Endoscopic Resection for Vascular Anomalies in Children: A New Standard.

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.

Curettage and sclerotherapy technique: A hybrid approach to superficial lymphatic malformation.

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.

miR-378c suppresses Wilms tumor development via negatively regulating CAMKK2.

Wilms tumor is a rare kidney malignancy primarily developed in children. Treatment for Wilms tumor includes surgery, radiotherapy, and chemotherapy. Recent studies have demonstrated that microRNAs (miRNAs) play important roles in regulating Wilms tumor development. In this study, we aimed to elucidate the expression and function of miR-378c in Wilms tumor. Quantitative real-time PCR (qRT-PCR) results showed that miR-378c was downregulated in Wilms tumor tissues and cell lines. Functionally, further CCK-8, would healing, and transwell assays revealed that overexpression of miR-378c impaired Wilms tumor cell growth and metastasis in vitro. In addition, xenograft assay showed that miR-378c overexpression inhibited Wilms tumor development in vivo. Mechanistically, luciferase reporter assay confirmed that miR-378c directly targets CAMKK2 in Wilms tumor. qRT-PCR and western blot assays demonstrated that CAMKK2 was highly expressed in Wilms tumor tissues and cell lines. Rescue experiments were performed to further evaluate the functional relationship between miR-378c and CAMKK2. Overexpression of miR-378c suppressed Wilms tumor cell metastasis via negatively regulating CAMKK2 expression. Consistently, inhibition of miR-378c enhanced Wilms tumor cell malignancy behavior via augmenting CAMKK2 expression, which could be abrogated by CAMKK2 knockdown. In summary, our findings suggest that miR-378c inhibits the development and metastasis of Wilms tumor via negatively regulating CAMKK2 expression, which could be utilized to develop new therapy strategy.

Propranolol induces regression of hemangioma cells via the down-regulation of the PI3K/Akt/eNOS/VEGF pathway.

BACKGROUND: Infantile hemangioma (IH) is a benign vascular neoplasm resulting from the abnormal proliferation of endothelial cells and pericytes in infants. Propranolol, a non-selective ß-adrenergic blocker, has recently emerged as an effective therapy for IH, causing regression. However, its potential therapeutic mechanism remains largely unknown. PROCEDURE: An XPTS-1 cell line was established by isolating hemangioma-derived endothelial cells (HemECs) from a specimen of human proliferating IH. Flow cytometer assay was performed to assess the effect of propranolol on cell cycle distribution. Western blot was employed to determine changes of protein expression. Matrigel invasion and tube formation assays were used to measure invasion ability and tube formation ability, respectively. Commercial kits were employed to quantify NO and VEGF levels. RESULTS: Propranolol blocked norepinephrine-induced HemECs cell cycle progression as well as the expression of cyclin A2 and cyclin D2; whereas p21 and p27 proteins were altered conversely. Propranolol inhibited norepinephrine-induced cell invasion by reducing the expression of MMP-9, VEGF, and p-cofilin. NO and VEGF release induced by norepinephrine was decreased by propranolol pretreatment, coincident with alterations in the phosphorylation of Akt, eNOS, and VEGFR-2. Tube formation ability and subsequent levels of NO and VEGF elevated by norepinephrine were distinctively counteracted in HemECs. CONCLUSIONS: The current study demonstrated the antiangiogenic properties of propranolol in vitro and that the drug was able to induce the regression of hemangioma cells via the inhibition of cell cycle progression, invasion, and tube formation, concomitantly with decreased NO and VEGF levels through the down-regulation of the PI3K/Akt/eNOS/VEGF pathway.

Management for lymphatic malformations of head and neck.

Background: To explore the management of lymphatic malformation in head and neck. Methods: This 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. Results: There 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. Conclusion: Surgical 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.

Gastrointestinal involvement in Klippel-Trénaunay syndrome: pathophysiology, evaluation, and management.

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.

Fibro-adipose vascular anomaly (FAVA) - diagnosis, staging and management.

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.

MicroRNA-27a-5p Inhibits Proliferation, Migration, and Invasion and Promotes Apoptosis of Wilms' Tumor Cell by Targeting PBOV1.

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.

Internal Iliac Vein Reflux: An Underrecognized Pathophysiology in Klippel-Trénaunay Syndrome With Pelvis Involvement.

