BackgroundNo large-scale study has compared the outcomes of soft-tissue endoscopic surgery (SOFTES) with conventional open surgery (OS) for vascular anomalies and benign soft tissue tumors. We aimed to compare the operative safety, efficacy, and outcomes of the two approaches for soft tissue lesions.MethodsPatients (n = 414) undergoing SOFTES or OS in two centers were retrospectively reviewed. Propensity score matching was used to minimize selection bias and group differences. The treatment outcomes were compared between the two groups.ResultsAfter matching, 150 patients (SOFTES: 75; OS: 75) were included. Compared with the OS group, the estimated blood loss in the SOFTES group was significantly lower [20 mL (1-500) vs. 50 mL (5-600); p = 0.001]; however, the operative duration was significantly longer [223 minutes (35-490) vs. 173 minutes (37-494); p = 0.008]. Major complications were not observed. The incidence of focal burn of skin in the SOFTES group was higher than that in the OS group (p = 0.048). The incidences of superficial peroneal nerve injury, postoperative bleeding, persistent lymph drainage, local sensory paralyses (<5 cm2), surgical site infection, hematoma/seroma, and residual mild pain were not significantly different between the two groups. However, the incidence of wound dehiscence [n = 0 (0.00%) vs. n = 7 (9.33%); p = 0.010] and flap necrosis [n = 1 (1.33%) vs. n = 11 (14.67%); p = 0.005] was significantly higher in the OS group than in the SOFTES group. Residual pain was cured or significantly improved. All patients achieved resolution of contracture had normal or near-normal joint motion.ConclusionsEndoscopic surgery is a safe and effective treatment option for various vascular anomalies and benign soft tissue tumors in selected patients. This paradigm shift has many advantages in terms of clinical outcomes and reduces postoperative complications.

RATIONALE AND OBJECTIVES:The pathophysiology of arteriolymphatic malformations (ALM) in somatic KRAS-related Parkes Weber syndrome (PWS) of the lower limbs has not been reported. This study aims to characterize the clinical, imaging, and genetic features of ALM in somatic KRAS-related PWS and to redefine this entity. MATERIALS AND METHODS:This retrospective observational study (2019-2024) analyzed patients from the Vascular Anomalies Center database who met the following criteria: (1) lower limb PWS confirmed clinically/radiologically; (2) lymphatic involvement; and (3) somatic KRAS mutation in lesional tissue. The exclusion criteria were incomplete records/no genetic testing. Diagnosis of ALM requires simultaneous arterial-phase opacification of the veins and lymphatics on computed tomography angiography and/or blood-tinged chyle on direct puncture, as confirmed by correlating femoral arteriography/lymphography. The data included demographics, imaging, and somatic genetic analyses. RESULTS:Twenty-seven patients (median 6 years) were included in the study. All 27 patients (100%) exhibited a discrepancy in the lower limb length (>1 cm). Somatic KRAS mutations: p.G12D (24/27, 89%), p.Q22K (3/27, 11%). ALM was radiologically confirmed. Chyle leakage occurred in 19/27 (70%) of patients. Lymphangiectasia was detected preoperatively in 18/27 (67%). Central conducting lymphatic incompetence was subclinical in 5/27 (19%). The associated anomalies included renal hypoplasia (1/27, 4%), horseshoe kidney (1/27, 4%), renal artery stenosis (1/27, 4%), femoral vein hypoplasia (2/27, 7%), and persistent sciatic vein (2/27, 7%). CONCLUSION:This cohort delineated a distinct subtype of somatic KRAS-related PWS characterized by peripheral ALM, limb overgrowth, specific KRAS variants, and the absence of systemic involvement or germline transmission, supporting the redefinition of capillary-lymphatic-arteriovenous malformation terminology.

