Eosinophilic Esophagitis and Achalasia: Two Distinct Nosologic Entities or a Possible Etiopathogenic Association?

BACKGROUND: Despite the fact that researchers have made significant progress in elucidating the pathophysiology of esophageal diseases, the understanding of esophageal motility alterations in patients with eosinophilic esophagitis (EoE) is in its infancy and current published medical literature remains rather scarce on this topic. A growing body of scientific data regarding associations between esophageal motor disorders, such as achalasia and EoE, exists nowadays. SUMMARY: It seems that the association of EoE and achalasia does not constitute a cause and effect relationship, as it is not clear whether esophageal motility abnormalities are the result of EoE or vice versa. As such, there is no universally accepted treatment algorithm for patients presenting with both of these entities. Key Messages: The aim of this article is to review the existing data on achalasia-like motility disorders in patients with EoE, highlighting a possible association between these 2 esophageal disorders. Moreover, we seek to describe the clinical presentation in such cases, diagnostic modalities to be used, and current treatment strategies in patients suspected to suffer from both disorders.

Abdominal aortic aneurysms and abdominal wall hernias - a systematic review and meta-analysis.

Background: Abdominal wall hernias (AWHs) share common epidemiological characteristics with abdominal aortic aneurysms (AAAs), typically presenting in male population and older ages. Prior reports have associated those two disease entities. Our objective was to perform a systematic review and meta-analysis and examine whether AAA rates are higher among patients with AWH vs controls and whether the incidence of AWH was higher among patients with AAA vs patients without AAA. Methods: We performed a systematic review and meta-analysis according to the PRISMA guidelines. The Medline database was searched up to July 31, 2020. A random effects meta-analysis was performed. Results: In total, 17 articles and 738,972 participants were included in the systematic review, while 107,578 patients were eligible for the meta-analysis. Among four studies investigating the incidence of AAA in patients with hernias, AAA was more common in patients with hernias, compared to patients without hernias. [OR: 2.53, 95% CI: 1.24-5.16, I2=81.6%]. Among thirteen studies that compared patients with known AAA vs no AAA, the incidence of hernias was higher in patients with AAA, compared with patients without AAA [OR: 2.27, 95% CI: 1.66-3.09, I2=84.6%]. Conclusions: Our study findings indicate that a strong association between AWH and AAA exists. AWHs could therefore be used as an additional selection criterion for screening patients for AAA, apart from age, gender, family history and smoking.

Combined hepatocellular-cholangiocarcinoma: An update on epidemiology, classification, diagnosis and management.

BACKGROUND: Combined hepatocellular-cholangiocarcinoma (CHC) is a rare subtype of primary hepatic malignancies, with variably reported incidence between 0.4%-14.2% of primary liver cancer cases. This study aimed to systematically review the epidemiological, clinicopathological, diagnostic and therapeutic data for this rare entity. DATA SOURCES: We reviewed the literature of diagnostic approach of CHC with special reference to its clinical, molecular and histopathological characteristics. Additional analysis of the recent literature in order to evaluate the results of surgical and systemic treatment of this entity has been accomplished. RESULTS: The median age at CHC's diagnosis appears to be between 50 and 75 years. Evaluation of tumor markers [alpha fetoprotein (AFP), carbohydrate antigen 19-9 (CA19-9) and carcinoembryonic antigen (CEA)] along with imaging patterns provides better opportunities for CHC's preoperative diagnosis. Reported clinicopathologic prognostic parameters possibly correlated with increased tumor recurrence and grimmer survival odds include advanced age, tumor size, nodal and distal metastases, vascular and regional organ invasion, multifocality, decreased capsule formation, stem-cell features verification and increased GGT as well as CA19-9 and CEA levels. In case of inoperable or recurrent disease, combinations of cholangiocarcinoma-directed systemic agents display superior results over sorafenib. Liver-directed methods, such as transarterial chemoembolization (TACE), percutaneous ethanol injection (PEI), hepatic arterial infusion chemotherapy (HAIC), radioembolization and ablative therapies, demonstrate inferior efficacy than in cases of hepatocellular carcinoma (HCC) due to CHC's common hypovascularity. CONCLUSIONS: CHC demonstrates an overlapping clinical and biological pattern between its malignant ingredients. Natural history of the disease seems to be determined by the predominant tumor element. Gold standard for diagnosis is histology of surgical specimens. Regarding therapeutic interventions, major hepatectomy is acknowledged as the cornerstone of treatment whereas minor hepatectomy and liver transplantation may be applied in patients with advanced cirrhosis. Despite all therapeutic attempts, prognosis of CHC remains dismal.

