Submucosal Location Research Articles

Intra-operative lymphatic mapping and sentinel node biopsy in laryngeal carcinoma using radiotracer injection.

The purpose of this study was to determine the value of sentinel lymph node biopsy (SLNB) in the laryngeal SCC, using intra-operative peri-tumoral injection of Tc-99m-phytate. Patients with biopsy-proven squamous cell carcinoma of the larynx were included. On the day of surgery, after anesthesia induction, suspension laryngoscopy was performed to inject 74MBq/0.4ml Tc-99m-phytate in four aliquots into the sub-mucosal peri-tumoral location. After a 10-min wait, a portable gamma probe was used to locate sentinel nodes. Subsequently, all patients underwent laryngectomy and neck dissection. Both sentinel nodes and non-sentinel nodes were examined using hematoxylin and eosin (H&E) staining. Twenty-six patients with a diagnosis of laryngeal carcinoma were included in the study. The SLN detection rate was 65.4%, with a 100% detection rate in the supraglottic region and a 52.6% detection rate for glottis/transglottic patients. Permanent pathology results showed lymph node involvement in four patients, but only one patient had a negative result in the SLNB, resulting in an overall false negative rate of 25%. The sensitivity of the SLN technique was 75% overall, 100% in the supraglottic region, and 67% in the glottis/transglottic region. The accuracy and feasibility of SLNB may be related to the location of the tumors in the larynx. For supraglottic tumors, the technique seems to be feasible with a low false negative rate. For glottis/transglottic tumors, both the detection rate and false negative rate seem to be suboptimal. Further studies are needed to validate our results.

CT findings in idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) and comparison to other colitides.

The purpose of this study is to determine computed tomography (CT) findings that aid in differentiating idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) from other colitides. Retrospective review of histiologic proven cases of IMHMV (n = 12) with contrast enhanced CT (n = 11) and/or computed tomography angiography (CTA) (n = 9) exams. Control groups comprised of CT of infectious colitis (n = 13), CT of inflammatory bowel disease (IBD) (n = 12), and CTA of other colitides (n = 13). CT exams reviewed by 2 blinded gastrointestinal radiologists for maximum bowel wall thickness, enhancement pattern, decreased bowel wall enhancement, submucosal attenuation value, presence and location of IMV occlusion, peripheral mesenteric venous occlusion, dilated pericolonic veins, subjective IMA dilation, maximum IMA diameter, maximum peripheral IMA branch diameter, ascites, and mesenteric edema. Presence of early filling veins was an additional finding evaluated on CTA exams. Statistically significant CT findings of IMHMV compared to control groups included greater maximum bowel wall thickness, decreased bowel enhancement, IMV occlusion, and peripheral mesenteric venous occlusion (p < 0.05). Dilated pericolonic veins were seen more frequently in IMHMV compared to the infectious colitis group (64% versus 15%, p = 0.02). Additional statistically significant finding on CTA included early filling veins in IMHMV compared to the CTA control group (100% versus 46%, p = 0.008). IMHMV is a rare chronic non-thrombotic ischemia predominantly involving the rectosigmoid colon. CT features that may aid in differentiating IMHMV from other causes of left-sided colitis include marked bowel wall thickening with decreased enhancement, IMV and peripheral mesenteric venous occlusion or tapering, and early filling of dilated veins on CTA.

Duodenal gangliocytic paraganglioma: A case report

Introduction: Duodenal gangliocytic paraganglioma (DGP) is a benign tumor that only warrants a surgical resection in the majority of cases. Case Report: We are reporting a case of 42-year-old man who consulted for significant weight loss, fatigue, anemia, and an on–off obstructive jaundice. Imageries showed a 5 cm peri-ampullary duodenal mass. We couldn’t have a diagnosis by endoscopic ultrasound (EUS), so we did a laparoscopic transduodenal tumorectomy and removed the entire mass, which came back as gangliocytic paraganglioma on pathology and immunohistochemistry. Gangliocytic paraganglioma is a benign tumor that most commonly occurs in men and is localized in duodenum. Neuroendocrine tumor, ganglioneuroma, paraganglioma, and schwannoma are the differential diagnoses. Histologic diagnosis is difficult to make. Immunohistochemistry is indispensable for the diagnosis. Often than not, we cannot make diagnosis by a simple biopsy, because the tumor has a submucosal location and three types of cells need to be present for diagnosis: spindle cells, ganglion cells, and epithelial cells. That’s why en-bloc resection of the tumor is frequently needed for accurate diagnosis. Endoscopic resection or laparoscopy is used depending on the characteristics of the tumor. In our case, the 5 cm peri-ampullary tumor warranted a laparoscopic resection. Generally, there is no role for adjuvant therapy in duodenal gangliocytic paraganglioma (DGP). But sometimes, radiotherapy, surgery, somatostatin analog are used for treatment depending on the features of DGP. There is no consensus on follow-up management, but experts agree on the necessity of frequent follow-ups. Conclusion: We believe it is important to include gangliocytic paraganglioma (GP) as a differential diagnosis in patients who present with duodenal masses, weight loss, and general state alteration.

