Background: The objective of this study was to examine the extent of surgical procedures, pathological findings, complications and outcome of patients treated in the last 12 years for gallbladder cancer.
Background
Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare malignancy that originated from the smooth muscle tissue of the vascular wall. Diagnoses, as well as, treatment of the disease are still challenging and to date, a radical surgical resection of the tumor is the only curative approach.
Case report
We report on the case of a 49-year old male patient who presented with suddenly experienced dyspnea. Besides bilateral pulmonary arterial embolism, a lesion close to the head of the pancreas was found using CT scan, infiltrating the infrahepatic IVC. Percutaneous ultrasound-guided biopsy revealed a low-grade LMS. Intraoperatively, a tumor of the IVC was observed without infiltration of surrounding organs or distant metastases. Consequently, the tumor was removed successfully, by en-bloc resection including prosthetic graft placement of the IVC. Histological workup revealed a completely resected (R0) moderately differentiated LMS of the IVC.
Conclusion
LMS of the infrahepatic IVC is an uncommon tumor, which may present with dyspnea as its first clinical sign. Patients benefit from radical tumor resection. However, due to the poor prognosis of vascular LMS, a careful follow-up is mandatory.
Measurement of indocyanine green plasma disappearance rate (PDR(ICG)) has been suggested as a meaningful liver function parameter. However, there are only very limited data concerning its value in the monitoring of graft dysfunction (GDF) and primary non-function (PNF) especially during molecular absorbent recirculating system (MARS) therapy. This study was therefore performed to evaluate the diagnostic accuracy to detect and monitor GDF with the measurement of the PDR(ICG) in direct comparison with conventional markers like bilirubin and prothrombin time (PT). Of the 19 liver recipients, four patients with GDF and two patients with PNF were treated with 38 MARS cycles. Only PDR(ICG) did reliably indicate liver function between patients with GDF/PNF and patients with sufficient graft function who served as controls. Moreover, receiver operating characteristic analysis showed the highest areas under the curve (AUC) for PDR(ICG) (AUC(PDRICG max): 0.840, AUC(PDRICG max): 0.822), followed by bilirubin (AUC(bilirubin): 0.528) and PT (AUC(PT): 0.546). In contrast to the decrease of the serum bilirubin concentration due to MARS, a noticeable improvement of PDR(ICG) was evident only in patients with GDF. Patients with acute fulminant failure and PNF had significantly lower PDR(ICG) values, which did not improve even during continuous MARS treatments. Conclusively, monitoring of PDR(ICG) is superior to bilirubin and PT measurements to determine the graft function especially in patients with PNF and GDF undergoing MARS therapy.
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