Epithelioid Inflammatory Myofibroblastic Sarcoma with Leukemoid Reaction.

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare and aggressive inflammatory myofibroblastic tumour (IMT) variant. This report identifies the first case of EIMS with leukemoid reaction. This is also the first case in which pancreatic infiltration occurred from the disease onset. A 14-year male patient presented with an 18×18×10 cm mass at the retroperitoneal space and a white blood cell (WBC) count of 85×109/L. The mass and the invaded tissues were surgically removed with tumour-free margins. Histopathology and bone marrow aspiration confirmed the diagnosis of EIMS with leukemoid reaction. The tumour recurred with hepatic and pulmonary metastasis one month after the surgery. WBC count also increased progressively with the tumour recurrence. There is no consensus on the treatment of EIMS. Since ALK rearrangement presents in all the EIMS cases, surgical resection combined with crizotinib or other targeted drugs may improve the prognosis. Key Words: Sarcoma, Soft tissue neoplasms, Leukemoid reaction, Crizotinib.

A better overall survival (OS) for total (ipsilateral) retroperitoneal lipectomy than standard complete resection in patients with retroperitoneal liposarcoma: a comparative multi-institutional study.

Background: Complete resection (CR) serves as the standard of surgical treatment for retroperitoneal liposarcoma (RPLS). Unfortunately, even at referral centers, recurrence rates are high, and CR may not address multifocal diseases, which are a common phenomenon in RPLS. We sought to retrospectively compare the clinical outcomes of RPLS patients treated with total (ipsilateral) retroperitoneal lipectomy (TRL) and CR. Because TRL remove potentially multifocal tumors in the fat, patients may have a better prognosis than CR. Methods: Patients with primary/first-recurrent RPLS who had been treated at 5 referral centers were recruited from December 2014 to June 2018. Multivariable Cox regression analyses were conducted to determine the effects of demographic, operative, and clinicopathological variables on the following primary endpoints: local recurrence (LR), local recurrence-free survival (LRFS), and overall survival (OS). Results: A total of 134 patients were enrolled in this retrospective study, 53 of whom underwent TRL, and 81 of whom underwent CR. The 2 groups were comparable in terms of age, gender, presentation (primary vs. first-recurrent RPLS), number of tumors (unifocal vs. multifocal) at presentation, and Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade. The TRL group had higher levels of preoperative hemoglobin (Hb) (13 vs. 12.5 g/dL; P=0.008) and a lower amount of intraoperative blood loss (400 vs. 500 mL; P=0.034), but there were no significant differences in the length of hospital stay (23 vs. 22 d; P=0.47) or complications (32 vs. 30; P=0.82) between the 2 groups. In a subset of patients with multifocal tumors at initial presentation, OS was more prolonged in those treated with TRL than those treated with CR (P=0.0272). Based on the multivariable analysis, primary liposarcoma and a low FNCLCC grade were associated with decreased LR and improved OS. Conclusions: TRL is a safe procedure that positively affects the OS of patients with multifocal RPLS. This novel strategy deserves further investigation in prospective studies.

Surgical management of leiomyosarcoma of the inferior vena cava.

The optimal surgical management of patients with leiomyosarcoma of inferior vena cava remains a controversy. From 1975 and 2009, five patients with leiomyosarcoma of inferior vena cava were treated at the Chinese PLA General Hospital and Beijing Shijitan Hospital. The age ranged 39-61 years and the duration of symptoms ranged from 18 to 36 months. Abdominal and back pain are the most common complaints. A combination of various imaging modalities is essential for treatment planning. R0, R1, R2, and biopsy only were accomplished in 2, 1, 1, and 1 case, respectively. Combined resections included inferior vena cava, right kidney, adrenal gland, psoas, colon, duodenal, gallbladder, liver, and/or aorta, without inferior vena cava reconstruction. No inferior vena cava-related postoperative complication was seen in our series.

[Applicability of anatomical vascular occlusion in hepatectomy for grand hepatocarcinoma].

