Between 2000 and 2008, 11 major orthopaedic surgeries for 7 congenital haemophilia patients with inhibitors were performed by the first author as the primary doctor using recombinant activated factor VII (rFVIIa). Orthopaedic surgical treatments were performed for six surgeries for four high-responder haemophilia A patients, three surgeries for two high-responder haemophilia B patients and two surgeries for one low-responder haemophilia B patient. This low-responder patient is allergic to factor IX products, so he usually uses rFVIIa as a haemostatic agent. All of the surgeries were major, such as joint arthroplasty, arthroscopic synovectomy, and a combination of both, and excellent surgical results were achieved. Seven cases were controlled by bolus infusion of rFVIIa, and the other four cases were controlled by combined bolus and continuous infusion of rFVIIa. An anti-fibrolytic agent was used for all cases. There were no thrombogenic adverse effects, only two bleeding episodes. As for haemostatic control, nine surgeries were excellent, one was good and one was fair. This report is the largest clinical report on major orthopaedic surgeries at a single institute. We have concluded that the combination of bolus and continuous infusion of rFVIIa is safe and effective, and more convenient to administer than simple bolus infusion therapy to achieve haemostasis at peri-operative periods. In addition, our data also concurs with the data of several previous reports which showed that orthopaedic surgery for haemophilia patients with inhibitors by means of rFVIIa is safe and effective.
Background
Neuroendocrine tumors (NETs) originate from neuroendocrine cells, which are found throughout the body. NETs occur principally in the gastrointestinal tract (approximately 65%) and bronchopulmonary tract (approximately 25%) but rarely occur in the presacral space. Aside from primary and metastatic lesions, there have been reports of NETs occurring in the presacral space arising from tailgut cysts, teratomas, and imperforate anus. We herein report a rare case of laparoscopic resection of a NET in the presacral space, which almost fully replaced tailgut cysts.
Case presentation
A 68-year-old woman was referred to our hospital for surgery of a right inguinal hernia, but preoperative computed tomography revealed an asymptomatic 43-mm mass in the presacral space. Magnetic resonance imaging showed a multilocular solid mass with clear boundaries and a slightly high signal intensity on T1- and T2-weighted images. Positron emission tomography showed 18F-fluorodeoxyglucose uptake. Thus, we suspected a malignant tumor and performed laparoscopic resection to obtain a definitive diagnosis. Macroscopically, the tumor was 43 mm in size with clear boundaries, and the cut surface was a gray-white solid component. Histopathological findings revealed that the tumor was composed of relatively uniform cells with fine chromatin, with round to oval nuclei arranged in solid, trabecular, or rosette-like growth patterns. Small cysts lined with stratified squamous epithelium and columnar epithelium were observed along with solid components of the tumor, which is a feature of tailgut cysts. Therefore, the final diagnosis was NET Grade 1 arising from tailgut cysts. No recurrence was observed within 1 year after surgery.
Conclusions
We performed en bloc laparoscopic resection of a NET arising from tailgut cysts in the presacral space without injury. In cases of a solid lesion in the presacral space, not only the primary disease but also the pathological condition with tissue transformation and replacement should be considered, as in this case.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.