Amyloidosis: Modern Cross-sectional Imaging.

Amyloidosis is a rare diverse condition caused by the pathologic extracellular deposition of abnormal insoluble proteins throughout the body. It may exist as a primary disease or, more commonly, may be secondary to a wide variety of pathologic processes ranging from chronic infection or inflammation to malignancy. Hereditary forms also exist. On the basis of the structure of the protein deposits, more than two dozen subtypes of amyloidosis have been described. A single organ or multiple organ systems may be affected. The radiologic manifestations of amyloidosis are varied and often nonspecific, making amyloidosis a diagnostic challenge for the radiologist. In the chest, the lungs, mediastinum, pleura, and heart may be involved. Lung involvement may manifest as diffuse reticulonodular interstitial thickening, consolidations, or solitary or multiple parenchymal nodules that may calcify, cavitate, and slowly enlarge. Pleural involvement most commonly manifests as pleural effusions. Tracheobronchial involvement may exhibit concentric airway thickening, mural and intraluminal nodules, submucosal calcification, and airway obstruction. Mediastinal and hilar lymph nodes may enlarge and frequently calcify. At cardiac magnetic resonance (MR) imaging, the left ventricular wall is typically thickened, with associated diastolic dysfunction. Delayed contrast material-enhanced cardiac MR imaging typically shows global transmural or subendocardial enhancement. The pathophysiology, classification, treatment, and prognosis of amyloidosis are reviewed, followed by case examples of the appearance of thoracic and cardiac amyloidosis on chest radiographs, computed tomographic (CT) images, and cardiac MR images.

Thyroid cancer in pregnancy: diagnosis, management, and treatment.

The evaluation and management of cancer during pregnancy requires special care to assure the health and safety of both the mother and fetus. The diagnosis and treatment of thyroid cancer in the non-pregnant patient often involves radioactive iodine exposure. However, radioactive iodine is contraindicated in pregnancy and surgical interventions pose risks to both the mother and fetus. Thus, the management of thyroid cancer during pregnancy is a unique clinical challenge. In this review, we discuss the imaging of thyroid nodules during pregnancy, including the role of CT, MRI, and nuclear Imaging, as well as that of Ultrasound and FNA. The staging and prognosis are discussed along with the management, treatment, and surveillance of thyroid cancer in pregnancy. Finally, the risks to the fetus through treatment are examined. Case examples are provided with an emphasis on the appropriate direction of care from a radiologist's perspective.

Delayed cancer diagnosis in the pregnant patient: navigating a complex medical and ethical dilemma.

Prompt diagnosis of cancer in pregnancy is necessary to ensure timely management and improve outcomes. However, there are a several reasons why diagnosis may be delayed in pregnancy. Three major contributors to delayed diagnosis and treatment are patient delay, provider delay, and referral delay. This article aims to (1) increase physician awareness of this problem by providing a detailed review of the main culprits of delayed diagnosis and treatment of cancer in the pregnant patient, (2) discuss the complex ethical issues at hand in these cases, and (3) provide suggestions on how to better address such cases with the goal of improving patient outcomes.

Melanoma in pregnancy.

Melanoma is one of the most common types of cancer diagnosed during pregnancy. Patients with advanced disease require frequent staging examinations (e.g., CT, PET, MRI, ultrasound), which, during pregnancy must be modified from routine protocol to minimize risk to the fetus. We will review the diagnostic and treatment approach to pregnant patients with melanoma, with a discussion and pictorial examples of imaging protocol modifications, and the appearance of metastatic melanoma on radiology exams using modified protocols due to pregnancy.

Diagnostic approach to primary retroperitoneal pathologies: what the radiologist needs to know.

Retroperitoneal soft tissue lesions represent a wide range of disease processes with overlapping imaging findings. Familiarity with the CT and MR characteristics of these conditions is important to guide clinical evaluation. We review the tissue types, characteristic clinical, demographic, and imaging features of retroperitoneal tumors and tumor-like non-neoplastic conditions with CT and MR correlation, including anatomic and imaging clues, and provide a diagnostic approach to aide the radiologist in making a specific diagnosis.

Can a simplified CT response criteria for vascular involvement in pancreatic adenocarcinoma after neoadjuvant therapy predict survival in patients who achieved subsequent R0 resection?

