RESUMO
Objective:To investigate the MRI and pathological features of intramuscular fibro-adipose vascular anomaly (FAVA).Methods:The clinical and imaging data of 44 patients with intramuscular FAVA confirmed by pathology from December 2012 to March 2021 in Henan Province People′s Hospital were retrospectively analyzed. Twenty-five females and 19 males were included, with the age of (15±6), from 5 to 29 years old. The clinical and MRI features including the type, location, boundary, signal intensity, enhancement mode and degree, and the vascular flow voids in the lesion were summarized and compared with pathological results.Results:The thigh and calf muscles were involved in 1 patient simultaneously, and 1 site was involved in 43 patients, including 20 calf muscles, 15 thigh muscles, 5 forearm muscles, 1 upper arm muscle, 1 gluteal muscle, and 1 shoulder muscle. The gastrocnemius muscle of lower leg was most commonly involved (13/44), followed by soleus muscle (10/44) and quadriceps femoris muscle (9/44). All the lesions were solid on MRI, including 24 cases of focal mass type, 15 cases of diffuse infiltration type and 5 cases of local infiltration type. The long axis of all the lesions were consistent with the long axis of the muscles. All lesions showed inhomogeneously moderate hyperintensity on T 1WI and T 2WI, and significantly hyperintensity on fat suppression T 2WI. All lesions showed tortuous and dilated abnormal vessels, of which 18 cases showed vascular flow voids. Thrombosis was found in 10 cases. On contrast-enhanced imaging, the lesions showed moderate to obvious inhomogeneous enhancement. Pathologically, the diseased skeletal muscle was infiltrated by fibrous tissue, fat components, irregular abnormal veins and vessels, which led to inhomogeneous MRI signals. Among the 7 patients who underwent human PIK3CA gene mutation detection, and 6 were mutant. Conclusions:Intramuscular FAVA has certain characteristics in clinic, MRI imaging and histopathology, and its MRI signal characteristics can reflect its complex pathological components.
RESUMO
Objective:To analyze efficacy of comprehensive surgical treatment for Kasabach-Merritt phenomenon (KMP) .Methods:Clinical data were collected from 18 patients with KMP who underwent surgical treatment in Department of Hemangioma, Henan Provincial People′s Hospital from November 2017 to March 2019, and analyzed retrospectively. Preoperative treatment included preoperative use of glucocorticoids and immunoglobulins to increase platelet counts in patients. For patients irresponsive to glucocorticoids, a single large-dose of platelets was infused 12-24 hours before surgery. The platelet dose for infusion should be 0.3 and 0.2 therapeutic dose (TD) /kg respectively for patients with platelet counts lower than 30 × 10 9/L and those with platelet counts higher than 30 × 10 9/L, and the total infusion dose should be no more than 1 TD (containing about 2.5 × 10 11 platelets in 200-250 ml of infusion solution) . During the operation, tumor tissues were removed as much as possible; if there were difficulties in suturing in patients with large tumors, in situ autologous skin grafting would be performed; for children with potential postoperative functional dysfunction, a clinical consultation with rehabilitation specialists was given, and individualized functional exercises were prescribed to promote functional restoration. Results:Eighteen patients were enrolled, including 9 males and 9 females, with an average age of 73 days (range, 7-354 days) . Skin lesions were located on the extremities in 3 cases, on the trunk in 11, and on the head, face and neck in 4. Surgeries were successful in 17 patients, but 1 died. Among the 17 patients with successful operation, platelet counts and coagulation function returned to normal within 1 week after the surgery in 16, and did not returned to normal until after regular oral administration of sirolimus in 1. Favorable movement ability was maintained in all the children.Conclusion:Comprehensive surgical treatment shows rapid efficacy with less adverse reactions in the therapy of KMP.
