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Abstract Background The Muir-Torre syndrome is a rare autosomal dominant condition and is currently considered a subtype of the more common hereditary nonpolyposis colorectal cancer syndrome, in which multiple primary malignancies occur together with sebaceous gland tumors. Case presentation We describe a case of a 62-year-old woman with three primary colorectal tumors, genital tumor, and sebaceous adenomas and present her family history of three generations. Our case represents the first case reported from Greece in the international literature. Conclusion Recognition of the syndrome in patients with sebaceous gland tumors should facilitate early detection of subsequent malignancies if the patient is entered into appropriate screening programs.

Purpose To evaluate the reliability of selective surveillance colonoscopy based on 6 specific perioperative risk factors in the early diagnosis of colonic ischemia (CI) after successful ruptured abdominal aortic aneurysm (rAAA) repair.Patients and Methods From 1999 to 2005, 62 consecutive patients underwent rAAA repair. In 59 of them, routine aggressive surveillance colonoscopy was offered every 12 hours within the first 48 hours, and CI was graded consistently. Patients with stage I or stage II CI were treated conservatively and were followed up with repeat colonoscopy, whereas patients with stage III CI underwent immediate laparotomy and colectomy. In parallel, 6 specific perioperative risk factors (PRFs) were retrospectively analyzed.Results Overall mortality was 33.9%. Nineteen patients (32.2%) developed CI and 12 (63.2%) of them survived.Thirteen (22%) had grade III CI and among these 6 survived. In patients with CI the mortality rate was 36.2%. Patients with less than 3 PRFs had no CI whereas all instances of CI could be diagnosed if colonoscopy was offered selectively in patients with more than 3 PRFs. The positive predictive value of CI increased with the number of PRFs. Patients with 5 or 6 PRFs were about 101 times more likely to develop CI compared with patients with 0 to 4 PRFs (P < .001).Conclusion Our study showed that CI is a frequent complication after successful rAAA repair and could reliably be early diagnosed if surveillance colonoscopy was offered selectively in patients with more than three PRFs. [ABSTRACT FROM AUTHOR]

Intestinal failure (IF) refers to the condition in which certain causes lead to derangements in nutrient absorption capacity. Gut adaptation occurs in response to IF and it is both morphologic and physiologic in nature and can be mediated by growth factors and nutrients. Our paper reviews certain trophic growth factors that have important interactions relevant for intestinal growth, function and adaptation. Data Source: The literature was reviewed (data from both animal and human studies) and certain trophic factors that modulate intestinal adaptation are summarized. The factors reviewed are: epidermal growth factor, insulin-like growth factor I and II, transforming growth factor α and ß, neurotensin, interleukin-11, glucagon-like peptide-2, keratinocyte growth factor, human growth hormone, short-chain fatty acids, and glutamine. Conclusions: Growth factors augment intestinal proliferation, diminish programmed apoptosis, and modulate the adaptive process. They also have the potential to improve nutrient absorption in some bowel disease. The enhancement of gut adaptation may allow patients to transition of parenteral/enteral to normal nutrition, in a shorter period of time, which reduce the rate of adverse effects caused by artificial nutrition and improve quality of life. [ABSTRACT FROM AUTHOR]

Abstract Background Gastrointestinal stromal tumors are mesenchymal tumors of the gastrointestinal tract of varying malignant potential that are believed to originate from neoplastic transformation of the interstitial cells of Cajal. They may occur anywhere along the gastrointestinal tract, but most commonly arise in the stomach or small intestine. They usually grow exophytically invading adjacent organs or perforating into the peritoneal cavity. They may also cause bleeding or obstructive symptoms. Intussusception and obstruction is a very uncommon presentation of these lesions because of their tendency to grow in an exraluminal fashion. Case presentation We present an unusual case of ileoileal intussusception in a 79-year-old female patient caused by a gastrointestinal stromal tumor located in the terminal ileum, and review the diagnostic and therapeutic approach highlighting the difficulty in diagnosing this entity preoperatively as a cause for intestinal obstruction. Conclusion This case presents an unusual malignant cause of adult intussusception and highlights the importance of computed tomography scanning in the accurate diagnosis of this rare entity.

