Analysis of angiographic characteristics of kaposiform hemangioendothelioma and investigation of the value of transcatheter arterial embolization therapy.

BACKGROUND: This study aimed to analyze the angiographic characteristics of kaposiform hemangioendothelioma (KHE) and investigate the value of transcatheter arterial embolization (TAE) therapy. METHODS: The clinical data of infants diagnosed with KHE at the department from June 2013 to June 2020 were retrospectively analyzed. Of these, 34 infants received TAE therapy. The efficacy of the treatment was evaluated 4 weeks after the therapy. The angiographic characteristics were analyzed by comparing them with the angiographic characteristics of infantile hemangioma (IH), and the times of TAE therapy and the platelet level after each TAE therapy in infants with KHE were summarized. RESULTS: The present study showed that the capillary blush of KHE was irregular with an obscure boundary and nonuniform distribution. Many fine feeding arteries were present. The diameter of the feeding arteries was disproportionate to the volume of the tumor blush. The normal arteries were usually embedded in the tumor blush. The angiography of common IH in infants also showed tumor blush, but it was usually round with a clear boundary and uniform staining, and was distributed on 1 side of the normal arterial trunk. The infants with KHE received TAE therapy for 2 to 5 times/case, with a total of 104.0 times, with an average of 3.1±0.8/case. Among which, the platelets continued to decline for 9 times after TAE therapy and the platelets increased to ≥100×109/L in 7.8±3.2 days for 95 times after TAE therapy, The average relapse time was 30.0±15.9 days. CONCLUSIONS: The feeding arteries of KHE were numerous and fine and were not easily embolized. The application of TAE may rapidly improve the platelet level, but the long-term effect is poor.

Clinical efficacy of absolute ethanol combined with n-butyl cyanoacrylate sclerotherapy in the treatment of Puig's classified advanced venous malformation in children.

The aim of the present retrospective study was to investigate the clinical safety and efficacy of absolute ethanol combined with n-butyl cyanoacrylate sclerotherapy in the treatment of Puig's classified advanced venous malformation. Sclerotherapy was performed in 121 children (52 males and 69 females; age range, 5 months to 16 years) with venous malformations under general anesthesia between April 2009 and October 2014 at the Department of Interventional Radiology and Vascular Anomalies, Guangzhou Women and Children's Medical Center, Guangzhou, China. The patients with venous malformations were diagnosed and classified according to the diagnostic criteria of the International Society for the Study of Vascular Anomalies. According to the characteristics of intraoperative percutaneous angiography, 21 patient cases (9 males and 12 females; age range, 6 months to 14 years) were classified as advanced Puig's venous malformation. These 21 patients were treated with absolute ethanol combined with n-butyl cyanoacrylate. The patients were followed-up for 6-24 months (average, 15 months) after treatment. Following treatment with absolute ethanol combined with n-butyl cyanoacrylate, 15 cases were controlled and the total effective rate was 71% (15/21). However, 1 patient developed skin ulcerations, which was classed as a minor complication, 1 patient developed ectopic embolism caused by n-butyl cyanoacrylate reflux, and 1 patient developed transient pulmonary hypertension, the latter two complications were classified as major. Notably, the incidence rate of minor and major complications were 14.3%. To conclude, the present findings indicated that absolute ethanol combined with n-butyl cyanoacrylate sclerotherapy was a safe and effective method with a low complication rate in the treatment of Puig's classified advanced venous malformation in patients.

Microwave ablation combined with transcatheter arterial chemoembolization is effective for treating unresectable hepatoblastoma in infants and children.

The present study is to evaluate the feasibility and efficacy of microwave ablation (MWA) combined with transcatheter arterial chemoembolization (TACE) in the treatment for unresectable hepatoblastoma in infants and children. A total of 17 patients with PRETEXT stage III and IV hepatoblastoma that was unresectable by conventional resection were included in the present study. The patients were treated with TACE, MWA, and chemotherapy. All cases were diagnosed by computed tomography (CT) and liver tumor biopsy before TACE procedure. All patients received 2 courses of TACE and 1 to 2 times of MWA. Finally, several cycles of chemotherapy were arranged. Among the 17 patients, 14 were alive and had normal alpha-fetoprotein (AFP) levels. The other 3 patients died from tumor progression. The follow-up periods ranged from 10 to 68 months. Complete ablation was achieved in the 14 patients (14/17, 82.35%). Most patients were well tolerated during the whole course except for 1 patient with pneumonedema after TACE and another 1 with biloma after MWA. No marked chemotherapeutic agent-induced toxicity occurred. After chemotherapy or TACE, transient blood indicators and symptoms were observed as follows: myelosuppression, abnormal liver function, gross hematuria, fever, and abdominal pain. Transient symptoms after MWA were fever, abdominal pain, and massive gross hematuria. The present study demonstrates that MWA combined with TACE is a safe and effective method for treating unresectable hepatoblastoma in infants and children with controllable side effects.

