Cocaine-Induced Chronic Bowel Ischemia Manifesting As Small Bowel Obstruction.

Cocaine represents one of the most frequently used recreational drugs worldwide. Cocaine-related disorders mostly affect the nervous and cardiovascular system, although gastrointestinal complications are not negligible and sometimes life-threatening. The most common gastrointestinal manifestations of cocaine abuse are ulceration, infarction, perforation, ischemic enterocolitis, and rarely hemorrhage, with mesenteric ischemia being the underlying pathophysiological mechanism. Herein, we report a rare case of cocaine-induced small bowel obstruction in a young female patient, caused by chronic mesenteric ischemia and excessive intestinal wall fibrosis.

Postoperative Bowel Obstruction as a Rare Complication of an Abdominal Drain.

Although routine intra-abdominal drain insertion following surgery represents a common practice worldwide, its utility has been questioned during the last decades. Several comparative studies have failed to document significant benefits from routine draining, and drain insertion has been correlated with various complications as well. Drain-related complications include, but are not limited, to infection, bleeding, and tissue erosion. Herein, we present the case of a 32-year-old patient with perforated peptic ulcer and purulent peritonitis, whose postoperative course was complicated by early mechanical bowel obstruction due to an abdominal drain. A high level of clinical suspicion, along with accurate imaging diagnosis, dictated prompt removal of the drain, which resulted in immediate resolution of the patient's symptoms. We aim to increase the clinical awareness of this rare complication related to intra-abdominal drain utilization with this report.

Performance of thoracic ultrasonography compared with chest radiography for the detection of rib fractures using computed tomography as a reference standard.

OBJECTIVE: Although there is growing evidence that ultrasonography is superior to X-ray for rib fractures' detection, X-ray is still indicated as the most appropriate method. This has partially been attributed to a lack of studies using an appropriate reference modality. We aimed to compare the diagnostic accuracy of ultrasonography and X-ray in the detection of rib fractures, considering CT as the reference standard. MATERIALS AND METHODS: Within a 2.5-year period, all consecutive patients with clinically suspected rib fracture(s) following blunt chest trauma and available posteroanterior/anteroposterior X-ray and thoracic CT were prospectively studied and planned to undergo thoracic ultrasonography, by a single operator. All imaging examinations were evaluated for cortical rib fracture(s), and their location was recorded. The cartilaginous rib portions were not assessed. CTs and X-rays were evaluated retrospectively. Concomitant thoracic/extra-thoracic injuries were assessed on CT. Comparisons were performed with the Mann-Whitney U test and Fisher's exact test. RESULTS: Fifty-nine patients (32 males, 27 females; mean age, 53.1 ± 16.6 years) were included. CT, ultrasonography, and X-ray (40 posteroanterior/19 anteroposterior views) diagnosed 136/122/42 rib fractures in 56/54/27 patients, respectively. Ultrasonography and X-ray had sensitivity of 100%/40% and specificity of 89.7%/30.9% for rib fractures' detection. Ultrasound accuracy was 94.9% compared to 35.4% for X-rays (P < .001) in detecting individual rib fractures. Most fractures involved the 4th-9th ribs. Upper rib fractures were most commonly overlooked on ultrasonography. Thoracic cage/spine fractures and haemothorax represented the most common concomitant injuries. CONCLUSION: Ultrasonography appeared to be superior to X-ray for the detection of rib fractures with regard to a reference CT.

Non-traumatic lower abdominal pain: ultrasonographic and clinical differential diagnosis.

Lower abdominal pain is frequently reported and has a diverse differential diagnosis. In cases with atypical presentation and nonspecific findings, further imaging evaluation is required to confirm the clinical suspicion and to distinguish between self-limiting disorders and those requiring immediate intervention. In line with European guidelines, transabdominal ultrasonography is recommended as a first-line imaging modality for clinically suspected acute appendicitis and acute diverticulitis, which respectively represent the predominant causes of right and left lower quadrant abdominal pain. It is similarly the preferred method for evaluating suspected obstetric/gynecologic and genitourinary diseases. Computed tomography is utilized as a secondary option when ultrasonography results are inconclusive. This pictorial essay illustrates the sonographic features of the most common conditions associated with lower abdominal pain and outlines the clinical characteristics of each entity.

Performance of Ultrasonography in the Diagnosis of Acute Colonic Diverticulitis.

