Vascular birthmarks are the most common disease.The morbidity is about 2.5%,most of the lesions occur in oral and maxillofacial regions which accounts for 40%-60% of the total lesions.In 1982,Mulliken and Glowacki pro...Vascular birthmarks are the most common disease.The morbidity is about 2.5%,most of the lesions occur in oral and maxillofacial regions which accounts for 40%-60% of the total lesions.In 1982,Mulliken and Glowacki proposed a biologic classification of vascular birthmarks on the basis of their clinical manifestations,histopathological features,and natural history.They defined hemangiomas as vascular tumors with a growth phase,marked by endothelial proliferation and hypercellularity,and an involutional phase.They recognized that many entities referred to as hemangiomas are actually structural malformations of the vasculature,derived from capillaries,veins,lymph vessels,or arteries or from a combination of these sources.The classification was confirmed and issued by International Society for the study of vascular anomality(ISSVA) in 1988.Waner and Suen amended the above category in 1995.This paper presents the new classification of vascular birthmarks and the developments in this field in re-cent years,including the pathology,clinical features and the therapy.For example,the classification of venular malformation categorized by Waner in 1989;the classification of lymphous malformation by Waner and Suen in 1995;and the treatments according to above classifications.展开更多
Keratocystic odontogenic tumors(KCOTs,previously known as odontogenic keratocysts) are aggressive,noninflammatory jaw lesions with a putative high growth potential and a propensity for recurrence.This article puts tog...Keratocystic odontogenic tumors(KCOTs,previously known as odontogenic keratocysts) are aggressive,noninflammatory jaw lesions with a putative high growth potential and a propensity for recurrence.This article puts together a summary of the serial studies related to KCOTs undertaken by the author’s research group in recent years.Intraosseous jaw cysts with a solely orthokeratinized lining epithe-lium have been suggested to differ from the typical KCOTs.We report 20 cases of such cyst type under the term of ’orthokeratinized odontogenic cyst(OOC)’.Apart from the presence of a keratinizing epithelial lining,the OOC lacks the other histological features of KCOT,exhibits little if any tendency to recur,has no apparent association with NBCCS,may be cured by simple enucleation,and may thus constitute its own clinical entity.Mutations in PTCH1 gene are responsible for NBCCS and are related in tumors associated with this syndrome.We have so far detected 26 PTCH1 mutations(2 mutations occurred twice) in 10 out of 34(29.4%) sporadic and 14 out of 16(87.5%) NBCCS-associated KCOTs.The 26 mutations consisted of 10 frameshift,2 nonsense,3 aberrant splicing,4 in-frame insertion/deletion/ duplication and 7 missense mutations.Two missense mutations in PTCH2 were also detected in 2 out of 15 NBCCS related KCOT patients.By contrast,no pathogenic mutation was detected in SMO.Thus,our data,together with reports from ther groups,indicate that defects of PTCH1 are involved in the pathogenesis of syndromic as well as sporadic KCOTs.The pathogenic role of PTCH2 requires further investigation.A series of in vitro studies on bone resorption of KCOTs and ameloblastomas were undertaken by this group.The results indicate that odontogenic lesions could promote bone resorption in vitro and it is likely to be related to some of the cytokines secreted by the lesions.展开更多
目的:检测parafibromin蛋白在人颌骨骨肉瘤(osteosarcoma of the jaws,JOS)中的表达情况,探讨其在JOS的发生、发展中的作用。方法:采用免疫组织化学的方法检测parafibromin蛋白在47例JOS和11例颌骨骨化纤维瘤(ossifying fibroma of the ...目的:检测parafibromin蛋白在人颌骨骨肉瘤(osteosarcoma of the jaws,JOS)中的表达情况,探讨其在JOS的发生、发展中的作用。方法:采用免疫组织化学的方法检测parafibromin蛋白在47例JOS和11例颌骨骨化纤维瘤(ossifying fibroma of the jaws,JOF)中的表达情况。结果:JOS中,parafibromin高表达、中等表达、低表达患者分别占48.9%(23/47)、40.4%(19/47)和10.7%(5/47);JOF中,三者所占的百分率分别为90.9%(10/11)、9.1%(1/11)和0.0%(0),与JOS相比,差异有统计学意义(U=147.5,P=0.012);parafibromin在高分化型JOS中的表达水平高于传统型JOS(U=71.0,P=0.021);parafibromin的表达情况与临床病理参数间无相关性(P>0.05)。结论:parafibromin蛋白在JOS中表达下调,这种表达的减少可能与JOS的分化和恶性程度有关。展开更多