Background and objective Internal iliac vein reflux (IIVR) has been underrecognized in Klippel-Trénaunay syndrome (KTS) with pelvis involvement. In this study, our aim was to report clinical and radiological characteristics, modified phlebography technique, and diagnostic and treatment algorithm and outcomes of IIVR in KTS patients with pelvis involvement. Materials and methods KTS patients diagnosed between May 2014 and January 2021 were retrospectively analyzed. The medical records and imaging studies of all patients with KTS of the lower extremities were included and reviewed. KTS was defined as the triad of capillary malformation, venous malformation, and limb overgrowth. Pelvis involvement was evaluated with MRI. Phlebography was performed if IIVR was suspected. IIVR ablation and sclerotherapy were performed if IIVR was confirmed in KTS patients with external genitalia/perineum manifestation and bleeding. Patients were followed up via outpatient consultations. Episodes of gross bleeding were specifically investigated. Results A total of 211 patients with lower limb KTS diagnosed by our team were included in the study. Unilateral IIVR was diagnosed in 97 patients, and bilateral IIVR in two patients; 117 KTS patients were managed with radiological intervention and/or hybrid surgery by our team. Eleven patients underwent an IIVR ablation procedure due to recurrent bleeding from pelvic organs. Postprocedural complications included transient fever (n=2) and mild anaphylactic reaction (n=1). A small hyperpigmented scar at the incision and/or accessing site was noticed in patients receiving bleomycin during the procedure (n=6). Bleeding episodes and anemia resolved in all patients during the follow-up period. Correspondingly, the involved IIV and its tributaries were found to have disappeared on imaging during the follow-up. Conclusion IIVR is common in KTS patients, and it can cause bleeding from pelvic organs. Bleeding can be managed with IIVR ablation and sclerotherapy in KTS with pelvis involvement.

Liposuction-Like Sclerotherapy Technique for Microcystic Lymphatic Malformation.

Background The treatment for microcystic lymphatic malformation (LM) remains challenging. We describe the liposuction-like sclerotherapy technique, a new treatment for extensive microcystic LM. Methods LM data was retrospectively reviewed. This study included patients with a microcystic LM component treated by liposuction-like technique with bleomycin sclerotherapy. Results Between June 2016 and October 2019, 39 consecutive patients (male/female ratio: 21:18; mean age, 33.6 months; range: 5 months to 15 years) with microcystic LM were treated by liposuction-like sclerotherapy (LS-LS) technique. Fifty-six sessions of LS-LS were performed (mean of 1.44 sessions per patient; range: one to four sessions). Follow-up ranged 6-30 months (mean of 21 months). We observed no major complications. Transient minor complications included: postoperative noninfectious fever, vomiting, temporary skin edema, pigmentation, mild local depressions, and/or irregularities, and a small hyperpigmented scar at the incision. No postoperative infection, skin ulcer, or necrosis occurred. The patients' symptoms were successfully resolved or stable. A sub-complete response and partial response were observed for 26 (76%) and 13 patients (33%), respectively. Conclusion The LS-LS technique for microcystic LMs is safe, feasible, and effective. This technique is an effective intervention with which it is possible to manage and potentially cure microcystic LM clinically.

Downregulation of miR-382 by propranolol inhibits the progression of infantile hemangioma via the PTEN-mediated AKT/mTOR pathway.

Approximately 10% of infantile hemangiomas (IHs) are the most common vascular tumors affecting children and are characterized by rapid growth, and can have destructive, disfiguring and even life-threatening consequences. Currently, propranolol is considered to be a safe and effective treatment option for problematic proliferating IHs. Recent studies have also revealed that microRNAs (miRNAs or miRs) play important roles in the regulation of angiogenesis. In this study, XPTS­1 cells were used as a hemangioma-derived endothelial cell line constructed in our laboratory. Through a series of experiments, we discovered that miR­382 is a novel miRNA associated with IHs, which was overexpressed in XPTS­1 cells and was conversely downregulated by treatment with propranolol. In addition, we found that miR­382 contributes to the progression of IHs. Our results revealed that propranolol inhibited XPTS­1 cell migration and proliferation, and promoted apoptosis, and these effects were reversed by the restoration of miR­382 expression by transfection of the cells with an miR­382 overexpression vector. Further experiments revealed that the above-mentioned effects were associated with the phosphatase and tensin homolog (PTEN)-mediated AKT/mammalian target of rapamycin (mTOR) signaling pathway. The expression of PTEN was upregulated, while that of p-AKT, p-mTOR and p-p70S6K was downregulated by propranolol; these effects were partly reversed by the overexpression of miR­382. On the whole, our study identified that the downregulation of miR­382 by propranolol inhibits the progression of IHs via the PTEN-mediated AKT/mTOR pathway.