Neuroendocrine carcinoma of the minor papilla with pancreas divisum: a case report and review of the literature

BackgroundNeuroendocrine tumors of the minor papilla are very rare, and only 20 cases have been reported in the literature. Neuroendocrine carcinoma of the minor papilla with pancreas divisum has not been reported previously, making this the first reported case. Neuroendocrine tumors of the minor papilla have been reported in association with pancreas divisum in about 50% of cases reported in the literature. We herein present our case of neuroendocrine carcinoma of the minor papilla with pancreas divisum in a 75-year-old male with a systematic literature review of the previous 20 reports of neuroendocrine tumors of the minor papilla.Case presentationA 75-year-old Asian man was referred to our hospital for evaluation of dilation of the main pancreatic duct noted on abdominal ultrasonography. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed a dilated dorsal pancreatic duct, which was not connected to the ventral pancreatic duct; however, it opened to the minor papilla, indicating pancreas divisum. The common bile duct had no communication with the pancreatic main duct and opened to the ampulla of Vater. A contrast-enhanced computed tomography scan showed a 12-mm hypervascular mass near the ampulla of Vater. Endoscopic ultrasonography showed a defined hypoechoic mass in the minor papilla with no invasion. The biopsies performed at the previous hospital found adenocarcinoma. The patient underwent a subtotal stomach-preserving pancreaticoduodenectomy. The pathological diagnosis was neuroendocrine carcinoma. At the 15-year follow-up visit, the patient was doing well with no evidence of tumor recurrence.ConclusionIn our case, because the tumor was discovered during a medical check-up relatively early in the course of disease, the patient was doing well at the 15-year follow-up visit, with no evidence of tumor recurrence. Diagnosing a tumor of the minor papilla is very difficult because of the relatively small size and submucosal location. Carcinoids and endocrine cell micronests in the minor papilla occur more frequently than generally thought. It is very important to include neuroendocrine tumors of the minor papilla in the differential diagnosis of patients with recurrent pancreatitis or pancreatitis of unknown cause, especially for patients with pancreas divisum.

Role of endoscopic ultrasound-guided tissue acquisition for the diagnosis of gastric wall thickening: a retrospective study with meta-analysis.

Tissue acquisition from a thickened gastric wall using biopsy forceps may not always lead to diagnosis, given the submucosal location of the pathology. Endoscopic ultrasound (EUS)-guided tissue acquisition (TA) may serve as a minimally invasive diagnostic tool in such cases. Hence, we aimed to assess the diagnostic outcome and safety of EUS-TA from thickened gastric walls. Data from patients with gastric wall thickening undergoing EUS-TA at 5 tertiary care centers from August 2020 to August 2022 were retrospectively analyzed. These data were pooled with studies obtained from a comprehensive search of Medline, Embase and Scopus from January 2000 to November 2022 and a meta-analysis was performed. Pooled event rates were calculated using an inverse variance model. The search strategy yielded 13 studies that were combined with data from 30 patients from our centers; a total of 399 patients were included in the analysis. The pooled rate of sample adequacy was 94.1% (95% confidence interval [CI] 90.0-98.2), while the pooled rate of diagnostic accuracy was 91.3% (95%CI 87.0-95.5). The pooled sensitivity and specificity for diagnosing malignant lesions with EUS-TA from gastric wall thickening were 94.8% (95%CI 91.3-97.2) and 100% (95%CI 93.6-100), respectively. There were no reported adverse events in any of the studies. EUS-TA offers a safe and accurate diagnostic modality for the etiological diagnosis of thickened gastric walls. Further research is required to identify the needle type and optimal technique for improving outcomes.