OBJECTIVES: Computed tomography is regarded as the reference-standard imaging modality for the assessment of acute left-sided colonic diverticulitis (ALCD). However, its utility may be impaired by cost issues, limited availability, radiation exposure, and contrast-related adverse effects. Ultrasonography is increasingly advocated as an alternative technique for evaluating ALCD, although there is variation regarding its accuracy in disease diagnosis and staging and in determining alternative diagnoses. The aim of this study was to assess the performance of ultrasonography in diagnosing ALCD, differentiating complicated from non-complicated disease and defining alternative diseases related to left lower quadrant pain. METHODS: Within a 2-year period, all consecutive adult patients with clinically suspected ALCD and available abdominal computed tomography were prospectively evaluated and planned to undergo an abdominal ultrasonographic examination, tailored to the assessment of left lower quadrant. Computed tomography (CT) was regarded as the reference standard. RESULTS: A total of 132 patients (60 males, 72 females; mean age: 61.3 ± 11 years) were included. The sensitivity, specificity, and area under curve of ultrasonography for diagnosing ALCD were 88.6, 84.9, and 86.8%, with positive and negative predictive values of 89.7 and 83.3%, respectively. The method had sensitivity, specificity, and area under curve of 77.8, 100, and 88.9%, respectively, for defining complicated disease. The area under the curve for the identification of alternative diseases in patients with left lower quadrant pain was 90.9%. CONCLUSIONS: Ultrasonography has high diagnostic accuracy for diagnosing ALCD, differentiating complicated from non-complicated disease and establishing alternative diagnoses related to left lower quadrant pain. A low threshold to get a CT should be maintained as not to miss cases that may mimic ALCD.

The Role of Imaging in the Pre- and Postoperative Evaluation of Inguinal Hernia.

Inguinal hernia repair is the most frequently performed surgical procedure. Imaging has a fundamental role in initial assessment and postoperative evaluation of inguinal hernias. Clinical implications of radiology in the preoperative setting include diagnosis of occult/complicated hernias and enhancement of diagnostic certainty. Postoperatively, imaging may assist the detection and characterization of complications. Familiarity with the normal appearance of the postoperative groin area and surgical techniques is required to avoid misinterpretation. This pictorial essay provides an overview of the expected postoperative imaging findings and highlights the role of imaging in the pre- and postoperative evaluation of inguinal hernias, with emphasis on ultrasonography.

Pattern of road traffic injuries in the Rethymnon region, Crete, Greece: a secondary hospital-based study.

INTRODUCTION: Despite the significant progress that has been made during the last decade, Greece still faces the effects of road traffic accidents (RTAs) to a great extent every year. This study represents an epidemiological approach to road traffic injuries (RTIs) presenting to a secondary hospital in a geographical area with very low-quality road network; it aims to indicate possible preventive measures and actions. METHODS: The present study was conducted in Rethymnon General Hospital between January 2018 and February 2020. Data from all trauma patients who presented to the emergency department (ED) after RTAs were retrospectively collected from patient medical records. The severity of injury sustained by road traffic victims was graded using the Injury Severity Score (ISS). RESULTS: During the study period, 1582 trauma patients presented to the ED after RTAs. There were 1079 men and 503 women and the median age was 30 years. In total, 818 motorcycle-related injuries (51.7%), 497 automobile-related injuries (31.4%), 165 bicycle-related injuries (10.4%) and 102 pedestrian injuries (6.4%) were documented. Of the 1582 trauma patients, 58.2% (921) presented with injuries to the extremities, 35.3% (554) presented with thoracic injuries, 34.4% (541) presented with head injuries, 30% (475) presented with spinal column injuries, 17.4% (271) presented with facial injuries, 12.9% (203) presented with abdominal injuries and 10.3% (162) presented with pelvic injuries. A significant increase in the number of incidents was noticed during the tourist season. Median ISS was 2 (IQR=4). 53.7% of patients who were transferred to the hospital by ambulance had an ISS≤4. In 41 cases, transportation to a tertiary hospital was necessary, six patients had emergency operations and 13 others died in the ED. The median length of hospital stay was 3 (IQR=6) days. CONCLUSION: Actions that could possibly mitigate the burden of RTIs in the district of Rethymnon include upgrading the level of law enforcement, implementing educational strategies and information campaigns against inappropriate ambulance use, encouraging facilities to develop trauma registries and ensuring adequate hospital staffing.