INTRODUCTION:This study aimed to characterize the clinical, radiological, and histopathological features of the rare and often misdiagnosed verrucous venous malformation-subcutaneous variant (VVM-SV) and evaluate its management outcomes. METHODS:We conducted a retrospective review of 33 patients with histopathologically confirmed VVM-SV from three centers (2010-2025). Collected data included demographics, clinical presentation, imaging findings, prior treatments, and surgical outcomes following open (n = 6) or endoscopic (n = 9) excision. RESULTS:Most patients (30/33) were initially misdiagnosed, commonly as a common venous malformation, and 29 had undergone unsuccessful sclerotherapy. Typical presentation was a solitary, firm, bluish subcutaneous nodules, most often on the finger (n = 21). Imaging and surgical findings revealed a well-circumscribed, nodular mass with minimal venous flow. Histopathology confirmed that adipose tissue was surrounded by well-demarcated venous channels. Surgical excision was curative in all 15 operated patients, with no recurrences over a median follow-up of 19 mo. CONCLUSIONS:VVM-SV is a distinct clinicopathological entity frequently misdiagnosed, leading to inappropriate treatment. Accurate diagnosis relies on clinicopathological correlation, recognizing its subcutaneous location and minimal flow. Surgical excision is the definitive curative treatment.

Purpose: Historically, open surgery has been the treatment of choice for soft tissue lesions. This study aims to report an innovative soft tissue endoscopic surgery for subcutaneous and intramuscular lesions at our center. Methods: For this retrospective review, data on sex, age, symptoms, diagnosis, sites of surgery, previous treatment, surgery, and follow-up were collected from our Vascular Anomalies Center database. Patients with soft tissue lesions who had undergone endoscopic surgery between September 2019 and March 2024 were included. Soft tissue endoscopic surgeries included totally endoscopic surgery, endoscopy-assisted surgery, and conversion to open surgery. Results: In total, 122 patients were included: 74 females and 48 males, with ages ranging from 1 to 38 years (median, 7.5 years). Conditions for endoscopic surgery included various vascular anomalies and benign soft tissue tumors. Surgical sites included the lower extremity, upper extremity, abdominal wall, thoracic wall, back, gluteal region, and face. One hundred patients underwent totally endoscopic surgery, and 22 underwent endoscopy-assisted or conversion to open surgery. The operative duration was 40-530 minutes (median, 222 minutes). Blood loss was 1-1400 mL (median, 30 mL). No hemorrhage or wound dehiscence was observed within 30 days after surgery. Conclusions: Various subcutaneous and intramuscular conditions can be successfully managed with soft tissue endoscopic surgery. We think that this novel treatment approach can provide valuable references for clinicians of many specialties, encompassing general surgery, pediatric surgery, vascular surgery, plastic surgery, dermatologic surgery, and orthopedics.

Congenital aplasia, atresia, and hypoplasia of the iliac and lower extremity veins are rare vascular anomalies characterized by disrupted venous drainage and compensatory collateralization. Despite the clinical significance of these anomalies, their embryological origins, anatomical patterns, and hemodynamic consequences remain understudied. This study aimed to elucidate the anatomical variability, collateral pathways, and clinical implications of these anomalies through a single-center retrospective analysis. Chinese patients with lower extremity congenital capillary-venous malformations and deep venous aplasia/atresia/hypoplasia were included. Imaging protocols included color Doppler ultrasound in all cases, supplemented by magnetic resonance and/or computed tomography venography in selected cases. An evaluation of the patient demographics demonstrated a male predominance (male:female, 40:24), mean age of 6.9 ± 6.7 years (2 months to 33 years), and 59.4

Klippel–Trénaunay syndrome (KTS) typically involves a combination of capillary, lymphatic, and venous malformations of the lower extremities. Genitourinary involvement is frequently observed in patients with KTS. Bleeding and pelvic pain are the most common complications. This condition has been increasingly reported in recent years. However, most authors have only depicted extreme presentations or various combinations of clinical findings. The underlying pathophysiology of genitourinary involvement in KTS remains unclear. Genitourinary involvement in female patients with KTS has a more complicated pathophysiology than that in male patients with KTS. After understanding its pathophysiology, some patients can be successfully managed by using a staged plan. Therefore, recognizing the pathophysiology of genitourinary involvement is necessary for practitioners to evaluate and determine adequate management. Owing to the complexity and rarity of this condition, a prospective controlled study involving a large cohort of patients is impossible. Based on a literature review and our practice, we discuss the pathophysiology, evaluation, and treatment strategies for genitourinary involvement in KTS.