文摘Vascular birthmarks are the most common disease.The morbidity is about 2.5%,most of the lesions occur in oral and maxillofacial regions which accounts for 40%-60% of the total lesions.In 1982,Mulliken and Glowacki proposed a biologic classification of vascular birthmarks on the basis of their clinical manifestations,histopathological features,and natural history.They defined hemangiomas as vascular tumors with a growth phase,marked by endothelial proliferation and hypercellularity,and an involutional phase.They recognized that many entities referred to as hemangiomas are actually structural malformations of the vasculature,derived from capillaries,veins,lymph vessels,or arteries or from a combination of these sources.The classification was confirmed and issued by International Society for the study of vascular anomality(ISSVA) in 1988.Waner and Suen amended the above category in 1995.This paper presents the new classification of vascular birthmarks and the developments in this field in re-cent years,including the pathology,clinical features and the therapy.For example,the classification of venular malformation categorized by Waner in 1989;the classification of lymphous malformation by Waner and Suen in 1995;and the treatments according to above classifications.
文摘Keratocystic odontogenic tumors(KCOTs,previously known as odontogenic keratocysts) are aggressive,noninflammatory jaw lesions with a putative high growth potential and a propensity for recurrence.This article puts together a summary of the serial studies related to KCOTs undertaken by the author’s research group in recent years.Intraosseous jaw cysts with a solely orthokeratinized lining epithe-lium have been suggested to differ from the typical KCOTs.We report 20 cases of such cyst type under the term of ’orthokeratinized odontogenic cyst(OOC)’.Apart from the presence of a keratinizing epithelial lining,the OOC lacks the other histological features of KCOT,exhibits little if any tendency to recur,has no apparent association with NBCCS,may be cured by simple enucleation,and may thus constitute its own clinical entity.Mutations in PTCH1 gene are responsible for NBCCS and are related in tumors associated with this syndrome.We have so far detected 26 PTCH1 mutations(2 mutations occurred twice) in 10 out of 34(29.4%) sporadic and 14 out of 16(87.5%) NBCCS-associated KCOTs.The 26 mutations consisted of 10 frameshift,2 nonsense,3 aberrant splicing,4 in-frame insertion/deletion/ duplication and 7 missense mutations.Two missense mutations in PTCH2 were also detected in 2 out of 15 NBCCS related KCOT patients.By contrast,no pathogenic mutation was detected in SMO.Thus,our data,together with reports from ther groups,indicate that defects of PTCH1 are involved in the pathogenesis of syndromic as well as sporadic KCOTs.The pathogenic role of PTCH2 requires further investigation.A series of in vitro studies on bone resorption of KCOTs and ameloblastomas were undertaken by this group.The results indicate that odontogenic lesions could promote bone resorption in vitro and it is likely to be related to some of the cytokines secreted by the lesions.
文摘目的:检测parafibromin蛋白在人颌骨骨肉瘤(osteosarcoma of the jaws,JOS)中的表达情况,探讨其在JOS的发生、发展中的作用。方法:采用免疫组织化学的方法检测parafibromin蛋白在47例JOS和11例颌骨骨化纤维瘤(ossifying fibroma of the jaws,JOF)中的表达情况。结果:JOS中,parafibromin高表达、中等表达、低表达患者分别占48.9%(23/47)、40.4%(19/47)和10.7%(5/47);JOF中,三者所占的百分率分别为90.9%(10/11)、9.1%(1/11)和0.0%(0),与JOS相比,差异有统计学意义(U=147.5,P=0.012);parafibromin在高分化型JOS中的表达水平高于传统型JOS(U=71.0,P=0.021);parafibromin的表达情况与临床病理参数间无相关性(P>0.05)。结论:parafibromin蛋白在JOS中表达下调,这种表达的减少可能与JOS的分化和恶性程度有关。