PURPOSE: To investigate if a simplified image based scoring system assessing treatment response after neoadjuvant therapy (NAT) can predict survival in patients with pancreatic ductal adenocarcinoma (PDAC) who achieved subsequent R0 resection. METHODS: Retrospective analysis of 57 PDAC patients (male = 29, 51%) with mean age of 64 at diagnosis (range 42-79) who received NAT and R0 resection. Post-NAT overall, arterial and venous imaging response was characterized as improved, similar, or worse by 2 readers independently followed by consensus review. Kaplan-Meier Analysis was performed to compare overall survival (OS) with post-NAT overall imaging response. A Multivariable Cox proportional hazards analysis was performed to evaluate the association of the following variables with OS: overall, arterial and venous radiology response, clinical staging, postoperative CA19-9, and patient age. RESULTS: At study conclusion, 30/57 patients were deceased (53%), 26/57 (46%) alive, and 1 patient unknown. Post-NAT, 39/57 (68.4%) had overall improved disease and 18/57 (31.6%) had similar disease. The median OS was 55.7 months (95% CI 33.4-not reached, NR) for those with improved disease vs. 53.9 months (95% CI 14.3-NR) with similar disease (p = 0.859) after NAT. Among all clinical parameters, only post-operative CA 19-9 level was associated with OS (p = 0.002) and PFS (p = 0.005), respectively. CONCLUSION: Pancreatic cancer patients who underwent R0 resection showed no difference in survival when comparing those with similar vs improved disease on post-NAT imaging.

Assessing the Impact of Referral on Multidisciplinary Tumor Board Outcomes in Patients With Hepatocellular Carcinoma.

BACKGROUND AND AIMS: Hepatocellular carcinoma (HCC) is a complex and unique cancer. At many tertiary care institutions, optimal treatment is planned at a multidisciplinary liver tumor board (MDLTB). This study examined the impact of referral on MDLTB outcomes for patients with HCC. METHODS: Chart review was performed of incident HCC cases presented over 3 years at an American College of Surgeons accredited MDLTB. Internal cases were defined as patients whose care originated within the tertiary care institution of the MDLTB; external cases were defined as patients who received initial care from outside institutions and referred to the tertiary care MDLTB for consultation. Internal and external cases were compared for differences in HCC diagnosis, treatment, and survival. RESULTS: There were 120 internal cases and 163 external cases. Compared with internal cases, external cases took significantly more time to be diagnosed with HCC (4.5 versus 37.5 days, P < .001) and to be discussed at MDLTB (19 versus 53 days, P < .001). Internal cases were more often diagnosed by imaging studies (77.5%), and external cases were more often diagnosed by biopsy (43%) or by consensus of experts at MDLTB (26%). CONCLUSION: Patients with HCC, whose care originated within the tertiary care institution of the MDLTB, were less likely to require biopsy and had shorter time from initial suspicion to diagnosis of HCC. The data suggest that referral of external HCC cases to a tertiary center MDLTB at initial suspicion may limit unnecessary diagnostic procedures, possibly resulting in decreased health care costs and reduced uncertainty for patients.

[Surgical treatment options of metastatic tumours in the gastrointestinal tract (stomach, small and large bowel)]. / A tápcsatorna (gyomor, vékonybél, vastagbél) másodlagos daganatainak sebészete.

Metastatic tumours in the gastrointestinal tract are infrequent findings and usually represent advanced disease at the time of their diagnosis. In these cases, radical surgical resection is rarely possible due to dissemination, but palliative surgery can be relatively effective. Operative treatment can be indicated as a life saving measure in patients with gastrointestinal bleeding, perforation or obstruction. Radical resection of metastases may prolong survival, if R0 resection is achieved. In these disease free patients, the surgical treatment should be tailored to the primary tumour and combined with neoadjuvant or adjuvant therapy. 1053 gastrointestinal tumours were treated surgically by the authors between 01.01.2000. and 31.12.2005. Of those, secondary tumours were found in 24 cases in the gastrointestinal tract (11 in the stomach, nine in the small bowel and four in the large bowel). The authors summarize their experience and review the relevant literature.

[Rectum carcinomas treated with Kraske procedure in elderly patients]. / Kraske-amputációval kezelt rectumcarcinomák idoskorú betegeken.

Kraske procedure is an option that can be chosen to treat rectal tumours in elderly patients with multiple co-morbidities, in order to minimize complications and stress caused by surgery. In the past six years, 382 rectal resections were performed in our department: 154 abdominoperineal rectum resections, 219 anterior rectum resections and 9 Kraske procedures in high anaesthetic risk patients. Kraske resection was applied for mid- and lower third rectal cancers, providing a short term survival for more than half of these patients. The authors discuss their experience with the Kraske procedure.

