BACKGROUND: This study aimed to evaluate the perioperative safety and efficacy of the Mini-open and trans-tubular approach in patients with spinal metastases who underwent decompression surgery.
METHODS: 37 consecutive patients with spinal metastases who underwent decompression surgery through a Mini-open or trans-tubular approach were retrospectively reviewed between June 2017 and June 2022. Thirty-four patients were included in this study. 19 underwent decompression surgery through the Mini-open approach, and 15 underwent the Trans-tubular approach. T-test and chi-square test were used to evaluate the difference between baseline data and primary and secondary outcomes.
RESULTS: Baseline characteristics did not differ significantly between Trans-tubular and Mini-open groups except for the Ambulatory status (P < 0.001). There was no significant difference in blood loss between the two groups (P = 0.061). Operative time, intraoperative blood transfusion, intraoperative complication (dural tear), and postoperative hospitalization were comparable in the two groups (P > 0.05). The trans-tubular group had significantly less amount of postoperative drainage (133.5 ± 30.9 ml vs. 364.5 ± 64.2 ml, p = 0.003), and the time of drainage (3.1 ± 0.2 days vs. 4.6 ± 0.5 days, p = 0.019) compared with Mini-open group (P < 0.05). Sub-group analysis showed that for patients with hypo-vascular tumors, the Trans-tubular group had significantly less blood loss than the Mini-open group (951.1 ± 171.7 ml vs. 1599.1 ± 105.7 ml, P = 0.026).
CONCLUSIONS: Decompression through Mini-open or Trans-tubular was safe and effective for patients with spinal metastases. The trans-tubular approach might be more suitable for patients with hypo-vascular tumors.

OBJECTIVE: Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare vascular tumors in children historically associated with significant morbidity and mortality. This study was conducted to determine first-line therapy in the absence of available prospective clinical trials.
METHODS: Patients from 17 institutions diagnosed with KHE/TA between 2005 and 2020 with more than 6 months of follow-up were included. Response rates to sirolimus and vincristine were compared at 3 and 6 months. Durability of response and response to other treatment modalities were also evaluated.
RESULTS: Of 159 unique KHE/TA subjects, Kasabach-Merritt phenomenon (KMP) was present in 64 (40.3%), and only two patients were deceased (1.3%). Over 60% (n = 96) demonstrated treatment response at 3 months, and more than 70% (n = 114) by 6 months (no significant difference across groups). The vincristine group had higher radiologic response at 3 months compared to sirolimus (72.7% vs. 20%, p = .03), but there were no differences between these groups at 6 months. There were no differences in rates of recurrent or progressive disease between vincristine and sirolimus.
CONCLUSIONS: In this large, multicenter cohort of 159 patients with KHE/TA, rates of KMP were consistent with historical literature, but the mortality rate (1.3%) was much lower. Overall treatment response rates were high (>70%), and there was no significant difference in treatment response or durability of disease comparing sirolimus to vincristine. Our results support individualized treatment decision plans depending on clinical scenario and patient/physician preferences. Response criteria and response rates reported here will be useful for guiding future treatment protocols for vascular tumors.

To investigate the association between CT signs and clinicopathological features and disease recurrence in patients with hepatoid adenocarcinoma of stomach (HAS).
Forty nine HAS patients undergoing radical surgery were retrospectively collected. Association between CT and clinicopathological features and disease recurrence was analyzed. Multivariate logistic model was constructed and evaluated for predicting recurrence by using receiver operating characteristic (ROC) curve. Survival curves between model-defined risk groups was compared using Kaplan-Meier method.
24(49.0%) patients developed disease recurrence. Multivariate logistic analysis results showed elevated serum CEA level, peritumoral fatty space invasion and positive pathological vascular tumor thrombus were independent factors for disease recurrence. Odds ratios were 10.87 (95%CI, 1.14-103.66), 6.83 (95%CI, 1.08-43.08) and 42.67 (95%CI, 3.66-496.85), respectively. The constructed model showed an area under ROC of 0.912 (95%CI,0.825-0.999). The model-defined high-risk group showed poorer overall survival and recurrence-free survival than the low-risk group (both P < 0.001).
Preoperative CT appearance of peritumoral fatty space invasion, elevated serum CEA level, and pathological vascular tumor thrombus indicated poor prognosis of HAS patients.

