients with relapsed prostate cancer (PC) with a low trigger prostate-specific antigen (PSA). MATERIALS AND METHODS: This institutional review board (IRB)-approved, Health Insurance Portability and Accountability Act (HIPAA)-compliant retrospective study included all 133 patients (mean age 68 years; range 45-88; median 69 months since original diagnosis; interquartile range [IQR]: 32-139) with hormone-sensitive PC (HSPC, n=28) or castration-resistant PC (CRPC, n=105) and trigger PSA <4 ng/ml, who underwent same-day bone scintigraphy and computed tomography (CT; total 224 time points) at Dana-Farber Cancer Institute from January to December 2013. Clinical and pathological data were obtained by manual review of the electronic medical records. All the included bone scintigraphs and CT images were reviewed by a fellowship-trained oncoradiologist to record the metastatic pattern and any clinically significant non-metastatic findings. RESULTS: Ninety-four of the 133 (71%) patients had metastatic disease (18/28 [64%] with HSPC, 76/105 [72%] with CRPC). Forty-one of the 133 (31%) patients developed new metastatic disease and 23/133 (17%) developed new clinically significant non-metastatic findings. The incidence of osseous, nodal, and visceral metastases, and clinically significant non-metastatic findings was similar across the HSPC and CRPC groups (p>0.05 for all). Fifty-seven of the 133 (43%) patients had findings seen only at CT, of which 37 had new extra-osseous findings. Only 2/133 (2%) had findings at bone scintigraphy not seen at CT, both in areas not covered on CT. CONCLUSION: Imaging frequently demonstrated new metastatic and non-metastatic findings in patients with a low trigger PSA. CT is valuable in these patients because extra-osseous findings not visible at bone scintigraphy are frequently seen.

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.

Multimodality imaging and clinical features in Castleman disease: single institute experience in 30 patients.

OBJECTIVE: To analyse imaging features of subtypes of Castleman disease (CD), emphasizing differentiating features from lymphoma. METHODS: Institutional review board-approved, Health Insurance Portability and Accountability Act compliant, retrospective study examined 30 patients with CD. 30 patients (females, 20; mean age, 46 years; range, 22-87 years) with histopathologically confirmed CD and pre-treatment imaging formed the analytic cohort. Imaging at presentation in all patients [CT, 30; positron emission tomography (PET)/CT, 5; MR, 4; ultrasound, 3] and subsequent imaging in three cases that developed lymphoma was reviewed by two radiologists in consensus. RESULTS: Subtypes: hyaline-vascular (n = 18); multicentric not otherwise specified (NOS) (n = 6); human herpesvirus 8 associated (n = 2); mixed unicentric (n = 2); pure plasma-cell variant (n = 1); and unicentric NOS (n = 1). Distribution: unicentric (n = 17); and multicentric (n = 13). Nodal sites-unicentric: 13 thoracic, 3 abdominal and 1 cervical; multicentric: 9 abdominal, 8 thoracic, 6 cervical, 5 inguinal, 4 axillary and 4 supraclavicular. On CT, differentiating features from lymphoma were calcification (n = 8; 26.7%) and heterogeneous enhancement (n = 5; 19.2%). No association between CD subtype, degree or enhancement pattern, or calcification was noted. On PET/CT (n = 5), nodes were typically fluorine-18 fludeoxyglucose avid (n = 4). On ultrasound (n = 3), nodes were hypoechoic, homogeneous with posterior acoustic enhancement. On MR (n = 4), nodes were hypointense (n = 2) to isointense (n = 2) on T1 weighted images and isointense (n = 1) to hyperintense (n = 3) on T2 weighted images. All (n = 4) demonstrated homogeneous enhancement. Three cases developed non-Hodgkin's lymphoma, two of the three had larger spleens, and these cases had effusions/ascites. CONCLUSION: CD can be unicentric or multicentric and involve nodes above and below the diaphragm. Patients with CD can develop lymphoma. ADVANCES IN KNOWLEDGE: Assessing individual risk of developing lymphoma in patients with CD is difficult, although the findings of splenomegaly, pleural effusion and ascites may be suggestive.

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.

Metastatic patterns of breast cancer subtypes: what radiologists should know in the era of personalized cancer medicine.

There is accumulating evidence that molecular phenotyping of breast cancer determines the timing, pattern, and outcome of metastatic disease. The most clinically relevant subtypes are hormonal-positive [oestrogen and progesterone receptor (ER/PR) positive], HER2 expressing, and triple-negative breast cancers (TNBCs). ER/PR-positive breast cancers demonstrate the best prognosis; however, metastases, in particular osseous disease, may develop much later. HER2-expressing breast cancers, although aggressive, have improved outcomes due to the advent of HER2-targeted therapies, with increased risk of central nervous system (CNS) relapses later. Finally, TNBCs present in younger women, BRCA1 mutations carriers, and carry the worst overall prognosis, with high incidence of CNS metastases, especially during the first 5 years of diagnosis. It is important for radiologists to understand the nuances of these breast cancer subtypes to predict metastatic behaviours and guide possible imaging surveillance.