BackgroundOpen surgery is the treatment of choice for verrucous venous malformation (VVM) and capillary arteriovenous malformation (CAVM) with overgrowth. The study aimed to report an innovative endoscopic resection technique for subcutaneous vascular malformations with superficial lesions at our center.MethodsWe retrospectively reviewed the medical records of patients who underwent endoscopic resection for VVM or CAVM between September 2019 and July 2024.ResultsThe current cohort includes 14 female and 4 male patients, with ages ranging from 1 year to 8 years. Diagnoses included classic VVM (n = 10), VVM—subcutaneous variant (n = 4), and CAVM (n = 4). Endoscopic surgery uses two or more small ports in a gas-inflated manner. Surgery included radical resection, partial resection of the subcutaneous mass, and debulking of adipose overgrowth. Technical success was achieved in all patients. Local skin necrosis (area <1 cm2) occurred in two patients. No recurrence was observed during follow-up.ConclusionEndoscopic resection is a minimally invasive, feasible, and safe technique for VVMs in selected CAVM. Better cosmetic results can be expected in patients with superficial lesions using this endoscopic surgical technique.

BackgroundCongenital hepatic hemangioma with arteriovenous fistula (HHAVF) is a rare condition in newborns that may manifest as respiratory distress, pulmonary hypertension, and heart failure shortly after birth. This report describes a case of HHAVF complicated by encephalomalacia identified after transcatheter arterial embolization (TAE). However, the condition improved with multidisciplinary management and long-term follow-up.Case presentationA full-term female infant presented with a cardiac murmur and pulmonary hypertension at birth. Contrast-enhanced CT demonstrated multiple hepatic hemangiomas with high-flow arteriovenous shunting. Pulmonary hypertension resolved after TAE; however, the recurrence of hepatic hemangioma required oral propranolol therapy, which led to complete regression by 18 months of age. Postprocedural imaging identified encephalomalacia in the right frontotemporal and parietal lobes, as well as the basal ganglia, concurrent with left-limb motor impairment. Long-term rehabilitation improved left-limb function and the extent of encephalomalacia stabilized.ConclusionsCurrent research primarily focuses on early cardiopulmonary complications in HHAVF, while multidisciplinary management strategies and long-term outcomes, particularly neurological manifestations, are rarely reported.

BACKGROUND:Open surgery is the standard treatment for fibroadipose vascular anomalies (FAVA). This study evaluated an endoscopic approach for FAVA, assessing its safety and feasibility. METHODS:Data from October 1, 2019, and May 1, 2024, were analyzed from the Vascular Anomalies Center database at our institution. Patients with FAVA Stage I (pain stage) or II (contracture stage) were included and divided into the endoscopic surgery group (ESG) and open surgery group (OSG). RESULTS:In total, 101 patients underwent 110 procedures. In ESG, 42 patients (16 males and 26 females; 31 with Stage I and 11 with Stage II) underwent radical excision (with tendon lengthening in 2 patients). Ten patients underwent an open surgery. In OSG, 63 patients (18 males and 45 females; 30 with Stage I and 33 with Stage II) underwent radical excision (with tendon lengthening in 12 patients). The OSG had significantly shorter operative time than the ESG (p = 0.009). Furthermore, The OSG had longer hospitalization time (p = 0.022) than the ESG. The blood loss in the OSG was greater than that in the ESG, but without statistical difference (p = 0.069). During follow-up, the incidence of wound complications (p = 0.121) and residual symptoms (p = 0.179) were slightly higher in the OSG. CONCLUSIONS:Endoscopic surgery is a safe and effective treatment for FAVA, promoting faster habilitation and improved patient satisfaction. This method may also serve as a reference for the excision of other benign muscle and soft tissue masses.