Congenital hepatic hemangiomas (CHHs) are benign vascular tumors whose clinical, histological, and genetic correlation has recently been described in patients with long-term survival, although no mortality risk factors have been identified to date. The aim of this study is to analyze predictors of mortality in patients with CHH. A retrospective single-center case-control study of consecutive CHH patients diagnosed in our institution between 1991 and 2021 was performed, who were classified into two groups according to their survival. Demographic, gestational, imaging, and laboratory data at diagnosis were collected and compared between both groups. A total of 29 patients were included (12 males; 17 females) of whom 5 died as a result of CHH evolution due to cardiac failure and coagulopathy, with a median age of 11 days until death. No differences in demographic or gestational data were reported. There were neither differences when comparing imaging tests, nor in location, number of affected liver segments, or CHH estimated volume. Upon laboratory data at diagnosis, deceased patients had a significant elevation of median liver enzymes [glutamic-oxaloacetic transaminase (359 u/L vs. 45 u/L; p < 0.01) and glutamic-pyruvic transaminase (313 u/L vs. 20 u/L; p < 0. 01)], as well as a decreased median platelet count (85,250/µL vs. 337,000/µL; p < 0.01), prothrombin activity (54% vs. 93%; p < 0.01), and fibrinogen (131 mg/dL vs. 284 mg/dL; p < 0.01), with no differences in blood count or biochemistry data.
CONCLUSIONS: CHH clinical behavior can be innocuous or life-threatening. Thrombocytopenia, coagulation disorders, and increased liver enzymes at diagnosis seem to be the main predictors of mortality.
BACKGROUND: • Congenital Hepatic Hemangiomas (CHHs) are benign vascular tumors whose clinical behavior can be innocuous or life-threatening.
BACKGROUND: • Thrombocytopenia, coagulation disorders and increased liver enzymes at diagnosis seem to be the main predictors of mortality in these patients.

BACKGROUND: Malignant peritoneal mesothelioma (MPM) is a rare malignant tumor with a high mortality rate and extremely poor prognosis. In-depth pathological analysis is essential to assess tumor biological behaviors and explore potential therapeutic targets of MPM. Nucleoplasmin 2 (NPM2) is a molecular chaperone that binds histones and may play a key role in the development and progression of tumors. This study aimed to analyze the correlation between the expression level of NPM2 and the main clinicopathological characteristics and prognosis of MPM.
METHODS: Ninety-two postoperative specimens from MPM patients following cytoreductive surgery were collected. Postoperative specimens were stained with immunohistochemistry. The expression level of NPM2 was quantitatively analyzed by QuPath-0.3.2 software. Univariate and multivariate analyses were conducted to investigate the correlation between NPM2 expression and other conventional clinicopathological characteristics.
RESULTS: Among the 92 MPM patients, there were 47 males (48.9%) and 45 females (51.1%), with a median age of 56 (range: 24-73). There were 70 (76.0%) cases with loss of NPM2 protein expression, 11 (12.0%) cases with low expression, and 11 (12.0%) cases with high expression. Univariate analysis showed that NPM2 protein expression level (negative vs. low expression vs. high expression) was negatively correlated with the following three clinicopathological factors: completeness of cytoreduction (CC) score, vascular tumor emboli, and serious adverse events (SAEs) (all P < 0.05). Multivariate analysis showed that NPM2 protein expression level (negative vs. low expression vs. high expression) was independently negatively correlated with the following two clinicopathological factors: CC score [odds ratio (OR) = 0.317, 95% CI: 0.317-0.959, P = 0.042] and vascular tumor emboli (OR = 0.092, 95% CI = 0.011-0.770, P = 0.028). Survival analysis showed that loss of NPM2 protein expression (negative vs. positive) was associated with poor prognosis of MPM.
CONCLUSIONS: Loss of NPM2 expression is a potential immunohistochemical marker for MPM.

Penile myointimoma is a rare, benign tumor occurring within the corpus spongiosum vasculature of the glans penis. Thus far, there have been twenty-three reported tumors in the literature. We present four additional tumors of this unique myointimal proliferation. Patients ranged in age from 20 to 68 years and presented with a firm mass on the glans penis. All four tumors displayed distinctive morphologic features consisting of a myointimal proliferation with plexiform architecture of bland myofibroblastic cells in a myxoid background in the corpus spongiosum vasculature. Characteristic cytoplasmic immunoreactivity of lesional cells with smooth muscle actin in addition to a desmin positive collarette of native vessel smooth muscle was seen in all four tumors. No disease was reported in any of the patients at last clinical follow-up (9 months to 15 years) after biopsy or excision. Myointimoma is part of a rare group of mesenchymal tumors that has been recently classified by its distinctive location, morphology, and immunohistochemical reactivity. For any nodular, spindle cell lesion of the corpus spongiosum, myointimoma should be included in the differential diagnosis given its unique characteristics and favorable clinical outcome.

