Chinese expert consensus on standardized treatment for presacral cysts.

Presacral cysts are cystic or cyst-solid lesions between the sacrum and rectum, almost involving adjacent pelvic floorstructures including sacrococcygeal fascia, rectum, and anal sphincter. Presacral cysts are usually benign, currently believed to arise from aberrant embryogenesis. Presacral cysts are clinically rare and the true incidence is unknown. Surgical resection remains the major treatment for presacral cysts. Unless the cysts are completely resected, recurrence is unavoidable. Recurrent cysts or hard-to-heal sinuses in the sacrococcyx cause patients extreme pain. However, the current knowledge of presacral cysts is insufficient. They are occasionally confused with other diseases such as ovarian cysts and perianal abscesses. Moreover, lack of the correct surgical concept and skills leads to palliative treatment for complex presacral cysts and serious complications such as impairing the function of the anal sphincter or important blood vessels and nerves. The consensus summarizes the opinions and experiences of multidisciplinary experts in presacral cysts and aims to provide clinicians with a more defined concept of the treatment, standardize the surgical approach, and improve the efficacy of presacral cysts.

Characteristics and outcomes of patients with primary abdominopelvic aggressive angiomyxoma: a retrospective review of 12 consecutive cases from a sarcoma referral center.

BACKGROUND: Aggressive angiomyxoma (AAM) is a rare mesenchymal tumor that mostly arises from the pelvic and perineal soft tissues. Few studies reported its characteristics and outcomes previously due to its rarity and challenges of treatments. This study aimed to investigate the clinical characteristics as well as surgical and short-term survival outcomes of primary abdominopelvic AAM. METHODS: Medical records of patients who were admitted to surgery with pathological confirmation of primary abdominopelvic AAM at Peking University International Hospital from January 2016 through December 2021 were retrospectively retrieved from our retroperitoneal tumor database. Demographics, operative outcomes and pathological findings were collected. Patients received followed-up routinely after the surgery. Survival probabilities were calculated and determined through Kaplan-Meier analysis. RESULTS: A total of 12 consecutive patients (male/female 4:8) were included in this study. The median age was 45 years old. The clinical presentation varied among individuals, consisting of 2 abdominal discomforts, 4 constipations, 1 lumbago, 1 prolonged menstruation, and 1 buttock swelling. R0/R1 resection was achieved in 100% of patients. Postoperatively, 50% of patients developed various complications including 3 fistulas and 3 wound infections. No operative mortality was observed. Histopathology of all patients was suggestive of AAM. Immunohistochemistry was done with a 91.7% positive rate for estrogen and progesterone receptors. The median recurrence-free survival time was 38 months. There were no cases of deceased or presented with distal metastasis during a median of 42 months' follow-up. CONCLUSIONS: The clinical manifestations of abdominopelvic AAM are mostly atypical. Surgical resection with curative intents remains the mainstay treatment of this disease, which was strongly suggested in experienced sarcoma centers due to the high probability of severe postoperative complications. In addition, long-term follow-up is necessary due to the high rate of local recurrences.

Prediction of intraoperative bleeding and blood transfusion in patients with recurrent retroperitoneal liposarcoma: a retrospective study.