OBJECTIVE: To retrospectively explore the clinical efficacies and applicability of anatomical vascular occlusion (AVO) in hepatectomy for grand primary hepatocarcinoma at different locations. METHODS: A total of 212 grand primary hepatocarcinoma cases undergoing hepatectomy were divided into 2 groups by vascular occlusion in the process of resection: AVO group (n = 97) and Pringle group (Pringle maneuver, n = 115). According to whether or not tumor was adjacent to main vessels, the cases were divided into 2 types: centrally (n = 98) and peripherally (n = 114) located lesions. And the perioperative outcomes were compared between 2 groups totally and by types respectively. RESULTS: No significance existed between the AVO and Pringle groups in the demographic characteristics and tumor background (P > 0.05). For total cases, there were no significant differences between 2 groups regarding the intraoperative blood loss volume ((632 ± 437) ml vs (546 ± 549) ml, P = 0.217) and the blood transfusion requirement (44.3% vs 33.0%, P = 0.092). The AVO group showed significantly better postoperative liver functions in terms of serum levels of total bilirubin and aminotransferase (P > 0.05). But no significant difference was found between 2 groups in the postoperative complication rate (18.6% vs 22.6%, P = 0.469) and hospital stay duration ((10.5 ± 4.8) vs (11.8 ± 5.6) days, P = 0.087). In centrally located lesions: the AVO group showed a significantly smaller intraoperative blood loss volume ((722 ± 492) ml vs (1032 ± 618) ml, P = 0.007) and blood transfusion requirement (45.6% vs 68.3%, P = 0.026). Also the AVO group showed significantly better postoperative liver functions in terms of serum levels of total bilirubin and aminotransferase (P < 0.01). As a consequence, the AVO group had a significantly lower postoperative complication rate (19.3% vs 39.0%, P = 0.031) and a shorter hospital stay duration ((10.7 ± 5.0) days vs (13.0 ± 6.2) days, P = 0.042). In peripheral located lesions: there were significantly larger intraoperative blood loss volume (504 ± 307 vs 278 ± 237 ml, P = 0.000) and blood transfusion requirement (42.5% vs 13.5%, P = 0.001) in the AVO group. The postoperative liver functions (total bilirubin and aminotransferase levels, P > 0.05), postoperative complication rate (17.5% vs 13.5%, P = 0.808) and hospital stay duration ((10.3 ± 4.6) days vs (11.1 ± 5.1) days, P = 0.429) showed no significant differences between 2 groups. CONCLUSION: The technique of AVO is unsuitable for all types of grand hepatocarcinoma. Whether or not the tumor is adjacent to main vessels is an important consideration of choosing the vascular control technique. Considering the risk of vascular damage in the process of hepatectomy, the AVO technique is indicated for the resection of central lesions but not for peripheral lesions.

[Application of selective and timely regional hepatic vascular occlusion for resection of large centrally located liver tumors: report of 133 cases].

OBJECTIVE: To improve the resection rate and increase operation safety for large centrally located liver tumors. METHODS: Clinical data from 133 patients with large centrally located liver tumors confirmed by surgery were analyzed retrospectively. Selective and timely regional hepatic vascular occlusion was used during the operation procedure. RESULTS: The resection rate was 100%. Perioperative death occurred in one patient. During operations, Forty-four patients underwent regional hepatic inflow occlusion ranging from 12 to 33 minutes. Twenty-three patients underwent left and right inflow occlusion, respectively, ranging from 8 to 50 minutes. One patient had right half-hepatic vascular exclusion for 40 minutes. The blood loss of 132 patients was (665 ± 424) ml (one patient experienced diffuse blood oozing and died in the next day). Among them, the blood loss of patients with liver cirrhosis was (723 ± 479) ml. On the contrary, those without liver cirrhosis was (458 ± 223) ml (P < 0.01). Liver function in 92.4% (122/132) patients recovered to Child-Pugh A within one week. No liver failure occurred. After operation, 3 patients presented ascites. Among them, two patients had liver cirrhosis and hepatocellular jaundice, one patient was accepted for transcatheter arterial chemoembolization preoperatively. Four patients had biliary fistula, one patient had gastroparesis, one patient had thrombus in the superior mesenteric vein and portal vein, and five patients had right pleural effusion. The 1-, 3- and 5-year survival rates of 112 patients were 89.1%, 57.7% and 36.9%, respectively. CONCLUSIONS: Selective and timely regional hepatic vascular occlusion is useful for the resection of large centrally located liver tumors. This kind of procedure can effectively control the blood loss during the operation and shorten the ischemic reperfusion time, beneficial for protecting the liver cell function. This procedure is a safe hepatic flow occlusion method.