Successful management of steroid-resistant vascular tumors associated with the Kasabach-Merritt phenomenon using sirolimus.

Vascular tumors associated with Kasabach-Merritt phenomenon (KMP) are life-threatening and the mortality is as high as 10-30%. Steroids are considered the primary choice for drug therapy. However, there are many steroid-resistant cases. In the present study, analyzed data are presented to support the use of sirolimus in clinical practise for the treatment of corticosteroid-resistant vascular tumors with KMP in eight infants between June 2015 and April 2017 in a single hospital. The time to initial response was 6.8 ± 2.7 days. The average stabilization time for the platelet count was 19.1 ± 8.5 days. At the time of publication, the average duration of sirolimus treatment was 14.1 ± 4.0 months, and the average time for sirolimus treatment as a single agent was 12.6 ± 4.2 months. The side-effects were tolerable and included oral ulcer, fever, pain, skin rash and transient ascension of serum transaminase and cholesterol. Our study indicated that sirolimus therapy is an effective and safe method for the treatment of corticosteroid resistant vascular tumors associated with KMP in infants.

Treatment of Corticosteroid-Resistant Vascular Tumors Associated with the Kasabach-Merritt Phenomenon in Infants: An Approach with Transcatheter Arterial Embolization Plus Vincristine Therapy.

PURPOSE: To investigate the effectiveness and application of transcatheter arterial embolization (TAE) plus systemic vincristine for treatment of corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon in infants. MATERIALS AND METHODS: TAE was performed in 17 infants (average age, 4.3 mo ± 2.4; range, 1-10 mo) with corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon, followed by intravenous vincristine once weekly for systemic chemotherapy. The effects and complications were observed and evaluated after a cycle (1 cycle: TAE plus treatment with vincristine every 4 weeks). Cycles were repeated in infants with platelet counts < 150 × 10(9)/L. RESULTS: In 17 patients, 36 treatment cycles were successfully performed. The platelet count for all patients increased to ≥ 100 × 10(9)/L for the first time at 6.0 days ± 3.5; the platelet level of 15 infants was maintained at levels > 150 × 10(9)/L at 57.5 days ± 16.5. Before treatment, two infants had a normal fibrinogen level (2.21 g/L and 2.34 g/L); the fibrinogen level in the other 15 infants was first found to be increased to ≥ 2.0 g/L at 7.0 days ± 3.4 and was stabilized at levels > 2.0 g/L at 55.9 days ± 13.8 after treatment. Complications were graded as major in four cases and as minor in 13 cases. CONCLUSIONS: TAE plus vincristine can rapidly improve levels of platelets and fibrinogen, and it is an effective method for treatment of corticosteroid-resistant vascular tumors associated with Kasabach-Merritt phenomenon in infants.

Preoperative transcatheter arterial chemoembolization of hepatoblastoma in infants.

PURPOSE: To evaluate the effect of preoperative transcatheter arterial chemoembolization of hepatoblastoma in infants. MATERIALS AND METHODS: Clinical data of 21 infants with hepatoblastoma treated between July 2008 and July 2012 in a single hospital were retrospectively analyzed. After preliminary diagnosis, surgical resection was performed in 9 infants (group I), and transcatheter arterial chemoembolization was performed in 12 infants (group II) before conventional resection. Surgical resection was performed when the tumor bulk appeared sufficiently reduced after transcatheter arterial chemoembolization alone or transcatheter arterial chemoembolization following chemotherapy in cases of pulmonary metastases. RESULTS: Tumor shrinkage ranged from 25%-91% with a mean reduction of 69% (t = 3.816, P = .003) in group II. α-Fetoprotein levels were markedly decreased from 49%-99% with a mean level of 95% (t = 4.871, P = .000) in group II. Specimens in group II showed massive necrosis with a mean percentage of 72% with no significant treatment-related toxicity. In group II, the surgical time was significantly shorter (t = 3.438, P = .003), intraoperative blood loss was considerably less (t = 3.459, P = .003), and the weight of the resected liver was significantly less (t = 3.785, P = .001). Of 21 patients, 16 survived for 50 months without recurrence. CONCLUSIONS: Transcatheter arterial chemoembolization effectively reduced tumor volume, decreased α-fetoprotein, and reduced intraoperative hemorrhage. It represents a safe and effective adjuvant bridge to successful surgery for hepatoblastoma in infants.

Comparison between absolute ethanol and bleomycin for the treatment of venous malformation in children.