RESUMO
Objective@#To compare the curative effect of non-surgical treatment, early operation and advanced operation on the Kaposiform hemagioendothelioma (KHE). To explore the optimal treatment for KHE.@*Methods@#The 106 cases of KHE patients admitted by our hospital from January 2004 to December 2016 were selected as the study subjects. The age is the minimum 1d and the maximum 43 years, median age 94.5 d. According to different treatment methods, they were divided into non-surgical treatment and surgical treatment: according to the onset of the disease and the day of the operation, the early operation was performed less than three months since the onset of the disease, and the advanced operation more than 3 months. Efficacy evaluation criteria: The recovery meant that the tumor disappeared or mostly disappeared, the platelets returned to normal, and there was no recurrence more than half a year after treatment. " Effective" indicated that tumors were shrunken significantly and the platelet counting rose significantly. " Ineffective" indicated that the tumor was not shrunken or continued to increase and platelet counting did not rise or continued to decrease.The three groups of patients were followed up, and the efficacy of the three groups after treatment and the changes of platelet counting before and after treatment on day 1, 7 and 14 were compared. The two groups of early and late treatment were compared for operative and postoperative conditions.@*Results@#All patients were followed up for 6-23 months, 24 cases were effective, 12 cases were ineffective, and 5 cases died. After 7 days of treatment, the platelet counting of the three groups were 68 (31, 157)×109/L in the non-surgical treatment group, (294.75±150.59)×109/L in the early operation group, (221.48±148.71)×109/L in the late operation group, with statistical significance (P < 0.05). After 14 days of treatment, the platelet counting of the three groups were 78 (25.51, 54.5) in the non-surgical treatment group, 281 (234, 356)×109/L in the early operation group, and in the late operation group (219.67±140.95)×109/L, with statistical significance (P < 0.05). After at least half a year of follow-up, the total effective rate of clinical efficacy in the three groups was the non-surgical treatment group 62.07%, the early operation group 96.34% and the late operation group 81.82%; the difference was statistically significant between two groups (P < 0.05); There was significant difference between the early operation group and the late operation group in the amount of bleeding and operation time, the time of platelet counting and the total hospitalization time (P< 0.05).@*Conclusions@#Early surgical treatment of Kaposiform hemangioendothelioma is superior to late-operative and non-surgical treatment.Surgical treatment will be the preferred option for treatment of Kaposiform hemangioendothelioma, and examinations should be operated to potential patients for early diagnosis.
RESUMO
Objective@#This study aims (1) to evaluate the surgical outcome of intramuscular venous malformations, and associated nerve and tendon encapsulated; (2) to summarize the surgical procedure, with the purpose of providing individualized surgical treatment.@*Methods@#This is a retrospective study of 114 patients of intramuscular venous malformations with associated nerve and tendon encapsulated. All the patients were admitted in the Henan Provincial People′s Hospital from January 2012 to December 2014. The surgeries were produced, according to the patient′s condition. The surgical outcome was evaluated after 1-3 years follow up, by the overall evaluation of radiological imaging, symptoms and functions of limbs. The evaluation criteria: (1) Invalid: clinical symptoms or radiological imaging showed no improvement or even worsen; (2) Improvement: clinical symptoms or radiological imaging was significantly improved; (3) Cure: clinical symptoms were gone or the radiological imaging was normal.@*Results@#All the 114 patients were followed up for 1-3 years, with the average of 1.8 years. Three cases were evaluated as invalid, 26 cases were improved, and 85 cases were cured. The effective rate was 97%, and the cure rate was 75% on 1 year after surgery.@*Conclusions@#It is difficult to treat intramuscular venous malformations as it often involves nerve and tendon. Universal treatment is few. Therefore, an individualized surgical plan is recommendable.