Background: Spontaneous rapture of a germ cell tumor (GCT) metastases causing massive hemoretroperitoneum in a patient without choriocarcinoma component who has not received previous systemic chemotherapy is an exceedingly rare event. In such a devastating case scenario, a high index of clinical suspicion for early diagnosis and appropriate management is crucial., Case Presentation: We report on a 25-year-old male patient with a 4-month history of orchiectomy for testicular GCT (tGCT), who presented in the emergency department with acute abdomen and hemodynamic instability. Urgent computed tomography scan depicted a retroperitoneal mass measuring approximately 13 × 11.4 × 15 cm and massive intraperitoneal hemorrhage. Hemoperitoneum caused by spontaneous rapture of the metastatic retroperitoneal mass was suspected. COVID-19 pandemic-related deviation from the oncologic surveillance standards combined with COVID-19-related patient's emotional distress and self-neglect had led to loss of opportunity for appropriate adjuvant chemotherapy, obviously leading to the development of this devastating complication. An emergency, surgical exploration was decided. The bleeding mass was adequately exposed following a Cattell-Braasch maneuver and active bleeding was controlled by a challenging resection of approximately 80% of the lymph node mass volume. Pathological evaluation of the specimen revealed teratoma with low volume of yolk sac tumor component and extensive necrosis, findings compatible with the patient's history. Postoperative recovery was uneventful, followed by early start of adjuvant chemotherapy. Two years after the operation the patient is doing well with no evidence of recurrent disease., Conclusions: Massive hemoperitoneum is a devastating event that exceedingly rarely can complicate the clinical course of patients with advanced tGCT. Emergency surgical intervention is usually necessary however, sound judgement and careful surgical techniques are required for a positive and uneventful outcome. During COVID-19 pandemic, first-line medical personnel push their limits further not only to ensure health care services standards but also, to manage unpredictable, life-threatening cancer-related complications, associated with COVID-19-related deviation from appropriate oncologic surveillance and care., (© 2023. The Author(s).)

Background: Hilar cholangiocarcinoma (HC), also referred to as Altemeier-Klatskins tumour, is a lethal primary extrahepatic carcinoma of biliary epithelial origin, arising within 2cm of the hilar confluence. Radical surgical excision provides the best chance for a cure; however, the management of patients with HC is challenging not only because of the need for a high level of skill in biliary and hepatic resections, but also because of the difficulty in reaching an accurate diagnosis preoperatively. In fact, the differential diagnosis of HC is a diagnostic dilemma which is currently persisting, as modern, sophisticated diagnostic modalities are not always able to provide a definitive preoperative diagnosis. This difficulty is compounded by the fact that alternative entities that mimic HC may be present in up to 25% of patients with hilar obstruction. This makes precise preoperative characterisation of a hilar stricture extremely important by preventing unnecessary, high-risk, major surgical procedures. Therefore, alternative benign entities masquerading as Altemeier-Klatskins tumour deserve an important place in the differential diagnosis of hilar obstruction., Purpose: Considering the important clinical implications that a precise diagnosis of the aetiological cause of a biliary obstruction at the liver hilum would have, this paper will focus on the differentiation between HC and benign hilar obstructions and will review benign tumours and pseudotumours masquerading as HC along with their specific diagnostic features.

Background: Endoscopic full-thickness resection (EFTR) using the full-thickness resection device (FTRD ® ) is an invasive treatment for colorectal lesions not resectable by conventional endoscopic techniques. This study presents the first Greek experience of the FTRD ® procedure, assessing the efficacy and safety of EFTR., Methods: We conducted a retrospective analysis of 17 consecutive patients treated with the FTRD ® at 2 referral centers from October 2015 through December 2018. The indications included difficult adenomas (non-lifting and/or at difficult locations), early adenocarcinomas and subepithelial tumors. Primary endpoints were technical success and R0 resection., Results: Technical success and R0 resection were achieved in 82.3% procedures (14/17) and in 87.5% of those with difficult adenomas (8 patients). In the subgroup with carcinomas (n=3), the rate of technical success and R0 resection was 66.6%, while in the subgroup with subepithelial tumors (n=6) the rate was 83.3%. Technical success and R0 resection were significantly lower for lesions >20 mm vs. ≤20 mm (P=0.0429). In the 17 patients a total of 3 adverse events occurred (17.6%) and one of the patients underwent laparoscopic appendectomy because of EFTR around the appendix., Conclusions: Our study showed favorable results concerning EFTR feasibility, efficacy and safety, especially for lesions ≤20 mm, non-lifting adenomas, and subepithelial tumors. Technical success, R0 resection, and adverse events rates were comparable with previously published data. Larger randomized studies are needed to better define the clinical benefit and long-term outcomes of EFTR in selected patients., Competing Interests: Conflict of Interest: None

Objective: Despite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population., Methods: Data were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018., Results: Overall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1-6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, p<0.001), which remained significant after adjustment for age and for NYHA functional class., Conclusions: Long-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population., Competing Interests: Competing interests: GG has acted as a consultant and/or received unrestricted educational or research grants from Actelion, Bayer, MSD, GlaxoSmithKline, Lilly, Pfizer and United Therapeutics. AF received travel grants from Actelion and GlaxoSmithKline., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Lipomatous hypertrophy of atrial septum (LHAS) is a rare benign cardiac condition characterized by fatty tissue infiltration located in the atrial septum. We presented a rare case of LHAS resulting in recurrent syncopal attacks., Competing Interests: There are no conflicts of interest