Propranolol represses infantile hemangioma cell growth through the ß2-adrenergic receptor in a HIF-1α-dependent manner.

Propranolol, as a non-selective blocker of the ß-adrenergic receptor (AR), is utilised as the first-line treatment for infantile hemangiomas. However, the underlying mechanism remains poorly understood. The present study was designed to investigate the molecular basis of propranolol on the regression of infantile hemangiomas using a proliferating infantile hemangioma-derived endothelial cell line. In infantile hemangioma patients, we found that propranolol significantly decreased the expression levels of the hypoxia inducible factor (HIF)-1α in serum and urine, as well as in hemangioma tissues. In vitro analysis revealed that propranolol reduces the expression of HIF-1α in hemangioma cells in a dose- and time-dependent manner, mainly by acting on ß2-AR. Interestingly, it was observed that overexpression of HIF-1α apparently abrogated the inhibitory effects of propranolol on vascular endothelial growth factor (VEGF) expression and cell growth. Our data further demonstrated that propranolol inhibited the signal transducer and activator of transcription 3 (STAT3), a critical oncogenic signaling molecule, and the anti-apoptotic protein Bcl-2. Additionally, overexpression of HIF-1α significantly reversed the inhibitory effects of propranolol on STAT3 signaling. In a mouse xenograft hemangioma model, overexpression of HIF-1α significantly attenuated the therapeutic effects of propranolol and inhibited propranolol-induced hemangioma cell apoptosis. Moreover, the protein levels of VEGF, phosphorylated STAT3, total STAT3 and Bcl-2 were significantly upregulated by HIF-1α overexpression in propranolol-treated nude mice bearing hemangiomas. Collectively, our data provide evidence that propranolol may regress infantile hemangiomas by suppressing VEGF and STAT3 signaling pathways in an HIF-1α-dependent manner.

Detection of autophagy in Hirschsprung's disease: implication for its role in aganglionosis.

Hirschsprung's disease (HD) is a common congenital gastrointestinal malformation, characterized by the lack of ganglion cells from the distal rectum to the proximal bowel, but the pathogenesis is not well understood. This paper evaluates the effects of autophagy in HD. Using electron microscopy, the autophagosomes were detected in three segments: narrow segment (NS), transitional segment (TS), and dilated segment (DS). Typical autophagosome structures are found in the Auerbach plexus of both NS and TS. Real-time PCR results showed that Beclin1 (NS vs. TS, P<0.01) and LC3 (NS vs. TS, P<0.05) mRNA were the highest in the NS, but p75 (NS vs. TS, P<0.01) was the highest in the DS. Correlation analysis results showed a positive correlation between Beclin1 and LC3 mRNA levels (R=0.736, P=0.000), whereas inverse correlations were found between p75 and Beclin1/LC3 mRNA levels (p75 vs. Beclin1: R=-0.714, P=0.000; p75 vs. LC3: R=-0.619, P=0.000). Immunohistochemistry analyses indicated a consistent result with mRNA levels, by increased Beclin1-positive and LC3-positive neurons, but reduced p75-positive neurons in the Auerbach plexus of TS compared with DS. These findings indicated that autophagy exists in the bowel of patients with HD. On the basis of the detection of the highest expression of the autophagy genes in NS, autophagy may additionally cause the lack of neurons.

[Therapeutic mechanism of bleomycin A5 on infancy hemangioma: an experimental study].