Repeat liver resection for recurrent colorectal metastases: a single-centre, 13-year experience.

OBJECTIVES: Isolated intrahepatic recurrence is noted in up to 40% of patients following curative liver resection for colorectal liver metastases (CLM). The aims of this study were to analyse the outcomes of repeat hepatectomy for recurrent CLM and to identify factors predicting survival. METHODS: Data for all liver resections for CLM carried out at one centre between 1998 and 2011 were analysed. RESULTS: A total of 1027 liver resections were performed for CLM. Of these, 58 were repeat liver resections performed in 53 patients. Median time intervals were 10.5 months between the primary resection and first hepatectomy, and 15.4 months between the first and repeat hepatectomies. The median tumour size was 3.0 cm and the median number of tumours was one. Six patients had a positive margin (R1) resection following first hepatectomy. There were no perioperative deaths. Significant complications included transient liver dysfunction in one and bile leak in two patients. Rates of 1-, 3- and 5-year overall survival following repeat liver resection were 85%, 61% and 52%, respectively, at a median follow-up of 23 months. R1 resection at first hepatectomy (P = 0.002), a shorter time interval between the first and second hepatectomies (P = 0.02) and the presence of extrahepatic disease (P = 0.02) were associated with significantly worse overall survival. CONCLUSIONS: Repeat resection of CLM is safe and can achieve longterm survival in carefully selected patients. A preoperative knowledge of poor prognostic factors helps to facilitate better patient selection.

Undifferentiated carcinoma with osteoclast-like giant cells of the pancreas: An individual participant data meta-analysis.

Undifferentiated carcinoma with osteoclast-like giant cells (UC-OGCs) of the pancreas is a rare neoplasm that accounts for less than 1% of all pancreatic malignancies. The aim of this study was to review the literature regarding UC-OGC, and to highlight its biological behavior, clinicopathologic characteristics, prognosis, and therapeutic options. A systematic review of the literature in PubMed/Medline and Scopus databases was performed (last search October 31st, 2023) for articles concerning pancreatic UC-OGC in the adult population. Fifty-seven studies met the inclusion criteria, involving 69 patients with a male-to-female ratio of 1.1:1 and a mean age of 62.96. Main symptoms included abdominal pain (33.3%), jaundice (14.5%), weight loss (8.7%), while fourteen patients (20.3%) were asymptomatic. Surgical resection was performed in 88.4% of cases. Survival rates at one, three, and five years were 58%, 44.7%, and 37.3% respectively. Sex, age, size (cut-off of 4 cm), location, and adjuvant treatment did not significantly affect patient survival. UC-OGC of the pancreas is a rare subtype of undifferentiated pancreatic carcinoma with a better prognosis than conventional pancreatic ductal adenocarcinoma or undifferentiated carcinoma without giant cells. The establishment of a dedicated patient registry is imperative to further delineate the optimal treatment for this uncommon clinical entity.

Aetio-pathogenesis and the management of spontaneous liver bleeding in the West: a 16-year single-centre experience.

BACKGROUND: Spontaneous liver bleeding (SLB) is a rare but potentially fatal complication. In contrast to the East, various benign pathologies are the source of SLB in the West. An accurate diagnosis and a timely implementation of appropriate treatment are crucial in the management of these patients. The present study presents a large Western experience of SLB from a specialist liver centre. METHODS: A retrospective analysis of patients presented with SLB between January 1995 and January 2011. RESULTS: Sixty-seven patients had SLB, 44 (66%) were female and the median age at presentation was 47 years. Abrupt onset upper abdominal pain was the presenting symptom in 65 (97%) patients. The aetiology for SLB was hepatic adenoma in 27 (40%), hepatocellular carcinoma (HCC) in 17 (25%) and various other liver pathologies in the rest. Emergency treatment included a conservative approach in 42 (64%), DSA and embolization in 6 (9%), a laparotomy and packing in 6 (9%) and a liver resection in 11 (16%) patients. Eleven (16%) patients had further planned treatments. Seven (10%) died during the same admission but the mortality was highest in patients with HELLP syndrome. At a median follow-up of 54 months all patients with benign disease are alive. The 1-, 3- and 5-year survival of patients with HCC was 59%, 35% and 17%, respectively. CONCLUSION: SLB is a life-threatening complication of various underlying conditions and may represent their first manifestation. The management should include initial haemostasis followed by appropriate staging investigations to provide a definitive treatment for each individual patient.