Beta adrenergic receptors (β-AR) play a key role in regulating several hallmark pathways of both benign and malignant human and canine tumors. There is scarce information on the expression of β-AR in canine vascular tumors. Therefore, the purpose of the present research work was to study the mRNA expression levels of the three subtypes of the β-AR genes (ADRB1, ADRB2, ADRB3) in hemangiosarcoma (HSA) and hemangioma (HA), as well as in vascular hamartomas (VH) from dogs.Fifty samples (n = 50) were obtained from 38 dogs. Twenty-three animals had HSA, eight animals HA and seven animals VH. HSA were auricular (n = 8), splenic (n = 5), cutaneous (n = 6), auricular and splenic (n = 2), cutaneous-muscular (n = 1) and disseminated (n = 1). There were seven cases of HSA that were divided into primary tumor and secondary (metastatic) tumor. Skin and muscle samples with a normal histological study were used as control group. ADRB gene expression was determinate in all samples by real-time quantitative PCR. Results showed that ADRB1, ADRB2 and ADRB3 were overexpressed in HSA when compared to the control group. ADRB2 was overexpressed in HA when compared to the control group. HSA express higher values of ADBR1 (p = 0.0178) compared to VH. There was a high inter-individual variability in the expression of the three subtypes of ADBR. No statistically significant difference in the expression of ADBR genes were observed between HSA primary when compared to metastatic or in different anatomical locations. In conclusion, canine HSA overexpress the three β-AR subtypes and canine HA β2-AR. High variability was observed in β-AR mRNA levels amongst HSA cases.

This study aimed to assess the feasibility of preoperative chemoradiotherapy using gemcitabine plus nab-paclitaxel (GnP) and to determine the recommended dose (RD) of nab-paclitaxel for patients with localized pancreatic ductal adenocarcinoma (PDAC).
The participants had localized PDAC with contact or invasion to major arteries. They received GnP on days 1, 15, 29, and 43. The dose of gemcitabine was fixed at 600 mg/m2, whereas that of nab-paclitaxel was at 3 dose levels in accordance with a standard 3 + 3 dose escalation scheme. Three-dimensional radiotherapy was administered concurrently to a total dose of 50.4 Gy per 28 fractions.
The study cohort comprised 15 patients. Grade 3 or 4 neutropenia was observed in 4 (26.7%), leukopenia in 1 (6.7%), biliary infection in 2 (13.3%), appetite loss and nausea in 1 (6.7%), and anaphylaxis in 1 (6.7%). The RD was determined as level 2 (gemcitabine, 600 mg/m2; nab-paclitaxel, 100 mg/m2). Three patients underwent pancreatectomy after additional chemotherapy and achieved R0 resection.
The RD of nab-paclitaxel in our chemoradiotherapy protocol using GnP was 100 mg/m2 with gemcitabine 600 mg/m2 and 3-dimensional conformal radiotherapy to a total dose of 50.4 Gy per 28 fractions for patients with localized PDAC.