Is There a Role for Color Doppler Ultrasonography in Acute Cholecystitis?

OBJECTIVE: The aim of this study was to evaluate the utility of the Pulsatility Index (PI) of the right hepatic artery, measured by color Doppler sonography, in the diagnosis of acute cholecystitis. METHODS: Seventy-five subjects were included in this study and divided into three groups, each consisting of 25 subjects: the cholecystitis group, the asymptomatic cholelithiasis group, and normal controls. Patients with acute cholecystitis fulfilled all the diagnostic criteria as stated in the latest Tokyo Guidelines. In all patients, the right hepatic artery was detected by color Doppler ultrasound and the PI was measured. RESULTS: Patients with acute cholecystitis were found to have significantly higher PI values compared to both normal controls and cholelithiasis patients. Regression analysis revealed a significant positive correlation between the PI and the cholecystitis outcome. CONCLUSION: Measurement of PI by color Doppler ultrasound represents a useful aid in the diagnostic process of acute cholecystitis. More studies are needed before this method is incorporated in the relevant guidelines.

Adrenal-sparing surgery: current concepts on a theme from the past.

Adrenal insufficiency represents a debilitating condition which mandates lifelong steroid replacement and which is associated with significant long-term morbidity, due to either inadequate or excessive replacement. The concept of preserving healthy cortical tissue by means of partial adrenalectomy has evolved as a means of avoiding the detrimental consequences of adrenal insufficiency. The advent of advanced technology in adrenal surgery has greatly facilitated the performance of partial adrenalectomy, enabling utilization of this method in an increasing number of endocrine diseases. Hereditary pheochromocytoma, Conn's syndrome, Cushing's syndrome, and non-functional adrenal masses represent the current indications for partial adrenalectomy, although the specific circumstances under which adrenal-sparing surgery should be proposed are still debatable. Partial adrenalectomy can be achieved by all types of minimally invasive surgery. In the absence of randomized, prospective, controlled studies designed to compare laparoscopic, retroperitoneoscopic, and robot-assisted partial adrenalectomy, none of these techniques has as yet been proven to be the gold standard for adrenal-sparing surgery. Apart from indications for surgery, results of surgery, and different types of partial adrenalectomy, controversial topics addressed in this review article include technical aspects such as the volume of residual adrenal tissue needed, ligation of adrenal vein, and means of tumor identification. Discussion of these controversial topics represents an attempt to define the role of partial adrenalectomy in modern adrenal surgery.

Current Controversies in Metabolic Surgery for Nonalcoholic Fatty Liver Disease.

Nonalcoholic fatty liver disease (NAFLD) represents the most common liver disease, and it is expected to become the leading cause of end-stage liver disease in the near future. Bariatric operations have beneficial effects on NAFLD, inducing histological resolution of liver damage through weight loss-dependent and weight loss-independent mechanisms. Due to lack of randomized controlled trials, no specific guidelines have been established so far. Yet there is growing evidence that NAFLD will eventually become a formal indication for metabolic surgery. Data regarding the choice of procedure are conflicting, although gastric bypass seems to be slightly superior to sleeve gastrectomy. The purpose of this review is to provide an update on the ongoing research regarding the role of metabolic surgery in NAFLD management.

Differential Expression of ß-Catenin, EGFR, CK7, CK20, MUC1, MUC2, and CDX2 in Intestinal and Pancreatobiliary-Type Ampullary Carcinomas.

INTRODUCTION: The purpose of this study was to associate immunohistochemical expression of ß-catenin, EGFR, CK7, CK20, MUC1, MUC2, and CDX2 in ampullary adenocarcinomas with the type of differentiation and prognosis. METHODS: Forty-seven patients with ampullary adenocarcinoma who underwent pancreatoduodenectomy with curative intent from 1997 to 2014 were included in this study. Nine patients with perioperative death were included in the association analysis but excluded from survival analysis. All tumors were classified as intestinal or pancreatobiliary type, according to histologic criteria, and immunohistochemically stained against the aforementioned markers. RESULTS: Eighteen carcinomas were classified as intestinal type and 29 carcinomas as pancreatobiliary type. Univariate analysis revealed that CK20 and CDX2 expression correlates with intestinal type, whereas MUC1 positivity indicates pancreatobiliary type. A marginally significant trend was shown for intestinal-type tumors toward larger size and more frequent MUC2 expression. Using multivariate analysis CK20 ( P = .003) and MUC1 ( P = .004) were identified as independent predictors of the intestinal and pancreatobiliary types, respectively. Mean and median survival was 90.3 and 55 months, respectively. Overall 5-year survival rate was 48%. On univariate survival analysis, overall survival was adversely influenced by the number of infiltrated lymph nodes, elevated Ca19-9 serum levels, jaundice, poor differentiation, T4 stage, N1 stage, TNM stage III, and CDX2 immunonegativity. Multivariate analysis identified TNM stage as the only independent prognostic factor in ampullary adenocarcinoma ( P = .048). CONCLUSIONS: Immunoreactivity against CK20 and MUC1 in ampullary carcinomas is a useful adjunct to histologic examination in determining histotype. None of the immunohistochemical markers studied had prognostic significance.