Primary Squamous Cell Carcinoma of Colon and Rectum: A Systematic Review of the Literature.

Background - Objective: Primary colorectal squamous cell carcinoma is an extremely rare neoplasm with an incidence of 0.1 to 0.25 per 1,000 diagnosed colorectal carcinomas.The objective of this study was to evaluate its biological behavior and highlight the role of a surgical approach for its management.MethodsPubMed and Cohrane databases were independently searched (last search: April 10th, 2020) for articles concerning colorectal squamous cell carcinoma in adult population.Results: Seventy-one studies met predefined inclusion criteria and involved 99 patients (54.5% females) with an age of 56.98 ± 12.19 years (mean ± SD). The most frequent site of occurrence was the rectum (63.5%). Open surgery was conducted at 95% of patients, while 21.4% and 30.3% received neoadjuvant and adjuvant therapy respectively.Postoperative complications were developed in 31.3% of patients, while 6.1% died withing the first month following operation. Five-years survival rate was 49.5% (95% CI: 33.7%-63.4%). Female sex (HR: 0.24; 95% CI: 0.11-0.54; p-value: 0.001) and presence of postoperative complications (HR: 4.10; 95%CI: 1.47-1.46; p-value: 0.007) significantly affected the survival.Conclusions Colorectal Squamous Cell Carcinoma is a rare tumor with an aggressive behavior. Surgery is the standard of treatment for the colontumors, while the role of chemoradiotherapy is promising especially for rectal tumors. Further clinical trials are necessary to determine the preferred treatment approach.

An extremely rare clinical manifestation of acute appendicitis in a nonagenarian patient: lessons still to be learned.

Abstract.

Primary Synovial Sarcoma of the Kidney: Diagnostic Approach and Therapeutic Modalities for a Rare Nosological Entity.

Synovial sarcoma (SS) is a rare mesenchymal entity that represents 5-10% among soft tissue sarcomas (STS). Primary renal synovial sarcoma (PRSS) is an uncommon, rapidly growing tumor, with potential metastatic dissemination. The main prognostic factors of PRSS include tumor size and histologic grade, while translocation t (X; 18) (p11.2; q11.2) (fusion of SYT gene -chromosome 18- with SSX genes (1, 2 or 4)-chromosome X) is the most common pathognomonic sign. Aggressive surgical resection of the tumor along with concomitant regional lymphadenectomy is the treatment of choice for PRSS, while additional en bloc resection of the adjacent affected organs is often performed. To date, the role of preoperative or postoperative chemotherapy remains equivocal. The prognosis of patients with PRSS is poor, as the 5-year survival rate is only 20-30% and further deteriorates when a high mitotic activity is detected. Local recurrence even after complete R0 surgical excision remains the most frequent cause of death. The aim of this review was to meticulously discuss clinical features, histogenesis, and morphological and immunochemical findings of PRSS, while the role of current diagnostic and therapeutic management of this aggressive neoplasm was emphasized.

Synchronous well differentiated neuroendocrine tumour and gastrointestinal stromal tumour of the stomach: a case report.

BACKGROUND: Well differentiated neuroendocrine tumours (carcinoids), arising from cells of the diffuse neuroendocrine system, represent the most commonly encountered gastric endocrine tumours. Gastrointestinal stromal tumours (GISTs), which stem from interstitial Cajal cells located within the wall of the gastrointestinal tract and have a characteristic immunoreactivity for CD117 (c-kit protein), account for the majority of gastrointestinal mesenchymal neoplasms. Simultaneous occurrence of a GIST with a well differentiated neuroendocrine tumour in the stomach is very rare. METHODS: Clinical history, endoscopy and histopathological findings were utilized for our diagnostic considerations. RESULTS: We report the coexistence of a high risk GIST with a well differentiated neuroendocrine tumour of benign clinical behavior, both located in the stomach, in a 62-year-old man previously operated for a gastric well differentiated neuroendocrine tumour with uncertain malignant behaviour. CONCLUSIONS: Even single well differentiated, sporadic, NETs of small size may coexist with GISTs. An appropriate initial therapeutic approach combined with a scrupulous follow-up seems to play a significant role in terms of preventing a metastatic disease.

Rupture of a mature cystic teratoma in a young girl caused by a car accident: an extremely rare case and review of the literature.