RESUMO
Objective@#To observe the effects of different concentrations and doses of urea on the proliferation and apoptosis of human hemangioma endothelial cells, in order to provide evidence for the further mechanism study of urea in the treatment of hemangioma.@*Methods@#Human hemangioma endothelial cells (HemECs) and normal endothelial cells (VE) were cultured in vitro. Cell viability was detected by CCK-8 after invention with different concentrations(40%, 50%, 60%, 70%) and doses(3, 6, 9 μl/ml) of urea. The apoptosis of HemECs was detected by flow cytometry dual-dye and propidium lodide single dye.@*Results@#The viability of HemECs was significantly lower than that of VE under different concentrations and doses of urea (P<0.05). The inhibition rate of 40% urea on HemECs increased with the increase of urea dose (P<0.05), and the inhibition effect was most obvious at 4 h and 12 h. The apoptosis of HemECs increased in a time and dosage dependent manner with the treatment of 40% urea. High dose(9 μl/ml)of 40% urea significantly promoted the apoptosis of HemECs(P<0.05).@*Conclusions@#Low dose of 40% urea significantly inhibited the proliferation of HemECs, and had no significant effect on VE. However, high doses of urea promoted apoptosis of HemECs.
RESUMO
Objective@#To investigate the clinical and pathologic features, diagnosis and differential diagnosis of hypertrophic port-wine stain (PWS).@*Methods@#Cases of hypertrophic PWS, collected from Henan Provincial People′s Hospital between 2012 and 2018, were retrospectively analyzed for their clinical and pathologic features, immunophenotype and histochemical data, and the relevant literature was reviewed.@*Results@#Twenty-four cases of PWS were included in this cohort, located in the head and neck region (20 cases), limbs (2 cases), and trunk (2 cases). The clinical presentations were mainly red or purple-red plaques or slow growing, painless nodules, or thickened and raised above the skin surface. Microscopically, deformed blood vessels showed honeycomb-like, plexiform or cluster-like growth pattern, and diffusely involved the dermis, skin appendages, subcutaneous fat tissue, and deep skeletal muscles; The vascular lumen of the deformed vessels was dilated (≥100 μm in diameter), and in 18 cases the lumen was greater than 400 μm. The superficial dermis mainly contained few deformed capillaries. The deep wall showed thickening of blood vessel wall and fibrous tissue hyperplasia. Elastic fiber and Masson staining indicated abnormal venous vessel, which in some cases contained small amount of abnormal arterioid vessel,without vascular endothelial cell proliferation in all cases. In 24 cases, 19 cases had epidermal atrophy, 6 with vascular chronic inflammation or epidermal ulcer, 4 with capillary hemangioma, 4 with sebaceous gland hyperplasia, 2 with epidermal papillary hyperplasia and 2 with vascular keratomas.@*Conclusions@#PWS is a common congenital capillary malformation. The number of histologically deformed capillaries is reduced and they usually locate in the superficial part. The deep vascular wall is increased with thick venous malformation, diffusely involving the dermis and deep skeletal muscle. Furthermore, PWS needs to be differentiated from infantile hemangioma, cavernous hemangioma and vascular keratomas.