Risk factors for perforation during colorectal endoscopic submucosal dissection

AbstractEndoscopic submucosal dissection (ESD) achieves higher en bloc resection rates than endoscopic mucosal resection in early colorectal neoplasms. However, perforation occurs more frequently during ESD. We aimed to identify the risk factors for perforation during colorectal ESD. A total of 161 patients treated with colorectal ESD at a tertiary center between January 2014 and December 2019 were enrolled in this retrospective study. Perforation was defined as a deep defect of the muscularis propria, with or without direct contact with connective tissue outside the colon. Clinical risk factors for perforation during ESD, including age, sex, tumor morphology, tumor size, tumor location, procedure time, en bloc resection rate, histology, and submucosal fibrosis, were analyzed. The mean size of the ESD specimens was 38.2 ± 24.6 mm. The overall en bloc resection rate was 98.1%. Perforations occurred during ESD in 31 out of 161 patients (19.3%). All perforations were successfully treated with endoscopic closure using the hemoclips and nonsurgical management. Emergency surgery was not required in our patients. On multivariate analysis, severe submucosal fibrosis (odds ratio [OR] 3.06; 95% confidence interval [CI] 1.23‐7.59; P = .016) and location in the right colon (OR 4.54; 95% CI 1.31‐15.79; P = .017) were independent risk factors. Perforation during colorectal ESD occurred in 31 patients (19.3%), but all patients had a good outcome without surgery. Submucosal fibrosis and its location are risk factors for perforation during ESD.

Jejunal Intussusception Secondary to Large Inflammatory Fibroid Polyp

Abstract Introduction/Objective Inflammatory fibroid polyp (IFP) is a benign mesenchymal lesion that can arise throughout the gastrointestinal tract but are most common in the stomach, followed by the ileum and more rarely in the duodenum and jejunum. Clinical manifestations include intestinal obstruction, abdominal pain, intussusception, and bleeding. Histologically these are characterized by submucosal location, bland spindle stromal cells, abundant eosinophils and lack of lineage specific markers. Methods/Case Report We report the case of a 68-year-old woman with history of recurrent small bowel obstruction due to Crohn’s disease who presented with jejunal intussusception that required ileocolic resection. Gross examination revealed a 4.2 cm well-circumscribed, lobulated mass with a gray-white and fleshy cut surface. Histologic sections showed a submucosal, nodular proliferation of bland spindle cells embedded within a loose, edematous stroma associated with abundant eosinophils. No hypercellularity, necrosis, hemorrhage, or mitotic figures were identified. Immunohistochemical studies revealed that the lesional cells were positive for vimentin and negative for desmin, SMA, S100, CD1a, CD117, DOG1, ALK, Melan-A, HMB-45, CD34, and STAT6. Ki-67 proliferative index was &amp;lt;1%. The mass was classified as an IFP. Results (if a Case Study enter NA) NA Conclusion IFPs usually express CD34 and negative cases have been described as atypical IFPs. Activating mutations of PDGFRA appear to define this lesion molecularly and are responsible for the recruitment of eosinophils. PDGFRA mutations have been detected in both CD34 positive and negative cases suggesting they represent variants of the same entity. The putative origin of this lesion is hypothesized to be a distinct type of stromal cell: the telocyte. Surgical excision is curative for symptomatic IFP.

Long term follow-up of uterine fibroids treated with microwave ablation: an up to 3-year observational study of volume, regrowth, and symptoms

Objective To study long-term effects in patients treated with microwave ablation (MWA) for symptomatic uterine fibroids and investigate fibroid characteristics predictive of successful treatment. Method Women who received MWA treatment for uterine fibroids in a previous study were included. A total of 16 patients underwent contrast enhanced MRI before treatment, postoperatively at 6 months and at long-term follow-up, to assess volumes of treated fibroids (n = 42). Long-term MRI was performed between 16 and 36 months after treatment [median 22 months, interquartile range (IQR) 18.5–27]. Validated questionnaires for evaluation of uterine fibroid symptoms and menstrual bleeding (UFS-QoL and PBAC) were used to assess long-term effects on symptoms. The degree of shrinkage was correlated to vascularization and T2 signal intensity (SI) at preoperative MRI and location of fibroids according to the FIGO classification, using the Mann–Whitney U test. Results Most patients (82%) reported improvement up to 3 years after treatment. Out of 42 treated fibroids, 35 (83%) continued to shrink over time with median relative volume reduction of 77% (IQR 39–95). For eight fibroids (19%) which showed low vascularization on the pretreatment MRI, there was less shrinkage compared to well-vascularized fibroids (p = 0.01). Most fibroids (79%) showed iso- to hyperintense T2 signal on preoperative MRI and showed a higher grade of shrinkage than hypointense fibroids (p = 0.02). Conclusion After microwave treatment improvement is maintained for most patients up to 36 months and most fibroids showed continuous shrinkage. Preoperative vascularization, high T2 SI and submucosal location predicted continuous volume reduction. However, to confirm this, larger studies are needed.

