Clinicopathological Features and Outcomes of Gastrointestinal Stromal Tumours in Oman: A multi-centre study.

OBJECTIVES: This study aimed to report the clinicopathological features, management and long-term outcomes of patients with gastrointestinal stromal tumours (GISTs) in Oman. METHODS: This retrospective study was conducted on patients treated for GIST between January 2003 and December 2017 at three tertiary referral centres in Muscat, Oman. All patients with confirmed histopathological diagnoses of GIST and followed-up at the centres during this period were included. Relevant information was retrieved from hospital records until April 2019. RESULTS: A total of 44 patients were included in the study. The median age was 55.5 years and 56.8% were female. The most common primary site of disease was the stomach (63.6%) followed by the jejunum/ileum (18.2%). Two patients (4.5%) had c-Kit-negative, discovered on GIST-1-positive disease. A total of 24 patients (54.5%) presented with localised disease and eight (33.3%) were classified as being at high risk of relapse. Patients with metastatic disease received imatinib in a palliative setting, whereas those with completely resected disease in the intermediate and high-risk groups were treated with adjuvant imatinib. Of the six patients (13.6%) with progressive metastatic disease, of which four had mutations on exon 11 and one on exon 9, while one had wild-type disease. Overall, rates of progression-free survival and overall survival (OS) at 100 months were 77.4% and 80.4%, respectively. Rates of OS for patients with localised and metastatic disease were 89.9% and 80.2%, respectively. CONCLUSION: The presenting features and outcomes of patients with GISTs in Oman were comparable to those reported in the regional and international literature.

Pneumorrhachis with Spontaneous Pneumomediastinum: Should It Raise Special Concerns?

Spontaneous pneumomediastinum (SPM) is a rare and benign condition predominantly seen in young males. Patients present with non-specific pleuritic chest pain, cough, and dyspnea. Hence, it is difficult to diagnose in the acute setting until other serious differential diagnoses involving the integrity of the aerodigestive tract have been excluded. This results in over investigation and overtreatment until a diagnosis has been confirmed. We report a case of a 21-year-old Omani female who presented with a complaint of cough. She was diagnosed based on clinical and radiological findings after exclusion of hollow viscous perforation to have SPM extending to the spinal canal (pneumorrhachis). Her condition improved following conservative management, and she was sent home after three days.

True Intramural Esophageal Duplication Cyst.

Esophageal duplication is the second most common site of gastrointestinal duplication and most cases present with complications. These complications include bleeding, infection, dysphagia, and dyspnea. We report an incidental case of a true intramural esophageal duplication cyst in a new military recruit. The patient was diagnosed in Armed Forces Hospital, Oman. The patient came for a pre-recruitment routine check-up, he was found to have a suspicious soft tissue lesion on chest X-ray. He was referred to the thoracic surgeon for further investigations. The investigations included computed tomography and magnetic resonance imaging chest scans, barium swallow, endoscopy and, finally, an endoscopic ultrasound. All workup pointed to a diagnosis of esophageal duplication cyst; therefore, the decision was made to excise the lesion after discussion with the patient about the possible diagnosis and nature of the treatment. The cyst was completely excised thoracoscopically with uneventful recovery. The patient was discharged a few days later and was doing well in subsequent visits to the outpatient department. The histopathological exam confirmed the diagnosis of a true congenital duplication cyst, which was lined by pseudostratified ciliated columnar epithelium overlying double layers of thick bundles of smooth muscle fibers.