Pleomorphic leiomyosarcoma of the maxilla with metastasis to the colon: A case report.

BACKGROUND: Pleomorphic leiomyosarcomas make up around 8.6% of all leiomyosarcomas. They behave aggressively and often have poor prognoses. They can affect the gastrointestinal tract and retroperitoneum. To date, pleomorphic leiomyosarcoma involving the mesocolon have been reported in nine patients. CASE SUMMARY: The patient was a 44-year-old man with a history of pleomorphic leiomyosarcoma of the left maxilla with metastasis to the lung and liver. His most recent positron emission tomography-computed tomography (PET-CT) scan showed uptake in the ascending and transverse colons. A colonoscopy revealed a 5.0 cm × 3.5 cm × 3.0 cm pedunculated polyp in the ascending colon. The polyp was removed using hot snare polypectomy technique and retrieved with Rothnet. Histopathologic examination of the polyp showed a metastatic pleomorphic leiomyosarcoma. CONCLUSION: Uptake(s) on PET-CT in a patient with pleomorphic leiomyosarcoma should raise suspicion for metastasis.

Per-oral endoscopic myotomy (POEM) training and skills evaluation tool: a pilot study.

Background and study aims Hands-on training for per-oral endoscopic myotomy (POEM) in the United States is limited and without a structured curriculum or assessment tool. Training to competency in POEM is critical and POEM trainees must acquire multiple cognitive and technical skills to achieve proficiency. The aim of this study was to develop a POEM training and skills evaluation tool. Patients and methods The training protocol included initial explant porcine models followed by live human cases, proctored by a single endoscopist experienced in POEM who prospectively graded trainees for each step ("skill") of the procedure on a 5-point scale. Procedural skills were divided into cognitive and technical skills. Acceptable passing level was considered a score ≥ 4 for each skill. Results Three trainees completed a total of 18 cases (8 cases on animal explant models and 10 human cases). Overall, cognitive skills were acquired early in training with scores of ≥ 4 achieved by ≤ 3 cases. Technical skills required more cases and direction with scores ≥ 4 in technical skills achieved by three porcine and eight human cases. Entry of the endoscope into the submucosal space and submucosal tunneling were the most challenging steps followed by myotomy. Conclusion This pilot study introduces a POEM training and skills evaluation tool for training to competency. Submucosal entry, tunneling, and myotomy were the most challenging to learn while cognitive skills were learned early in training. Evaluation of more trainees at multiple sites will be necessary to further validate the utility of this tool.

Health-related quality of life and long-term outcomes after endoscopic therapy for walled-off pancreatic necrosis.

BACKGROUND AND AIM: Walled-off pancreatic necrosis (WON) frequently develops after necrotizing pancreatitis. Endoscopic drainage has become the preferred modality for symptomatic or infected WON. The aim of the present study was to assess health-related quality of life (HR-QOL) and long-term outcomes in patients undergoing endoscopic drainage for WON. METHODS: Patients undergoing endoscopic drainage of WON from January 2006 to May 2016 were identified. Data recorded included demographic information, and the incidence of long-term sequelae including pancreatic endocrine and exocrine insufficiency. Attempts were made to contact all patients. HR-QOL was assessed using the SF-36 questionnaire. RESULTS: Eighty patients were analyzed, 41 (51.3%) of whom completed the SF-36. One-year all-cause mortality was 6.2%, and disease-related mortality was 3.7%. A notable proportion of patients developed exocrine insufficiency (32.5%), endocrine insufficiency (27.7%), and long-term opiate use (42.5%). Development of exocrine insufficiency was predictive of lower total SF-36 scores (P = 0.016). Patients with WON had better HR-QOL compared with cohorts of irritable bowel syndrome (IBS) and inflammatory bowel disease (IBD). In patients developing exocrine insufficiency versus healthy controls, poorer scores in the physical role (P < 0.001), general health (P < 0.001), vitality (P = 0.001), and emotional role (P = 0.029) domains were observed. Exocrine insufficiency patients had better HR-QOL than the IBS and IBD cohorts, although these differences were less pronounced. CONCLUSION: After undergoing endoscopic drainage for WON, patients have relatively preserved HR-QOL. The subset of patients that develop exocrine insufficiency have significantly poorer HR-QOL compared to healthy controls, although not to the degree of chronic gastrointestinal disorders such as IBS and IBD.