Imaging of uncommon esophageal malignancies.

Malignant esophageal neoplasms other than squamous cell carcinoma and adenocarcinoma are uncommon and include endocrine tumors, lymphoid malignancies, melanoma, malignant stromal tumors, and secondary tumors (metastases). Imaging, though not diagnostic in many cases, helps in selecting the appropriate treatment strategy by determining the anatomic extent of the tumor and locoregional and distant spread. In this article, we provide a comprehensive review of the imaging features of these uncommon esophageal malignancies.

Differences in CT features of peritoneal carcinomatosis, sarcomatosis, and lymphomatosis: retrospective analysis of 122 cases at a tertiary cancer institution.

AIMS: To study the differences in the imaging features of spread from the three cancer cell lines, namely epithelial, sarcomatoid, and lymphoid, resulting in peritoneal carcinomatosis, peritoneal sarcomatosis, and peritoneal lymphomatosis, respectively. MATERIALS AND METHODS: In this institutional review board-approved Health Insurance Portability and Accountability Act (HIPAA)-compliant retrospective study, an electronic radiology database was searched to identify patients with peritoneal tumour spread who underwent CT imaging at Dana-Farber Cancer Institute, a tertiary cancer institution, between January 2011 and December 2012. Out of 1214 patients with possible peritoneal tumour spread on the radiology reports, 122 patients were included with histopathologically confirmed peritoneal disease (50 randomly selected patients with peritoneal carcinomatosis and sarcomatosis each, and all 22 patients with lymphomatosis). Two blinded, fellowship-trained radiologists in consensus reviewed the CT images in random order and recorded the imaging findings of peritoneal tumour spread. The statistical analysis was performed in two steps: the first comparing incidence of various features in each group and the second step was a pairwise analysis between each cohort. RESULTS: Peritoneal carcinomatosis more frequently had ascites, peritoneal thickening, and omental cake (all p ≤ 0.001). Measurable nodules were less common in peritoneal carcinomatosis (p < 0.001), and when present, were ill-defined and had an irregular outline (p ≤ 0.002). Peritoneal sarcomatosis more often had discrete nodules that were well defined and had a smooth outline and less frequently had ascites, peritoneal thickening, omental caking, serosal implants, and lymphadenopathy (all p ≤ 0.005). Peritoneal lymphomatosis frequently involved the omentum and mesentery, and often had associated lymphadenopathy and splenomegaly (all p ≤ 0.002). CONCLUSION: Peritoneal carcinomatosis, sarcomatosis, and lymphomatosis have distinctive patterns on imaging, which can help the radiologists to differentiate between them.

Diagnosis and management of intrahepatic cholangiocarcinoma: a comprehensive update for the radiologist.

There is increasing focus on intrahepatic cholangiocarcinoma (IHCC) due to its rising incidence worldwide and relatively poor prognosis, with the revised TNM classification (2009) introducing a separate staging system for IHCC for the first time. In this article, we comprehensively review the current role of the radiologist in the diagnosis and management of patients with IHCC.

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.

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.

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.

Multidetector-row CT of tumour-bowel fistula: Experience at a tertiary cancer centre.

AIM: To study the clinical and multidetector computed tomography (MDCT) features of tumour-bowel fistula (TBF). MATERIALS AND METHODS: Fifty-one patients (27 women; mean age 57.4 years, range 30-77years) with TBF presenting to our institution between January 2005 and February 2012 were identified retrospectively from the radiology database. MDCT images before, at, and subsequent to diagnosis of TBF were reviewed by three radiologists in consensus; clinical presentation, management, and outcome were documented from electronic medical records. RESULTS: Of 51 patients, small bowel (n = 22) was the most common site with gastrointestinal stromal tumour (GIST) being the most common sarcoma subtype (n = 10). TBF was treatment-associated (TTBF) in 40 patients [78%; 22 of whom had received molecular targeted therapy (MTT)], and spontaneous (STBF) in 11 patients (22%). Thirty-one patients (61%) were symptomatic at the time of TBF detection. TTBF was more often asymptomatic (19/40 versus 1/11; Fisher's exact test p = 0.03). In the TTBF group, 16 had a partial response, seven had stable disease, and 17 had progressive disease. Treatment was discontinued or changed to an alternative regimen in 27/40 patients, and 13/40 patients continued with the same regimen. TBF persisted in 27/33 patients (82%) who underwent CT follow-up. Thirty-one of the 51 patients were deceased at the time of analysis. Time from diagnosis of TBF to death was shorter with STBF (1.8 months) than with TTBF (6.4 months). CONCLUSION: TBF is often associated with MTT and can be seen with treatment response or progression. TTBF is more frequently asymptomatic. TBF is usually managed conservatively by discontinuing treatment, but often persists on CT follow-up.