Клинические и морфологические особенности простой и клеточной миомы

Introduction. The current trend of shifting the time of delivery to an older age requires the improvement of methods, algorithms for diagnosis, and choice of management for organ-preserving treatment of uterine fibroids. There are few reports in which data obtained using preoperative imaging methods are compared with the results of histological examination of leiomyomata. The aim of the study was to investigate the features of morphotype and clinical course of the disease in patients with common and cellular uterine leiomyoma. Materials and methods. We divided 80 patients with uterine leiomyoma into 2 groups after comprehensive clinical and morphological examination and treatment. Group 1 involved 50 patients with conventional uterine leiomyoma; group 2 included 30 patients with cellular uterine leiomyoma. All patients had a patient-specific personalized three-dimensional mathematical and graphical model of uterine fibroids built before surgery. Leiomyomata were subjected to a standard pathological examination with hematoxylin and eosin staining, immunohistochemical study with Ki-67 antibody, and Van Gieson picrofucsin to assess fibrosis. Results. The frequency of dysmenorrhea, menorrhagia, primary infertility, and endometrial hyperplastic processes significantly exceeded (p&lt;0.05) those in the group of patients with cellular myoma. In 64.6% of patients of group 2, the indications for surgery were location of myomatous nodes and their enlargement; in group 1, the percentage of such patients was 26.0% (p&lt;0.05). Large uterine fibroids and multiple locations of nodes were detected in more than 50% of patients in group 2, while those in patients of group 1 were determined only in 14%. In patients with conventional leiomyoma, the Ki-67 index was 0–1–2%, whereas in patients with cellular leiomyoma it was 3–4–5%. In group 1, the fibrosis area was 20–40%; in group 2, this indicator reached up to 10%. Conclusion. Uterine cell myoma is characterized by rapid growth, large node sizes, a higher frequency of both submucosal location and infertility, a combination with hyperplastic endometrial processes, uterine bleeding, pronounced pain syndrome, and a younger age. Keywords: uterine leiomyoma, conventional uterine leiomyoma, cellular uterine leiomyoma, 3D modeling, surgical navigation

Uterine Artery Embolization of Uterine Leiomyomas: Predictive MRI Features of Volumetric Response

OBJECTIVE. The purpose of this article was to evaluate MRI features of uterine leiomyomas that predict volumetric response after uterine artery embolization (UAE). MATERIALS AND METHODS. This retrospective study included 75 patients with 212 uterine leiomyomas who were successfully treated between August 2013 and December 2018. To predict uterine volumetric response, age, number of lesions, and baseline uterine volume were assessed. To predict leiomyoma volumetric response, a multivariate regression analysis was performed to evaluate six predictive factors: location, baseline leiomyoma volume, signal intensity on T1-weighted and T2-weighted MRI, heterogeneity of signal intensity on T2-weighted MRI, and vascularity on subtraction imaging (SI). A five-variable predictive ROC model was developed to evaluate the diagnostic accuracy of the signal intensity ratio on T2-weighted MRI, enhancement ratio, heterogeneity ratio on T2-weighted MRI, location, and baseline leiomyoma volume in predicting at least 40% leiomyoma volumetric response. RESULTS. Age, number of leiomyomas, and baseline uterine volume were not predictive of uterine volumetric response. A submucosal location was the best predictive factor of leiomyoma volumetric response, and it showed 32.2% more leiomyoma volumetric response compared with a nonsubmucosal location (p < .001). Hyperintensity on T2-weighted MRI was the second best predictive factor of leiomyoma volumetric response, and it showed 16.9% more volumetric response compared with hypointense leiomyomas (p = .013). A small baseline leiomyoma volume (< 58 cm3) was associated with 10.2% more leiomyoma volumetric response compared with larger leiomyomas (p = .01). Leiomyomas that were hyperintense on SI showed 7.9% more leiomyoma volumetric response compared with those that were hypointense (p = .014). The five-variable ROC model showed high diagnostic accuracy with an AUC of 0.85, sensitivity of 82%, and specificity of 71%. CONCLUSION. A submucosal location, hyperintensity on T2-weighted MRI, small baseline leiomyoma volume (< 58 cm3), and hyperintense leiomyoma on subtraction imaging are the main independent favorable predictors of leiomyoma volumetric response after UAE. An accurate predictive ROC model was developed that may help in selecting patients suitable for UAE. Quantitative assessment of heterogeneity on T2-weighted MRI showed promising results as a predictor of volumetric response, and further research in this area using texture analysis and radiomics is suggested.

Imaging of Malignant Minor Salivary Gland Tumors of the Head and Neck.