Death receptor 5 (DR5) and a 5-gene apoptotic biomarker panel with significant differential diagnostic potential in colorectal cancer.

High expression of Inhibitor of apoptosis proteins (IAPs) has been related to colorectal cancer (CRC) progression, resistance to treatment and poor prognosis. TRAIL (TNF-related apoptosis-inducing ligand) through its receptors DR4 (TRAIL-R1) and DR5 (TRAIL-R2) can selectively induce cancer cell apoptosis. The mRNA expression of DR4, DR5, c-IAP1, c-IAP2, XIAP and BIRC5/Survivin genes was examined in 100 paired (cancerous-normal) colorectal tissue specimens by real-time PCR, 50 of which were KRAS wild-type and 50 KRAS-mutant. DR5, XIAP and BIRC5/Survivin genes are significantly up-regulated (p < 0.0001, p = 0.012 and p = 0.0003, respectively), whereas c-IAP1 and c-IAP2 genes are significantly down-regulated at mRNA and protein levels in CRC (p < 0.0001 for both). ROC analyses showed that DR5, cIAP1 and cIAP2 expression has discriminatory value between CRC and normal tissue (AUC = 0.700, p < 0.0001 for DR5; AUC = 0.628, p = 0.011 for cIAP1; AUC = 0.673, p < 0.0001 for cIAP2). Combinatorial ROC analysis revealed the marginally fair discriminatory value of 5 genes as a panel (AUC = 0.685, p < 0.0001). Kaplan-Meier survival curves revealed significant association of cIAP2 down-regulation in CRC with lower overall survival probability of CRC patients (p = 0.0098). DR5, BIRC5/Survivin, XIAP, c-IAP1 and c-IAP2 mRNA expression are significantly deregulated in CRC and could provide a panel of markers with significant discriminatory value between CRC and normal colorectal tissue.

Differentiation and prognostic markers in ampullary cancer: Role of p53, MDM2, CDX2, mucins and cytokeratins.

INTRODUCTION: Subclassification of ampullary adenocarcinomas into intestinal and pancreatobiliary type has prognostic and therapeutic implications. Immunohistochemical staining against specific biomarkers has been proven to be a useful adjunct in determining the exact histotype. Furthermore the immunohistochemical profile is suggestive of the molecular pathogenic mechanisms through which the tumor evolved. The aim of this study was to correlate p53, MDM2, CK7, CK20, MUC1, MUC2 and CDX2 expression in ampullary adenocarcinomas with the type of differentiation and patients' survival. MATERIAL AND METHODS: Forty-seven radically resected ampullary adenocarcinomas were included in this study. Thirty-eight of them were eligible for survival analysis. Patients' data were retrospectively collected. All tumors were classified as intestinal or pancreatobiliary type, according to histologic criteria, and immunohistochemically stained against the aforementioned markers. RESULTS: There were 18 intestinal and 29 pancreatobiliary type ampullary adenocarcinomas. A trend was found between intestinal type tumors and large tumor size. CK20, MUC2 and CDX2 expression was more prevalent in intestinal type tumors, while MUC1 was more frequently expressed in pancreatobiliary type tumors. Neither p53 nor MDM2 differential expression between the two histotypes reached statistical significance. Multivariate analysis indicated CK20 and MUC1 as independent predictors of the histotype. Mean and median survival was 90.3 and 55 months respectively. Overall 5-year survival rate was 48%. Survival analysis indicated TNM stage as the only independent prognostic factor. Although significant difference in survival rates among the two histotypes was implied based on survival plots, this difference could not gain statistical significance. CONCLUSION: Immunoreactivity against CK20 and MUC1 in ampullary carcinomas is a useful adjunct to histologic examination in determining histotype. None of the immunohistochemical markers studied has prognostic significance. Future studies focused on other signaling pathways should seek further evidence of distinct tumorigenic mechanisms between histotypes of ampullary adenocarcinoma.

