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1.
J Dermatol ; 2021 Feb 19.
Artigo em Inglês | MEDLINE | ID: mdl-33608936

RESUMO

Kaposiform haemangioendothelioma (KHE) and tufted angioma (TA) are rare vascular tumors that can cause life-threatening Kasabach-Merritt phenomenon. No evidence-based treatment strategies have yet been established, and its management is still a challenge. The purpose of this multicenter prospective randomized controlled study was to evaluate and compare the efficacy of corticosteroid and vincristine (VCR) in the treatment of KHE and TA. All patients with KHE/TA who met the diagnostic criteria were consecutively recruited. The patients were randomized into a methylprednisolone (MP) group and a VCR group. The primary outcome was the single main parameter effective rate and overall effective rate of corticosteroid and VCR over 1 month after treatment. The single main parameters included platelets, fibrinogen, tumor size, texture, and appearance. From May 2016 to April 2018, a total of 59 patients completed the clinical trial, including 29 in the MP group and 30 in the VCR group. The results showed that VCR was superior to corticosteroid in the improvement of platelet (80.0% vs 44.0%, P = 0.019) and tumor texture (68.9% vs 30.8%, P = 0.007). Although the efficacy of VCR on fibrinogen (23.3% vs 20.7%, P = 1.000), tumor size (23.3% vs 13.8%, P = 0.273), and appearance (65.5% vs 46.2%, P = 0.120) was higher than that of corticosteroid, there was no significant difference (P > 0.05). Meanwhile, the overall effective rate of VCR was higher than that of corticosteroid (56.7% vs 31.0%), but the difference was also not statistically significant (P = 0.067). In conclusion, the therapeutic effect of VCR was significantly better than that of corticosteroid with regard to treating thrombocytopenia and tumor texture. We recommend that VCR could be an option for first-line treatment in KHE/TA patients.

2.
Pediatr Dermatol ; 37(4): 677-680, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32212177

RESUMO

We present a retrospective case series of 3 patients with retroperitoneal kaposiform hemangioendothelioma (KHE) complicated by Kasabach-Merritt phenomenon (KMP) and biliary obstruction. We found sirolimus to be a safe and effective treatment for these patients who were refractory to other treatment modalities. However, our patients were slow to respond in comparison to published reports of sirolimus use for KHE without biliary obstruction. We postulate that therapeutic serum levels of sirolimus may be affected by biliary obstruction and improved with surgical alleviation of the obstruction.

3.
Dermatology ; 236(3): 262-270, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31896113

RESUMO

BACKGROUND: Mammalian target of rapamycin (mTOR) inhibitors have been shown to have excellent effects in the management of kaposiform hemangioendothelioma (KHE); however, the mechanism of action is unclear. This study identified the expressions of mTOR pathway-related proteins in different vascular tumors to provide insight into the pathogenesis of KHE. METHODS: We retrospectively reviewed the pathologic specimens of 30 patients (KHE, 15; tufted angioma [TA], 5; infantile hemangioma [IH], 5; and lymphatic malformation [LM], 5). The immunohistochemical expression of mTOR-related proteins tuberous sclerosis complex 2 (TSC2), phosphatase and tensin homologue (PTEN), phosphorylated eukaryotic translation initiation factor 4E binding protein 1 (p-4EBP1), phosphorylated mTOR (p-mTOR), and phosphorylated ribosomal protein S6 kinase B1 (p-P70S6K) were analyzed using Image-Pro Plus software. KHE had the following pattern of expression in the spindle vascular endothelial cells: TSC2 (-); PTEN (-); p-4EBP1 (+); p-mTOR (+); and p-P70S6K (+). RESULTS: All 3 patients treated with sirolimus had good responses. The TA results were similar to KHE with no significant differences (p-4EBP1: p = 0.0687; p-mTOR: p = 0.0832). The expressions of TSC2, PTEN, p-4EBP1, p-mTOR, and p-P70S6K were negative or weakly positive in IH with a statistically significant difference compared to KHE (p-4EBP1: p < 0.001; p-mTOR: p < 0.001; p-P70S6K: p < 0.001). LM had no significant differences when compared to KHE. CONCLUSIONS: The absence of TSC2 and PTEN caused abnormal activation of the mTOR signaling pathway and may be involved in the pathogenesis of KHE. The expression of mTOR-related proteins in TA and LM was similar to KHE, unlike IH. The KHE pattern of expression [PTEN (-), TSC2 (-), p-mTOR (+), p-P70S6K (+), and p-4EBP1 (+)] suggested that sirolimus may be a good therapeutic choice.