Background: Surgery is the main treatment for recurrent retroperitoneal liposarcoma (RPLS). The aim of the present study was to explore the factors associated with blood loss during surgery for recurrent RPLS. Methods: This retrospective study included patients with first recurrence of RPLS who were treated at our hospital between January 2015 and December 2019. Factors associated with intraoperative blood loss were identified by univariate and multivariate logistic regression analyses. Receiver-operating characteristic (ROC) curve analyses were conducted to evaluate whether tumor size and number of tumor-containing abdominal/pelvic zones were predictive of the need for blood transfusion. Results: The study included 67 cases. The number of zones containing tumors was 1 in 4 cases (6%), 2 in 36 cases (53.7%), 3 in 14 cases (20.9%), and 4 in 13 cases (19.4%). Tumor size was associated with blood loss >500 mL [odds ratio (OR): 1.153, 95% confidence interval (CI): 1.051-1.266, P=0.003]. The number of tumor-containing zones was associated with blood loss >1,000 mL (OR: 3.161, 95% CI: 1.248-8.003, P=0.015) and >1,500 mL (OR: 2.674, 95% CI: 1.061-6.739, P=0.037). Multiple tumors were associated with blood loss >2,000 mL (OR: 3.161, 95% CI: 1.092-13.133, P=0.036) and >2,500 mL (OR: 2.674, 95% CI: 1.243-16.299, P=0.022). Tumor dedifferentiation was associated with blood loss >1,000 mL (OR: 4.802, 95% CI: 1.287-17.916, P=0.019) and >1,500 mL (OR: 9.249, 95% CI: 1.927-44.39, P=0.005). ROC curve analysis showed that tumor size >15.25 cm [area under the ROC curve (AUC): 0.772, P<0.001] and the number of tumor-containing zones >2.5 (AUC: 0.670; P=0.023) were predictive of the need for blood transfusion. Conclusions: The main finding of the present study was that a larger tumor size, a larger number of tumor-containing zones, multiple tumors, and dedifferentiation were independently associated with a larger volume of intraoperative blood loss in patients with recurrent RPLS. The tumor size >15.25 cm and the tumor area >2.5 areas predicted the need for blood transfusion. Formulating the intraoperative blood transfusion plan for recurrent RPLS, it is necessary to pay attention to two spatial factors, tumor size and affected area, rather than one of them.

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.

Transcriptome analysis of tumor-derived mesenchymal progenitor cells shows that CHST15 is a fibrosis regulator of retroperitoneal liposarcoma.

Background: Retroperitoneal liposarcoma (RPLS) is a rare, biologically heterogeneous tumor with distinct clinical characteristics, such as frequent local recurrence, repeated relapse, and rare distant metastasis. No effective targeted therapy is available for RPLS. Here, we aim to determine the pathological functions and therapeutic potential of carbohydrate sulfotransferase 15 (CHST15) in RPLS. Methods: Tumor-derived mesenchymal progenitor cells (MPCs) and normal adipose derived mesenchymal stem cells (MSCs) were obtained from patients with RPLS. MPCs and MSCs were isolated and characterized based on surface markers, proliferation, and differentiation using flow cytometry and molecular staining. Transcriptome analysis was performed to decipher expression profile of differentiation-related genes in 3 paired MSCs and MPCs. Further confirmation of genes were performed using quantitative real-time polymerase chain reaction (qRT-PCR). Plasmids overexpressing CHST15 were transfected into adipose MSCs to examine fibrosis-related gene expression at mRNA level by real-time PCR. Results: The tumor stromal-derived MPCs expressed CD105, CD73, and CD90, and exhibited osteogenic and adipogenic differentiation potential in vitro. The proliferation of tumor-derived MPCs was significantly lower than that of normal adipose-derived MSCs (P<0.001). Transcriptome analysis revealed upregulation of IL-7R, ALPL, PKNOX2, and CHST15 in tumor-derived MPCs. CHST15 was highly expressed in tumor-derived MPCs (P<0.001). CHST15 mediated fibrosis-related FGF2 gene expression in MSCs (P<0.05) and MPCs (P<0.001). Conclusions: CHST15 is upregulated in tumor-derived MPCs and regulates fibrosis in RPLS. This provides clues for development of novel therapeutic strategies by targeting CHST15-induced MPC activation in RPLS.

KRT15 promotes colorectal cancer cell migration and invasion through ß-catenin/MMP-7 signaling pathway.

KRT15 has been reported to act as an oncogene in colorectal cancer. However, whether KRT15 promotes colorectal cancer migration and invasion remain unclear. In this study, western blot and qRT-PCR assay were used to determine the expression of KRT15 in colorectal cancer cells. Wound-healing and transwell migration assay were performed to assess the migration of colorectal cancer cells. Matrigel transwell invasion assay was employed to examine the invasion of colorectal cancer cells. We found that KRT15 was highly expressed in colorectal cancer cells. Ectopic expression of KRT15 dramatically promoted colorectal cancer cell migration and invasion. Conversely, silencing KRT15 remarkably suppressed the migration and invasion of colorectal cancer cells. Importantly, we found that MMP-7 was crucial for KRT15-induced migration and invasion of colorectal cancer cells. Knockdown of MMP-7 significantly diminished the migration and invasion induced by KRT15; overexpression of MMP-7 almost completely rescued the inhibitory effects of KRT15 shRNAs on colorectal cancer cell migration and invasion. In addition, by gain- and loss-of function, we confirmed that ß-catenin was responsible for the increased expression of MMP-7 induced by KRT15 colorectal cancer cell lines. In conclusion, KRT15 promotes migration and invasion of colorectal cancer cell at least partly through ß-catenin/MMP7 signaling pathway, suggesting KRT15 is a potential therapeutic target for patients with metastatic colorectal cancer.