Purpose We aimed to report an innovative single-site endoscopic surgery for soft tissue lesions performed at our center. Methods All patients who underwent soft tissue surgery were reviewed. All consecutive patients who underwent single-site endoscopic surgery between September 2019 and March 2024 were included in the study. Data were extracted from our medical records database, including sex, age, diagnosis, sites of surgery, surgery, operation time, blood loss, and follow-up. Results There were 10 females and five males in the current study, with ages ranging from one year to 26 years (median = nine years). Conditions for surgery included enlargement of the leg (n = 5), fibro-adipose vascular anomaly (n = 4), microcystic lymphatic malformation (n = 3), venous malformation with thrombosis (n = 3), and borderline tumor (n = 2). Surgical sites included the lower extremity (n = 13) and upper extremity (n = 2). Perioperative thrombosis prophylaxis included elastic compression and subcutaneous low-molecular-weight heparin in patients who had venous malformation and localized intravascular coagulopathy for three or more days. Surgery included tumor en bloc resection, tumor partial resection, lymphedema debulking, microcystic lymphatic malformation debulking, limb debulking, intramuscular lesion radical resection, thrombectomy, Achilles lengthening, relaxation of the ankle capsule, gastrocnemius recession, and tendon transfer. Technical success was obtained in all patients. Operative duration ranged from 66 to 455 minutes (median = 183 minutes). Blood loss ranged from 5 to 700 mL (median = 50 mL). One patient received a blood transfusion. Conclusions This retrospective review demonstrates the feasibility of using a single-site endoscopic approach to resection of subcutaneous, muscular and tendinous lesions and proof of principle for future soft tissue surgery.

BackgroundTo explore the management of lymphatic malformation in head and neck.MethodsThis is a retrospective study at a single center. Data on demographic, surgery, sclerotherapy and follow-up information were collected from our Vascular Anomalies Center database. Patients with lymphatic malformation of head and neck who had undergone surgery and sclerotherapy between March 2020 and March 2024 were included.ResultsThere were 94 patients in this study, the lesion sites included head (n = 60), tongue (n = 7), neck (n = 41), pharynx (n = 7), and head and neck (n = 7). Symptoms included bleeding (n = 6), infection (n = 2), dyspnea (n = 2), dysphonia (n = 4), and dysphagia (n = 4). Lymphatic malformation included macrocystic (n = 61), microcystic (n = 12) and mixed (n = 21). Surgeries for LM included radical resection, subtotal or partial resection and staged surgeries. Sclerotherapies included bleomycin monotherapy and combined sclerotherapy with ethanol and bleomycin, under ultrasound or fluoroscopy guidance. The follow-up period was from 3 months to 1 year. The therapeutic effect was evaluated according to the size of the treatment area. 55 patients, 21 patients, 11 patients and 7 patients were evaluated with excellent, good, moderate and no response, respectively.ConclusionSurgical resection, sclerotherapy and the combination of the two are efficacious treatment modalities for head and neck LM. Combined with oral drugs and other new therapies may be warranted in future for challenging conditions.

Background The diagnosis and treatment of fibro-adipose vascular anomaly (FAVA) of the limb remains challenging since this entity is rare and complex. This paper is aimed to describe the clinical and imaging features, staging and management of this underrecognized disease of the limb. Material and method Patients diagnosed with FAVA and managed between September 2019 and May 2022 in department of pediatric surgery & vascular anomalies of Xi’an international medical center hospital were retrospectively reviewed. Data extracted include age at presentation, previous diagnosis, affected muscles, symptoms, previous treatment, our management, and follow-up. Results Thirty-two patients with FAVA were diagnosed and managed in our center. There was a female sex predominance, with 23 female (72%) and 9 male (28%) in the cohort. Only one lesion was noticed during infancy; the remaining presented at age 1 to 20 years (median, 7 years). The most commonly involved muscles were gastrocnemius (14/32, 44%) and soleus (13/32, 40%). Swelling (mass), pain and contractures were the most common presentations. MRI featured a heterogeneous and ill-defined intramuscular high signal intensity. Diseases were staged according to clinical features: stage I (pain stage, n = 4), stage II (contracture stage, n = 20) and stage III (deformity stage, n = 8). Patients with stage I disease underwent radical resection and obtained a cure. Patients with stage II disease received radical resection and possible Achilles lengthening, having an outcome of cure. Personalized treatment was required in patients with stage III disease, including radical/partial/staged resection, Achilles lengthening/tenotomy, joint capsulotomy, neurolysis/neurectomy, tendon transfer, stretching exercises, and oral sirolimus/alpelisib. Significant improvement of symptoms was achieved in most. Conclusion The most distinct features of FAVA include enlarging mass, severe pain and contracture. Based on distinct clinical and radiologic features, it is not difficult to make the diagnosis of FAVA. Earlier awareness of this disease can reduce misdiagnoses. Surgery-based comprehensive management can typically improve pain and contracture. Oral sirolimus or alpelisib plays an important role in treatment of unresectable lesions and major nerve involvement. Surgery alone can be curative in early stage FAVA.