Objective: To investigate the clinicopathological features of hepatic vascular tumors in children. Methods The clinical characteristics, histology and immunohistochemical staining results were summarized and analyzed in 22 cases of hepatic vascular tumors in children at Guangzhou Women and Children\'s Medical Center from September 2007 to November 2020. Results: The 22 patients aged from 1.0 month to 2.5 years (mean age 9 months). There were 10 males and 12 females. Five cases were found in premature and had low birth weight infants; three cases were discovered in the antenatal period; one patient also had cutanous hemangioma; six patients had associated anemia; Kasabach-Merritt phenomenon was not seen in any patient. CT examination showed 17 tumors were solitary and five were multifocal lesions. Macroscopically, the tumors size ranged from was 0.6 cm to 11.0 cm; the cut surface was solid, gray red and brown in color, and in six cases there were hemorrhage and necrosis in the central area. Microscopically,15 cases of solitary congenital hepatic hemangiomas showed characteristic necrosis in the central area, with loose fibrous tissues at periphery. Proliferation of capillaries, residual bile ducts between the vascular lumens, and dilated thrombosed vascular channels were seen, and contained extramedullary hematopoietic foci and calcification. Five cases of multiple hepatic infantile hemangiomas showed capillaries of different sizes composing of plump endothelium and pericytes and were arranged in lobular or diffuse patterns. Two cases of cavernous hemangioma (venous malformation) consisted of dilated thin-walled blood vessels with branch-like pattern lined with flat endothelial cells. Immunohistochemically, all 22 case expressed vascular endothelial markers CD31 and CD34, but D2-40 was negative. Glut1 was positive in five cases of multiple hepatic infantile hemangiomas, and the other cases were negative. Conclusion: Hepatic vascular tumors in children are rare, and their classification is different from that of adults. It is of great significance to make clear pathologic diagnosis.
目的： 探讨儿童肝脏血管源性肿瘤的临床病理特征。 方法： 对2007年9月至2020年11月广州市妇女儿童医疗中心22例儿童肝脏血管源性肿瘤患者的临床特征、标本的组织学及免疫组织化学染色结果进行总结分析。 结果： 22例儿童肝脏血管源性肿瘤，年龄1个月至2.5岁，平均年龄9个月。男10例，女12例。早产儿及低出生体重儿5例，产前发现肝脏病变3例，伴有皮肤血管瘤1例，伴有贫血6例，均未出现Kasabach-Merritt现象。CT检查肝组织内孤立性病变17例，多灶性5例。病理学检查：肿瘤直径0.6~11.0 cm，切面实性，灰红灰褐色，6例中央见出血坏死。显微镜下，15例孤立性先天性肝血管瘤见特征性的中央坏死区，周围组织疏松，可见毛细血管增生，管腔之间较多残留小胆管，扩张的血管内血栓形成，其中可见髓外造血及钙化。5例多灶性肝婴儿血管瘤见毛细血管分叶状或弥漫排列，管腔大小不等，可见较肥胖的血管内皮细胞及血管周细胞。2例海绵状血管瘤（静脉畸形）由扩张的分支状薄壁血管组成，内衬扁平的内皮细胞。22例肝脏血管瘤均表达血管内皮标志物CD31及CD34，不表达D2-40。5例多灶性肝婴儿血管瘤Glut1阳性，其余Glut1均阴性。 结论： 儿童肝脏血管源性肿瘤罕见，其分型与成人不同，明确病理诊断具有重要意义。.

OBJECTIVE: To explore the surgical method and safety of modified one stage posterior approach total en block spondylectomy combined with pedicle screw fixation and titanium mesh reconstruction for the treatment of invasive thoracic vascular tumor.
METHODS: The clinical data of 12 patients with invasive thoracic vasculay tumor from December 2012 to May 2015 was retrospectively analyzed. There were 8 males and 4 females, aged from 40 to 62 years with an average of 51.2 years, the course of disease was 2 months to 8 years with an average of 3.4 years. The lesions involved vertebral bodies:1 case of T2, 4 cases of T4, 1 case of T5, 2 cases of T6, 2 cases of T8, and 2 cases of T10. According to Tomita classification, there were 3 cases of typeⅠ, 3 cases of typeⅡ, 1 case of type Ⅲ, and 5 cases of type Ⅳ. The Japanese Orthopaedic Association (JOA) score was 8.0±2.7 before operation. One patient with T10 lesions had sensory dyskinesia below the umbilicus, and the muscle strength of both lower limbs was grade Ⅰ-Ⅱ, and the others 11 patients were grade Ⅲ-Ⅳ. All 12 patients underwent one stage posterior approach total en block spondylectomy and the pedicle screw fixation combined with titanium mesh reconstruction under general anesthesia and continuous motor evoked potential (MEP) spinal cord electrophysiological monitoring throughout the operation. The operation time, intraoperative blood loss and transfusion, postoperative pain and recovery of spinal cord function, bone graft fusion, tumor recurrence and other complications were followed up.
RESULTS: All the operations were successful. The average operation time, intraoperative blood loss and blood transfusion were 5.5 h (4.5 to 6.0 h), 1 850 ml (1 650 to 2 500 ml), 1 050 ml (600 to 1 500 ml), respectively. All 12 patients were followed up for 5 months to 2.5 years with an average of 21 months. Local pain and lower limb muscle strength were improved to varying, and the nerve compression symptoms disappeared. The JOA score at 6 months after operation was 12.0±3.4, which was statistically significant difference compared with the preoperative 8.0±2.7 (t=3.20, P<0.05). Titanium mesh bone grafts were all fused in phaseⅠ, with an average fusion time of 4.5 months (3 to 7 months). During the follow-up period, there was no tumor recurrence, loosening or breaking of nails, sinking and displacement of titanium mesh.
CONCLUSIONS: Modified one stage posterior approach total en block spondylectomy is an ideal surgical method for the treatment of invasive thoracic vascular tumors, which has a safe, reliable and long lasting efficacy.