The aim of this study was to investigate the therapeutic efficacies and treatment effects of absolute ethanol and bleomycin for the treatment of venous malformation (VM) in children. A total of 138 children with VM were randomly divided into two groups; 75 patients were treated with absolute ethanol, while a further 63 were treated with bleomycin under general anesthesia between February 2009 and February 2012. The treatment outcome and complications were observed in the two groups and the treatment efficacy was classified as one of four categories: cured, markedly effective, effective and ineffective. The curative effect was analyzed 6-24 months after treatment, with a mean of 15 months. Absolute ethanol was effective (cured, markedly effective or effective) in 71 cases and bleomycin was effective in 41 cases, and the difference between the effective rates was considered to be statistically significant (χ2=19.6, P<0.05). In the absolute ethanol group there were 14 cases with skin necrosis, 17 patients had serious localized swelling which required additional treatment, three patients developed muscle fibrosis and one patient suffered a brain embolism. In the bleomycin group there were five cases with skin necrosis and the difference in the incidence of adverse reactions was considered to be statistically significant (χ2=18.8, P<0.05). The curative effect of sclerotherapy for VM is clear, and absolute ethanol is the most effective sclerosing agent, but has a greater incidence of adverse side-effects than bleomycin. The major side-effect is skin necrosis. The choice of sclerotherapy depends on the classification of VM in children.

An effective approach for treating unresectable hepatoblastoma in infants and children: Pre-operative transcatheter arterial chemoembolization.

The objective of the present study was to investigate the feasibility and efficacy of pre-operative transcatheter arterial chemoembolization (TACE) for unresectable hepatoblastoma in infants and children. A total of 24 patients (14 males and 10 females) with unresectable hepatoblastoma, aged between 26 days and 41 months, were treated with pre-operative TACE between March 2007 and March 2011. All cases were confirmed by computed tomography (CT) and liver tumor biopsy prior to TACE. Arteriography was performed and the chemoembolization mixture (pirarubicin and cisplatin emulsified in lipiodol) was injected, followed by polyvinyl alcohol (PVA). The procedure was performed one to four times depending on the patient's response. There was a significant reduction in tumor volume associated with decreased α-fetoprotein (AFP) levels following TACE. Tumor volumes decreased by between 46.1 and 90.2%, with a mean value of 72%. The AFP levels fell by between 63.8 and 99.9%, with a mean value of 95.7%. A total of 22 cases underwent subsequent safe complete surgical resection and the remaining two patients accepted a partial resection. To evaluate the toxicity of TACE, the alanine aminotransferase (ALT), serum creatinine (Cr) and creatine kinase (CK) levels of the patients were measured to assess liver, renal and cardiac function, respectively. The results showed that no marked chemotherapeutic agent-induced toxicity occurred during TACE. It may be concluded that TACE is an effective and feasible pre-operative therapeutic approach for treating unresectable hepatoblastoma and that it may improve the resectability of bulky liver tumors.

Successful treatment of Kasabach-Merritt syndrome with transarterial embolization and corticosteroids.

Kasabach-Merritt syndrome (KMS) refers to the combination of large neonatal vascular tumors and thrombocytopenic coagulopathy. However, a standard treatment regimen for KMS has not yet been established. We report a case of a 6-week-old male infant with life-threatening KMS who was successfully treated with transarterial embolization and corticosteroids. One week after initiating the corticosteroid treatment, his platelet counts recovered, and the lesion growth halted. The approach with corticosteroid therapy resulted in an excellent response that was maintained long enough for us to perform transarterial embolization therapy. The combination of transarterial embolization and corticosteroid therapy should be considered as an option for Kasabach-Merritt syndrome.

Interventional embolization therapy of puerile congenital deep femoral arteriovenous fistula.

This study aimed to investigate the treatment efficiency of interventional embolization therapy in puerile congenital deep femoral arteriovenous fistula. A retrospective analysis was conducted for 9 cases of congenital deep femoral arteriovenous fistulae treated in our department in the past 5 years. B-ultrasound examination indicated that all puerile patients suffered from deep femoral arteriovenous fistulae, which was confirmed by angiography examination. For all patients, endovascular interventional embolization therapy was conducted and angiography re-examination was implemented after 4 weeks. If there were residual orificium fistulae, the interventional embolization therapy was conducted again. In the 6 month to 2 year follow-up period, improvement of clinical symptoms was observed. Following interventional embolization, 9 cases of deep femoral arteriovenous fistulae were completely occluded and the clinical symptoms were improved. No relapses occurred. In addition, after three embolization treatments, the disease condition of one case was controlled well and the disease condition did not progress. Interventional embolization therapy has a number of advantages, including simple surgery and reliable treatment efficacy. Therefore, it is worthy of promotion and application in the clinic.