Assuntos
Hemangioendotelioma/metabolismo , Imuno-Histoquímica/métodos , Síndrome de Kasabach-Merritt/metabolismo , Sarcoma de Kaposi/metabolismo , Serina-Treonina Quinases TOR/biossíntese , Antineoplásicos/uso terapêutico , Células Endoteliais/metabolismo , Hemangioendotelioma/tratamento farmacológico , Hemangioma/metabolismo , Humanos , Síndrome de Kasabach-Merritt/tratamento farmacológico , Anormalidades Linfáticas/metabolismo , Estudos Retrospectivos , Sarcoma de Kaposi/tratamento farmacológico , Transdução de Sinais , Sirolimo/uso terapêutico , Serina-Treonina Quinases TOR/metabolismo
4.
J Dermatol ; 46(11): 956-961, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31489702

RESUMO

Mammalian target of rapamycin inhibitors have shown promising results in the management of kaposiform hemangioendothelioma (KHE). The purpose of this study was to present our experience involving sirolimus therapy for KHE. A retrospective study was conducted to review the medical documents of 26 patients with KHE who were treated with sirolimus at our hospital between March 2012 and December 2016. Fifteen males and 11 females manifested KHE in infancy with an average age of 2.9 ± 1.8 months. Multiple anatomical sites were involved. Four patients had multifocal lesions, while 22 patients had solitary lesions. Twenty-five patients had Kasabach-Merritt phenomenon (KMP). Twenty patients completed sirolimus therapy in 28.3 ± 12.5 months. Nineteen KHE lesions reduced to small residuals with platelet counts reaching normal levels 3.7 ± 2.8 weeks after treatment; one KHE lesion had no response to therapy. One patient with multifocal lesions died due to a severe infection, although the patient had previously responded to sirolimus. Five patients remained in treatment and had good responses with normal platelet counts. Nineteen patients with anemia had normal hemoglobin levels after 3.5 ± 1.9 weeks of treatment. Mild side effects were observed. The median follow-up time was 32 months (26-60 months), with no evidence of recurrences. Sirolimus was shown to be efficacious in the management of KHE with an average course of 28 months. The time-to-response was variable, with an average of 1 week. After 4 weeks of treatment, the platelet count and hemoglobin level had normalized. Multifocal KHE with KMP is more severe than solitary KHE.


Assuntos
Antibióticos Antineoplásicos/uso terapêutico , Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Sirolimo/uso terapêutico , Feminino , Seguimentos , Hemangioendotelioma/patologia , Humanos , Lactente , Recém-Nascido , Síndrome de Kasabach-Merritt/patologia , Masculino , Estudos Retrospectivos , Sarcoma de Kaposi/patologia
5.
J Pediatr Surg ; 54(12): 2589-2594, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31522796

RESUMO

BACKGROUND: Neuroblastoma is the most common extracranial solid tumor in childhood. In this work, the clinical value of long noncoding RNA LINC01296 was evaluated in patients with neuroblastoma. METHODS: LncRNA microarray was conducted to identify differentially expressed lncRNAs in 5 early stage and 5 advanced stage tumor tissues of neuroblastoma. RT-qPCR was carried out to validate the result of microarray. Clinical information was reviewed to analyze the relationship between lncRNA and clinical characteristics. The public database R2 was used to analyze prognosis. RESULTS: 765 differentially expressed lncRNAs were identified. LINC01296 was the most overexpressed lncRNA in advanced stage patients. RT-qPCR was conducted in 28 tumor tissues, confirming the tendency with microarray. Higher expression of LINC01296 was associated with age > 18 months (p = 0.004) and advanced stage (p = 0.021). Furthermore, LINC01296 was correlated with tumor size (r = 0.4060, p = 0.0321), LDH level (r = 0.6904, p = 0.0002), and NSE level (r = 0.5772, p = 0.0013). The neuroblastoma dataset shows patients with overexpression of LINC01296 obtained a shorter overall survival than low expression (n = 88, log-rank: P < 0.0001). CONCLUSION: LINC01296 is associated with clinical characteristics of neuroblastoma and may function as a prognostic predictor. LEVEL OF EVIDENCE: II.