RESUMO
To investigate the relationship between glucocorticoid receptor (GRα, GRβ) expression and hormone sensitivity in kaposiform hemangioendothelioma (KHE) patients complicated by Kasabach-Merritt phenomenon (KMP).Methods@#In this study, 25 cases of KHE with KMP (mean age 2.4±1.5 months), including 9 males and 16 females at Henan Provincial People′s Hospital between May 2013 and May 2016 were included. All patients underwent surgical resection after being treated with hormone for one week, and were divided into sensitive group (10 cases) and resistance group (15 cases) according to the efficacy evaluation criteria. Normal tissues collected from 15 patients received surgical excision of lipoma was performed as a control group.Immunohistrochemical SP method was adopted in detecting GRα and GRβ expression in all groups. The expression intensity and positive rate were analyzed. Statistical significance was determined using the Wilcoxon rank sum test for the group samples and the Kruskal-Wallis test for multiple samples. Values of P<0.05 were considered statistically significant.@*Results@#①There was no significant difference among the control group, the resistance group and the sensitive group in expression intensity of GRα (control group 4.20±1.01, resistance group 4.10±0.99, sensitive group 3.53±0.52, P=0.632). The number of GRα positive cells in the sensitive group(46.20±2.57)was higher than that in the resistance group (40.93±5.18, P=0.032). ②The expression intensity of GRβ in the resistance group(5.40±0.51)was significantly higher than that in the sensitive group(2.60±0.52)and the control group(2.87±0.64, P=0.000, 0.002); there was significant difference among the sensitive group(29.70±2.50), the control group(36.07±3.47)and the resistance group (47.27±5.59)in the number of GRβ positive cells (P=0.000). ③The ratio of GRα/Grβ expression intensity was significantly lower in the resistance group than in the sensitive group and the control group; The ratio of GRα/GRβ positive cells was gradually decreased in the sensitive group, control group and resistance group, and the difference was statistically significant (P=0.000).@*Conclusions@#In the tumor tissue of KHE children with KMP, the increase of GRβ expression negatively regulats GRα, leading to imbalance of the expression ratio of the two, which may be an important cause for GC resistance.
RESUMO
Objective@#To assess the safety and effectiveness of sufficient, short-term platelet (PLT) transfusion for the surgery preparation of the infantile patients with Kasabach-Merritt phenomenon, who were insensitive to glucocorticoids.@*Methods@#The infantile cases were retrospectively analyzed during May 2011 to December 2016, who were clinically diagnosed as KMP and insensitive to glucocorticoids, received PLT transfusion and surgical resection. PLT transfusion in patients whose PLTC was less than 30×109/L, was 0.3 therapeutic dose(TD)/kg, and 0.2 TD/kg in PLTC≥30×109/L group. The maximum was 1 TD.Criteria of the PLT transrusion: 1 hour after the transfusion, the PLT count (PLTC) were tested and the corrected count increment of platelet (CCI) and practical platelet recovery (PPR) was calculated. PLTC ≥100×109/L, CCI>7.5×109/L and PPR>30% were defined as effective; while PLTC=(50-99)×109/L, CCI>7.5×109/L and PPR>30% as partial effective; PLTC<50×109/L, or CCI≤7.5×109/L, or PPR ≤30% were defined as ineffective. By reviewing the method and response of their PLT infusions, to figure out the most effective way in rising PLT, as a part of pre-operation treatment.@*Results@#There were 46 cases in the research. Based on the PLTC, CCI and PPR 1 hour after PLT transfusion, there were 44 effective transfusion, 2 patients with partial effectiveness, and no ineffective case. There was no allergic or heart failure happened in any cases. No critical potential complications of PLT transfusion occurred, including fluid and iron overload, alloimmunization to human leukocyte antigen and/or PLT antigen.@*Conclusions@#Pre-operative sufficient and short-term PLT infusions are more effective than low dose and long-term ones. They can create a more optimistic opportunity for surgical resections.
RESUMO
Objective@#To define a new subtype of vascular anomaly, named fibro-adipose vascular anomaly(FAVA)and to discuss the methods of diagnosis and treatment in order to decrease the misdiagnosis rate and improve the recovery rate.@*Methods@#From Jan. 2011 to Dec. 2015, 34 cases with FAVA on the lower extremities received surgical treatment in our center. The clinical data were collected to summarize the imaging and pathological characteristics for diagnosis.@*Results@#The misdiagnosis rate was 76.5%(26/34) in all these 34 patients. The cure rate was 100% after operation. The patients were followed-up for 1-3 years(average, 19 months) with normal function and no recurrence.@*Conclusions@#FAVA is usually misdiagnosed as hemangioma or vascular malformation. The disease should be well defined to help the diagnosis and treatment. Surgical excision is one of the optional treatments.