Minor salivary gland carcinomas (MSGCs) are nonminor tumors in the head and neck region and account for about half of all salivary gland carcinomas. Imaging evaluation based on the anatomy of minor salivary and mucous glands in the head and neck region as well as invasion patterns in each site helps in identifying optimal treatment modalities and planning suitable treatment strategies. MSGCs can be divided radiologically into localized and invasive subtypes on the basis of the clinical utility of such categorization. Characteristic invasion patterns of the invasive type include deep submucosal extension, bone marrow infiltration, and perineural spread, which are difficult to assess clinically. MSGCs easily invade adjacent structures because of their submucosal location and may spread along the muscles, nerves, periosteum, and dura mater. Moreover, the tumor may spread into the bone marrow without obvious bone destruction. In addition to imaging classification, examining the anatomy and distribution of the minor salivary glands, including the palatal, lingual, buccal, labial, and retromolar glands, as well as other mucous glands, including the glands of the sinonasal cavity and nasopharynx, lacrimal glands, ceruminous glands, laryngeal glands, and tracheal glands, facilitates MSGC diagnosis and tumor extension assessment. The authors review the precise anatomy of the minor salivary and mucous glands in the head and neck region, discuss the MSGC imaging classifications, and describe how to evaluate the extent of MSGCs in each site on the basis of the imaging classification and invasion patterns. ©RSNA, 2020.

S3038 Peri-Gastric Venous Hemangioma: A Tale of Discordant Cytology and Histology on EUS-Core Biopsy

INTRODUCTION: Hemangiomas are highly vascular tumors, without known malignant potential. They are extremely rare to arise from the upper gastrointestinal (GI) tract and may present with variable symptoms. Due to low sensitivity of radiological imaging, their diagnosis in the GI tract is often challenging. We present a case of a peri-gastric hemangioma adequately diagnosed with endoscopic ultrasound (EUS)-guided biopsy. CASE DESCRIPTION/METHODS: A 48-year old lady with hypertension and diabetes mellitus, sought care for a 3-month history of left upper quadrant (LUQ) abdominal pain, with normal hemoglobin and comprehensive metabolic panel. Abdominal computed tomography (CT) demonstrated a 7cm lobulated, exophytic LUQ mass suspected to be arising externally from the stomach, raising suspicion for gastrointestinal stromal tumor (GIST). She was referred to us for EUS, which revealed a 8.9 × 5.5 cm heterogeneously hypoechoic, irregularly marginated mass, thought to be exophytic and abutting the proximal portion of the stomach. Core biopsies were obtained, and sent for both cytology (in CytoLyt) and histology (in Formalin). The cytology showed proliferation of bland spindle cells concerning for spindle cell neoplasm, however, the histology demonstrated abnormal venous structures with prominent spindle cell smooth muscle walls and luminal CD34 + endothelial cells supporting the diagnosis of benign venous hemangioma. The patient remained symptomatic with LUQ discomfort and underwent surgical resection. Final post-operative pathology confirmed the diagnosis of venous hemangioma. DISCUSSION: Contrast has higher specific gravity than blood and there is slow blood flow within hemangiomas; therefore, radiographic imaging may be inconclusive as hemangiomas may appear as non-specific heterogeneous lesions. EUS can determine the anatomy of the lesion, its origin and vascularity. Tissue biopsies are challenging due to the high vascularity of these lesions and their submucosal location, but histology can provide a definitive diagnosis. A few cases of endoscopic resection of intraluminal small venous and cavernous hemangiomas have been reported, but surgical resection is the most common treatment modality for symptomatic extra-luminal GI tract venous hemangiomas. Our case educates endoscopists that histopathological evaluation with immunohistochemistry staining is more accurate than cytological analysis for diagnosis of venous hemangiomas, and hence core sample should be sent in formalin rather than for cytology alone.Figure 1.: Histology of a peri-gastric mass showing (A) abnormal venous structures with prominent spindled smooth muscle walls and luminal CD34 positive benign endothelial cells (inset). The lesion signed out as venous hemangioma. Concurrent cytology (B) showed proliferation of bland spindle cells raising concern for spindle cell neoplasm.

S3393 Granular Cell Tumor of the Colon - Surprise Beneath the Mucosa!