Assuntos
Neuroblastoma , RNA Longo não Codificante , Biomarcadores Tumorais/análise , Biomarcadores Tumorais/genética , Biomarcadores Tumorais/metabolismo , Humanos , Neuroblastoma/epidemiologia , Neuroblastoma/genética , Neuroblastoma/metabolismo , Neuroblastoma/mortalidade , Prognóstico , RNA Longo não Codificante/análise , RNA Longo não Codificante/genética , RNA Longo não Codificante/metabolismo
6.
J Pediatr Surg ; 54(12): 2585-2588, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31521373

RESUMO

BACKGROUND: Stage 4S neuroblastoma is a unique category of metastatic disease in infants with a favorable outlook. The purpose of this study was to clarify the prognostic factors of patients with stage 4S neuroblastoma. METHOD: Data were retrospectively collected from infant patients with stage 4S neuroblastoma in our department from May 2000 to May 2018. Patient characteristics, operative variables, perioperative outcomes, overall survival (OS), and recurrence were evaluated. Univariate and multivariate analyses were performed to identify the prognostic factors of stage 4S neuroblastoma. RESULT: A total of 28 infant patients (71. 4% males) with a mean age of 3.7 ±â€¯2.7 months were recruited. The involved metastatic sites included liver (n = 18), skin (n = 9), and bone marrow (n = 5). Nine patients received biopsy, and 14 patients underwent original lesions resection followed by postchemotherapy. Five patients accompanied with abdominal compartment syndrome (ACS) were given experiential chemotherapy. The follow-up time ranged from 12 M to 156 M, with a mean of 32 months. Twenty-two patients completed treatment and survived. Two patients were still under treatment. Four patients died, including three with ACS. No recurrence was observed. According to Kaplan-Meier method, the 5-year overall survival was 84.4%. ACS (p = 0.001) and chemotherapy sensitivity (p < 0.001) were associated with all causes of mortality of stage 4S neuroblastoma, while neuroblastoma liver metastasis (P = 0.107), skin metastasis (P = 0.137), bone marrow metastasis (P = 0.89), tumor radical resection (P = 0.202), prenatal diagnosis (P = 0.314), and younger than 2 months of age (P = 0.683) did not emerge as prognostic factors. CONCLUSION: ACS and chemotherapy sensitivity were highly important factors that had an association with the prognosis of stage 4S neuroblastoma. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level IV.


Assuntos
Neuroblastoma , Feminino , Humanos , Lactente , Masculino , Neuroblastoma/diagnóstico , Neuroblastoma/epidemiologia , Neuroblastoma/patologia , Prognóstico , Estudos Retrospectivos
7.
Eur J Pediatr Surg ; 29(5): 401-407, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30372769

RESUMO

OBJECTIVE: To evaluate the outcome and safety of corticosteroids and vincristine (VCR) in the treatment of kaposiform hemangioendothelioma (KHE) and tufted angioma (TA). MATERIALS AND METHODS: Clinical studies involving corticosteroids and VCR therapies in treating KHE/TA were identified by using PubMed, Cochrane Library, OVID, EBSCO, CNKI, VIP, and Wanfang databases from their establishment date to December 2017. Randomized controlled trials, case-control, or case series with more than five cases were included. The following data were extracted: study sample, demographics, responses rate, recurrence rate, and adverse reactions. Two reviewers completed screening and extraction. Methodological quality was evaluated with quality appraisal tool. RESULTS: A total of 266 studies were found, and 27 studies were finally included in this research; quality of all studies was low. Seven studies with a total of 123 participants, which compared the effect of systemic corticosteroids with that of VCR, were performed for the meta-analysis. The results indicated that the effect of VCR was significantly higher than that of corticosteroids (relative risk [RR] = 2.08, 95% confidence interval [CI]: 1.38-3.16). The recurrence rate of VCR (11.1%) was lower than that of corticosteroids (50%), but there was no statistical difference between the two therapies (p = 0.1312). The result of pooled adverse reactions response rate for VCR was 18.2%, significantly lower than that for corticosteroids, which was 52.0%. CONCLUSION: The present profile shows that VCR is relatively more effective and safer in treating KHE/TA than corticosteroids are. So, we believe VCR could be used as a first-line medication agent in the treatment of KHE/TA.


