Abstract. The objective of this study was to investigate the effect of administration of recombinant human growth hormone (hGH) in patients with Noonan syndrome. hGH was administered (0.5 IU/kg/week) to 15 patients with Noonan syndrome over a 2 year period. Average patient age prior to therapy was 7.5 ± 2.5 (mean ± SD) yr, the height SD score was -2.8 ± 0.7, and the pretreatment height velocity and bone age were 4.8 ± 1.0 cm/yr and 5.8 ± 2.1 yr, respectively. The height velocity in the year prior to treatment, and 0-12 and 12-24 months after commencing treatment was 4.8 ± 1.0 cm/ yr, 7.0 ± 1.2 cm/yr, and 5.5 ± 0.6 cm/yr, respectively. The height velocity in the first year of treatment was significantly greater (P = 0.0001, n = 14) than the pretreatment value, but there was no significant difference in the second year. The height SD scores at the commencement of treatment, and after 12 and 24 months of treatment were -2.8 ± 0.7, -2.4 ± 0.7, and -2.2 ± 0.5, respectively. Bone age advanced by 1.1 ± 0.5 yr in the 12 months after commencing treatment. We conclude that the use of hGH may be beneficial in the treatment of Noonan syndrome, although further research is required.

The nomogram validation was shown to be moderately predictive. PHC decreased the performance of the nomogram and might produce no DFS benefit in patients with low recurrent risk.

This prospective study showed that combined chemotherapy based on K-ras status can facilitate conversion to resection in patients with unresectable CRLM.

BACKGROUNDPostoperative pancreatic leakage readily results in intractable pancreatic fistula and subsequent intraperitoneal abscess. This refractory complication can be fatal; therefore, intensive treatment is important. Continuous local lavage (CLL) has recently been reevaluated as effective treatment for severe infected pancreatitis, and we report three patients with postoperative intractable pancreatic fistula successfully treated by CLL. We also discuss our institutional protocol for CLL for postoperative pancreatic fistula.CASE SUMMARYThe first patient underwent subtotal stomach-preserving pancreaticoduodenectomy, and pancreatic leakage was observed postoperatively. Intractable pancreatic fistula led to intraperitoneal abscess, and CLL near the pancreaticojejunostomy site was instituted from postoperative day (POD) 8. The abscess resolved after 7 d of CLL. The second patient underwent distal pancreatectomy. Pancreatic leakage was observed, and intractable pancreatic fistula led to intraperitoneal abscess near the pancreatic stump. CLL was instituted from POD 9, and the abscess resolved after 4 d of CLL. The third patient underwent aneurysmectomy and splenectomy with wide exposure of the pancreatic parenchyma. Endoscopic retrograde pancreatic drainage was performed on POD 15 to treat pancreatic fistula; however, intraperitoneal abscess was detected on POD 59. We performed CLL endoscopically via the transgastric route because the percutaneous approach was difficult. CLL was instituted from POD 63, and the abscess resolved after 1 wk of CLL.CONCLUSIONCLL has therapeutic potential for postoperative pancreatic fistula.

A solitary fibrous tumor is a ubiquitous mesenchymal fibroblastic tumor that was previously considered limited to the pleural cavity. Here, we report a rare case of a large solitary fibrous tumor of the mesorectum, which was successfully resected laparoscopically. A 56-year-old woman was referred to our hospital for a giant pelvic mass. Pelvic MRI showed a well-circumscribed mass, 12 cm in diameter, with heterogeneous signal intensity on T -weighted images. It was diagnosed as a benign mesorectal tumor of unknown origin. We successfully resected the entire tumor laparoscopically. Histological examination revealed it to be an extrapleural solitary fibrous tumor. For large tumors in the pelvis, the laparoscopic approach is preferable in terms of intraoperative hemorrhage, as long as they do not invade surrounding tissues.