Contrast Agents for MR Imaging.

Contrast media are essential to the practice of MR imaging. An increasing variety of agents have been approved for clinical use, specific contrast agents can often be tailored to a specific clinical question. Compared with CT contrast media, MR imaging contrast is well tolerated with an excellent safety record and a low incidence of adverse events. In this article, we review the pharmacology, indications, and the common adverse events of the intravenous and oral MR contrast agents most commonly used in contemporary imaging practice, including gadolinium-based contrast, manganese and iron-based agents and the most common oral contrast agents.

MRI in pelvic inflammatory disease: a pictorial review.

Pelvic inflammatory disease (PID) is an ascending infection of the female genital tract caused by the spread of bacteria from the vagina to the pelvic reproductive organs and occasionally the peritoneum. The most common causative organisms are sexually transmitted. PID is a significant source of morbidity among reproductive age women both as a cause of abdominal pain and as a common cause of infertility. Its clinical presentation is often nonspecific, and the correct diagnosis may first come to light based on the results of imaging studies. MRI is well suited for the evaluation of PID and its complications due to its superior soft tissue contrast and high sensitivity for inflammation. MRI findings in acute PID include cervicitis, endometritis, salpingitis/oophoritis, and inflammation in the pelvic soft tissues. Acute complications include pyosalpinx, tuboovarian abscess, peritonitis, and perihepatitis. Hydrosalpinx, pelvic inclusion cysts and ureteral obstruction may develop as chronic sequela of PID. The pathophysiology, classification, treatment, and prognosis of PID are reviewed, followed by case examples of the appearance of acute and subclinical PID on MR images.

[Leiomyosarcoma of the oesophagus: a case report]. / Nyelocso leiomyosarcomákról, egy operált esetünk kapcsán.

Authors treated a oesophageal sarcoma in a 46 year old female patient in July 1999. The tumor caused retrosternal pain and weight loss but did not disturb swallowing. After resecting the tumor, an intrathoracic oesophago-gastrostomy was performed. On the 18th postoperative day the patient went home without any complaints. Histologically the tumor proved to be a leiomyosarcoma. The patient has had no complaints in the past 6 years. On account of this case report the authors review the literature on this rare disease.

MRI of ovarian torsion: Correlation of imaging features with the presence of perifollicular hemorrhage and ovarian viability.

PURPOSE: The purpose of our study is to test for: (a) correlation between the presence of a perifollicular T2-hypointense rim on MRI with the presence of perifollicular hemorrhage on histology; and (b) correlation between this finding and diminished ovarian viability after intra-operative detorsion. METHODS AND MATERIALS: Our IRB-approved, retrospective study evaluated 780 patients between August 2012 and February 2016 with ovarian torsion as a diagnostic consideration on the emergency department note. Patients were included if they had preoperative MRI and intraoperatively confirmed case of ovarian torsion. MRIs were retrospectively reviewed for presence of perifollicular T2 hypointense rim in the torsed ovary. Two arms of analysis were performed: (a) assessment of perifollicular hemorrhage on histological exam; and (b) assessment of ovarian viability after intra-operative detorsion. Sensitivity, specificity, positive predictive value, and negative predictive value of MRI for predicting ovarian viability in the setting of torsion was performed. κ test assessed level of agreement between readers. RESULTS: 24 patients included in one of the two arms; 20 in viability analysis and 12 in perifollicular hemorrhage analysis (8 in both). The presence of T2-hypointense rim on MRI demonstrated 88.9% sensitivity and 66.7% specificity for the diagnosis of perifollicular hemorrhage on histology, and 91.7% sensitivity and 100% specificity for predicting intraoperative viability. CONCLUSION: The presence of a perifollicular T2 hypointense rim on MRI in the setting of ovarian torsion correlates with perifollicular hemorrhage on histopathologic exam, and may also be a useful predictor of ovarian viability in patients presenting with ovarian torsion.

Long-term outcome of uterine artery embolization of leiomyomata.