Assuntos
Corticosteroides/uso terapêutico , Hemangioendotelioma/tratamento farmacológico , Hemangioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Vincristina/uso terapêutico , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Observacionais como Assunto , Recidiva , Resultado do Tratamento
8.
Pediatr Surg Int ; 35(3): 365-371, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30392130

RESUMO

PURPOSE: The purpose of this study was to review the clinical characteristics and prognosis of children with adrenocortical tumors (ACT). METHODS: We retrospectively reviewed the medical records of 28 patients with ACT at our hospital between March 2010 and March 2017. RESULTS: The main clinical presentations were sexual prematurity (n = 17) and Cushing's syndrome (n = 15). All patients without metastasis underwent complete resection by laparotomy (n = 19) or laparoscopic surgery (n = 9). Pathological diagnosis confirmed adrenocortical carcinomas (ACC, n = 12) and adrenocortical adenomas (ACA, n = 16). Dehydroepiandrosterone (939.4 ± 148.2 µg/dl vs 630.9 ± 376.3 µg/dl; p = 0.031) and testosterone (235.7 ± 89.1 ng/dl vs 164.6 ± 47.5 ng/dl; p = 0.012) were significantly increased in ACC compared with ACA. The ACC tumor volumes were larger than those in ACA (107.5 ± 69 vs 25.5 ± 23.1 cm3; average diameter 6 cm vs 4 cm p = 0.001) and the immunochemical expression of Ki-67 was higher in ACC than in ACA (30.2 ± 22.7 vs 9.9 ± 4.9 p = 0.013). The mean follow-up of patients with ACA was 40 ± 23 months without recurrence. Seven patients with ACC had postoperative distant metastases and five patients died within 2 years. Five patients with ACC survived with a median follow-up of 27 months. The 2-year overall survival was 44.6%. CONCLUSIONS: Patients with ACC had significantly larger tumor volumes than those with ACA. The discordantly elevated serum levels of sexual corticosteroid hormones and lactate dehydrogenase may predict the malignant nature of these tumors. The prognosis of patients with ACA was good, while those with ACC had high postoperative metastasis and mortality rates.


Assuntos
Neoplasias do Córtex Suprarrenal/diagnóstico , Laparoscopia/métodos , Estadiamento de Neoplasias , Carga Tumoral , Adolescente , Neoplasias do Córtex Suprarrenal/mortalidade , Neoplasias do Córtex Suprarrenal/cirurgia , Adrenalectomia , Criança , Pré-Escolar , China/epidemiologia , Feminino , Humanos , Incidência , Masculino , Recidiva Local de Neoplasia/epidemiologia , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida/tendências
10.
Oncol Lett ; 13(6): 4727-4733, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28599474

RESUMO

MicroRNA (miR/miRNA)-21 is a well-known oncogenic miRNA that is overexpressed in various types of tumors. The tumor-suppressor genes programmed cell death 4 (PDCD4) and phosphatase tensin homologue (PTEN), are targets of miR-21, and are underexpressed in several types of cancer. However, the expression of miR-21 and its target genes in neuroblastoma (NB) remains unclear. In the present study, a miR-21 inhibitor oligonucleotide was transfected into the SK-N-SH cell line, and the expression of miR-21, PTEN and PDCD4 was detected through quantitative polymerase chain reaction analysis. Western blotting was used to examine levels of PTEN, PDCD4 and caspase-3 proteins. The expression of PTEN and PDCD4 in the SK-N-SH cell line transfected with the miR-21 inhibitor was significantly increased compared with untransfected SK-N-SH and negative control-transfected cells. Cell proliferation was inhibited and the apoptotic ratio was significantly increased in miR-21 inhibitor-transfected cells compared with untransfected SK-N-SH and negative control-transfected cells. Western blot analysis revealed a significant increase in caspase-3 expression compared with untransfected SK-N-SH and negative control-transfected cells. The results of the present study indicate that miR-21 may serve an oncogenic role in the cellular processes underlying NB development and thus may be a novel therapeutic target for the treatment of patients with NB.