Mature cystic teratomas (MCT) of the ovary or dermoid cysts are commonly encountered benign ovarian lesions accounting for  approximately 70% of benign tumors and originating from germ cells. A rare case of peritonitis in a 17-year-old female patient caused by traumatic rupture of an MCT of the right ovary is herein presented. A meticulous search of the literature has also been performed. Due to deterioration of patient's clinical condition, she was urgently led to surgery and intraoperative investigation of the peritoneal cavity revealed an inflamed ruptured ovarian cyst along with extensive peritoneal adhesions and purulent peritoneal fluid. A cyst resec-tion was performed with preservation of the ovary followed by adhesiolysis and meticulous abdominal washing and the patient had an uneventful recovery. Despite its rarity, traumatic rupture of ovarian cyst should be considered at evaluation of trauma patients with special attention to cases with a discovered or known presence of ovarian cyst.

Gastrointestinal stromal tumors (GISTs): an updated experience.

BACKGROUND: Gastrointestinal stromal tumors (GISTs) are relatively common mesenchymal tumors of the digestive tract characterized by c-KIT mutations. This is a comprehensive review of the current data of the literature on the various aspects of the diagnosis and treatment of these tumors. METHODS: The stomach is the most commonly involved site for these tumors in the digestive tract. Computed tomography and endoscopy can usually establish the diagnosis. The study of certain specific immunohistochemical markers may contribute to better characterization of these tumors. RESULTS: Surgical resection of GISTs has been the most effective therapy. In addition, targeted therapy with tyrosine kinase inhibitors may reduce the development of recurrence or decrease the disease progression in patients with metastatic disease. CONCLUSIONS: The introduction of tyrosine kinase inhibitors has resulted in significant improvement in the overall prognosis of these patients. Furthermore, preoperative imatinib can decrease tumor volume and is associated with complete surgical resection in locally advanced primary GISTs.

Supravesical hernias: a systematic review of the literature.

BACKGROUND: A supravesical hernia (SH) is a rare abdominal wall hernia that is defined by the protrusion of abdominal viscera through the supravesical fossa. The objective of this study was to review current literature on SHs and elucidate their clinical manifestations and preferred therapeutic approach. METHODS: PubMed, Embase and Cochrane bibliographical databases were searched (last search: 30 March 2020) for articles reporting on SHs. RESULTS: Thirty-four studies met our inclusion criteria and involved collectively 38 patients (94.7% males) with an age of 59.57 ± 14.84 (mean ± standard deviation) years. Abdominal pain (81.6%) and nausea and vomit (71.1%) were the most frequently encountered symptoms. The majority of patients (78.1%) had signs of bowel obstruction. Twenty-nine patients (76.3%) suffered from an internal SH, while nine (23.7%) had an external one. An open hernia repair was conducted at 29 patients (76.3%), while eight (21.1%) had a laparoscopic approach and in one case (2.6%) the laparoscopic operation was converted to an open one. A mesh was applied at 14.3% of included cases. Eight patients (21.1%) underwent a bowel resection and hernia sac was resected at six (15.8%) cases. Four patients (11.8%) experienced postoperative complications and one patient (2.9%) died on first postoperative day. CONCLUSION: SH is an extremely rare type of hernia occurring through the supravesical fossa between the remnants of urachus and foetal umbilical arteries. All clinicians should be aware of this entity and further studies are necessary in order to clarify their aetiology, true incidence and optimal therapeutic approach.

Aortoiliac endograft-enteric fistula due to an ingested toothpick.

Foreign body ingestion is not uncommon, but in most patients the object passes without sequelae. In very few patients, the ingested foreign objects may perforate the gastrointestinal tract, causing potentially life-threatening complications. Pointed ingested objects such as toothpicks are the most common. Vascular perforation due to toothpick ingestion has rarely been reported. We present a patient with lower gastrointestinal bleeding secondary to simultaneous perforation of the sigmoid colon and the right iliac limb of an aortobiiliac endograft by an ingested toothpick. This arterioenteric fistula was treated in two stages. First as a bridging operation, a stent graft was inserted into the ipsilateral limb of the previous aortoiliac endograft to control the bleeding. Second, the aortoiliac endograft was removed, the aorta was oversewn, and an extra-anatomic axillobifemoral bypass restored the flow to the lower limbs. The colon perforation was treated with a proximal temporary loop colostomy. To our knowledge, this the first case of aortoiliac endograft-enteric fistula after endovascular abdominal aortic aneurysm repair caused by this extraordinary mechanism.

Mixed Adenoneuroendocrine Carcinoma (MANEC) of the Gallbladder: A Systematic Review of Outcomes Following Surgical Management.

