Malignant peripheral nerve sheath tumour of the oesophagus Review of the literature and report of a case with lymph node and distant metastases.

Oesophageal sarcomas are very rare while various histological types have occasionally been reported. Malignant Peripheral Nerve Sheath Tumour (MPNST) of the oesophagus is an exceedingly rare type of oesophageal sarcoma with only thirteen cases previously reported in the world literature. However, it should be included in the differential diagnosis of oesophageal neoplasias. Due to the small number of reported cases, the information about the biological behaviour of this entity is still insufficient. While MPNST is generally considered an aggressive type of tumour with high recurrence rates after surgical treatment and poor prognosis, previous reports of cases with oesophageal localization have recorded satisfactory outcomes overall even with less aggressive therapeutic approaches, although a long-term follow-up is lacking. Herein, we present the case of a 76-year-old female patient with oesophageal MPNST who presented with lymph node and distant metastases at the time of diagnosis, accounting for the second time only that this unusual presentation of this extremely uncommon disease has been reported. In our case, the course of disease was extremely aggressive which resulted in the second recorded death from this entity in the literature. The case presentation is followed by an extensive review of the world literature for the so far reported cases, aiming to highlight all relevant aspects such as demographics, clinical features, diagnostic assessment and findings, histological parameters, treatment and prognosis, and extract valuable previously unpublished conclusions for this rare entity. KEY WORDS: Lymph node metastasis, Malignant Peripheral Nerve Sheath Tumour, Malignant schwannoma, Neurogenic sarcoma, Oesophagus, S100.

Bouveret syndrome-the rarest variant of gallstone ileus: a case report and literature review.

We present a case report of a patient with Bouveret syndrome with interesting radiological findings and successful surgical treatment after failure of the endoscopic techniques. The report is followed by a review of the literature regarding the diagnostic means and proper treatment of this rare entity. Bouveret syndrome refers to the condition of gastric outlet obstruction caused by the impaction of a large gallstone into the duodenum after passage through a cholecystoduodenal fistula. Many endoscopic and surgical techniques have been described in the management of this syndrome. This is a case of a 78-year-old patient with severe medical history who presented in bad general condition with an 8-day history of nausea, multiple bilious vomiting episodes, anorexia, discomfort in the right hypochondrium and epigastrium, and fever up to 38,5°C. The diagnosis of Bouveret syndrome was set after performing the proper imaging studies. An initial endoscopic effort to resolve the obstruction was performed without success. Surgical treatment managed to extract the impacted gallstone through an enterotomy after removal into the first part of the jejunum.