Neoadjuvant therapy for advanced pancreatic neuroendocrine tumors: an emerging treatment modality?

OBJECTIVE: Complete surgical resection is the only potentially curative treatment of localized pancreatic neuroendocrine tumors. Unfortunately, a significant proportion of these patients present with unresectable locally advanced tumors or massive metastatic disease. Recently, a new therapeutic approach for this subset of patients has emerged consisting of neoadjuvant therapy followed by surgical exploration in responders. DESIGN: We searched MEDLINE for the purpose of identifying reports regarding neoadjuvant treatment modalities for advanced pancreatic neuroendocrine tumors. RESULTS: We identified 12 studies, the vast majority of which were either case reports or small case series. Treatment options included chemotherapy, radiotherapy, peptide receptor radionuclide therapy, biological agents or various combinations of them. CONCLUSIONS: Increasing evidence supports the application of neoadjuvant protocols in advanced pancreatic neuroendocrine tumors aiming at tumor downsizing, thus rendering curative resection feasible. Given that prospective and controlled randomized clinical trials from high-volume institutions are not feasible, expert panel consensus is needed to define the optimal treatment algorithm.

Surgical treatment of potentially primary malignant adrenal tumors: an unresolved issue.

Although the great majority of incidentalomas are adrenocortical adenomas, a number of them, depending on the size and radiological characteristics of the lesions, will turn out to be carcinomas. These tumors may present as suspicious on initial evaluation and potentially malignant or malignant on histology. Adrenocortical carcinoma is a rare and aggressive malignancy with evolving diagnostic and therapeutic approaches. Laparoscopic surgery has become the gold standard for surgery of benign adrenal tumors. Despite the extensive experience gained in laparoscopic adrenalectomy, controversy still remains in the management of adrenal tumors with high suspicion or evidence of malignancy. The aim of this review is to update the existing information regarding the diagnostic approach and surgical management of suspicious and potentially malignant primary adrenal tumors.The interpretation of radiologic characteristics is a cornerstone in pre-operative assessment of large adrenal masses, since open surgery remains the preferred procedure when malignancy is suspected in large tumors with possible local invasion. Despite the improvement of imaging techniques, they lack sufficient accuracy to exclude primary malignancy in tumors from 4 cm to 10 cm in size. An initial laparoscopic approach can be used in this group of patients, but early conversion to open technique is mandatory if curative resection cannot be performed. Adrenal tumors >10 cm of malignant potential should be treated by the open approach from the start. Solitary adrenal metastasis from another primary malignancy is usually amenable to laparoscopic surgery. Patients with suspected adrenal cancer should be referred to tertiary centers that perform laparoscopic and open adrenal surgery with minimal morbidity and mortality.

Five-year actual survival after pancreatoduodenectomy for pancreatic head cancer.

BACKGROUND: The aim of this study was to analyse retrospectively the long-term results of patients who were operated for adenocarcinoma of the pancreatic head and identify significant prognostic factors. METHODS: Eighty patients who were surgically treated for adenocarcinoma of the pancreatic head between 1995 and 2006 met the inclusion criteria and were subject to retrospective analysis. Possible prognostic factors were evaluated and independent predictors of survival were determined. RESULTS: A classic Whipple procedure was performed in 47 patients and a pylorus-preserving pancreatoduodenectomy in 32 patients; one patient underwent total pancreatectomy. Five-year survival rate in this group of patients was 13.6%. Median survival time was 24 months. Univariable analysis demonstrated stage of disease, tumour size and grade and nodal status as significant predictive factors of survival. Multivariable analysis indicated tumour size, nodal status and disease stage as significant prognostic indicators in terms of survival. CONCLUSIONS: Long-term survival in pancreatic cancer is still very low. Prognostic factors include differentiation of the tumour, disease stage and nodal status. So far, there has been no reliable method that can accurately predict which patient will mostly benefit from surgical resection. This means that pancreatic cancer resection should nearly always be attempted.