BACKGROUND/AIM: Mixed adenoneuroendocrine carcinomas (MANEC) are uncommon tumors exhibiting both adenocarcinomatous and neuroendocrine differentiation. They most commonly arise in the colon, appendix, rectum or stomach, however, a limited number of MANECs have been reported to originate in the gallbladder (gMANEC). The aim of our systematic review was to accumulate the existing data on gMANEC with special attention to the clinicopathological characteristics, surgical approach, recurrence and survival rates of patients diagnosed with this rare malignancy. MATERIALS AND METHODS: A comprehensive search of the literature was undertaken. RESULTS: A total of 15 studies (14 case reports and 1 case series), which comprised 19 patients who successfully underwent surgical treatment for gMANEC were included in our systematic review. During a median follow-up of 8 months (range=2-48 months) the overall survival was 87% and the recurrence rate was 21%. CONCLUSION: Achievement of complete surgical resection is the mainstay of the therapeutic management. Additionally, the stage of the disease and the histopathological mapping of these tumors affect decision-making for adjuvant chemotherapy and seem to define the prognostic course of each patient.

Small bowel perforation with multiple intestinal metastases from lung carcinoma: A case report.

The present report describes the case of an 81-year-old woman who underwent an emergency explorative laparotomy due to small bowel perforation. Diffuse feculent peritonitis due to perforation of the jejunum was identified. In addition, six distinct tumors of the jejunum in close proximity to the perforation site, as well as two more lesions caudally at the ileum, were identified. A separate stenotic tumor was identified at the sigmoid colon, causing moderate dilatation of the whole intestine. An enterectomy, including the perforation site and the proximal six tumors, was performed, followed by limited enterectomies for the distal two tumors and side-to-side anastomoses of the small intestine. All resected tumors were identified at histology as metastatic from lung carcinoma. Postoperatively, the patient remained in a severe septic condition and succumbed the following day. Metastatic lesions from lung carcinoma in the small bowel wall are a rare finding predisposing to bowel perforation. When an acute condition coexists with multiple intestinal metastases, the mortality rate is high despite prompt surgical intervention.

Advents in the Diagnosis and Management of Ischemic Colitis.

BACKGROUND: Ischemic colitis (IC) is a common type of ischemic insult, resulting from decreased arterial blood flow to the colon. This disease can be caused from either atherosclerotic occlusive vascular disease or non-occlusive disease. The aim of this study is to present the diagnostic methodology and management of this severe disease based on current literature. METHODS: A literature search has been done including articles referring to modern diagnosis and management of IC. RESULTS: IC is usually a transient disease, but it can also cause gangrene of the colon, requiring emergency surgical exploration. Diagnosis is troublesome and is based on imaging examinations, mainly computerized tomography, which in association with colonoscopy can delineate the distribution pattern and severity of disease. CONCLUSION: The majority of patients with mild disease have usually complete clinical recovery within a short period. The severe forms of the disease carry high morbidity and mortality rates and prompt surgical intervention is the only way to improve the associated severe prognosis.

Human epidermal growth factor receptor-2 gene amplification in gastric cancer using tissue microarray technology.

AIM: To assess human epidermal growth factor receptor-2 (HER2)-status in gastric cancer and matched lymph node metastases by immunohistochemistry (IHC) and chromogenic in situ hybridization (CISH). METHODS: 120 cases of primary gastric carcinomas and 45 matched lymph node metastases from patients with full clinicopathological features were mounted onto multiple-punch and single-punch tissue microarrays, respectively, and examined for HER2 overexpression and gene amplification by IHC and CISH. RESULTS: Twenty-four tumors (20%) expressed HER2 immunohistochemically. An IHC score of ≥ 2+ was observed in 20 tumors (16.6%). HER2 amplification was detected by CISH in 19 tumors (15.8%) and in their matched lymph node metastases. A high concordance rate was found between HER2 positivity (as detected by IHC) and HER2 gene amplification (as detected by CISH), since 19 of the 20 IHC positive cases were amplified (95%). All amplified cases had 2+ or 3+ IHC results. Amplification was associated with intestinal phenotype (P < 0.05). No association with grading, staging or survival was found. CONCLUSION: In gastric cancer, HER2 amplification is the main mechanism for HER2 protein overexpression and is preserved in lymph node metastases.

Localized peripancreatic plasma cell Castleman disease.

Castleman disease (CD) is a rare, benign, and usually systemic lymphoproliferative disorder. Unicentric Castleman disease of the pancreas is extremely rare, with only less than 10 cases described in the literature. We describe a case of an isolated peripancreatic localization of a plasma cell-type Castleman disease, its clinical presentation, the diagnostic evaluation, and the cure of disease by surgical excision.