RESUMO
Objective@#To analyze the pain caused by intramuscular venous malformation, so as to avoid misdiagnosis.@*Methods@#We retrospectively analyzed 173 patients who received surgical treatments in our department between Jan.2012 to Dec.2014, with the main complaint of local pain and were diagnosed as intramuscular venous malformation. The mechanisms of the local pain, based on the image data, intra-operative findings, pathology reports and the comparison of the Visual Analogue Scale(VAS) data before and after operation were summarized. The surgical interventions included simple excision or excision + adhesiolysis or excision + adhesiolysis + nerve decompression.@*Results@#The reasons of local pain can be divided into 4 categories: ①lesion located in the tendon insertions; ②lesion involving the local nerve, inducing the thickening and tensing of its epineurium and the increasing of its diameter; ③lesion infiltrated to the periosteum; ④phlebolith in it. All the 173 patients received surgical treatments and got varying degrees of relieving from local pain. 63 patients got a decrease of the VAS by 5 or more, and 95 cases′ VAS number decreased by 3-4, the rest 15 patients′ VAS cut down by 1-2.@*Conclusions@#Intramuscular venous malformation is an important reason for local pain and should not be neglected. Surgical treatment can be an effective method to remove the lesion and relieve local pain.
RESUMO
Kasabach-Merritt phenomenon (KMP) is characterized by thrombocytopenia with enlarging vascular tumour,KMP usually develops in infancy and is associated with significant morbidity and mortality,the mortality rate is reported as high as 30％.It commonly reported sites of tumor include extremities,trunk,retroperitoneum and neck.There is no consensus in treatment and various regimens have been used by different authors.This report is aim to learn the pathophysiology of the KMP and its diagnosis and treatment.
RESUMO
Objective To describe and examine the clinical characteristics and spectrum of tufted angiomas (TA) in infants so as to explore the therapeutic approaches to Kasabach-Merritt phenomenon (KMP).Methods The clinical and follow-up data of 24 patients with TA were retrospectively analyzed between Jan.2009 and Mar.2013.The median age of the patients was 7.5 months(ranged from 18 days to 2 years),including l0 male and 14 female.Surgical excision and observation were chosen according to the lesion and conditions of the patients.The mean follow-up period was 3.6 years (1.2 to 5.4 years).The changes in the patients' s condition were established by evaluating platelet counts,and the size of lesion.Results Common clinical features included dusky red or violaceous infiltrating cutaneous lesion,thrombocytopenia,pain or decreased function and hyperhidrosis or hypertrichosis.The following 3 clinical patterns of TA progress were described:spontaneous complete or partial regression (n =2,8.3％),TA without complications and persistence over the years(n =9,37.5％),and TA complicated by KMP(n =13,54.2％).The average interval of development of KMP for delayed cases was 45.2 days(ranged 0 d to 4 months).Each of the 13 patients who developed KMP subsequent to initial presentation was symptomatic at the time KMP was detected(enlarged lesion,n =8;increased lesion firmness with change in cutaneous stain,n =3 ;and respiratory distress,n =2).All of 13 patients were cured by surgery.Complete surgical resections were performed on 10 cases.The thrombocyte count was back to normal within 1-3 days post operation,and hemoglobin and blood coagulation function gradually returned to normal within 1 to 2 weeks.Other 3 cases received major resection surgery.The number of platelets in the patients were unstable,but significantly higher than that of preoperational stage.The platelet count remained above 60 × 109/L.The residue lesions in 2 cases disappeared gradually after the operation and medication were given within 3-6 months.And the other case died of multiple organ failure post-operation.Conclusions Surgical intervention can be applied to TA that severely makes damage to children's appearance or looks or may be complicated with KMP or functional abnormality.A closely monitored policy seems appropriate for the early small tumor without severe complications.And it is necessary to monitor the number of the platelet regularly and find the KMP by as early as possible.TA associated with KMP is vitol to infants.And surgical therapy after clear diagnosis should be done as early as possible.The surgical therapy is a reliable management with higher curative rate,short disease period and minimal side-effect.