OBJECTIVE: To determine the long-term outcome from uterine artery embolization for leiomyomata. METHODS: In a prospective study, 200 consecutive patients treated with uterine embolization were each followed for 5 years. Outcome, including symptom status compared with baseline, reinterventions, menstrual status, and satisfaction were recorded. Summary statistics were used to report baseline characteristics and outcome at each interval. Predictors of subsequent interventions, failure, and satisfaction with treatment were analyzed using logistic regression and Cox proportional hazards models. Failure was defined as subsequent hysterectomy, definitive myomectomy, repeat embolization, or failure of symptom improvement at the patient's final follow-up interval. RESULTS: Of the 200 patients initially treated, 5-year follow-up was completed in 182 (91%), with 18 patients missing. At 5 years after treatment, 73% had continued symptom control, whereas 36 (20%) had failed or recurred. There had been 25 hysterectomies (13.7%), 8 myomectomies (4.4%), and 3 repeat embolizations (1.6%). Long-term failure was more likely in those not improved at 1 year (relative risk [RR] 5.73; 95% confidence interval [CI] 2.32-14.12, P < .001) and in those with baseline leiomyoma volumes greater than the median (RR 2.18; 95% CI 1.05-4.51, P = .036). After adjustment, patients in the first tertile of leiomyoma volume reduction (< or = 30.5%) were 3 times more likely to be dissatisfied with outcome compared with women in the third tertile (> or = 56.3% volume reduction) (RR 3.23; 95% CI 1 07-9.81, P = .037). CONCLUSION: Uterine embolization provides durable symptom relief for most patients, with a 25% chance of failure of symptom control or recurrence over the course of a 5-year follow-up. LEVEL OF EVIDENCE: II-3.

Serum estrone concentration, estrone sulfate/estrone ratio and BMI are associated with human epidermal growth factor receptor 2 and progesterone receptor status in postmenopausal primary breast cancer patients suffering invasive ductal carcinoma.

BACKGROUND: We investigated in postmenopausal women with primary breast cancer prior to surgical intervention whether, serum levels of different steroid hormones and hormonal precursors associated with tumor tissue estrogen receptor (ER), progesterone receptor (PR) and human epidermal growth factor receptor 2 (HER2) status. METHODS: We enrolled 1,042 patients suffering invasive ductal carcinoma undergoing surgical resection in the National Institute of Oncology, Hungary between 2003 and 2011. Serum parameters were measured by RIA/IRMA assays; tumor tissue ER, PR and HER2 status was assessed histologically. Patients were classified according to tumor receptor status. Case-case analysis subjects were categorized into four subgroups based on serum hormone concentrations in ER, PR and HER2 receptor-negative cases, respectively. RESULTS: Serum estrone sulfate and dehydroepiandrosterone sulfate levels correlated with each other and also with serum estrone and estradiol levels. According to case-case study the odds ratios in the highest quartile were 1.517 (p = 0.0305, Ptrend = 0.0394) for androstenedione, 1.495 (p = 0.0317, Ptrend < 0.0105) for estrone and 0.654 (p = 0.0273, Ptrend < 0.0151) for estrone sulfate/estrone ratio in PR+ vs. PR- tumors. Regarding HER2 status (HER2+ vs. HER2-), the odds ratios for estrone, estrone sulfate and estrone sulfate/estrone ratio were 0.530 (p = 0.0234, Ptrend = 0.0595), 2.438 (p = 0.0042, Ptrend < 0.0066) and 3.118 (p = 0.0001, Ptrend < 0.0001) in the highest quartile, respectively. Of note significantly increased BMI associates with PR+ and ER +/PR+ status while significantly decreased BMI was observed in HER2+ cases. CONCLUSIONS: Taken together, measurement of serum estrone and estrone sulfate concentrations prior to surgical intervention might support the individualization of regime in postmenopausal primary breast cancer patients.

[Different histological findings in two specimens from pulmonary metastasectomy in the same patient]. / Tüdómetastasectomia során eltávolított több góc különbözó szöveti szerkezettel.

INTRODUCTION: Out of the 3310 thoracic surgical procedures performed between 1980 and 2000 at the Department of Surgery of the National Institute of Oncology in Budapest, 258 were pulmonary metastasectomies involving 236 patients. Primary tumors were the following in order of decreasing frequency: testicular cancer, colorectal cancer, renal cancer, soft tissue tumor, breast cancer and others. METHODS: In the present study the authors report two patients with multiple pulmonary metastases. The primary tumor was non-seminoma testicular cancer in case one and endometrial cancer in case two (previously treated for thyroid cancer). Histological examination of resected specimens revealed unsuspected focal inactive tuberculosis in the first case and medullary thyroid cancer in the second. CONCLUSIONS: In the reported two cases the following conditions of metastasectomy were given: 1. satisfactory cardiopulmonary status, 2. possibility of surgical radicality, 3. locoregional disease control, 4. prior chemotherapy in chemosensitive tumor (case one). The generally accepted condition of metastasectomy-lack of clinically manifest disease in other distant organs--was not fulfilled in case two (suspected liver metastases).