Primary intrahepatic malignant epithelioid mesothelioma.

INTRODUCTION: Primary malignant hepatic mesotheliomas are extremely rare. We report the case of a patient with primary intrahepatic malignant mesothelioma who was treated in our department. PRESENTATION OF CASE: A 66-year old male patient was admitted to our department for the evaluation of anemia. An abdominal computed tomography scan revealed a large space occupying lesion in the right liver lobe. DISCUSSION: The tumor was subsequently resected and a diagnosis of primary intrahepatic malignant mesothelioma was made after pathologic examination. The patient did not receive adjuvant therapy and is currently alive and free of disease, 36 months after the resection. CONCLUSION: To our knowledge this is the eighth adult case of primary intrahepatic malignant mesothelioma reported in the literature. These tumors are rarely diagnosed preoperatively. Absence of previous asbestos exposure does not exclude malignant mesothelioma from the differential diagnosis. Proper surgical treatment may offer prolonged survival to the patient, without adjuvant therapy.

Subclinical Cushing's syndrome: current concepts and trends.

Clinically inapparent adrenal masses which are incidentally detected have become a common problem in everyday practice. Approximately 5-20% of adrenal incidentalomas present subclinical cortisol hypersecretion which is characterized by subtle alterations of the hypothalamic-pituitary-adrenal axis due to adrenal autonomy. This disorder has been described as subclinical Cushing's syndrome, since there is no typical clinical phenotype. The diagnosis of subclinical Cushing's syndrome is based on biochemical evaluation; however, there is still no consensus for the biochemical diagnostic criteria. An abnormal 1mg dexamethasone suppression test (DST) as initial screening test in combination with at least one other abnormal test of the hypothalamic-pituitary-adrenal axis has been advocated by most experts for the diagnosis of subclinical Cushing's syndrome. DST is the main method of establishing the diagnosis, while there is inhomogeneity of the information that other tests provide. Arterial hypertension, diabetes mellitus type 2 or impaired glucose tolerance, central obesity, osteoporosis/vertebral fractures and dyslipidemia are considered as detrimental effects of chronic subtle cortisol excess, although there is no proven causal relationship between subclinical cortisol hypersecretion and these morbidities. Therapeutic strategies include careful observation along with medical treatment of morbidities potentially related to subtle cortisol hypersecretion versus laparoscopic adrenalectomy. The optimal management of patients with subclinical Cushing's syndrome is not yet defined. The conservative approach is appropriate for the majority of these patients; however, the duration of follow-up and the frequency of periodical evaluation still remain open issues. Surgical resection may be beneficial for patients with hypertension, diabetes mellitus type 2 or abnormal glucose tolerance and obesity.

Ampullary cancer--a separate clinical entity?

AIMS: Ampullary cancer is a relatively uncommon tumour, with a better prognosis than pancreatic cancer. The purpose of this study was to review the recent literature on ampullary adenocarcinoma, focusing on histological types and prognostic factors. METHODS AND RESULTS: Using PubMed, we carried out a comprehensive search of the literature, which was extended to April 2013 to retrieve all additional publications. Ampullary cancer comprises two main histological subtypes, the pancreatobiliary type and the intestinal type. These subtypes have different pathogenetic and clinical characteristics. Clinical and histological parameters as well as immunohistochemical markers have been identified as significant prognostic factors in ampullary cancer. Moreover, several immunohistochemical markers have been studied, not only as prognostic factors but as a means of differentiating ampullary from other peri-ampullary tumours, and of identifying the exact histological subtype. CONCLUSIONS: The considerable differences in the frequencies of the two subtypes of ampullary tumours reported in literature reinforce the necessity to define molecular markers to distinguish them. Until then, the significance of the histological subtype as a prognostic factor should be evaluated cautiously. Future research on the pathogenesis of ampullary cancer will possibly suggest that we should stop treating this type of cancer as a separate entity.

Metastatic leiomyosarcoma of the intrapancreatic bile duct.

We report the case of a patient with a history of surgically treated pulmonary leiomyosarcoma, presenting with recurrent acute cholangitis and metastatic leiomyosarcoma of the common bile duct. Preoperative examinations had revealed a high grade malignant neoplasm and bilateral lung metastases. The patient underwent pylorus-preserving pancreaticoduodenectomy and survived for 5.5 years after the first diagnosis.