Objective: To describe the curettage and sclerotherapy technique, a hybrid approach to treatment for superficial lymphatic malformations. Methods: A retrospective analysis of a lymphatic malformation data base was performed. Patients with superficial lymphatic malformation treated by curettage and sclerotherapy technique with bleomycin were included in this study. Safety and efficacy of the curettage and sclerotherapy technique with bleomycin were evaluated. Results: Between September 2019 and October 2021, 10 consecutive patients (male/female ratio: 4:6; mean age, 10.9 years; range, 3-35 years) presented with superficial lymphatic malformations that were subsequently treated by curettage and sclerotherapy technique with bleomycin. All lesions were located on the trunk and extremities. Each patient received one treatment session and a mean follow-up of 12 months. We observed minor complications. No postoperative infections occurred in this series. No patient developed skin ulceration or necrosis. Scarring and recurrence occurred in one patient. Complete regression was confirmed for all 10 patients by photographic evaluation. Conclusion: A curettage and sclerotherapy technique is proposed to treat superficial lymphatic malformation in this study. This technique seems to be safe and highly effective.

Klippel-Trénaunay syndrome is typically a complex combined capillary-lymphatic-venous malformation in lower limb. Gastrointestinal involvement is not infrequent in Klippel-Trénaunay syndrome. Rectal bleeding is the most common complication. In recent years, this condition has been increasingly reported. However, most authors simply described extreme manifestations or various combinations of clinical observations. The underlying pathophysiology of gastrointestinal involvement in Klippel-Trénaunay syndrome has been underrecognized. Pathophysiologically, some seemingly adequate managements are pitfalls in treatment. Anorectosigmoid vascular malformations in KTS have distinct and more complicated pathophysiologies than anorectal vascular malformation. Once understanding the pathophysiology, some patients can be successfully managed with a staged plan in our practice. Therefore, recognizing the pathophysiologies of gastrointestinal involvement is needed to evaluate, prevent pitfalls, and determine adequate managements for practitioners. Because of the complexity and rarity of this condition, prospective controlled study or a large cohort of patients is impossible. Based on literature review and our practice, we discuss pathophysiologies, evaluation, pitfalls, and treatment strategies for gastrointestinal involvement in Klippel-Trénaunay syndrome.

目的 探讨胸腔镜下介入治疗纵隔淋巴管畸形(LM)的安全性及有效性.方法 收集2017年1月至2021年1月西安交通大学第二附属医院、西安国际医学中心医院收治的9例纵隔LM患者的临床资料.2019年9月之前4例患者治疗方法以手术切除为主,创面喷洒平阳霉素.2019年9月之后由于杂交手术室的使用,5例患者以胸腔镜下介入治疗为主,术中采用无水乙醇冲洗囊腔后注射造影剂与平阳霉素的混合物,必要时多点注射,术后1周再次经猪尾导管注射平阳霉素,统计并发症发生情况及临床治疗效果评分.结果 手术治疗后轻度并发症为(4.8±1.0)次,高于介入治疗后的(2.6±0.9)次(P﹤0.05).手术治疗的临床治疗效果评分为(4.0±0.8)分,与介入治疗的(3.8±0.4)分比较,差异无统计学意义(P﹥0.05).结论 胸腔镜下介入治疗大囊型、混合型纵隔LM是一种安全、有效的治疗方式.

OBJECTIVE:To report an innovative endoscopic surgery for subcutaneous vascular malformations and intramuscular fibro-adipose vascular anomaly (FAVA) at our center. BACKGROUND:Historically, open surgical resection has been the treatment of choice. Recent advances in minimally invasive surgery have led to the successful application of endoscopic resection techniques for the surgical management of diseases of soft tissue. METHODS:Patients who underwent endoscopic resection of vascular anomalies were included in this retrospective review. Data were extracted from our Vascular Anomalies Center database between September 2019 and October 2022, including sex, age, symptoms, diagnosis, sites of surgery, previous treatment, surgery, and follow-up. RESULTS:There were 13 females and 15 males in the current study, with ages ranging from 1 to 17 years. The diagnoses included microcystic lymphatic malformation (LM) (n = 8), Klippel-Trénaunay syndrome (n = 7), venous malformation (n = 6), FAVA (n = 6), and mixed cystic LM (n = 1). Surgical sites included the lower extremity (n = 24), abdominal wall (n = 2), upper extremity (n = 1), and thoracic wall (n = 1). Five patients had an intramuscular lesion (FAVA). The endoscopic technique used 2 or 3 small ports in a gas inflation manner. Surgery included thrombectomy, radical resection, and debulking of vascular anomalies. Postoperative sclerotherapy with bleomycin was performed through a drainage tube in 6 patients with microcystic LM. Technical success was obtained in 27 patients. The conversion to open surgery was performed in one patient owing to the deep location of the lesion. No wound-related complication was observed. CONCLUSIONS:Endoscopic surgery is a minimally invasive, effective, and safe treatment for subcutaneous vascular malformations and intramuscular FAVA. This approach can set a new standard that minimizes wound complications and reduces recovery time in patients undergoing resection for benign soft-tissue lesions.