Adjuvant radiotherapy in central hepatocellular carcinoma after narrow-margin hepatectomy: A 10-year real-world evidence.

OBJECTIVE: A prospective randomized control study investigated the feasibility and efficacy of adjuvant radiotherapy on patients with central hepatocellular carcinoma (HCC) after narrow-margin hepatectomy (<1 cm). This study presents an updated 10-year real-world evidence to further characterize the role of adjuvant radiotherapy. METHODS: Patients with central HCC after narrow-margin hepatectomy (<1 cm) were prospectively assigned to adjuvant radiotherapy group and control group. Patients' outcome, adverse events, long-term recurrence and survival rates were investigated. RESULTS: The 1-, 5-, and 10-year recurrence-free survival (RFS) rates were 81.0%, 43.9%, and 38.7%, respectively in adjuvant radiotherapy group and 71.7%, 35.8%, and 24.2%, respectively in control group (log-rank test, P=0.09). The 1-, 5-, and 10-year overall survival (OS) rates were 96.6%, 54.7%, and 42.8%, respectively in adjuvant radiotherapy group and 90.2%, 55.1%, and 30.0%, respectively in control group (log-rank test, P=0.20). The 1-, 5-, and 10-year RFS rates for patients with small HCC (≤5 cm) were 91.1%, 51.6%, and 48.4%, respectively in adjuvant radiotherapy group and 80.0%, 36.6%, and 26.6%, respectively in control group (log-rank test, P=0.03). Landmark analysis demonstrated that patients with small HCC in adjuvant radiotherapy group had a significantly improved OS in second five years after treatment in comparison to patients in control group (log-rank test, P=0.05). CONCLUSIONS: Our updated results showed a sustained clinical benefit on reducing recurrence, improving long-term survival for small central HCC by adjuvant radiotherapy after narrow-margin hepatectomy. Long-term survival data also indicated that hepatectomy is an optimal treatment for selected patients with central HCC.

Difference in left renal vein pressure: an indicator for free of reconstruction after ligation in retroperitoneal tumor patients.

We hypothesized that the left renal vein pressure difference (ΔP) before and after the ligation can serve as an objective indicator for free of reconstruction after resection of a retroperitoneal tumor with renal segment of inferior vena cava and right kidney. After established a model of left renal vein compression, 45 miniature pigs were operated on experimental procedures including renal segment of inferior vena cava resection, right nephrectomy, and left renal vein ligation. The ΔPs of left renal vein before and after the ligation were measured. Safe ΔP variation without causing acute kidney injury was calculated using regression analysis. In human the safety range of ΔP before and after ligation of the left renal vein was calculated by diuretic response test. The safety range of ΔP in animals or human was 0-11.9 or 0-17.5 cm H2O, respectively. The renal function changed dramatically (p < 0.01), characterized by a significant increase in the rate of acute kidney injury when the ΔP was beyond the upper limit of the safety range. In conclusion, ΔP can predict free of reconstruction after resection of a retroperitoneal tumor with the renal segment of the inferior vena cava and the right kidney.

Association of FPGS genetic polymorphisms with primary retroperitoneal liposarcoma.

Primary retroperitoneal liposarcoma is generally regarded as a genetic disorder. We have retrospectively genotyped 8 single nucleotide polymorphisms (SNPs) in 6 candidate genes (MDM2, CDK4, CDC27, FPGS, IGFN1, and PRAMEF13) in 138 patients and 131 healthy control subjects to evaluate the effects of genetic factors on individual susceptibility to primary retroperitoneal liposarcoma in Chinese population. Three SNPs (rs2870820, rs1695147, rs3730536) of MDM2 showed significant differences in single-loci genotypes and allele frequencies between case and control groups (p < 0.05). The minor allele G of SNP rs10760502 in FPGS (folylpolyglutamate synthase) gene was significantly associated with increased risk for primary retroperitoneal liposarcoma, compared with major allele A. Our data suggest that FPGS variant in Chinese population may affect individual susceptibility to primary retroperitoneal liposarcoma.

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.