INTRODUCTION: Granular Cell Tumors (GCT) are a rare soft tissue neoplasm that arise from Schwann cells. Even though these tumors are generally benign, 1-2% of the cases have been reported to be malignant. Although usually found in the oral cavity or subcutaneous tissue, 8% of cases manifest in the gastrointestinal (GI) tract. In those patients, the esophagus is the most frequently affected organ, followed by the duodenum, anus, stomach. The least common sites are the colon and rectum. To date ∼ 150 cases of Colonic GCT have been reported in the English literature. Most GCT tend to be asymptomatic and less than 2cm in size, hence they are usually discovered incidentally during routine colonoscopy or abdominal imaging. Herein we report a unique case of GCT of the ascending colon measuring 11mm in size in a patient presenting with constipation that was treated successfully with endoscopic resection. CASE DESCRIPTION/METHODS: A 55-year old male with a history of external hemorrhoids presented to the GI clinic with constipation for two weeks. His previous colonoscopy eight months prior revealed a one cm polyp in the ascending colon, but it was not removed due to poor preparation. Repeat Colonoscopy revealed an 11 mm semi pedunculated polyp in the ascending colon. The polyp was removed with hot snare polypectomy followed by the placement of hemostatic clips. (Figure#1). Histopathologic examination of the resected polyp showed an intramucosal and submucosal tumor composed of polygonal cells with abundant amphophilic granular cytoplasm and centrally or eccentrically located small round or irregularly shaped nuclei (Figure#2). Margins of the resected polyp were free of tumor. Immunohistochemical analysis showed tumor cells were positive for S100 (Figure#3), CD68, and inhibin and negative for CD117 and neurofilament. On the basis of the above findings, the tumor was diagnosed as GCT. He followed up with a gastroenterologist as an outpatient one month later and had reported no symptoms. DISCUSSION: Colonic GCT are usually discovered at the time of colonoscopy as small, sessile, yellowish-white nodules or polyps covered by normal-appearing mucosa. Definitive diagnosis can be made by histopathology and immunohistochemical analysis. Endoscopic removal is the safest and most effective treatment. Though typically benign, gastroenterologists should be aware of malignant features such as a size greater than 4cm or rapid growth. It is important to consider GCT when one encounters a subepithelial lesion during colonoscopy.Figure 1.: A-11mm semi-pedunculated polyp in the ascending colon B-Prophylactic application of resolution clip at the polyp removal site.Figure 2.: The morphology of granular cell tumor in intramucosal and focally submucosal location in a colon polyp (H&E: 10x). Infiltrative growth pattern with nested or trabecular cellular clusters or sheets are appreciated. The cells are with abundant amphophilic granular cytoplasm and centrally or eccentrically located small round or irregularly shaped nuclei. No necrosis or mitosis is appreciated.Figure 3.: S100 positive staining highlighted the infiltrating trabecular growth pattern of this granular cell tumor (H&E: 10x).

Adenocarcinoma arising from a heterotopic pancreas in the first portion of the duodenum: a case report

BackgroundHeterotopic pancreas (HP) is defined as pancreatic tissue in organs with no anatomical continuity with the orthotopic pancreas. Based on the number of cases reported in the literature between the year 2000 and 2020, HP is rarely found causing malignant transformation of the duodenum. We herein report a case of adenocarcinoma arising from the HP in the first portion of the duodenum.Case presentationA 77-year-old Japanese man presented to our hospital with epigastric pain. Despite having undergone laparoscopic surgery for early sigmoid colon cancer a month earlier, serum levels of tumor-specific antigens, such as CA19-9, were elevated. After undergoing a series of radiologic examinations, the first portion of the duodenum was found thickened. However, a biopsy of the lesion showed no malignancy. Four months later, follow-up computed tomography (CT) scans showed that the lesion was thicker and involved the gastroduodenal artery (GDA), suggesting tumor invasion. A new biopsy did not detect the malignancy. However, serum tumor-specific antigen levels increased, especially duke pancreatic monoclonal antigen type 2 (5287 U/mL), in the absence of tumor in the orthotopic pancreas. The follow-up CT imaging showed a malignant tumor in the first portion of the duodenum. Five months later, we performed a subtotal stomach-preserving pancreaticoduodenectomy (SSPPD) for duodenal or HP cancer in the first portion of the duodenum, finding a lesion from the pyloric bulbs to the first portion of the duodenum, which invaded the adjacent pancreas and GDA. The pathological examination of the specimens revealed adenocarcinoma arising from HP. Nine months after surgery, no recurrence was found by radiologic imaging or tumor-specific antigen laboratory testing.ConclusionsHP adenocarcinoma is rare and difficult to diagnose preoperatively due to its submucosal location. Therefore, a careful follow-up with blood testing and radiologic imaging, as well as diagnostic surgery, is recommended.