Objective:To explore the diagnosis, staging, and treatment of fibro-adipose vascular anomaly(FAVA).Methods:The data of the patients with FAVA admitted to Xi’an International Medical Center Hospital between October 2019 and February 2023 were retrospectively reviewed. Ultrasound and magnetic resonance imaging (MRI) were routinely performed. X-ray and CT were performed for patients with unequal length of lower limbs, lesions involving joints, and obvious joint deformities. The treatment plan was made according to the stage: stage Ⅰ (pain stage), open or laparoscopi radical resection of the lesion was performed; stage Ⅱ (contracture stage), radical surgery was performed to remove the lesion, sometimes combined with Achilles tendon lengthening or tenolysis, and rehabilitation training was performed 2 weeks after surgery; stage Ⅲ(deformity stage), comprehensive treatment based on surgical resection was adopted, combined with joint capsule release, Achilles tendon lengthening or dissection, tendon transfer and oral sirolimus (each time 0.08 mg/m 2, twice daily ) before and after the operation. For patients with lesions involving multiple anatomical regions, staged surgery was performed, and in principle, only one anatomical region was operated per time. Patients were followed up for pain, joint activity and recurrence. Results:A total of 42 patients were admitted, including 18 males and 24 females. The onset age was (7.3±5.0) years, but the average age of diagnosis was (12.5±6.0) years. The lower limbs were involved in 38 cases, the upper limbs in 4 cases. There were 17 cases of stage Ⅰ, 17 cases of stage Ⅱ and 8 cases of stage Ⅲ. Only 4 cases had no misdiagnosis experience, and the misdiagnosis rate was 90.5%(38/42). Persistent pain, muscle contractures and joint deformities were the main clinical symptoms of the disease. MRI showed heterogeneous high and low signal intensity on T1-weighted images, and the high signal intensity was the same as that of subcutaneous adipose tissue. T2 fat-suppressed sequences showed stronger heterogeneous hyperintensity. The follow-up time was (14.6±10.8) months. Patients who took sirolimus orally before or after surgery experienced significant relief of pain symptoms. Of the 42 patients, the symptoms of 31 patients were completely relieved after the operation, and 11 patients still had residual pain or joint movement disorder or even deformity after the operation. Sixteen of 17 stage Ⅰ patients were cured, the lesion was further expanded and the pain recurred in 1 case after the operation. Of the 17 stage Ⅱ patients, 15 were cured, and 2 had mild limitation of ankle movement after the operation. Eight stage Ⅲ patients had varying degrees of pain or joint movement disorder after surgery, postoperative oral sirolimus significantly relieved symptoms. All 10 patients with stage Ⅰ and Ⅱ who underwent endoscopic resection were cured.Conclusion:FAVA usually occurred in school-age and adolescent children. Pain, muscle contracture and joint deformity were the characteristic clinical features. Combined with MRI features, the diagnosis can be confirmed. FAVA staging system could guide treatment and predict prognosis. For stage Ⅰ and Ⅱ patients, surgery should be performed as soon as possible and the prognosis was good. For stage Ⅲ patients, surgery-based comprehensive treatment could improve symptoms, but it was difficult to cure them radically. Oral sirolimus was also required after surgery.