[Change of attitude in the surgical treatment of lung metastases]. / Szemléletünk alakulása a tüdoáttétek sebészi kezelésében.

There has been no major change in surgical technique of lung metastasectomy till the last decades. In the latest years the variety of diagnostic tools and methods grew significantly. There are more chemotherapy possibilities and more palliative surgical techniques (VATS, ITP, RFTA) to treat certain types of lung metastases. Based on data of the International Registry of Lung Metastases the most important prognostic factor for metastasectomy is how radical the procedure was (clear resection margin). The more aggressive approach (even pneumonectomy for R0 resection) is becoming more popular.

[Angiosarcomas of the chest wall and breast after radiotherapy: The questions of radiogenic origin, diagnosis and treatment] / Mellkasfali és emlô angiosarcoma sugárkezelés után: a radiogén eredet, a diagnózis és a kezelés kérdései.

PURPOSE: To present medical history of secondary chest wall and breast angiosarcomas (AS) developed after radiotherapy, and to discuss the questions of radiogenic origin, diagnosis and treatment by the review of the literature. METHODS: Report of two cases and MEDLINE search for relevant publications. RESULTS: Secondary AS occured in a previously irradiated field after a long (6 and 8 years) latency period in both cases. Detailed histopathological and immunohistochemical examinations from the biopsy and/or surgical specimens confirmed the diagnosis as AS. The first patient with moderately differentiated AS was treated successfully with radical surgery. The second patient with irresecable AS died of rapid local progression within 4 months. The incidence of chest wall and breast AS is increased after irradiation, however, controversial data exist in the literature. The incidence of chest wall and breast AS after radiotherapy was found to be 0.39% in our patient population, which means an estimated odds ratio of 2.4 for secondary AS. Stewart-Treves syndrome is not of radiogenic origin, since postoperative lymphoedema has been considered as primary etiological factor. CONCLUSIONS: Patients treated with surgery and/or radiotherapy for primary breast cancer are at higher risk for developing secondary AS, compared to the healthy population. An etiological relationship between radiotherapy and subsequent AS of chest wall and breast is likely, but still controversial. Initial radical surgery is the only effective treatment for achieving long term survival. Further adjuvant radiotherapy is no longer feasible, due to the previous irradiation. Chemotherapy has only palliative effect. These very rare cases deserve special attention due to the atypical clinical appearance, difficulties of differential diagnosis and poor prognosis.

Free-breathing, motion-corrected late gadolinium enhancement is robust and extends risk stratification to vulnerable patients.

BACKGROUND: Routine clinical use of novel free-breathing, motion-corrected, averaged late-gadolinium-enhancement (moco-LGE) cardiovascular MR may have advantages over conventional breath-held LGE (bh-LGE), especially in vulnerable patients. METHODS AND RESULTS: In 390 consecutive patients, we collected bh-LGE and moco-LGE with identical image matrix parameters. In 41 patients, bh-LGE was abandoned because of image quality issues, including 10 with myocardial infarction. When both were acquired, myocardial infarction detection was similar (McNemar test, P=0.4) with high agreement (κ=0.95). With artifact-free bh-LGE images, pixelwise myocardial infarction measures correlated highly (R(2)=0.96) without bias. Moco-LGE was faster, and image quality and diagnostic confidence were higher on blinded review (P<0.001 for all). During a median of 1.2 years, 20 heart failure hospitalizations and 18 deaths occurred. For bh-LGE, but not moco-LGE, inferior image quality and bh-LGE nonacquisition were linked to patient vulnerability confirmed by adverse outcomes (log-rank P<0.001). Moco-LGE significantly stratified risk in the full cohort (log-rank P<0.001), but bh-LGE did not (log-rank P=0.056) because a significant number of vulnerable patients did not receive bh-LGE (because of arrhythmia or inability to hold breath). CONCLUSIONS: Myocardial infarction detection and quantification are similar between moco-LGE and bh-LGE when bh-LGE can be acquired well, but bh-LGE quality deteriorates with patient vulnerability. Acquisition time, image quality, diagnostic confidence, and the number of successfully scanned patients are superior with moco-LGE, which extends LGE-based risk stratification to include patients with vulnerability confirmed by outcomes. Moco-LGE may be suitable for routine clinical use.