A174 RARE BUT DEADLY: AN APPROACH TO MANAGEMENT OF DUODENAL VARICEAL HEMORRHAGE

Abstract Background Common etiologies of upper gastrointestinal bleeding (UGIB) in cirrhotic patients with portal hypertension include gastroesophageal varices (GOV), portal hypertensive gastropathy (PHG) and gastric antral vascular ectasia (GAVE). Less commonly, patients with portal hypertension develop varices in ectopic sites, including the rectum, biliary tree and duodenum. Ectopic varices are rare, contributing to 1–5% of all variceal bleeding, of which 17% is from duodenal varices. Aims To describe the management of duodenal variceal hemorrhage. Methods A case report and literature review was performed. Results Case report: We present a case of recurrent UGIB in a 59-year-old male with decompensated cirrhosis due to non-alcoholic steatohepatitis (CP-C; Meld 14). Initial endoscopy was negative for GOV, peptic ulcer, PHG, and GAVE, but an erosion over a mucosal bulge in the third segment of the duodenum was identified, raising possibility of vascular malformation versus ectopic varix. There was active bleeding after water provocation, so clips were deployed for hemostasis. CT imaging showed mesenteric venous collaterals abutting the duodenum, again raising possibility of duodenal varix, which was ultimately confirmed by endoscopic ultrasound (EUS). Patient had recurrent overt bleeding despite beta-blockage for prophylaxis, endoscopic clipping on four separate occasions, attempted angioembolization by interventional radiology, and cyanoacrylate glue. Transjugular intrahepatic porto-systemic shunt (TIPS) was not possible due to portal vein occlusion, so he underwent EUS-guided cyanoacrylate glue a second time. Literature review: Ectopic varices are rare, contributing to 1–5% of all variceal bleeding, of which 17% is from duodenal varices. Duodenal variceal hemorrhage can lead to hemorrhagic shock, and is potentially life threatening, with quoted mortality rates of 40%. Unfortunately, duodenal varices can be difficult to identify. Diagnosis is often delayed due to a combination of lower awareness and endoscopic challenges given the unusual serosal and submucosal location. Evidence-based guidelines for the management of ectopic varices are limited. For this reason, our current management strategies rely heavily on local expertise. Splanchnic vasoconstrictor medication, endoscopic ligation, EUS guided gluing, interventional radiology guided embolization, TIPS, balloon retrograde transvenous obliteration and surgical shunts are potential therapeutic options to manage the acutely bleeding varix. Following a variceal bleed, liver transplantation should be considered in eligible patients with no other contraindications. Conclusions Duodenal varices are a rare, potentially fatal, and underrecognized cause of gastrointestinal bleeding in patients with portal hypertension. Definitive therapy currently relies upon local expertise in the absence of clear guideline-based therapy. Funding Agencies None

Improving target localization during trans-oral surgery with use of intraoperative imaging.

Trans-oral surgery provides a less invasive means for the surgical management of upper aerodigestive tract malignancies but is limited in its ability to readily assess submucosal tumor extent and location of critical structures intraoperatively. We sought to determine surgeons' baseline target localization accuracy during operative laryngoscopy with preoperative imaging alone and then assess for improvement in localization accuracy when presented with intraoperative CT imaging capturing soft tissue deformation. Fiducial beads were placed submucosally in four cadaver heads. "Preoperative" (PO) and "intraoperative" (IO) neck CTs were acquired before and during suspension laryngoscopy using a CT-compatible laryngoscopy system. Surgeons attempted to localize submucosal fiducials beads using pins based on sequential review of PO and IO images. Mean total error (TE) decreased from 12.8 ± 9.9 to 10 ± 7.5mm from PO to IO (P < 0.001), respectively. TE for base of tongue and vallecula decreased by 1.7 ± 6.7mm (P = 0.015). Right-sided structures were most exposed by scope positioning and experienced a TE reduction of 4.8 ± 9.3mm (P < 0.001). Task completion time decreased from PO to IO by 26% (P < 0.001). Intraoperative imaging significantly improves localization accuracy and task efficiency when targeting submucosal beads in cadaver heads during operative laryngoscopy.

Gastric tuberculosis presenting as non healing ulcer: A case report

Tuberculosis is a major health problem in India. Gastrointestinal tuberculosis is the sixth most common causes of extrapulmonary tuberculosis and it mostly involves the ileocaecal region. Primary gastric tuberculosis in immunocompetent person is very rare. Stomach as its site is rare and is the sixth most common site of gastrointestinal tuberculosis. It mostly presents as a cases of non healing ulcer or gastric outlet obstruction. Yield of endoscopic biopsies for granuloma is low due to submucosal location of these lesions and mostly they are diagnosed after surgical intervention. We report a case of isolated gastric tuberculosis in a middle age immunocompetent female who present as a cases of non healing ulcer and responded well to standard antitubercular treatment. A high index of its suspicion should be kept in mind in any chronic infiltrative lesions of stomach like non healing ulcers and gastric outlet obstruction for its early diagnosis and treatment.

Arteriovenous Malformation with Thrombosis Formation in the Jugal Mucosa: a Clinical Case

Arteriovenous malformation (AVM) is a congenital disorder of blood vessels, causing abnormal passage of blood between the artery and vein, which most often results in abnormal development of the vascular system in embryonic life. Thrombosis is the clotting of blood within the vessels caused by platelet aggregation. We present the case of a 22-year-old white woman, who complained of facial asymmetry on the left side, painless, with a 6-month evolution. Clinical examination revealed a palpable nodule measuring approximately 25 mm, soft, painless, of submucosal location; thus, the initial hypothesis was lipoma. Ultrasonography was requested, which indicated the hypothesis of a vascular lesion. The patient was submitted to excisional biopsy, preceded by puncture and aspiration (white puncture). The microscopic diagnosis was of arteriovenous malformation with thrombus formation, and an excisional biopsy resolved this case. The patient has been followed for 3 months and is without signs of relapse.