Objective:To explore the diagnostic essentials and therapeutic principle of fibro-adipose vascular anomaly(FAVA).Methods:Clinical data were retrospectively reviewed for 13 FAVA children.And the relevant data included gender, age at diagnosis, clinical features, anatomic involvement, previous diagnosis and management, radiological features, management, histopathological findings, rehabilitation and follow-up plan.Results:From September 2019 to December 2020, a total of 13 cases of FAVA were diagnosed and treated in our center. Early diagnosis included: venous malformation ( n=8), intramyaloid hemangioma ( n=2), muscular lymphatic malformation ( n=1), arteriovenous malformation ( n=1) and cavernous hemangioma ( n=1) . There were 4 males and 9 females; the age at diagnosis ranged from 1 year and 2 months to 18 years old, with an average age of 10 years. 11 cases of lower extremity lesions, 2 cases of upper extremity. None of the cases involved the trunk. 5 cases were accompanied by hypersensitivity of the skin at the swollen part, and the pain was severe when lightly pressed. 11 cases were associated with reduced social activities, and the patients had a strong sense of inferiority and reduced social interaction with their peers. One case of upper limb patient was relieved of pain after oral administration of sirolimus; the remaining 12 cases underwent surgical treatment, and all of them underwent radical resection, that is, under the premise of protecting important blood vessels and nerves, enlarged resection of localized lesions; diffuse lesions resection of involved lesions The entire muscle and myofascia are affected; if important nerves are involved, incision and release of the epineurium are required; if tendon contracture causes joint mobility impairment, after removal of the entire affected muscle and myofascia, tendon lengthening/transfer is required to Reconstruction function; 12 cases were indwelling wound negative pressure drainage; tendon lengthening/transfer, joint ankylosing correction, plaster immobilization for 1 to 4 weeks. The postoperative follow-up was 1-16 months, and the joint mobility disorder was cured or improved significantly. Conclusion:The most distinct features of FAVA include pain, contracture and joint deformity.Based upon unique clinical and radiologic features, it is not difficult to make a definite diagnosis of FAVA.The management strategy for FAVA should be formulated according to staging.With appropriate surgical approaches surgery-based management plan is efficacious for relief of pain, contractures and function improvement.

Wilms' tumor is the most common type of renal tumor in children. MicroRNAs (miRNAs) are small noncoding RNAs that play crucial regulatory roles in tumorigenesis. We aimed to study the expression profile and function of miR-27a-5p in Wilms' tumor. miR-27a-5p expression was downregulated in human Wilms' tumor tissues. Functionally, overexpression of miR-27a-5p promoted cell apoptosis of Wilms' tumor cells. Furthermore, upregulated miR-27a-5p delayed xenograft Wilms' tumor tumorigenesis in vivo. Bioinformatics analysis predicted that miR-27a-5p directly targeted the 3'-untranslated region (3'-UTR) of PBOV1, and luciferase reporter assay confirmed the interaction between miR-27a-5p and PBOV1. The function of PBOV1 in Wilms' tumor was evaluated in vitro, and knockdown of PBOV1 dampened cell migration. In addition, overexpression of PBOV1 antagonized the tumor-suppressive effect of miR-27a-5p in Wilms' tumor cells. Collectively, our findings reveal the regulatory axis of miR-27a-5p/PBOV1 in Wilms' tumor, and miR-27a-5p might serve as a novel therapeutic target in Wilms' tumor.

