LAUSR

A 30-year-old male patient with acute diffuse abdominal pain, distension, vomiting, bloating, and lack of flatus was admitted to the emergency department. His medical history included a genetic diagnosis of type 1 neurofibromatosis (NF1) b (mutation R192X in NF1 gene). The patient had previous multiple NF spinal nodes resections and bilateral cornea transplant. The abdomen was painful, distended, without peritoneal signs. Initial vital signs were a heart rate of 120 bpm, blood pressure of 110/90 mmHg, and respiratory rate of 12 breaths per minute. Blood laboratories showed aspartate aminotransferase 73 U/L, alanine aminotransferase 116 U/L, gamma-glutamyl transferase 329 U/L, alkaline phosphatase 183 U/L, and cholinesterase 4,577 U/L. A chest and abdomen computed tomography (CT) scan with contrast showed a huge retroperitoneal mass (15 18 13 cm) compressing liver, pancreas, stomach, and right kidney; the celiac trunk with its branches, and inferior vena cava, resulted stretched and dislocated (Figs. 1 and 2). Multiple nodules compressing the esophagus were identified in the mediastinum; others were detected within the right costodiaphragmatic recess. Multiple enlarged lymph nodes in the chest and in the abdomen were also present. After initial management, a progressive deterioration of the respiratory function,