11.
Zhongguo Ying Yong Sheng Li Xue Za Zhi ; 33(3): 214-217, 2017 Mar 08.
Artigo em Chinês | MEDLINE | ID: mdl-29931934

RESUMO

OBJECTIVE: To investigate the effects of 4-week hypoxia training on index related to endurance capacity and lymphocyte sub-jects of male football players. METHODS: Football players from a sport university were evenly divided into 4 groups:training group, hypoxic group, hypoxia training group and control group. The control group completed a 4-week cycling training with the intensity of 65%~75% VO2max, while the hypoxia training group did the same training in hypoxia environment (14.7% oxygen concentration);Low oxygen groups for 4 weeks daily 60 min, 5 times a week in a low oxygen (14.7% oxygen concentration) environment, did not cycle training;the control group without any intervention. RESULTS: In hypoxia training group, the red blood cell count and hemoglobin were significantly higher than those of the control group, training group and hypoxia group(P < 0.05);there was significant difference in VO2max and the performance of 3 000 m running between hypoxia training group and other groups(P < 0.05); the number of CD3+ level in hypoxia training group was significantly higher than that of the control group and hypoxia group (P < 0.05). CONCLUSIONS: Compared to other methods environment, a hypoxia training program of 60 min/d, 5 d/w, 4 weeks in total contributed to improve the endurance capacity of male football players, and it's also probably beneficial for improvement of immunity.


Assuntos
Hipóxia , Consumo de Oxigênio , Condicionamento Físico Humano , Resistência Física , Linfócitos T/citologia , Desempenho Atlético , Humanos , Masculino , Oxigênio , Corrida , Futebol
12.
J Pediatr Surg ; 51(12): 2086-2090, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27686480

RESUMO

BACKGROUND: It has been reported that multifocal and diffuse hepatic hemangiomas are true infantile hemangiomas for which a continuum probably exists. We determined the similarities and fine differences between the two types of hemangioma and identified the multifocal subgroup of type, which needs timely treatment. MATERIAL AND METHODS: Twenty-four patients (4 males and 20 females; age 114±142days) with multifocal or diffuse hepatic hemangiomas who were treated between January 2000 and June 2015 were studied. For the multifocal type, patients were divided into countable (MC) and uncountable (MU) subgroups. The medical data were analyzed retrospectively. RESULTS: The clinical presentations included hepatomegaly (n=11), dyspnea (n=7), heart failure (n=9), hypothyroidism (n=6), and anemia (n=1). There were 19 and 5 patients with multifocal and diffuse types, and 6 and 13 patients in the MC and MU groups, respectively. There were significant differences between the multifocal and diffuse types with respect to hepatomegaly, heart failure, dyspnea, and hypothyroidism. However, there was no difference between the MU group and the diffuse type except for hypothyroidism. Observation was commonly recommended for patients with the multifocal type, and their survival rate was clearly higher than for patients with the diffuse type. Both the MU and diffuse-type groups needed treatment, unlike MC patients. The complete remission rate was higher in the MC group than in the MU and diffuse-type groups. Patients with the diffuse type were more likely to die. CONCLUSIONS: Patients with diffuse-type hepatic hemangioma are at high risk and need active treatment. The MU group for the multifocal type is unique and has high similarities to the diffuse type with respect to clinical presentation and treatment, which suggests that a continuum of the disease phenotypes exists. LEVEL OF EVIDENCE: Case-control study, level III.


Assuntos
Hemangioma/diagnóstico , Neoplasias Hepáticas/diagnóstico , Estadiamento de Neoplasias , Estudos de Casos e Controles , China/epidemiologia , Feminino , Hemangioma/mortalidade , Humanos , Lactente , Recém-Nascido , Neoplasias Hepáticas/mortalidade , Masculino , Estudos Retrospectivos , Taxa de Sobrevida/tendências
13.
Acta Biochim Biophys Sin (Shanghai) ; 48(8): 762-7, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27353319

RESUMO

Neuroblastoma is the most common abdominal malignant tumor in childhood. Immunotoxin (IT) that targets the tumor cell surface receptor is a new supplementary therapeutic treatment approach. The purpose of this study is to detect the expression of epidermal growth factor receptor (EGFR) in neuroblastoma cell lines and tissues, and to explore if IT therapy can be used to treat refractory neuroblastoma. The EGFR expression in human neuroblastoma tissue samples was detected by immunohistochemistry staining. The positive rate of EGFR expression was 81.0% in neuroblastoma tissue and 50.0% in gangliocytoma, respectively, but without statistical significance between them (P > 0.05). The positive rate of EGFR expression in favorable type and unfavorable type was 62.5% and 92.3%, respectively, but they were not statistically different (P > 0.05). Results from pre-chemotherapy and post-chemotherapy samples showed that there was no significant statistical difference (P > 0.05) between them in the EGFR expression. Furthermore, the EGFR expression levels in five neuroblastoma cell lines were measured using cell-based ELISA assay and western blot analysis. The results showed that the expression of EGFR was higher in KP-N-NS and BE(2)-C than those in other cell lines. Our results revealed that there are consistent and widespread expressions of EGFR in neuroblastoma tissues as well as in neuroblastoma cell lines, suggesting that it is possible to develop future treatment strategies of neuroblastoma by targeting at the EGFR.


