Harika Tirumani

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.

Metastatic pattern of uterine leiomyosarcoma: retrospective analysis of the predictors and outcome in 113 patients.

Objective To describe metastatic pattern of uterine leiomyosarcomas (ULMS) and correlate it with clinical and histopathologic parameters. Methods We included 113 women (mean age, 53 years; range, 29 to 72 years) with histopathology-confirmed ULMS from 2000 to 2012. Distribution of metastases was noted from imaging by two radiologists in consensus. Predictors of development of metastases were analyzed with univariate and multivariate analysis. Impact of various clinical and histopathologic parameters on survival was compared using Log-rank test and Cox proportional hazard regression model. Results Distant metastases were seen in 81.4% (92/113) of the patients after median interval of 7 months (interquartile range, 1 to 21). Lung was most common site of metastases (74%) followed by peritoneum (41%), bones (33%), and liver (27%). Local tumor recurrence was noted in 57 patients (50%), 51 of whom had distant metastases. Statistically significant correlation was noted between local recurrence and peritoneal metastases (p<0.001) and between lung and other common sites of hematogeneous metastases (p<0.05). Age, serosal involvement, local recurrence, and the International Federation of Gynecology and Obstetrics (FIGO) stage were predictive factors for metastases. At the time of reporting, 65% (74/113) of the patients have died; median survival was 45 months. Stage, local recurrence, and age were poor prognostic factors. Conclusion ULMS metastasizes most frequently to lung, peritoneum, bone, and liver. Local recurrence was associated with peritoneal spread and lung metastases with other sites of hematogeneous metastases. Age, FIGO stage and local recurrence predicted metastatic disease and advanced stage, older age and local recurrence predicted poor outcome.

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.

Esophageal Carcinoma: Current Concepts in the Role of Imaging in Staging and Management

Over the past few decades, the survival of esophageal cancer patients has improved owing to early detection and advances in multimodality treatment strategies. Imaging plays an important role in every step in the management of esophageal cancer, including diagnosis, staging, assessment of treatment response, and post-treatment surveillance. In this article, we provide a comprehensive review of the role of imaging in these various time points of esophageal cancer management.

Small bowel obstruction in the emergency department: MDCT features of common and uncommon causes.

Small bowel obstruction is a common condition encountered by the radiologist in the emergency department. Though intestinal adhesions and obstructed hernias are the most common causes of acute small bowel obstruction, a variety of uncommon and rare conditions can cause acute and subacute bowel obstruction. Imaging plays a key role in the workup of bowel obstruction by identifying the cause, level, and degree of bowel obstruction. In this article, we present a comprehensive review of the multi-detector computed tomography features of common and uncommon causes of acute and subacute small bowel obstruction.

Primary Extremity Liposarcoma: MRI Features, Histopathology, and Clinical Outcomes.

Objective This study aimed to describe magnetic resonance imaging (MRI) features of extremity liposarcoma (LPS) subtypes, correlating with histopathology and clinical outcomes. Methods In this retrospective study, we included 125 patients (80 men, 45 women; mean age, 53 years) with extremity LPS [23 atypical lipomatous tumor (ALT), 9 dedifferentiated (DDLPS), 70 myxoid (MLPS), 23 pleomorphic (PLPS)]. Pretreatment MRI of primary tumors in 56 patients (10 ALT, 4 DDLPS, 28 MLPS, 14 PLPS) was reviewed. Results All subtypes were predominantly T1 isointense relative to skeletal muscle (DDLPS = 3/4, MLPS = 28/28, PLPS = 13/14) and T2 hyperintense (ALT = 10/10, DDLPS = 3/4, MLPS = 28/28, PLPS = 14/14) except for ALT which were T1 hyperintense (8/10). Within MLPS, high grade was associated with unencapsulated margins (P = 0.05) and solid, nodular enhancement (P < 0.0001). Peritumoral edema (P = 0.03) and T2 heterogeneity (P = 0.05) predicted pulmonary (rather than extrapulmonary) metastases in MLPS. Tumor subtype correlated with mortality (P = 0.04). Conclusions The MRI features can help to distinguish between extremity LPS subtypes, and can predict histopathologic grade and metastatic pattern in myxoid LPS.

Incarcerated Morgagni Hernia: An Unusual Cause of Large Bowel Obstruction

A 67-year-old woman presented to the emergency department (ED) with a 2-day history of constipation and abdominal distension and 4 h of epigastric pain. Her history was remarkable for diabetes and hypertension and absence of any surgery. Clinical examination was notable for a distended abdomen with decreased bowel sounds and absence of peritoneal signs. Laboratory investigations revealed leukocytosis and normal liver function test and pancreatic enzymes. A plain abdominal radiograph revealed an elevated right hemidiaphragm and dilated, fecal matter loaded loop of large bowel in the right flank that could be traced up to the midline in the epigastric region, where there was an abrupt cutoff of the colon (Figure 1). Other than a few loops in the left upper quadrant, the rest of the colon and all of the small bowel loops were free of any air in the lumen. Although a diagnosis of large bowel obstruction was suspected, the cause was uncertain. Computed tomography (CT) scan of the abdomen and pelvis with i.v. contrast and without oral contrast was performed. It demonstrated a large retrosternal anterior diaphragmatic hernia containing a dilated loop of transverse colon, as well as omentum with luminal narrowing of the colon at the point of entry and mild soft tissue stranding in the hernia sac, consistent with incarcerated Morgagni hernia (Figure 2). The colon

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.

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.

Basal Cell Carcinoma: A Comprehensive Review for the Radiologist

OBJECTIVE. Basal cell carcinoma (BCC) is the most common malignancy in the United States. The purpose of this article is to provide a comprehensive description of the clinicopathologic features, diagnostic workup, staging, treatment, and follow-up of BCC. CONCLUSION. Radiology plays an important role in the evaluation and staging of locally advanced and metastatic BCC. MRI is the modality of choice for assessing perineural disease and is equivalent or superior to CT for evaluating bony involvement. CT and PET/CT are used to evaluate metastatic disease.