OBJECTIVE: To investigate the therapeutic mechanism of Bleomycin A5 on infancy hemangioma. METHODS: After intralesional injection of Bleomycin A5 into the tumor of animal model of infancy hemangioma, the variation of tumor form was and the variation of tumor structure were observed using light microscope and electron microscope, the variation of tumor gene expression spectra was also tested by DNA microarray technique. RESULTS: After treatment, the tumor gradually shrunk, hardened, disappeared one month later. The tumor lost appearance of infancy hemangioma and replaced by lamellar collagen fibers and cellular nucleus scattered in the fibers, and almost all cells were necrotic and dissolved. Under electron microscope, only large stretches of dissolved cell could be seen without intact cells and blood vessels, but apoptotic cells and bodies could also be found. The results of DNA microarray analysis showed that 9 genes associated with apoptosis (murine double minute 2, heat-labile enterotoxin B subunit, lymphotoxin B receptor, tumor necrosis factor ligand superfamily 7, tumor necrosis factor receptor superfamily 21, tumor necrosis factor receptor superfamily 1A, myeloid cell leukemia-1, caspase3), 13 genes associated with cell proliferation and cell cycle (cell division cycle27, cell division cycle37, CDC28 protein kinase 1B, cycling B1, cullin 2, cullin 3, cullin 4A, growth arrest and DNA damage-inducible 45A, meiotic recombination 11 homolog B, forkhead box M1, minichromosome maintenance 7, antigen identified by monoclonal antibody ki 67, proliferating cell nuclear antigen), and 11 genes associated with cellular stress and toxic reaction (glutathione peroxidase 1, metallothioneins, superoxide dismutase-1, heat shock protein A1A, heat shock protein A2, heat shock protein A4, heat shock protein A5, heat shock protein 9B, heat shock protein CA, macrophage migration inhibitory factor, plasminogen activator inhibitor)were up or down regulated more than 2 folds in tumors treated with Bleomycin A5 compared with controls. CONCLUSIONS: The therapeutic effect of Bleomycin A5 on infancy hemangioma is the synthetic results of multiple factors. Bleomycin A5 could not only induce apoptosis and inhibit cell proliferation, but also depressed the ability of cell stress and toxic reaction.

[Effect of bleomycin A5 on the hemangioma-derived endothelial cell line XTPS-1].

OBJECTIVE: To investigate the effect of bleomycin A5 on the hemangioma-derived endothelial cell line XTPS-1. METHODS: Hemangioma-derived endothelial cell line XTPS-1 was cultured with different concentration of bleomycin A5 (1000, 100, 10, 1, 0 mg/L), and then the survival rate was measured by methyl thiazolyl terazolium (MTT), the variation of cell morphology was observed using inverted phase contrast microscope and electron microscope, the variation of cell cycle and apoptosis rate were measured using flow cytometry. RESULTS: After 24 hours culture the cell survival rate was (92.96 ± 3.66)% and (99.86 ± 0.12)% in lower saturation group (10 and 1 mg/L), but (34.08 ± 3.11)% and (43.28 ± 2.88)% in higher saturation group (1000 and 100 mg/L). The difference between them was more significant (P < 0.01). Lower saturation of bleomycin A5 (10 and 1 mg/L)could induce apoptosis but had almost no cytotoxic effect. Higher saturation of bleomycin A5 (1000 and 100 mg/L) not only induced apoptosis, but also had strong cytotoxic effect, which was concentration dependent. CONCLUSIONS: bleomycin A5 could induce apoptosis, inhibit cell proliferation and has direct cytotoxic effect.

[Establishment of human infancy hemangioma-derived endothelial cell line XPTS-1 and animal model of human infancy hemangioma].

OBJECTIVE: To establish an immortalized human infancy hemangioma-derived endothelial cell line (HemEC) and animal model of human infancy hemangioma. METHODS: Hemangioma-derived endothelial cells from specimen of human infancy hemangioma were cultured in vitro and monocloed, and then its growth curve was made, karyomorphism of chromosome analyzed, morphologic characteristics observe, factor VIII related antigen identified by immunohistochemical method.Vascular endothelial growth factor receptor 2 (VEGFR-2) was detected by flow cytometry. HemEC were inoculated subcutaneously in athymic mouse to establish animal model of infancy hemangioma. The animal model was observed closely and its pathological characteristic was also studied. RESULTS: The cultural cells grew active, and immortalized spontaneously when they were subcultured on sixteenth generation. This cell line was cultivated for more than 70 times within one year and in good condition after freezing and resuscitating once and again, and had the morphologic character of HemEC. The cell population doubling time was 22 h. Factor VIII and VEGFR-2 were expressed positively. Karyo type analysis of the cell line showed abnormal diploid with the modal chromosomal number varying between diploid and triploid. The cell line was then named XPTS-1. The animal model of infancy hemangioma was successfully established and its character of histopathology was similar with that of infancy hemangioma. CONCLUSIONS: The cell line of HemEC was successfully established and immortalized spontaneously, and had the morphologic and biological character of HemEC. The animal model of infancy hemangioma was successfully established and showed the character of histopathology similar with that of infancy hemangioma.