Assuntos
Receptores ErbB/metabolismo , Neuroblastoma/metabolismo , Linhagem Celular Tumoral , Ensaio de Imunoadsorção Enzimática , Humanos , Neuroblastoma/patologia
14.
Pediatr Surg Int ; 32(3): 239-43, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26590817

RESUMO

PURPOSE: Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm. The purpose of this study was to review the clinical characteristics, imaging and pathological features, and outcomes of children with IMTs from a single center in China. METHODS: A retrospective file review was conducted involving 23 cases of pathologically confirmed IMTs treated at the Children's Hospital between April 2003 and April 2014. RESULTS: The tumor locations included multiple anatomic sites, as follows: abdomen or pelvis (n = 17); lungs (n = 2); head and neck (n = 1); trunk (n = 1); and extremities (n = 2). The tumors were associated with various clinical presentations. The predominant symptoms included an anemic appearance, fevers, and an asymptomatic mass. Computed tomography scanning showed solid, heterogeneous, well-demarcated masses; the appearance of enhancement was variable. MRI appeared hypointense on T1-weighted images and hypointense or hyperintense on T2-weighted images. Immunohistochemical staining revealed anaplastic lymphoma kinase was negative in 11 of 13 cases tested. One patient quit treatment for the unresectable mass after biopsy and died 2 years later, and another patient with incompletely resection is alive at 30 months following chemotherapy. The remaining 21 cases had complete resections; one patient died due to a recurrence, and the other 20 patients survived and were tumor free. The follow-up ranged from 7 to 141 months, with a mean of 56 months. The 3-year OS was 88 % (95 % CI, 57-97 %). CONCLUSIONS: IMT is a benign neoplasm that rarely presents with malignant features. Complete resection is curative in most patients. ALK+ is variable for diagnosis. Close follow-up is necessary for patients who undergo surgical resection.


Assuntos
Miofibroma/diagnóstico por imagem , Miofibroma/patologia , Avaliação de Resultados em Cuidados de Saúde/estatística & dados numéricos , Adolescente , Criança , Pré-Escolar , China , Feminino , Seguimentos , Humanos , Lactente , Imagem por Ressonância Magnética , Masculino , Miofibroma/terapia , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
16.
J Dermatol ; 42(4): 401-4, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25728547

RESUMO

Kasabach-Merritt phenomenon (KMP) is a rare and life-threatening disease involving a vascular tumor combined with severe consumptive coagulopathy. We present for the first time a case of KMP with the vascular tumor involving two anatomical sites; the patient failed to respond to steroids and vincristine. Following sirolimus therapy at a dose of 0.8 mg/m(2) twice daily, the lesions shrank and the platelet count improved and remained normal 4 months after initial therapy. Current treatments for KMP are not particularly effective. Sirolimus at 0.8 mg/m(2) per dose, administrated twice daily, appears to be a safe and effective management option. It appears to be an interesting therapeutic option in refractory KMP, but the time to response is variable.


Assuntos
Síndrome de Kasabach-Merritt/tratamento farmacológico , Sirolimo/uso terapêutico , Humanos , Lactente , Síndrome de Kasabach-Merritt/sangue , Síndrome de Kasabach-Merritt/patologia , Masculino , Contagem de Plaquetas , Sirolimo/administração & dosagem , Serina-Treonina Quinases TOR/antagonistas & inibidores
17.
Pediatr Blood Cancer ; 62(7): 1291-3, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25598153

RESUMO

Generalized lymphatic anomaly (GLA) is a rare and often fatal congenital lymphatic disorder that also commonly affects bone. Kaposiform lymphangiomatosis (KLA) is a novel subtype of GLA with poor prognosis and no proper treatment guidelines. A 9-year-old male with recurrent pleural effusion was clinically diagnosed as KLA. Following sirolimus therapy at a dose of 0.8 mg/m(2) twice daily, pleural effusion was significantly decreased and the general status of the patient markedly improved. The clinical course indicates that sirolimus may present an effective therapeutic option in KLA. Moreover, KLA should be considered in differential diagnosis for cases of GLA with coagulopathy.