Clinicopathologic Features of Submucosal Esophageal Squamous Cell Carcinoma

The prognoses of submucosal esophageal squamous cell carcinoma patients vary. Patients with favorable prognoses may receive less invasive or nonsurgical interventions, whereas patients with poor prognoses or advanced esophageal cancer may require aggressive treatments. We sought to identify prognostic factors for patients with submucosal esophageal squamous cell carcinoma, focusing on lymph node metastasis and recurrence. We included 137 submucosal esophageal squamous cell carcinoma patients who had undergone transthoracic esophagectomy with systematic extended lymph node dissection. Submucosal tumors were classified as SM1, SM2, and SM3 according to the depth of invasion. Prognostic factors were determined by univariable and multivariable analyses. Lymph node metastasis was observed in 18.8%, 30.5%, and 50.0% of SM1, SM2, and SM3 cases, respectively. The overall 5-year recurrence rate was 21.9%; the rates for SM1, SM2, and SM3 tumors were 9.4%, 18.6%, and 34.8%, respectively. The SM1 tumors all recurred locoregionally; distant metastasis occurred in SM2 and SM3 cases. The 5-year overall survival rates were 83%, 77%, and 59% for SM1, SM2, and SM3 cases, respectively. On univariable analysis, lymph node metastasis, depth of submucosal invasion (SM3 versus SM1/2), and tumor location (upper thoracic versus mid/lower thoracic) were poor prognostic factors for overall survival. Multivariable Cox regression analyses identified depth of submucosal invasion(hazard ratio 2.51, 95% confidence interval: 1.37 to 4.61) and tumor location (hazard ratio 2.43, 95% confidence interval: 1.18 to 4.63) as preoperative prognostic factors. Tumor location (upper thoracic) and infiltration (SM3) are the worse prognostic factors of submucosal esophageal squamous cell carcinoma, but lymph node metastasis is not a predictor of poorer prognosis.

Duodenal Gangliocytic Paraganglioma: A Rare Neuroendocrine Tumor

Gangliocytic paragangliomas, predominantly found in the second part of duodenum, are rare, usually benign tumors with occasional lymph node metastasis. Clinical presentation varies from incidental finding upon endoscopy, or abdominal pain and upper gastrointestinal bleeding. A 73-year old woman with past medical history of non-Hodgkin's lymphoma, presented with intermittent post-prandial epigastric pain, radiating to right upper quadrant, without any weight loss or melena. Physical examination was unremarkable. Laboratory analysis was normal. Cross-sectional imaging of abdomen revealed a possible duodenal diverticulum. Esophagogastroduodenoscopy revealed 17mm, sessile polyp in second part of duodenum. Endoscopic ultrasound (EUS) showed a hypoechoic mass, measuring 17mm x 13mm, extending from mucosa to submucosa and having well-defined margins. EUS-guided fine needle aspiration (FNA) cytology was consistent with a neuroendocrine tumor with immunohistochemical stains positive for synaptophysin, chromogranin and CD56 antibodies. A decision was made to proceed with endoscopic mucosal resection (EMR) which revealed a hypoechoic intramural lesion, appearing to originate from submucosa, overlaid by focal ulcerating flattened duodenal mucosa. The pathology was remarkable for variable admixture of bundles of spindle cells, clusters of ganglion cells and epithelial cells. Immunohistochemical stains showed positive S100 protein in the spindle cells; the epithelioid cells were positive for synaptophysin and CD56 consistent with neuroendocrine differentiation; and the ganglion cells positive for calretinin. Gangliocytic paragangliomas, first reported by Dahl et al. in 1957, is a peculiar neuroendocrine tumor thought to be derived from neural crest with 88% reported lesions located in the second part of duodenum. These tumors are benign and consist of variable admixture of spindle cells, epithelioid cells, and ganglion cells. Rare malignant cases have been reported with regional lymph node metastasis. The epithelioid component was found to be present in the patients with lymph node metastasis; the causal relationship of which is unclear. Due to the submucosal location of the tumor, diagnosis is difficult on endoscopic biopsy which emphasizes the role of EMR in the diagnosis. As most of these tumors are benign and non-functional, EMR is therefore the treatment of choice especially in cases in which the tumor may be removed in its entirety with negative margins.Figure: A 17mm x 13mm, hypoechoic intramural lesion, appearing to originate from submucosa.Figure: Duodenal mass showing variable admixture of ganglion cells, spindle cells, and epithelioid cells (arrow shows ganglion cell). H&E X 100.Figure: Well-controlled immunostain with antibody to synaptophysin shows strong positivity for epithelioid cells. Synaptophysin X 100.