Solid pseudopapillary neoplasm (SPN) is a rare tumor with malignant potential which is generally located in the tail of pancreas. The prevalence of SPN has increased with widespread use of cross sectional imaging. SPN is often misdiagnosed due to nonspecific clinical presentation and accurate diagnosis is essential for optimal management. Endoscopic ultrasound-FNA with immunohistochemistry can help in preoperative diagnosis. Surgery is the treatment of choice and a successful R0 resection is curative. Overall, SPN has a good prognosis. This review article focuses on pathogenesis, diagnosis and management of SPN.

Bone morphogenetic proteins (BMPs) are highly conserved morphogens that are essential for normal development. BMP-2 is highly expressed in the majority of non-small cell lung carcinomas (NSCLC) but not in normal lung tissue or benign lung tumors. The effects of the BMP signaling cascade on the growth and survival of cancer cells is poorly understood. We show that BMP signaling is basally active in lung cancer cell lines, which can be effectively inhibited with selective antagonists of the BMP type I receptors. Lung cancer cell lines express alk2, alk3, and alk6 and inhibition of a single BMP receptor was not sufficient to decrease signaling. Inhibition of more than one type I receptor was required to decrease BMP signaling in lung cancer cell lines. BMP receptor antagonists and silencing of BMP type I receptors with siRNA induced cell death, inhibited cell growth, and caused a significant decrease in the expression of inhibitor of differentiation (Id1, Id2, and Id3) family members, which are known to regulate cell growth and survival in many types of cancers. BMP receptor antagonists also decreased clonogenic cell growth. Knockdown of Id3 significantly decreased cell growth and induced cell death of lung cancer cells. H1299 cells stably overexpressing Id3 were resistant to growth suppression and induction of cell death induced by the BMP antagonist DMH2. These studies suggest that BMP signaling promotes cell growth and survival of lung cancer cells, which is mediated through its regulation of Id family members. Selective antagonists of the BMP type I receptors represents a potential means to pharmacologically treat NSCLC and other carcinomas with an activated BMP signaling cascade.

Unless indicated otherwise by family or personal history, PC screening under the age of 50 is low yield. Linear EUS may be the preferred modality for initial PC screening.

Air embolism is rarely diagnosed and is often fatal. The diagnosis is often not made in a timely manner given the rapid and severe clinical deterioration that often develops, frequently leading to cardiac arrest. Many patients are only diagnosed post-mortem. With the increasing use of endoscopic retrograde cholangiopancreatography, air embolism should be considered in the differential diagnosis in patients who experience sudden clinical deterioration during or immediately after the procedure. Clinical suspicion is key in the diagnosis and management of air embolism. Use of precordial Doppler ultrasound and transesophageal echocardiogram can aid in the diagnosis of air embolism. Once the diagnosis is made, supportive management of airway, breathing and circulation is pivotal. Advanced cardiac life support should be initiated when necessary. Fluid resuscitation and vasopressors can improve cardiac output. Hyperbaric oxygen therapy should be considered when possible in cases of suspected cerebral air embolism cases to improve neurological outcome. A multidisciplinary team approach and effective communication with experts, potentially including an anesthesiologist, cardiologist, intensivist, radiologist and surgeon, can improve the outcome in air embolism.

Gastrointestinal stromal tumors (GISTs) are rare but most common nonepithelial tumor of gastrointestinal tract. They are often found incidentally on computed tomography and endoscopic investigations. Increasing knowledge of the pathogenesis of GISTs and the advent of tyrosine kinase inhibitors revolutionized the management of GISTs. The newer advanced endoscopic techniques have challenged the conventional surgery although the true efficacy and safety of endoscopic approach is not clear at this time. This review article focuses on pathogenesis, diagnosis and management of GISTs.

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BACKGROUND Pancreatic paragangliomas (PPGL) are rare benign neuroendocrine neoplasms but malignancy can occur. PPGL are often misdiagnosed as pancreatic neuroendocrine tumor or pancreatic adenocarcinoma. CASE SUMMARY We reviewed 47 case reports of PPGL published in PubMed to date. Fifteen patients (15/47) with PPGL underwent endoscopic ultrasound-guided fine needle aspiration (EUS-FNA). Only six (6/15) were correctly diagnosed as PPGL. All patients with PPGL underwent surgical resection except three (one patient surgery was aborted because of hypertensive crisis, two patients had metastasis or involvement of major vessels). Our patient remained on close surveillance as she was asymptomatic. CONCLUSION Accurate preoperative diagnosis of PPGL can be safely achieved by EUS-FNA with immunohistochemistry. Multidisciplinary team approach should be considered to bring the optimal results in the management of PPGL.

Plummer-Vinson syndrome (PVS) also known as Paterson-Brown-Kelly syndrome is a rare syndrome which comprises iron deficiency anemia, dysphagia, and esophageal webs. The pathogenesis of PVS is not clear. Iron deficiency anemia is essential for diagnosis of PVS. If left untreated, there is an increased risk of developing pharyngeal or esophageal cancer in about 10% of patients. There are no strict guidelines for endoscopic surveillance in patients with PVS. Iron replacement can improve dysphagia and potentially lead to regression of esophageal webs. In this case report, we present a patient who had long-standing dysphagia for years which progressed to squamous cell cancer of esophagus by the time she sought medical treatment.