Assuntos
Antibióticos Antineoplásicos/uso terapêutico , Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Derrame Pleural/prevenção & controle , Sarcoma de Kaposi/tratamento farmacológico , Sirolimo/uso terapêutico , Criança , Humanos , Masculino , Prognóstico
18.
Pediatr Blood Cancer ; 62(4): 577-80, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25346262

RESUMO

BACKGROUND: Kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP) still remains a particular and life-threatening disease. The purpose of this study was to evaluate the efficacy of vincristine (VCR) and the possibility of replacement with steroids in the treatment of steroid-resistant KHE with KMP. PROCEDURE: We retrospectively reviewed the medical records of 37 patients with steroid-resistant KHE who were treated at the Children's Hospital of Fudan University between March 2003 and March 2013. RESULTS: The age of initial diagnosis with KHE was between 1 day and 10 months. Eight and 29 cases were located in the superficial and deep soft tissues, respectively. Thirty-seven KHE lesions did not respond well to steroids before starting VCR treatment. Twenty-six KHE lesions achieved complete remission, with platelet counts reaching normal levels within7.6 ± 5.2 weeks after VCR treatment. The vascular tumor began to decrease in size or soften at an average of 4.9 ± 2.7 weeks. Two KHE lesions had partial responses and one remains in treatment. Eight KHE lesions had no apparent response to VCR and thus received other therapies. Twenty-eight patients have ended treatment with VCR; the average length of treatment was 31.2 ± 5.9 weeks. Side effects occurred in 48.6% of patients who received steroids, and in 11.4% of patients who received VCR treatment. The mean follow-up time was 3.5 years. No recurrences have been reported. CONCLUSIONS: VCR appears to be a safe and effective treatment option in the management of steroid-resistant KHE with KMP, and recommended as first-choice treatment.


Assuntos
Antineoplásicos Fitogênicos/administração & dosagem , Resistencia a Medicamentos Antineoplásicos/efeitos dos fármacos , Hemangioendotelioma/tratamento farmacológico , Síndrome de Kasabach-Merritt/tratamento farmacológico , Sarcoma de Kaposi/tratamento farmacológico , Esteroides/administração & dosagem , Vincristina/administração & dosagem , Feminino , Seguimentos , Hemangioendotelioma/sangue , Hemangioendotelioma/patologia , Humanos , Recém-Nascido , Síndrome de Kasabach-Merritt/sangue , Síndrome de Kasabach-Merritt/patologia , Masculino , Contagem de Plaquetas , Estudos Retrospectivos , Sarcoma de Kaposi/sangue , Sarcoma de Kaposi/patologia , Fatores de Tempo
20.
Oncol Lett ; 8(5): 2007-2011, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25295083

RESUMO

Congenital mesoblastic nephroma (CMN) is a mesenchymal renal tumor. The aim of the present study was to review the clinical characteristics and outcome of CMN in infants. A retrospective file review was conducted of eight cases of CMN treated at the Children's Hospital of Fudan University between 2004 and 2012. Ultrasound and computerized tomography scans had been performed on all eight patients. Two cases presented with a solid tumor and exhibited pathological features consistent with those of classic CMN, five cases exhibited cystic, hemorrhagic and necrotic characteristics, with calcification and pathology consistent with the cellular variant of CMN and one case presented with a solid tumor, which exhibited pathological features consistent with ceullular CMN. Histology confirmed classic CMN in two patients and cellular CMN in six patients. For surgical intervention, four cases had radical nephrectomy, one case had a half nephrectomy and three cases had tumor enucleation performed. Two cases had received pre-operative chemotherapy, but exhibited no response, and three cases received post-operative chemotherapy. Two patients were lost to follow-up, but the remaining six patients survived to the end of follow-up without further complications. The mean follow-up time was 24.6 months. In conclusion, the differential diagnosis between CMN and Wilms' tumor is critical. Imaging characteristics are partially correlated with pathological characteristics. Surgery is the main treatment for CMN, but pre-operative chemotherapy is not particularly effective. The efficacy of post-operative chemotherapy requires further investigation, but the prognosis is positive.

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