To the Editor: We read with great interest the recent report "Intralesional bleomycin injection for localized verrucous venous malformations" (VVMs) by Chang et al.1Chang S.J. Qian L. Yang X. et al.Intralesional bleomycin injection for localized verrucous venous malformations.J Am Acad Dermatol. 2021; 85: 991-992Abstract Full Text Full Text PDF PubMed Scopus (0) Google Scholar We would like to comment on the management and response evaluation for VVMs. 1. A localized VVM is a distinct vascular malformation from common venous malformation. Recurrence after surgery should not occur if adequate margins are obtained and the full depth of the VVM is resected.2Calduch L. Ortega C. Navarro V. Martínez E. Molina I. Jordá E. Verrucous hemangioma: report of two cases and review of the literature.Pediatr Dermatol. 2000; 17: 213-217Crossref PubMed Scopus (41) Google Scholar High recurrence rate is because of inadequate excision. Therefore, local resection is still the first-line treatment for localized VVMs.2Calduch L. Ortega C. Navarro V. Martínez E. Molina I. Jordá E. Verrucous hemangioma: report of two cases and review of the literature.Pediatr Dermatol. 2000; 17: 213-217Crossref PubMed Scopus (41) Google Scholar 2. The VVM lesions typically involve the epidermis, dermis, and subcutaneous fat.2Calduch L. Ortega C. Navarro V. Martínez E. Molina I. Jordá E. Verrucous hemangioma: report of two cases and review of the literature.Pediatr Dermatol. 2000; 17: 213-217Crossref PubMed Scopus (41) Google Scholar Cosmetic concern is the main issue, and complications such as bleeding, pain, and functional impairment are rare. In this case series, 14 of 15 patients have no symptoms. Generally, the absence of symptoms is not indicative of intervention. The main goal of intervention is usually cosmetic outcomes and not the reduction of the subcutaneous part of VVMs. Until now, corresponding improvement in appearance has been rarely observed following intralesional bleomycin injection for ablating the subcutaneous part of VVMs in the literature. Imaging responses cannot be interpreted to improve cosmetic concerns and subjective symptoms in patients. Furthermore, posttreatment pain (n = 2), skin ulcers (n = 5), and scarring (n = 2) occurred in 9 of the 15 patients in this report.1Chang S.J. Qian L. Yang X. et al.Intralesional bleomycin injection for localized verrucous venous malformations.J Am Acad Dermatol. 2021; 85: 991-992Abstract Full Text Full Text PDF PubMed Scopus (0) Google Scholar However, those patients had no symptoms prior to treatment.1Chang S.J. Qian L. Yang X. et al.Intralesional bleomycin injection for localized verrucous venous malformations.J Am Acad Dermatol. 2021; 85: 991-992Abstract Full Text Full Text PDF PubMed Scopus (0) Google Scholar In our experience, skin ulcers following intralesional bleomycin injection for VVMs are often very difficult to cure and may persist for months, even more than a year, despite specialized dressing change (Fig 1). We recommend that the indications and responses of intervention in VVMs should be comprehensively evaluated. 3. The primary outcome of this prospective trial was evaluated by photographs1Chang S.J. Qian L. Yang X. et al.Intralesional bleomycin injection for localized verrucous venous malformations.J Am Acad Dermatol. 2021; 85: 991-992Abstract Full Text Full Text PDF PubMed Scopus (0) Google Scholar; however, the authors did not provide pretreatment and posttreatment photographs as the primary evaluation. 4. The statement "intralesional bleomycin treatment could delay its progression"1Chang S.J. Qian L. Yang X. et al.Intralesional bleomycin injection for localized verrucous venous malformations.J Am Acad Dermatol. 2021; 85: 991-992Abstract Full Text Full Text PDF PubMed Scopus (0) Google Scholar cannot be concluded from the results of this article. In patients with localized VVMs, esthetic concerns are the main indication for treatment. The intervention is mainly aimed at improving local appearance. In our opinion, complete surgical excision remains the first treatment of choice when feasible for localized VVMs. The side effects of bleomycin, including local ulceration, pigmentation, and potential pulmonary fibrosis, should be considered in advocating intralesional injection. In the article by Chang et al,1Chang S.J. Qian L. Yang X. et al.Intralesional bleomycin injection for localized verrucous venous malformations.J Am Acad Dermatol. 2021; 85: 991-992Abstract Full Text Full Text PDF PubMed Scopus (0) Google Scholar the lack of clinical photographs (the primary objective) makes it difficult for the reader to evaluate the reported responses. Responses should be mainly evaluated by photographs and symptom improvement. None disclosed. Authors' reply: Intralesional bleomycin injection for localized verrucous venous malformationsJournal of the American Academy of DermatologyVol. 87Issue 3PreviewTo the Editor: We thank Wang et al1 for their comment on our study. Verrucous venous malformations (VVMs) are a tenacious form of vascular malformation. They typically present as red or purple nonkeratinized patches in their early stages, which then gradually increase in size and subcutaneous depth and develop hyperkeratotic skin surfaces without regression. Hyperkeratotic skin surfaces commonly develop in childhood and can prompt bleeding, oozing, infections, ulcerations, etc. Most of the patients in our study had not yet developed thick, warty keratinized skin surfaces at the time of treatment. Full-Text PDF